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Late effects of paralytic poliomyelitis in Olmstead County, Minnesota

Late effects of paralytic poliomyelitis in Olmsted County, Minnesota – PubMed – NCBI

PubMed

Abstract

Neurology. 1991 Apr 41(4):501-7.

Late effects of paralytic poliomyelitis in Olmsted County, Minnesota. Windebank AJ”, Litchy WJ, Daube JR, Kurland LT, Codd MB, Iverson R.

Author information

We identified a cohort of 300 individuals who had paralytic polio between 1935 and 1955. All lived

in Olmsted County, Minnesota, From the 247 survivors, we selected 50 subjects for detailed historical, functional, psychological, clinical, and electrophysiologic evaluation. Sixty-four percent of these 50 survivors complained of new symptoms of muscle pain, fatigue, and weakness after a period of prolonged stability. This led to changes in lifestyle or activity in only 18%. The likelihood of expressing new complaints was not related to present age or interval since polio, and electrophysiologic testing did not distinguish between those with or without new problems. The development of new difficulties in a limb was most strongly predicted by significant paralysis of that limb at the time of the acute illness. Patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness. Elevated creatine kinase levels were present only in those with new complaints.

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Coping Styles

RECOGNIZING TYPICAL COPING STYLES OF POLIO SURVIVORS CAN IMPROVE RE-REHABILITATION

A Commentary¹

Frederick M. Maynard,² MD and Sunny Roller, MA

Am. J. Phys. Med. Rehabil. Vol. 70, No. 2, April 1991

Lincolnshire Post-Polio Library copy by kind permission of the author Key Words: Psychological Coping Behaviour Adaptions, Poliomyelitis, Rehabilitation, Post-Polio

During the past ten years many polio survivors in the U.S. have actively been seeking professional help for a wide range of new physical problems, commonly referred to as the late effects of polio. Often these persons require re-rehabilitation in order to continue their accustomed social roles.[1] In our experience at the Post-Polio Program of the University of Michigan Medical Center, we have come to recognize among polio survivors three distinct patterns of emotional reaction to the need for re-rehabilitation. These patterns appear to result from characteristic styles of living with a chronic disability. We propose a model for categorizing polio survivors that is based on our observations. Although it is limited by overgeneralization, we have found that polio survivors themselves have verbally validated our proposed categories at many post-polio conferences. A 1963 study of children with polio and their families also describes early coping behaviors that are compatible with our model.[2]

The model designates polio survivors as Passers, Minimizers and Identifiers. These labels characterize typical attitudes and behaviors that were adopted in order to cope with long-term mild, moderate or severe disability. Passers had a disability that was so mild it could be easily hidden in casual social interactions. They could pass for nondisabled. Minimizers had a moderate disability that was readily recognized by other people. They often used visible adaptive equipment or had to do physical tasks differently in order to optimally function. They typically minimized the importance of their physical differences. Identifiers were severely disabled by acute polio. They generally needed wheelchairs for independent mobility. Some also used respiratory equipment. They needed to fully identify with their disability in order to make major lifestyle adaptations and successfully cope. A closer look at each group’s coping style is presented in order to clarify the typical patterns of emotional reaction that occur when polio survivors experience disabling late effects.

Passers worked directly to hide their long-term disability. Many of their acquaintances probably did not know they were disabled in any way. Although intimate family members and friends may have known, on the whole Passers became psychologically invested in hiding their disability from other people. Even

today, they do not like to have to explain it or to talk about it. They don’t want to think of themselves as having a disability. By using denial, they have been able to put their disability out of existence mentally and physically and to create an image that completely fools the casual onlooker. Passers may hide a paralyzed hand by keeping it constantly in a pants pocket or cover slightly imperfect body parts with stylistically camouflaging clothing. Passing is a coping style that requires constant vigilance and attention to the nondisabled disguise. Good Passers believe they cannot “blow their cover” or they might get stigmatized as part of society’s disabled minority.

We administered an attitudes survey to 100 polio survivors and discovered that Passers were the group who were most distressed in having to adjust to the late effects of polio. They were more likely to be emotionally overwhelmed by the physical changes from the late effects than any of their postpolio counterparts. It is important for helping professionals to know that among post-polio people, it is the Passers who have the greatest resistance to making, and the most emotional difficulty in accepting, some of the relatively minor lifestyle adaptations that are needed to cope with the late effects of polio. Passers who are confronted with post-polio sequelae often have their self-image threatened because they cannot pass any longer. Their disability has become undeniable and suddenly they must give in to it. They may become frightened because they do not know how far it will progress. Typical thoughts may include: “Wearing a brace could lead to using two crutches, and a year later to a wheelchair, or who knows what after that… and now that the disability is obvious, what will other people think…?”

When confronted with polio’s late effects, Passers often must alter their self-perceptions and life-style in order to continue successful coping. Their former coping style may no longer be effective and new attitudes and behaviors must be learned. Clinically, Passers can often be fully rehabilitated because their new disabilities are less severe. They can be reassured that modern orthotics, such as plastic braces, can be nicely worn under clothing and completely hidden under shoes. Passers may require an unexpected amount of understanding, patience and empathetic support from health care professionals because of strong emotional reactions that are not only triggered by the impending public nature of their new disability, but also by memories of past polio-related experiences.[3] When their disability progresses from mild to moderate, they become undeniably disabled for the first time. This can be a harsh reality for them to finally face, accept and adapt to.

Minimizers are post-polio people who have had a moderate disability that was always apparent to themselves and to others. They have coped with polio’s first effects by minimizing the negative and accentuating the positive. Minimizers may say, “So what if I use braces and crutches and I can’t walk in a normal fashion, look at all the other things I can do.” Minimizers have adapted by de-emphasizing physical pain, deformity and functional shortcomings. Many have pursued intellectual vocations and avocations in place of more physical or athletic activities. They often have been high achievers who have pushed themselves to their limits. Minimizers have learned how to tune out their bodies in order to ignore physical imperfections, a process called “devaluing physique.”[4] This practical approach to living with an obvious physical disability has often been helpful for effective coping in a society which emphasizes physical beauty and prowess. However, Minimizers are often so adept at this form of denial that they recognize polio’s late effects only when physical symptoms become unbearable and insurmountable. In order to survive and function at peak capacity they may have learned to use minimizing as a defense mechanism to such an extent that they are quite insensitive to their own pain, sadness, weakness and anger. This perception can occasionally generalize to become an insensitivity to similar conditions and feelings in other people, including persons with a more severe disability.

When asked to respond to the survey statement, “I feel uncomfortable around other disabled people,” Minimizers endorsed it more than the other groups. We found that they often had negative attitudes about

severely disabled individuals as a group, particularly wheelchair users. Therefore, they may feel that to personally begin using a wheelchair signals joining a social group that they have previously devalued and/or implies defeat, helplessness and not fighting vigorously enough against polio’s disabling effects. Minimizers sometimes admit to difficulty being socially linked with someone in a wheelchair because the very association might somehow generate their own need to use one. It is useful for professionals to recognize these phobic-like reactions when they occur and employ techniques for helping Minimizers change their perceptions of wheelchairs and wheelchair users. Indeed, these post-polio patients are the most likely to physically benefit from beginning to use a wheelchair.

Minimizers may have difficulty verbally describing new physical symptoms because they are skilled at ignoring and/or denying such problems. They need coaching and encouragement to fully focus on their body sensations and reactions and to become what might be called “wise hypochondriacs.” Health care providers must listen closely to Minimizers for the slightest mention of new medical problems and give them permission to elaborate. Minimizers most commonly feel guilty about causing others, including

health care professionals, inconvenience related to their new disabilities. Additionally, they are likely to have intense angry feelings about having to deal with new disabilities and re-rehabilitation.[5]

In spite of many negative emotional reactions, Minimizers know how to set goals and achieve them with persistence and determination. The astute health care professional will encourage and help empower the Minimizer to use these qualities to refocus on what is important in life; to take another look at how to be successful; to set new goals and achieve them in new ways. Health care professionals must be patient in helping Minimizers work through understandable resistance, fears and anger with rerehabilitation. They must respect, remember and sometimes remind Minimizers that they are experienced copers who have a well-proven capacity to adapt effectively.

Identifiers are post-polio people who have usually been sufficiently disabled since their acute polio to require wheelchairs for mobility. They have needed to more fully integrate their disability into their self image in order to create successful and meaningful lives. Through identifying with others having physical disabilities, they have gained the strength to tolerate social prejudices and architectural barriers. Not surprisingly, many became disabled rights activists and helped start the independent living movement. Among the three groups sampled through the attitudes survey, Identifiers most strongly endorsed the statement, “high achievement is a requirement for survival as a disabled person.” They also most intensely believed that taking an active role in the disabled rights movement was necessary to their future well being in society, and that fully acknowledging their disability will help them cope with it more effectively.

With the onset of polio’s late effects many Identifiers confront the loss of their independence. The smallest functional forfeiture can be extremely distressing to a person who has been chronically severely disabled. If breathing function becomes significantly impaired, death may be a realistic threat. For Identifiers who have had to work diligently to learn to feed themselves and perform other simple self-care activities, independence in daily living activities may be one of the most important accomplishments of their lives. Therefore, if post-polio sequelae threaten a decline in strength, they can be expected to appear extremely distressed. Effective helping professionals need to anticipate the Identifier’s concerns and recognize that his/her intense interest in autonomy and control of his/her environment is not pathologic. Identifiers have needed to develop a heightened concern about physical independence and about personal choice with how required help is given in order to attain high self-esteem and survive with their severe disability. When their freedom to control their life activities is threatened by new physical limitations or even by temporary dependency imposed by a hospital setting, Identifiers may experience a threat to their whole life and purpose for living. This reaction often leaves Identifiers vulnerable to other’s false perceptions of them as being overly controlling, difficult and demanding people. In reality, they simply know what they need and

are not too timid to ask for it. The informed health care professional will accept this and will do everything possible to let them continue to feel, and actually be, in charge of what happens to them.

Passers, Minimizers and Identifiers each adopted a characteristic coping style in the past that worked to create some of our generation’s most successful and resilient survivors of physically disabling illness. The onset of new post-polio problems can present a challenge to their previously successful methods of coping and create significant emotional distress and pain. Health care professionals need to be aware of polio survivors’ typical past coping styles and of their need to employ different tactics for coping during the re rehabilitation process. They can point out to polio survivors that it is possible to find opportunity in their time of change. Passers can “come out of the closet,” relax and enjoy a little more freedom with their very acceptable natural physiques and identities. Minimizers can also be empowered to live life with a greater sense of wholeness through more fully recognizing, accepting and integrating all aspects of their bodies. By relinquishing their struggle for physical independence and accepting new personal and technological assistance, Identifiers can gain the time and energy to develop new pursuits and cultivate other realms of interest. It is in this honest and supportive spirit of healthy transition that successful re-rehabilitation can occur for the ever-adapting group of polio survivors.

REFERENCES

Maynard F: Late effects of polio create large demand for re-rehabilitation. Rehabil Rep 1986;2:2-3. 2. Davis F: Passage Through Crisis. Indianapolis, The Bobbs Merrill Co., Inc., 1963. 3. Backman M: The post-polio patient: psychological issues. J Rehabil 1987;53:4:23-26. 4. Frick N, Bruno R: Post-polio sequelae: physiological and psychological overview. Rehabil Lit 1986;47(5&6):106-111. [Lincolnshire Library Full Text]
Roller S: Post-polio memorandum. Polio Perspectives 1987;2:3:1-2. (Available through The Brandon Township Public Library, Post-Polio Collection, 476 Mill Street P.O. Box 489, Ortonville, MI 48462)

0894-9115/91/7002-0070$03.00/0

AMERICAN JOURNAL OF PHYSICAL MEDICINE & REHABILITATION

¹ From the Post-Polio Research and Training Program, Department of Physical Medicine and Rehabilitation, University of Michigan Medical Center, Ann Arbor, Michigan.

² To whom all correspondence and requests for reprints should be addressed at: Post-Polio Research and Training Program, Department of Physical Medicine and Rehabilitation, University of Michigan Medical Center, NI2A09 300 N. Ingalls Building, Ann Arbor, MI 48109-0491.

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A Ten Year Experience

Stanley K. Yarnell, M.D., Saint Mary’s Hospital Post-Polio Clinic San Francisco, Calif.

The following paper was prepared for Post-Polio Conference III–Perspectives for the Nineties, held February 1-2, 1991 at Oakland Hyatt Regency in Oakland, Ca. USA. It has been condensed here with permission of Gazette International Networking Institute (G.I.N.I.); 5100 Oakland Ave. #206; St. Louis, MO 63119; (314) 534-0475

SERVIS

THE ORIGINAL EFFECTS OF THE POLIO VIRUS

To more easily understand the late effects of polio it is important to understand the original effects of the poliomyelitis virus. The portal of entry for the polio virus was oral, and the infection in its first stage was in the gut cells lining the intestine. Many people infected with the virus at this stage thought they simply had gastrointestinal flu with symptoms of diarrhea and nausea. For a significant percentage of the people infected, the disease ended at this point.

But for some the virus continued to multiply and spilled over into the blood stream, signaling the viremia stage of the infection. Symptoms then were not unlike a more generalized influenza, with fever, chills, malaise and achiness. Once again for a significant percentage of patients, the disease process ended there. However, in a small percentage of the patients, the virus crossed over into the central nervous system and infected the anterior horn cells. These individuals contracted “paralytic” polio.

The distribution of weakness or paralysis depended on which anterior horn cells were involved. Spinal anterior horn cell involvement resulted in weakness or paralysis in the arms, legs, and trunk to one degree or another. Bulbar (brainstem) anterior horn cell involvement resulted in visual, swallowing, or breathing difficulties in any combination.

It is this period of fever, weakness, paralysis, and muscle pain that many polio survivors (or their parents) remember. This was the time when infected motor neurons were dying, leaving all the muscle fibers they innervated orphaned (or denervated). If all the motor neurons that supplied a particular muscle died, the result was complete paralysis of that muscle. If only a percentage of the motor neurons

died, the muscle was only weakened, but not completely paralyzed.

Many polio survivors remember having muscles completely paralyzed that started to move again, although weakly, after a period of a few weeks. That occurred because some of the anterior horn cells were simply shocked when neighboring anterior horn cells died. These nonfunctioning anterior horn cells were not dead but stunned by the swelling caused by adjacent nerve cell destruction. As days and weeks passed, the swelling subsided and the surviving anterior horn cells revived. Kenny hot packs and stretching helped control the pain and contracture of the denervated muscle. With time and exercise, the muscle got stronger.

Over the course of months and years, many polio survivors got stronger in a previously weakened or paralyzed muscle and were able to discard braces or wheelchairs. This was not only because of hard work and exercise but also because of a phenomenon called “sprouting”. Denervated or orphaned muscle fibers within the same muscle sent out a chemical distress signal that caused tiny sprouts to grow from the terminal nerve branches of neighboring, surviving motor neurons. Very slowly these sprouts grew over to the orphaned muscle fibers and reinnervated them, causing the motor unit to grow in size. In fact some motor units sprouted to innervate three to four times as many muscle fibers as Mother Nature had originally intended. As a result polio survivors felt stronger, and this pattern of strength and weakness remained stable for the next twenty to thirty years.

THE LATE EFFECTS OF POLIO

Since 1981, four hundred eighty-three (483) post-polio patients have been seen at St. Mary’s Hospital Post-Polio Clinic. When the data collected by Barbara L. Bammann, M.D., at Alta Bates-Herrich Rehabilitation Hospital and by Michael Berlly, M.D., at Santa Clara Valley Medical Center are added, there is a wealth of statistics and information. In all, seven hundred eleven (711) patients have been examined in the Bay Area Post-Polio Clinics. The ages range between thirty and seventy-six, the following material is based in large part on these statistics.

FATIGUE: The most frequent symptom was unaccustomed fatigue (79%). During the past decade, a number of investigators have looked at the problem using a variety of research tools. The picture that has emerged from this data suggests that after thirty years, the metabolically overburdened surviving motor neurons appear to have become incapable of supporting the integrity of all distal nerve terminals, resulting in defective neuromuscular transmission.

To better understand this, visualize what happens at the junction of the nerve and muscle under the usual circumstances. The electrical impulse travels from the anterior horn cell, down the nerve fiber to the tiny terminal nerve endings, which contact the individual muscle fibers. The arrival of the electrical i pulse at the terminal nerve ending causes the release of a chemical (acetylchloine), which in turn causes the electrical impulse to penetrate into the muscle fiber, resulting in contraction of that tiny muscle fiber. In polio survivors, repeated discharges of the large reinnervated motor unit results in a failure of some of the muscle fibers to fire, thereby not contributing to the force of contraction. Polio survivors experience this as fatigue or a loss of stamina.

WEAKNESS: A significant number of the patients had new or increasing weakness for reasons not directly related to their polio residuals. Having stated that, we note that there were a large number of patients who did have increasing weakness which was directly attributable to the late effects of polio. Some clinicians would argue that ‘acute’ overwork weakness and post-polio muscular atrophy are one and the same. We have chosen to separate them for record keeping purposes and for prognostic reasons.

Clinically, polio survivors who have engaged in excessively strenuous activities like tennis, weight-lifting, stair climbing, etc., have noted the sudden onset of weakness in particular muscle groups overused in that particular activity. Even though it may take months and with proper rest, the strength may return nearly to the level it was prior to the strenuous activity. This reversible weakness is what we refer to as ‘acute’ overwork weakness.

Cumulative research suggests that the old overburdened motor neuron, which has been driving many more muscle fibers than it was meant to drive, is so metabolically exhausted by the strenuous activity that some of the terminal nerve endings die back, leaving many of the muscle fibers of the same motor unit orphaned again. If the weakened muscle group is allowed to rest (by stopping the offending activity or bracing, for example), new sprouts will grow and recapture the lost muscle fibers.

‘Chronic’ overwork weakness, or what has been called post-polio muscular atrophy (PPMA), differs inasmuch as there is no singular event of strenuous activity or exercise that signals the onset of increasing weakness. Rather there is a very slow and gradual loss of strength with accompanying atrophy of a particular muscle group. And no amount of rest will reverse the weakness. Rather there is a very slow and gradual loss of strength with accompanying atrophy of a particular muscle group. And no amount of rest will reverse the weakness, the overburdened motor neuron is unable to send out new sprouts to capture the orphaned muscle fibers, which have become denervated slowly from “pushing” in order to complete ordinary day-today activities. RESPIRATORY PROBLEMS: Of the total number of patients seen in the Bay Area’s Post- Polio Clinics, only one hundred fifteen (16%) were classified as having worsening respiratory problems.

Recurrent bronchitis was a frequent enough problem to warrant mentioning, even though the patients for whom it was a problem had a perfectly adequate cough and no difficulty handling secretions. The problem seems to be more of a chemical bronchitis (as opposed to infection) caused by night-time aspiration of tiny amounts of stomach acid, which refluxes from the stomach into the esophagus because of hiatus hernia. Fully one hundred sixty-seven polio survivors (23%) have symptomatic indigestion secondary to hiatus hernia. Despite reports of sleep apnea in polio survivors, we have seen only one documented case.

JOINT AND MUSCLE PAIN: Various musculoskeletal aches and pains were a problem for five hundred fifty-nine polio survivors (74%). Most polio survivors suffer from chronic pain, which for some can be quite disabling. Nearly all polio survivors (99% of our patients) had scoliosis. Scoliosis per se does not hurt, but the long standing effects of rotation and curvature of the spine have been associated with conditions that do hurt. We found that 34% had facet arthropathy or wear and tear arthritis of the little joints of the spine; 41% had degenerative disc disease; 12% had pain in the back and legs or in the neck and arms; 11% had lumbar spinal stenosis, all of whom were over the age of sixty. The other joints in addition to the ones of the spine showed signs of wear and tear arthritis. The shoulder was affected in 22%, while the knee was affected in 21%. Degenerative arthritis was present in the hip in 11%, in the ankle/foot in 6%, and in the wrist in 4%. Interestingly, if a leg was braced because of weakness, the arthritis generally developed on the unbraced side, which had been the side of major weight bearing for many years. COLD INTOLERANCE: Forty one percent of the polio survivors complained of worsening cold intolerance. For nearly everyone it was not a new problem, but one that had been present since the onset of polio to a less bothersome degree. It appears that some of the sympathetic nerve fibers were affected originally, and these fibers are the ones that constrict the blood vessels of the skin in response to cold. Normally, when we are exposed to cold, the blood vessels of the skin constrict to shunt blood away form the surface of the skin down to the underlying bone and muscle where heat can be conserved and core body temperature maintained at 98.6 degrees. Some polio survivors have lost that ability to shunt blood away from the skin, and so heat radiates away from the affected limb and it cools down. The best treatment is an elastic stocking and good woolly socks. The other part of the problem is decreased muscular performance in the presence of cold. Many polio survivors find they are unable to swim in an unheated pool, or they find that they have more trouble walking in cold winter weather. This is explained by the fact that when muscle is cooled, defective neuromuscular transmission is made worse. If you suffer from cold intolerance, you can only be advised to try to avoid cold exposure and “button up your overcoat.”

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Assessment of Behavioral Features

Postpoliomyelitis Syndrome: Assessment of Behavioral Features

Donald L. Freidenberg, David Freeman, Steven J. Huber, Jacquelin Perry, Armin Fischer, Wilfred G. Van Gorp and Jeffrey L. Cummings

Neuropsychiatry, Neuropsychology, and Behavioral Neurology Vol. 2, No. 4, pp 272-281. 1989

Lincolnshire Post-Polio Library copy by kind permission of Dr. Perry

Summary: Postpoliomyelitis syndrome (PPS) is an increasingly recognized phenomenon characterized by late-onset weakness, pain, and fatigue. Psychiatric and cognitive disturbances have been noted in postpoliomyelitis patients, but the relationship of these symptoms to PPS is unknown. We examined postpoliomyelitis patients with and without PPS using objective neuropsychological and neuropsychiatric procedures. Our results suggest that disturbances of mood were common and that subtle cognitive deficits also occured in postpoliomyelitis patients. However, patients with PPS did not have greater depression or cognitive deficits compared to postpoliomyelitis patients without PPS. Key Words: Postpoliomyelitis syndrome, Mood disturbances, Cognitive deficits. NNBN 2:272-281, 1989

*Department of Neurology, Ohio State University, Columbus, Ohio;

¶Departments of Psychiatry and Behavioral Sciences, University of California at Los Angeles (UCLA) and **Neuropsychology Laboratory, and §Department of Neurology, UCLA and Neurobehavior Unit West LA VAMC (Brentwood Division), Los Angeles; and

***Departments of Pathokinesiology and ¥Pulmonary Medicine, Ranch Los Amigos Medical Center, University of Southern California School of Medicine, Downey, California, U.S.A.

Address correspondence and [original publication] reprint requests to Dr. D. L. Freidberg at Department of Neurology, Ohio State University, Means Hall Rm 417, 1654 Upham Drive, Columbus, OH 43210, U.S.A.

Recent reports have described the emergence of late-onset symptoms in patients who had previously had acute poliomyelitis (Dalakas et al., 1986; Cashman et al., 1987; Dalakas, 1988; Eulbert et el., 1988; Klingman et al., 1988). The new onset of lower motor neuron symptoms, including pain, weakness, and fatigue, has been referred to as postpoliomyelitis syndrome (PPS) (Cashman et al., 1987). Other symptoms, possibly unrelated to lower motor neuron involvement associated with delayed onset, have also been described, including parkinsonian symptoms (Vincent and Meyers, 1978), memory and concentration difficulties (Lane et al., 1974), mood and emotional disturbances (Lane et al., 1974; Kohl, 1987), pain alone (Smith et al., 1987), and central and peripheral sleep apnea (Fischer, 1987). Lane et al. (1974) suggested that complaints of late-onset depression and memory difficulty are associated with late onset ventilatory insufficiency.

The association of nonmotor symptoms, including mood and cognitive changes, and PPS is unknown. In

this study, clinical and objective procedures were used to examine possible neuropsychiatric and neuropsychological differences between postpoliomyelitis patients with and without PPS.

METHODS

Subjects

Thirty consecutive patients from a large orthopedic outpatient poliomyelitis clinic were examined after obtaining informed consent. All patients had acquired poliomyelitis between 1923 and 1958, except for one patient who, having failed to be vaccinated, acquired the illness in 1965. The diagnosis of poliomyelitis was confirmed by neurological examination and by review of the history. Patients were considered to have had poliomyelitis if they had sustained an acute onset of fever and paralysis with the physical findings of asymmetrical weakness, hyporeflexia, muscle atrophy, lack of sensory involvement, and a prolonged stable course after convalescence. None of the patients had a history of familial amyotrophy, and none had a history of psychiatric disease prior to the onset of poliomyelitis. Five potential study candidates were excluded: two for failing to adequately complete the examinations, one due to a history of a basilar skull fracture, and two for failing to meet criteria for confirmation of paralytic poliomyelitis. Of the 25 patients included in the study, 20 were women and 5 were men.

Clinical Examination

Complete history and neurological examinations were done by the same neurologist, who was experienced in the evaluation of neuromuscular disease and neuropsychiatric disorders. Specific data concerning recent onset of symptoms, including fatigue, pain, weakness, and loss of function were elicited. Patients were considered to have PPS if they had: (1) a credible history of poliomyelitis; (2) initial partial recovery; (3) at least 10 years of stability; (4) subsequent development of progressive weakness, pain, and fatigue; (5) a change in function as a result of increasing pain, weakness, and fatigue; (6) weakness on exam in areas corresponding to symptomatic complaints; and (7) no other cause for progressive weakness and pain, such as arthritic changes or nerve entrapment. The Karnofsky scale was used to rate current disability (Grieco and Long, 1984).

Psychiatric history was obtained by a semistructured clinical interview, concentrating on symptoms related to mood, psychosis, and personality disorders using Diagnostic and Statistical Manual of Mental Disorders criteria (third edition, revised)(APA, 1987).

Pulmonary Function Tests

Assessment of pulmonary function included measurement of forced vital capacity (FVC) via a Collins Vitalometer. Percent FVC was determined by the method proposed by Comroe et al. (1955). P_aCO₂ determinations were made on a Beckman LBI Medical Gas Analyzer utilizing rebreathing techniques. P_VCO₂ was calculated by multiplying the P_VCO₂ value by 0.8 (Powels, 1978).

Neuropsychometric Tests

Attention and Psychometer Speed

The Digit Span subtest of the Wechsler Adult Intelligence Test — Revised (Wechsler, 1981) was used to measure verbal attentional abilities, and the Trail-Making Test Part A (Reitan, 1958) was used to assess attention, visual scanning, and psychometer speed.

Mental Set Function

The Stroop Color Name Test (Stroop, 1935), and the Trail Making Test Part B (Reitan, 1958) were used to measure response inhibition and ability to shift mental set, respectively. The Symbol Digit Test (oral presentation) was administered to assess mental set function as well as psychomotor speed and novel problem solving ability (Smith, 1968).

Memory

Recent verbal memory was evaluated using the Rey Auditory Verbal Learning Test (RAVLT) (Taylor, 1959) and recall of the Rey Osterreith Complex Figure (ROCF) (Lezak, 1983) following a 3-min delay was used to assess nonverbal memory.

Word List Generation

The Controlled Word Association Test (FAS) (Benton, 1973) was used to assess speed of retrieval from long-term lexical memory.

Visuospatial Ability

Copy of the ROCF was used to assess constructional skills (Lezak, 1983).

Mood and Personality

The Beck Depression Inventory (BDI) (Beck et al., 1961) and Minnesota Multiphasic Personality Inventory (MMPI) (Dahlstrom et al., 1975) were used in addition to the clinical psychiatric interview to examine mood and personality.

RESULTS

There was a strikingly high incidence of mood disorders in the postpoliomyelitis patients. Forty-six percent of those with PPS and 50% of those without PPS had evidence of depression (adjustment disorder, depressive episode, recurrent depression, bipolar episode), and 8% of the patients in each group had attempted suicide. Even when those with adjustment disorders are eliminated, the rate of mood disorder is substantial (39% in PPS patients, 33% in patients without PPS). Mean BDI scores were moderately elevated (11.5 and 12.1 in the PPS and no PPS groups, respectively). The most commonly endorsed items on the BDI in the group as a whole were related to somatic features, including fatigue (84%), difficulty working (72%), and concerns related to physical appearance (64%). Irritability (60%) and difficulty with sleep (64%) were also common. In contrast, feelings of guilt (12%) or of being punished (12%), as well as symptoms of anorexia (16%)and weight loss (20%), were least common. It is possible that somatic features were most common due to intrinsic features of the disease: however, there was no significant correlation between BDI scores and physical disability (r = -0.28, n.s.), suggesting that mood impairment was not simply a reaction to disability. Additionally, patients with positive psychiatric histories on clinical interview had higher average BDI scores (14.9) compared to those without positive histories (8.4), but this failed to reach statistical significance, t(23) = 2.02, p< 0.06.

The group as a whole performed within the average range on the majority of neuropsychological measures compared to existing normative data. There was, however, borderline impairment on nonverbal recall (8th percentile) (Lezak, 1983) and low average performance on a timid color-naming task (Stroop Part B)(10th percentile) (Nehemkis and Lewinsohn, 1972).

As illustrated in Table 1, when the patient group was divided into those with (n = 13) and those without PPS (n = 12), there was no significant difference between the two patient groups with respect to age, education, age at onset of acute illness, overall disability, and gender distribution. Clinical history data related to the proportion of patients with current complaints of memory or concentration difficulties were also not significantly different between the two groups. Pulmonary function studies indicated that percent FVC was not different between the two groups. However, patients with PPS had significantly elevated P_aCO₂ levels compared to patients without PPS. In the group as a whole, P_aCO₂ of patients with complaints of decreased memory [t(20) = 2.06] or concentration [t(20) = 1.29] did not differ from those without such complaints.

TABLE 1. Demographic, clinical, and pulmonary data in patients with and without PPS
		PPS (n = 13)	No PPS (n = 12)	Significance
Demographics
	Age	49.6 ± 3.2	45.4 ± 2.8	t = 1.0, NS
	Education	14.9 ± 1.0	13.3 ± 0.9	t = 1.2, NS
	Age at onset	8.4 ± 2.1	8.6 ± 1.7	t = -0.1, NS
	Disability	70.0 ± 2.5	64.2 ± 4.2	t = 1.2, NS
	Male/Female	10/3	10/2	x² = 0.2, NS
Clincal

	DSMIII(R)
		Adjustment disorder with depressed mood	15	17	z = -0.06, NS
		Major depressive episode – single	23	25	z = -0.02, NS
		Major depressive episode – recurrent	08	08	NA*
		Bipolar disorder	08	00	z = 1.10, NS
		Suicide attempts	08	08	NA*
	Cognitive complaints
		Memory	54	42	z = 0.60, NS
		Concentration	46	33	z = 0.70, NS
Pulmonary
	P_aCO₂		37.7 ± 1.3	33.7 ± 0.7	t = 2.7, p < 0.01
	FVC		99.1 ± 4.2	106.2 ± 4.2	t = -1.2, NS
* NA, not analyzed. Demographic and pulmonary data are presneted as means ± SEM and clinical data as the percentage of patients with positive symptoms.

Primary data analysis involved comparisons of the two patient groups on the objective neuropsychological and neuropsychiatric measures. Sidak’s procedure was used to adjust the level of statistical significance for multiple pair-wise comparisons. A p-value of 0.002 defined the corrected level of statistical significance. Due to the large number of comparisons and the lack of power associated with the small sample sizes, any differences between the groups may most accurately be interpreted as trends.

As seen in Table 2, none of the scores from the neuropsychiatric or neuropsychological measures were significantly different between the two groups. There was, however, a trend for patients with PPS to perform better on both the Symbol-Digit and Trails B tasks. These procedures are related to attention, speed of mental processing, and ability to shift mental set. There was a trend for patients with PPS to have higher scores on the MMPI scale related to introversion and lower scores on the scale related to symptoms of hypomania. Despite failing to meet statistical significance, the fact that these two neuropsychological and the two neuropsychiatric procedures measure similar functions enhances the reliability of the findings.

TABLE 2. Comparison of neuropsychological and neuropsychiatric measures between patients with

and without PPS.

Measures					PPS (n = 13)	No PPS (n = 12)	t Test
Neuropsychological
	Attention/psychomotor speed
		Digits Forward			6.9 ± 0.4	6.8 ± 0.5	0.14, NS
		Digits backward			4.9 ± 0.3	4.5 ± 0.4	0.87, NS
		Stroop
			A		44.5 ± 2.1	50.4 ± 3.5	-1.47, NS
			B		58.3 ± 1.7	62.8 ± 2.5	-1.53, NS
		Trail Making A			29.6 ± 2.2	31.9 ± 2.6	-0.68, NS
	Mental set functions
		Trail making B			67.8 ± 4.2	92.0 ± 7.2	-2.98, p<0.007
		Symbol digit			52.7 ± 1.4	44.8 ± 2.4	2.90, p<0.008
		Stroop C			114.8 ± 4.5	116.8 ± 8.2	-0.23, NS
	Memory
		Verbal memory
			RAVLT
				Trial 1	7.6 ± 0.5	7.0 ± 0.8	0.7, NS
				Trial 5	12.6 ± 0.7	12.1 ± 0.9	0.5, NS
				Recall	10.6 ± 0.8	9.8 ± 1.0	0.68, NS
				Recognition	14.1 ± 0.3	13.8 ± 0.3	0.57, NS
				False positives	1.0 ± 0.3	1.2 ± 0.5	-0.43, NS
		Visual memory
			ROCF		14.5 ± 1.4	15.9 ± 1.9	-0.59, NS
	Word list generation
		FAS			39.2 ± 3.0	37.8 ± 3.7	0.29, NS
	Visuospatial ability
		ROCF (copy)			31.5 ± 0.6	32.6 ± 0.9	-1.04, NS

Neuropsychiatric
	BDI		11.5 ± 2.7	12.1 ± 2.2	-0.16, NS
	MMPI
		1	72.3 ± 2.6	64.9 ± 2.7	1.97, NS
		2	72.5 ± 3.9	60.7 ± 4.4	2.01, NS
		3	70.4 ± 2.4	68.6 ± 3.4	0.43, NS
		4	61.2 ± 3.7	61.5 ± 3.2	-0.07, NS
		5	48.8 ± 1.9	52.1 ± 4.6	-0.66, NS
		6	60.6 ± 2.7	58.3 ± 3.5	0.53, NS
		7	63.0 ± 3.5	59.0 ± 3.2	0.84, NS
		8	65.5 ± 3.3	63.2 ± 2.6	0.56, NS
		9	50.9 ± 2.4	61.7 ± 3.2	-2.72, p<0.01
		10	63.1 ± 3.8	50.8 ± 2.2	2.74, p<0.01
Data are presented as means ± SEM.

DISCUSSION

Similar to a recent study by Conrady et al. (1989). who noted more psychiatric distress in women with postpoliomyelitis than in those with metastatic breast cancer, our postpoliomyelitis patients, when examined as a single group, had a high prevalence of mood disturbance, including somatic concerns and

feelings of irritability. Performance on cognitive tasks appeared to be normal, except for borderline impairment for nonverbal memory and speed of color naming.

However, when comparing patients with and without PPS, complaints of memory and concentration difficulties, as well as psychiatric histories, failed to differentiate the two patient groups. Additionally, no significant differences between the two groups were seen on any of the objective neuropsychological and neuropsychiatric measures. Despite the late onset of increasing weakness and pain, patients with PPS failed to have signiticantly higher scores of depression than those without PPS. In less clearly defined subgroups of postpoliomyelitis patients, Conrady et al. (1989) also reported no significant difference of psychiatric symptoms in those patients seeking assistance for physical disability and those in support groups presumed to be more physically stable.

Lane et al. (1974), however, previously reported late-onset mental changes in postpoliomyelitis patients. They found that 21 of 55 patients had a syndrome of fatigue often accompanied by headache, depression, and decreased ability to concentrate. Exertional dyspnea and abnormal sensitivity to cold were also noted in their patients. Fourteen of these 21 patients had mildly elevated P_aCO₂ levels, with associated reduction in vital capacity. The authors suggested that the emergence of this syndrome may be secondary to late onset ventilatory insufficiency. Although their study was completed prior to the more recent criteria for

PPS, their patients may have had this disorder.

In the present study, patients with PPS had significantly higher P_aCO₂ levels, but did not have increased memory or concentration difficulties assessed either by interview or by objective neuropsychological procedures. In addition, when the entire sample was considered, P_aCO₂ levels were not in the clinically elevated range and no differences in P_aCO₂ levels between patients with and without complaints of decreased memory or concentration occurred. Thus, our study fails to confirm the findings of Lane et al. (1974); however, more advanced degrees of ventilatory insufficiency in their patients may have contributed to these differences.

The cause of mood disturbances and cognitive complaints in postpoliomyelitis patients is unknown. Ventilatory changes (Lane et al., 1974), sleep disturbances (Fischer. 1987), and emotional reactions to disability (Kohl, 1987), previously reported, need to be further evaluated. Additionally, these symptoms may relate to the pathological changes of the brain that are associated with poliomyelitis. With the acute infection, these pathological changes were associated with feelings of apprehension, anxiety, and fear, as well as symptoms of hypomania (Noran, 1968). Residual psychic disturbances have also been observed (Bodian, 1947). Acute histopathology (Bodian, 1947, 1949; Matzke and Baker, 1951) and viral immunofluorescence studies (Torack and Morris, 1988) have shown involvement of the thalamus, hypothalamus, and brainstem reticular formation — structures that contain important elements of the limbic and reticular activating systems. In addition to the behavioral changes noted in acute poliomyelitis, other forms of pathology involving these areas have produced alterations of attention and concentration as well as psychiatric symptoms (Trimble and Cummings, 1981; Cummings, 1988[sic]; Torack and Morris, 1988). Recently, Pezeshkpour and Dalakas (1988) examined the pathology of postpoliomyelitis patients with and without PPS. They reported the presence of active gliosis, inflammatory cells, and neuronal atrophy in the spinal cords of both groups of postpoliomyelitis patients, but suggested that an ongoing neuronal reaction continues for many years and may contribute to the late-onset weakness associated with PPS. It remains unclear, however, whether this inflammation is causative or reflects secondary effects of cell death associated with aging. Although only the spinal cords were evaluated in this study, the same slow inflammatory reaction may also be present in the diencephalon and nonmotor areas of the brainstem and might be expected to contribute to nonmotor symptoms. The high incidence of depression noted in the entire group of postpoliomyelitis patients might be attributable to brainstem changes sustained at the time of the original infection or to on-going cell loss. Depression has been reported previously in patients with lesions involving this region (Cummings, 1986), and source nuclei for serotonin and norepinephrine, transmitters implicated in the mediation of depression (van Praag, 1982), are located in the same area. The trend noted in this study for PPS patients to perform better than those without PPS on two procedures evaluating concentration and the ability to shift mental set may reflect less intense original injury in patients now beginning to develop PPS. A similar mechanism has been suggested to explain less intense, although new onset, weakness in PPS patients (Klingman et al., 1988).

Our results suggest that PPS is not consistently associated with specific cognitive or personality disturbances. These findings suggest that either nonmotor structures in survivors of acute poliomyelitis were originally less severely involved, compensated more than motor areas, and/or have greater functional reserve, permitting relative sparing of nonmotor behavior with the onset of PPS. Delayed inflammatory changes, if associated with PPS, may essentially be restricted to lower motor neurons despite evidence for more widespread pathological involvement of the central nervous system noted acutely. It should be pointed out, however, that other explanations of these data exist. As half the fibers of a muscle need to be affected before clinical weakness occurs (Klingman et al., 1988), the non-PPS patient group may have included patients with subclinical escalating weakness who are destined to develop PPS. Our patients were examined on only one occasion, so that progressive weakness and atrophy could not be documented.

Longitudinal studies are required to adequately address these issues.

The delayed onset of new symptoms in this large population of patients is an important medical problem, and systematic investigation of nonmotor involvement has clinical and pathogenetic implications.

Acknowledgment: We wish to thank Julie A. Fishman. Ph.D., for assistance in neuropsychological evaluations. REFERENCES

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Wechsler, D. WAIS-R manual. Psychological Corporation, New York, 1981. The Lincolnshire Post-Polio Network

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Occupational Therapy and the Postpolio Syndrome

Occupational Therapy and the Postpolio Syndrome Grace R. Young

American Journal of Occupational Therapy, Vol 43, No. 2, February 1989, 97-103 Lincolnshire Post-Polio Library copy by kind permission of the author

Key Words: occupational therapy services, physical disabilities, occupational therapy, poliomyelitis, rehabilitation.

Approximately 75,000 polio survivors are experiencing new weakness, pain, and fatigue that are related to their initial disease. These problems affect their functional ability; therefore, they are of concern to occupational therapists. Overwork of a weakened neuromuscular system is believed to be the cause of these late symptoms. This article reviews current writings on the late effects of poliomyelitis. An understanding of the symptoms, causality, and psychosocial ramifications of this phenomenon facilitates effective occupational therapy intervention. Guidelines for occupational therapy assessment and treatment are included.

Grace R. Young, OTR, is Senior Occupational Therapist at Kaiser Permanente Medical Center, Downey, California 90242. She is also enrolled in the advanced master’s degree program of the Department of Occupational Therapy at the University of Southern California, Los Angeles, California.

This article was accepted for publication April 13, 1988.

A variety of new symptoms are now affecting at least 75,000 persons who recovered from poliomyelitis many years ago. Caspar’s (1985) article was the first to make occupational therapists aware of this problem. Because these new symptoms are causing serious health and functional problems, it is likely that occupational therapists will increasingly be called on to evaluate and treat these persons. This article discusses the nature of the acute disease, symptoms of the late effects, and current theories on causality, and makes suggestions for treatments. Because the coping mechanisms learned during the initial disease have had such a profound effect on polio survivors, the importance of the psychosocial ramifications of the postpolio syndrome is stressed.

Description of Acute Poliomyelitis

Acute poliomyelitis (polio) is a viral disease that affects the anterior horn cells, or motor neurons, in the spinal cord. Some of the motor neurons are destroyed, some are not affected, and some are affected but recover with residual scarring (Wiechers, 1985).

The clinical symptoms include flaccid paralysis and a loss of reflexes, with resulting atrophy in the affected muscles. Although the lower extremities are affected more often than the upper extremities, the paralysis may be asymmetrical and patchy in character and may result in complete or partial monoplegia, hemiplegia, paraplegia, or quadriplegia. Sensation is not affected (Hopkins & Smith, 1978). A loss of motor neurons in the brain stem (bulbar polio) may lead to respiratory involvement. Bulbar polio was the major cause of death for early polio victims.

At the turn of the century, the disease earned the name infantile paralysis because it tended to strike young children. However, over the years there was a steady increase in the age of onset. A survey of 201 persons by Halstead, Wiechers, and Rossi (1985) showed that the median age of the respondents who acquired polio before 1949 was 8 years compared with a median age of 16 years for those affected in 1949 or later. Older victims manifested more severe symptoms than younger victims did. Sixteen percent of those who acquired polio before the age of 10 years required breathing equipment compared with 35.7% who were 10 years of age or older at the onset of the disease (Halstead et al., 1985). Weinstein, Shelekov, and Seltser (1952) reported that, out of 259 patients hospitalized with poliomyelitis, monoplegia occurred more often in persons under 16 years of age than in adults, but quadriplegia occurred 2.5 to 3 times more often in adults than in children.

The Salk vaccine, which consists of inactivated, or killed, poliovirus, was introduced in the mid-1950s, followed in 1960 by the Sabin oral vaccine, which consists of live attenuated poliovirus (Laurie, Maynard, Fischer, & Raymond, 1984). The annual number of reported polio cases in the United States dropped from a high of 56,000 in 1952 (Codd & Kurland, 1985) to an average of 12 since the vaccine was created. From 1982 through 1983, the 21 cases reported were all related to inoculation with the live vaccine, and 3 of those cases occurred in immune-deficient persons (Fischer, 1985). The scourge of poliomyelitis appeared to have become a chapter of medical history.

Description of the Late Effects of Polio

Until recent years, poliomyelitis was considered to be a stable disease, although occasional reports of late occurring muscle weakness and atrophy have appeared in the medical literature since 1875 (Codd & Kurland, 1985). Recently, however, it has become clear that some polio survivors may experience a gradual decline in health and in functional ability decades after the acute attack.

The question of appropriate nomenclature has not been resolved. Late effects of polio, postpolio sequelae, and progressive postpolio muscular atrophy (PPMA) are among the terms used. The phrase post-polio syndrome is used in a general sense to describe those symptoms or conditions that are directly or indirectly related to the late effects of polio.

Researchers divide health problems related to previous poliomyelitis into two categories (Codd & Kurland, 1985; Dalakas, Sever, et al., 1985). The first group includes symptoms that have a definable cause and that are primarily musculoskeletal rather than neurological in origin. These include joint, limb, or trunk deformities that can cause degenerative arthritis, nerve entrapment, pain, decreased stamina, frequent falls, unstable gait, or increased bracing needs. The second category, often referred to as PPMA, involves symptoms without a readily identifiable cause. These include slowly progressive muscular weakness both in previously weakened muscles and in muscles apparently spared during the acute attack. This weakness is often accompanied by muscle pain, fatigue, cold intolerance, or new difficulties in breathing (Codd & Kurland, 1985; Dalakas, Sever, et al., 1985; Halstead et al., 1985; Owen, 1985).

Of all these new health problems, fatigue can be the most distressing because it imposes limits on activity

but its existence is not obvious to others. The fatigue, a systemic, generalized exhaustion, may be so severe and so out of proportion to the level of activity that such persons feel an overwhelming need to “just stop dead in their tracks” (Raymond, 1986, p. 1398). Dr. Lauro Halstead, a polio survivor, describes a phenomenon called the polio wall, which is a sudden onset of symptoms such as intense fatigue, headache, weakness, hot and cold rashes, sweating, or a feeling like “hitting a wall” (Halstead et al., 1985, p. 1398).

Concomitant with the development of new symptoms is an increase in difficulties with activities of daily living. Most frequently reported were difficulties with walking and climbing stairs and with transfers (e.g., wheelchair to bed). Other activities of daily living affected were homemaking, driving, bathing, dressing, eating/swallowing, and bladder/bowel function (Brooke, Stolov, Shillam, & Kelly, 1987; Halstead et al., 1985; Kaufert, Syrotuik, Kaufert, & Gilbert, 1985).

Etiological Theories

Several theories attempt to explain the progression of disability from a disease that was once believed to be stable.

Amyotrophic Lateral Sclerosis

At one time, some researchers thought that the late effects of polio might actually be a form of amyotrophic lateral sclerosis (ALS), which bears a clinical similarity to the postpolio syndrome. However, ALS is characterized by new, rapidly progressive, generalized muscle weakness and by the presence of bulbar signs, respiratory difficulties, and upper motor neuron signs such as increased deep tendon reflexes, spasticity, and abnormal Babinski signs. Patients with ALS die within an average of 3 years after the onset of the disease (Mulder, Rosenbaum, & Layton, 1972). The postpolio syndrome progresses slowly, and upper motor neuron signs are rarely present (Dalakas, Elder, et al., 1986; Mulder et al., 1972). No evidence, either from clinical or experimental studies, suggests that the two conditions are related (Laurie et al., 1984).

The Dormant Virus Theory

Although some researchers have demonstrated that the poliovirus can persist in humans under very specific circumstances, there is no evidence linking viral persistence with the late effects of polio (Cashman, Siegel, & Antel, 1987; Codd & Kurland, 1985; Dalakas, Elder, et al., 1986; Johnson, 1984).

The Aging Process

Decreased muscle strength due to a loss of anterior horn cells is a normal part of the aging process. Since polio survivors already have a decreased number of anterior horn cells, some researchers believe that the natural aging process quickly depletes the remaining anterior horn cells and leads to progressive weakness (Holman, 1986). However, Tomlinson and Irving (1985) have shown that there is no significant loss of motor neurons before the age of 60, yet many postpolio individuals experience serious changes at a much younger age. This lessens the credibility of the theory that aging alone can explain the late effects of polio.

Overwork Weakness

A motor unit consists of an anterior horn cell or motor neuron, its axon, and all of the individual muscle

fibers innervated by that motor neuron. When a motor neuron dies, Wallerian degeneration occurs in the axon, which results in the loss of trophic influence to the muscle fibers (Wiechers, 1985). Motor units that are still functioning send axon sprouts to the orphaned muscle fibers, and a new neuromuscular junction is formed. This process, called reinnervation by terminal axon sprouting, may continue for 2 years or more after the acute infection (Wiechers, 1985). If there are no functioning motor units nearby, the orphaned muscle fibers fibrillate and atrophy until they die (Wiechers, 1985).

Poliomyelitis causes an overall loss of motor units; therefore, the remaining units must innervate many more muscle fibers than they did originally. A normal motor neuron drives 200 to 500 individual muscle fibers, whereas a reinnervated motor neuron may drive 1,000 to 5,000 or more individual muscle fibers (Wiechers, 1985).

Increased muscle strength during recovery from paralytic poliomyelitis can result from (a) recovery of motor units that were initially affected but that regained function; (b) strengthening of the remaining functioning muscles by exercise; or (c) reinnervation by terminal axon sprouting of those muscle fibers that were orphaned (Wiechers, 1985).

Evidence indicates that the anterior horn cells that were initially affected but later recovered do not recover normally. Bodian’s (1985) research with rhesus monkeys enabled him to study changes in the spinal cord from a few days to 1 year after inoculation with the poliovirus. An examination of 40 separate limb regions in 11 animals sacrificed 2 to 5 days after the onset of paralysis showed that only 3% to 4% of the anterior horn cells appeared normal even when the extremities were clinically normal or only mildly paralyzed. However, at the end of 1 month, 90% of the surviving cells had regained a normal appearance, even though the vast majority had been affected.

This scarcity of normal-appearing motor neurons early in the paralytic process, even in cases with mild paralysis, supports evidence that many cells are invaded by the virus and later appear to recover. These become the parent motor neurons for those muscle fibers that were orphaned initially.

Some researchers postulate that the surviving motor neurons carry permanent scars from the infection and are unable to keep pace with the metabolic demands of innervating their overload of muscle fibers. This results in a dying back of the axons that have been overused (Dalakas, Sever, et al., 1985; Wiechers, 1985). Tomlinson and Irving (1985) found that many of these surviving neurons are smaller than normal. Other researchers have found evidence of ongoing denervation in the muscles of polio survivors who, at the time of the investigation, showed no late symptoms (Cashman, Maselli et al., 1987; Dalakas, Elder, et al., 1986; Martinez, Conde, & Ferrer, 1983).

In summary, most researchers believe that PPMA is caused primarily by the overwork of abnormal motor neurons that must innervate an excessively high number of muscle fibers. After many years of excessive metabolic demands, this results in the death of the terminal axon sprouts, leading to a significant and ongoing decrease in muscle strength.

Scope of the Problem

The number of polio survivors in the United States has been estimated at 254,000. According to the National Center for Health Statistics, polio is the Number 2 cause of paralysis in America, second only to stroke (Speier, Owen, Knapp, & Canine, 1987). The percentage of people experiencing the late effects of poliomyelitis is difficult to determine. Those on whom many of the epidemiological studies have been based have been contacted through recently formed networks of polio survivor groups or rehabilitation centers. These studies may tend to overrepresent those groups with the highest levels of disability and

those experiencing the more severe postpolio sequelae because these people have the greatest amount of contact with rehabilitation facilities and support programs. Conversely, these studies may tend to underrepresent those who recovered so completely that they are no longer counted within the polio disabled population (Kaufert et al., 1985).

A study of polio patients treated at the Mayo Clinic between 1935 and 1955 showed that 22.4% of the 125 respondents had developed new symptoms related to their previous poliomyelitis (Codd, Mulder, Kurland, Beard, & O’Fallon, 1985). A similar study located 670 polio patients who had been treated at the Sister Kenny Institute in Minneapolis, Minnesota, between July 1, 1952, and June 30, 1953. Of 327 respondents, 41% reported late symptoms (Speier et al., 1987). Because these surveys studied patients treated at specific facilities within a particular time frame, they may be more heterogenous than the studies previously mentioned. However, a bias may still exist because those persons currently experiencing late sequelae might have a greater tendency to respond to questionnaires. In summary, it appears that 75,000 or more persons may be experiencing serious changes in health and function as a result of their previous poliomyelitis.

Adaptations of Persons Disabled by Polio

Polio survivors are their own best advocates. They designed many of the equipment adaptations that minimized their dependence on others (Raymond, 1986). They catalyzed the movement for the rights of disabled people (Laurie, 1982). Various studies have shown that a high percentage of polio survivors, including those with severe residual disabilities, have successfully coped with disability as measured by education, employment, and marriage (Affeldt, West, Landauer, Wendland, & Arata, 1958; Bailey, 1985; Halstead et al., 1985).

During recovery from the acute attack, polio patients were pushed to exercise and to wean themselves away from equipment. Mailhot (1980) stated, “Persistence in strenuous activity was the great rehabilitation virtue and the pursuit of ambulation second only to the quest for the Holy Grail” (p. 7). Restoration of function was achieved by focusing on a goal rather than on the effort needed to reach that goal. Pain and fatigue were disregarded. The continued use of equipment represented the lack of a cure. Some patients used their canes, crutches, or braces only when they were not with their parents out of fear their parents would destroy the aids (Kohl, 1987).

Now many polio survivors have had to start using ambulation aids, change from crutches to wheelchairs, or exchange manual chairs for motorized chairs. Others have had to resume or increase the use of respiratory equipment. This need to reduce activity levels or increase equipment use means more to these persons than just a change of life-style. It represents becoming disabled by the same disease for a second time (Frick, 1985). Polio survivors traditionally have not afffiliated with each other as a disability group. That fact, coupled with the physicians’ lack of knowledge, has left most people experiencing postpolio sequelae bereft of emotional or medical support and with a heightened sense of isolation. The combination of isolation and the loss of physical abilities thought to be permanent has made the psychological effects of the postpolio syndrome devastating (Frick, 1985).

It is important to understand the coping mechanisms used during the acute phase of the disease in order to understand the current psychological stresses. Denial was useful when one expected complete recovery from an acute polio attack. If complete recovery did not occur, denial changed to minimization. This reduced patients’ overwhelming fear about the future and allowed them to believe that life could still be meaningful and that normal activities were still possible. Overcompensation was another helpful mechanism used by polio survivors. It contributed to their assertiveness and brought them to the highest

possible level of functioning. The fear of not being as good as others was a challenge that pushed many polio survivors to become overachievers. However, although these coping strategies worked well in the early stages of the disease, they could be detrimental if used by persons suffering from the late effects of polio (Carsey & Tepley, 1986).

According to Carsey and Tepley (1986), social workers who are also polio survivors, there are benefits to seeing oneself as disabled. It allows one to be pleased with what one has accomplished. It allows one to join support groups that provide an environment in which to express the painful feelings that accompany physical loss. It allows one to shed the false pride that has prevented equipment use and thus gain extra energy to meet the demands of life.

Frick (1985) describes a process that polio survivors can use to adjust to a second period of disability. The first step, mourning, is appropriate no matter how often disability occurs in a person’s life. During this period, the person may reject equipment because it symbolizes the loss of physical ability. Succeeding stages, which Frick calls devaluing physique, enlarging the scope of values, and upholding asset evaluation, help the person to minimize the importance of physical achievement and appearance while maximizing feelings of self-worth by participating in study, work, political, leisure, and other nonphysical activities. At the same time, the person learns to value achievements within the limits of his or her disability instead of judging activities by normal standards that are inappropriate.

Occupational Therapy Intervention

The following guidelines are based on my experiences as a clinician and a polio survivor as well as on an extensive review of the literature.

Evaluation

A comprehensive evaluation should start with an interview that clarifies the patient’s present occupational role (e.g., student, worker, homemaker, volunteer, retiree). The person should be asked which activities produce pain, weakness, or fatigue; at what times of the day these symptoms occur; and how these problems interfere with present occupational roles. Equipment that the patient currently uses, such as splints, mobile arm supports, and adaptive aids, should be checked for fit and function.

If muscles in either upper extremity are weak, a manual muscle test should be performed on both upper extremities. An important factor to consider is that a polio-weakened muscle is much weaker than its clinical appearance indicates.

Perry (1985) found that polio muscles that appear normal (Grade 5) or good (Grade 4) clinically, as evaluated by manual muscle testing, may in fact have significant denervation and weakness when tested with electromyography. Patients with good or normal strength on a manual muscle test are expected to function in a normal manner. However, quantitative testing showed that Grade 5 in a polio muscle is actually only 75% as strong as Grade 5 in a nonpolio muscle, and Grade 4 in a polio muscle is only 40% as strong as Grade 4 in a nonpolio muscle. Therefore, a polio muscle that tests as 4/5 on a manual muscle test actually has to work 2.5 times as hard as a normal muscle to do the same job (Perry, 1985). Using an advanced technique, called macroelectromyography, Wiechers (1985) also observed that muscles that were of fair, or antigravity, strength (Grade 3) actually had a very low number of individual motor units.

While performing manual muscle tests, the therapist should keep in mind that upper extremity strength varies markedly throughout the range of motion. Elbow flexors that are Grade 4 at 90° flexion can be

Grade 2 when the elbow is fully extended; shoulders may have greater strength at a lower elevation than when raised above 45° (Perry, 1985). This may indicate that the person can perform functional activities most efficiently with the elbow flexed and the arm at the side.

Treatment

The role of exercise. Whether exercise is beneficial or detrimental in PPMA is controversial. A Swedish study reported improvement in the quadriceps muscles of 12 postpolio subjects after a 6-week high intensity strengthening program (Einarsson & Grimby, 1987). Wiechers, in a commentary on the study of Einarsson and Grimby, suggested the possibility that this study would have long-range negative effects (i.e., that hyperfunctioning of residual motor neurons might lead to decreased strength and cost the subjects additional ambulation time in the future) (Einarsson and Grimby, p. 283).

Feldman and Soskolne (1987) initiated a 6-month program of rigidly controlled, nonfatiguing exercises for 32 muscles in six patients. Fourteen muscles showed improved strength, 17 maintained strength, and 1 lost strength. At the end of the strengthening program, patients were placed on a maintenance program and given strict instructions on life-style changes and the need to avoid fatigue and keep within the limits imposed by their disability.

Maynard (1985) reported that efforts to increase strength by controlled strengthening programs often aggravate the pain problems. His approach is to maintain strength by continuing functional activities such as self-care, ambulation, and work or recreational activities. He questions the role of strengthening exercises for a muscle already too weakened by a degenerative process to meet its normal requirements, and eschews even gentle strengthening exercises for a weakened muscle that is being used, perhaps exhaustively, on a regular basis in normal activities of daily living (e.g., the quadriceps in walking). Herbison, Jaweed, and Ditunno (1985) also supported the hypothesis that overwork to muscles that have a decreased number of motor units may contribute to damage.

If muscles have weakened from disuse (e.g., from immobilization following surgery or a fracture), a nonfatiguing trial strengthening program may be instituted by the occupational therapist. However, most researchers believe that muscles that are already being used extensively on a daily basis should not be stressed further. Because the long-range effects of exercise are unknown, any exercise program must be undertaken with extreme caution. Exercise should never be performed to the point of pain or muscle cramps (Cashman, Maselli, et al., 1987). Perry (1985) cautioned patients who are on home exercise programs to stop exercising immediately if they develop persistent pain, muscle spasms, chronic fatigue, or further weakness.

The role of equipment and life-style modifications. Patients experiencing the late sequelae of poliomyelitis must be taught life-style changes that will help them avoid overusing their muscles. The focus of occupational therapy for this stage should be on evaluation of and retraining in activities of daily living, use of assistive devices, modifications of the work and home environments, and education in work simplification and energy conservation.

Occupational therapy intervention with the postpolio population may challenge some of our profession’s long-held beliefs, such as the importance of independence in self-care activities. The patient’s occupational roles need to be prioritized. If the role of worker is the most meaningful to the patient but getting ready for work each day causes fatigue that interferes with job performance, then independence in self-care is not a realistic goal. In his book about President Franklin D. Roosevelt, Gallagher (1985) wrote

Roosevelt was quite capable of dressing himself. With ingenuity and practice the paraplegic

learns how. Dressing, however, can never be as easy and unconscious a business as it is for the nonhandicapped. It can be done from a wheelchair, but it takes effort, thought, and time. Roosevelt, like any other polio or, indeed, like any other person, had limits to his energy and his strength…. Over the seven years of his rehabilitation, FDR had shown that he had fully as much motivation to be independent as any other polio [survivor]. … Now, as President, faced with virtually unlimited demands upon his energy and interests, he chose not to waste his limited muscle power on things like dressing. (pp. 163-164)

The occupational therapist’s creativity will be challenged to help the patient modify his or her life-style to enable the continuation of the most valued occupational roles. This challenge is heightened when a patient must start using orthoses or adaptive aids or finds that equipment that has functioned well for many years is no longer adequate due to progressive muscle weakening. For example, a person’s upper extremities may have weakened to the extent that a mobile arm support no longer can be used for both feeding and written communication. If writing, typing, or computer use is a more valued activity than self-feeding, the mobile arm support should be adjusted to facilitate performance of the former activity, and the latter may be delegated to a caretaker.

The key to successful life-style modification is patient education. If a person does not understand his or her disorder, it is very difficult to implement the necessary interventions and strategies.

Conclusion

As many as 75,000 polio survivors in the United States are suffering from debilitating symptoms related to their original disease. Muscle weakening and atrophy, pain, and severe fatigue are some of the late sequelae that are decreasing functional levels. Because overwork weakness is thought to be the precipitating factor, strengthening activities may be precluded. Life-style changes incorporating assistive or orthotic devices and education in energy conservation and work simplification are the focus of treatment. Occupational therapy intervention can help these patients modify their life-styles so they can continue to perform those activities they find most valuable.

Acknowledgments

I thank Elizabeth J. Yerxa, EdD, OTR, FAOTA, Emeritus Professor, Department of Occupational Therapy, University of Southern California, for her guidance and encouragement, and Jacquelin Perry, MD, Chief, Pathokinesiology Service, Rancho Los Amigos Hospital, for reviewing this article.

References

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Brooke, M., Stolov, W., Shillam, L., & Kelly, B. (1987). The importance of symptom pattern in evaluating post-polio neuromuscular changes. In L. Halstead & D. Wiechers (Eds.), Research and clinical aspects of

the late effects of poliomyelitis (pp. 49-53).White Plains, NY: March of Dimes Birth Defects Foundation. [PubMed Abstract]

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Cashman, N., Maselli, R., Wollmann, R., Roos, R., Simon, R., & Antel, J. (1987). Late denervation in patients with antecedent paralytic poliomyelitis. New England Journal of Medicine, 317(1), 7-12. [PubMed Abstract]

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Caspar, N. (1985, June). The late effects of polio: A personal and professional perspective. Occupational Therapy News, p. 9.

Codd, M., & Kurland, L. (1985). Polio’s late effects. In 1986 Medical and Health Annual, pp. 249-252.

Codd, M., Mulder, D., Kurland, L., Beard, D., & O’Fallon, W. (1985). Poliomyelitis in Rochester, Minnesota, 1935-1955: Epidemiology and long-term sequelae: A preliminary report. In L. Halstead & D. Wiechers (Eds.), Late effects of poliomyelitis (pp. 121-134). Miami: Symposia Foundation .

Dalakas, M., Elder, G., Hallett, M., Ravits, J., Baker, M., Papadopoulos, N., Albrecht, P., & Sever, J. (1986). A long term follow-up study of patients with post-poliomyelitis neuromuscular symptoms. New England Journal of Medicine, 314(15), 959-963. [PubMed Abstract]

Dalakas, M., Sever, J., Fletcher, M., Madden, D., Papadopoulos, N., Cunningham, G., & Albrecht, P. (1985). Neuromuscular symptoms in patients with old poliomyelitis: Clinical, virological and immunological studies. In L. Halstead & D. Wiechers (Eds)., Late effects of poliomyelitis (pp. 73-89). Miami: Symposia Foundation.

Einarsson, G., & Grimby, G. (1987). Strengthening exercise program in post-polio subjects. In L. Halstead & D. Wiechers (Eds.), Research and clinical aspects of the late effects of poliomyelitis (pp. 275-283). White Plains, NY: March of Dimes Birth Defects Foundation. [PubMed Abstract]

Feldman, R., & Soskolne, C. (1987). The use of non-fatiguing strengthening exercises in post-polio syndrome. In L. Halstead & D. Wiechers (Eds.), Research and clinical aspects of the late effects of poliomyelitis (pp. 335-341). White Plains, NY: March of Dimes Birth Defects Foundation. [PubMed Abstract]

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Herbison, G., Jaweed, M., & Ditunno, J. (1985). Clinical management of partially innervated muscle. In

Halstead L., & Wiechers, D. (Eds.), Late effects of poliomyelitis (pp 171-177). Miami: Symposia Foundation.

Holman, K. (1986). Post-polio syndrome: The battle with an old foe resumes. Postgraduate Medicine, 79(8), 44-53. [PubMed Abstract]

Hopkins, H., & Smith, H. (1978). Willard and Spackman’s occupational therapy (5th ed.). Philadelphia: Lippincott.

Johnson, R. (1984). Late progression of poliomyelitis paralysis: Discussion of pathogenesis. Reviews of Infectious Diseases, 6, 568-570. [PubMed Abstract]

Kaufert, J., Syrotuik, J., Kaufert, P., & Gilbert, P. (1985). Epidemiological issues in follow-up studies of the impact of poliomyelitis. In L. Halstead & D. Wiechers (Eds.), Late effects of poliomyelitis (pp 135- 152). Miami: Symposia Foundation.

Kohl, S. (1987). Emotional responses to the late effects of poliomyelitis. In L. Halstead & D. Wiechers (Eds.), Research and clinical aspects of the late effects of poliomyelitis (pp. 135-143). White Plains, NY: March of Dimes Birth Defects Foundation. [PubMed Abstract]

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Laurie, G., Maynard, F., Fischer, D., & Raymond, J. (1984). Handbook on the late effects of poliomyelitis for physicians and survivors St. Louis: Gazette International Networking Institute.

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Martinez, A., Conde, M., & Ferrer, M. (1983). Chronic partial denervation is more widespread than is suspected clinically in paralytic poliomyelitis. European Neurology, 22, 314-321.

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Perry, J. (1985). Orthopedic management of post-polio sequelae. In L. Halstead & D. Wiechers (Eds.), Late effects of poliomyelitis (pp 193-205). Miami: Symposia Foundation.

Raymond, C. (1986). Medical news and perspectives. Journal of the American Medical Association, 25, 1397-1404.

Speier, J., Owen, R., Knapp, M., & Canine, J. (1987). Occurrence of post-polio sequelae in an epidemic population. In L. Halstead & D. Wiechers (Eds.), Research and clinical aspects of the late effects of poliomyelitis (pp. 39-48): White Plains, NY: March of Dimes Birth Defects Foundation. [PubMed Abstract]

Tomlinson, B., & Irving, D. (1985). Changes in spinal cord motor neurons of possible relevance to the late

effects of poliomyelitis. In L. Halstead & D. Wiechers (Eds.), Late effects of poliomyelitis (pp. 57-70). Miami: Symposia Foundation.

Weinstein, L., Shelekov, A., & Seltser, R. (1952). A comparison of the clinical features of poliomyelitis in adults and in children. New England Journal of Medicine, 246(8), 296-302.

Wiechers, D. (1985). Pathophysiology and late changes of the motor unit after poliomyelitis. In L. Halstead & D. Wiechers (Eds.), Late effects of poliomyelitis (pp. 91-94). Miami: Symposia Foundation.

Occupational Therapy and the Postpolio Syndrome Read More »

Stress and Type A Behaviours as Precipitants of Post Polio Sequelae The Felician Columbia Survey

Stress and “Type A” Behavior as Precipitants of Post-Polio Sequelae: The Felician/Columbia Survey Richard L. Bruno, PhD, and Nancy M. Frick, MDiv, LhD

In LS Halstead and DO Wiechers (Eds.): Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains: March of Dimes Research Foundation, 1987.

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

A behavioral profile has begun to emerge from studies of persons who survived acute poliomyelitis and are now experiencing post-polio sequelae. Persons who had polio have been shown to be employed full time at four times the rate of the general disabled population (1,2). Persons who had polio have more years of formal education on average than the general population (3), and marry at approximately the same rate as those who are not disabled (4). These data, combined with our own experience with thousands of persons who had polio, indicated that “polio survivors” are competent, hard-driving and time-conscious overachievers who demand perfection in all aspects of their personal, professional, and social lives. It appeared that those who survived polio exhibit “Type A” behavior and would therefore experience chronic emotional stress.

The notion that individuals who had polio exhibit “Type A” behavior and experience chronic stress was thought to be extremely important for an understanding of the pathophysiology and treatment of postpolio sequelae (PPS). Animal studies have demonstrated that stress accelerates the onset of muscle fatigue (5), augments age-related decreases in the terminal axon branch number (6), and accelerates age-related losses of neurons (7). Therefore, this study was designed to test 2 hypotheses: 1) persons who had poliomyelitis exhibit “Type A” behavior and symptoms pathognomonic of chronic stress, and 2) “Type A” behavior and stress precipitate or exacerbate PPS.

The Felician/Columbia Survey

Research funded by the Joel Leff Foundation.

METHODS

In order to test the above-stated hypotheses, a self-administered survey was designed to record demographic data, quantify “Type A” behavior, document psychophysiologic symptoms that are recognized as concomitants of chronic stress (including disturbed sleep), and identify the conditions that precipitated or exacerbated PPS.

Quantification of “Type A” behavior. To quantify “Type A” behavior. the Young and Barboriak brief “Type A” questionnaire (8) was included as part of the survey. This instrument consists of 10 questions that elicit responses characteristic of “Type A” behaviors and attitudes (Table 1). It was tested by its authors on a sample of nondisabled males (mostly professional or paraprofessional hospital employees) who were employed full time and who were without cardiovascular disease. They obtained a mean score of 35.6 (+/- 14.0) for this control sample, and 80% agreement was found between their brief “Type A” score and that obtained using the 65-question Jenkins Activity Survey.

All “Type A” questionnaires, including the Young and Barboriak instrument (8), are designed to quantify the behavior of men who are employed full time outside of the home. Since the post-polio population includes men and women, some of whom are employed part time, in the home, unemployed, or receiving social security disability, it was recognized that some of the post-polio respondents would not be able to complete all 10 questions. Therefore, the scoring of the Young and Barboriak questionnaire (8) was modified in consultation with its authors. It was decided that all questionnaires would be scored by assigning 10 points to each “Type A” response, summing those responses, and dividing by the total number of questions answered. This provided a “Type A” percentage on the basis of 10 questions, in persons who were employed full time, and on the basis of 7 questions (eliminating questions 2, 5, and 8) in persons who were not employed full time. The 7-question “Type A” score has been found by the authors of the questionnaire to correlate well with the 10-question score (Young, personal communication).

Documentation of psychophysiologic symptoms. To document the occurrence of psychophysiologic symptoms that are recognized as concomitants of chronic stress, subjects were asked if they experienced frequent feelings of anxiety, headaches, muscle & [sic], spasms, and “difficulty in failing asleep because the mind is racing.” They were also asked if they had been diagnosed as having asthma, hypertension, coronary artery disease, ulcer, or colitis. In addition, subjects were asked if they were experiencing “generalized random myoclonus” (GRM), the slow contraction and rapid twitching that occur randomly in limb and trunk muscles during sleep and especially at sleep onset (9). They were also asked if their sleep was disturbed by GRM.

Psychologic stress and other precipitants of PPS. Subjects were asked whether emotional stress or “upset” precipitated or exacerbated the 3 most frequently reported and least well understood PPS-muscle weakness, muscle pain, and unaccustomed fatigue. They were also asked if these symptoms were precipitated or exacerbated by physical overexertion, exercise, or exposure to cold and hot ambient temperatures. Finally, the subjects were asked if PPS interfered with their ability to participate in social activities, complete or perform work, and perform self-care activities; they were also asked about their attitudes concerning their new symptoms and the general topic of disability. (Functional and attitudinal data will be presented elsewhere.)

PROCEDURE

Distribution of the Survey

On April 1, 1985, 1,200 surveys were mailed to all self-identified post-polio clinics and support groups in the continental United States. Respondents were instructed to complete the survey after April 15 and to return it by June 30, 1985. A copy of the survey was obtained by a national organization that provides services for persons with disabilities. The organization reproduced the survey and mailed it to their offices across the country for distribution without our knowledge. While this unexpected distribution probably increased the number of persons who obtained the survey, control of the sample was lost and no meaningful response rate can be reported.

Surveys that did not include a completed “Type A” questionnaire or reported a co-existing medical condition that could cause muscle weakness, muscle pain, or fatigue (eg, arthritis, cancer, CVA, hyperthyroidism) were not included in the analysis.

Data Analysis

Orthogonal analysis of variance and independent groups t-tests were applied to compare parametric variables between groups. The chi-squared statistic was applied to compare the frequency of nonparametric variables between groups. A value of P < 0.05 was considered statistically significant.

RESULTS

Demographics, New Symptoms and Level of Functioning

The respondents were remarkably similar to those in other surveys of persons who had polio (Table 2). The average respondent was a 52-year-old female, who had acute poliomyelitis in 1948 at age 10.5 years. She was experiencing unaccustomed fatigue, muscle weakness (in muscles originally affected by the polio), and muscle pain. She ambulated without orthotics or ambulatory aids prior to developing new symptoms but required some assistive device (brace, cane or crutches) with the onset of PPS (Table 3). It should be noted that the ability to ambulate distinguishes these respondents from those in other surveys. The percentage of respondents who ambulated unassisted prior to PPS was 1.4 times greater than in other surveys. The percentage who used ambulatory aids or a wheelchair either prior to or following PPS onset was at least 2 times less than in other surveys.

“Type A” Score in Post-Polio Subjects

Between 88% and 91% of the respondents reported new decreases in muscle strength, increased muscle pain, or new or increased fatigue. The “Type A ” score was significantly higher in respondents reporting muscle pain and fatigue (but not decreased muscle strength) as compared to subjects without these symptoms (Figs. 1-3). Symptoms pathognomonic of chronic stress were reported by 49% to 58% of the respondents (Fig. 4). The “Type A” score was significantly higher in subjects who reported these psychophysiologic symptoms than in those who did not.

GRM was reported in 32.8% of the respondents and 29.9% reported both GRM and that their “sleep was disturbed by muscle twitching.” It is noteworthy that there was no relationship between GRM or GRMdisturbed sleep and reports of daytime fatigue.

Stress and Other Precipitants of PPS

“Emotional stress” was the second most frequently reported cause of fatigue (61 % of respondents) and the third most frequently reported cause of decreased muscle strength and muscle pain (45% and 51 % of respondents, respectively) (Fig. 5). The “Type A” score was significantly higher in subjects reporting that

their symptoms were exacerbated by emotional stress as compared to those who were unaffected by stress.

Exposure to cold ambient temperatures was the second most frequently reported cause of decreased muscle strength and muscle pain (62% and 60% of respondents, respectively). Exposure to cold or to heat was reported by 39% of the respondents as the third most frequently reported cause of fatigue. As in all other studies, the most frequently reported cause of decreased muscle strength, muscle pain, and fatigue was physical overexertion or exercise (reported by 92% to 95% of respondents).

DISCUSSION

The data indicate that both hypotheses should be accepted: 1) persons who had poliomyelitis demonstrate significantly more “Type A” behavior than do nondisabled controls, and evidence psychophysiologic symptoms pathognomonic of chronic stress, and 2) PPS are initiated or exacerbated by stress. In addition, there is an interrelationship between these 2 hypotheses . “Type A” scores are significantly higher in respondents who report that stress initiates or exacerbates PPS and in those subjects reporting psychophysiologic symptoms, new muscle pain, and unaccustomed fatigue.

Genesis of “Type A” Behavior in Persons Who Had Polio

There are a number of hypotheses as to why persons who had poliomyelitis exhibit “Type A” behavior and experience symptoms of chronic stress. It is possible that adults and even children who exhibited “Type A” behavior and were experiencing stress were more susceptible to infection by polio viruses because of stress-induced immunosuppression. It is also possible that to survive the acute polio infection and then thrive despite paralysis in a totally inaccessible world, the special drive of the “Type A” personality was required. It might also be the case that persons with disabilities must learn “Type A” behavior in order to succeed in a “barrier-full” society. For example, physical limitations might require one to be come “timeconscious” to perform common tasks that require more time to complete than for persons who are not disabled. Social prejudice might require persons with disabilities to become “hard-driving overachievers”

– personality, professionally, and especially physically – to be accepted by peers and employers.

This last hypothesis raises the question as to whether orthopedically disabled persons in general, but especially those disabled early in life, exhibit “Type A” behavior, experience chronic stress, and may also have late-onset problems. A recently completed study has identified late-onset problems in adults with spina bifida who, although younger than the post-polio population, are similar in educational level and ambulatory ability (Table 4) (Gingher, personal communication). It is interesting to note that muscle weakness, joint pain, and hypertension were reported about half as frequently in the spina bifida sample as in persons who had polio, while fatigue and muscle pain were not reported at all. We are presently conducting a survey of adults with spina bifida to document “Type A” behavior, psychophysiologic symptoms, and late-onset problems.

Stress and the Pathophysiology of PPS

The mechanism whereby stress induces or exacerbates PPS has not yet been described. Stress in animals has been shown to cause a variety of abnormalities that may contribute significantly to the pathophysiology of PPS. Stress has been shown to accelerate the onset of muscle fatigue and shorten lifespan (5). Stress has also been shown to augment age-related decreases in the number of terminal axon branches innervating both hind-limb and diaphragm muscles (6). A decrease in the number of functional terminal axon branches may be responsible for the shrinkage of motor unit territories seen in persons who had polio (12) and has been implicated as a probable cause of post-polio muscle weakness (13,14).

Corticosterone secretion, a specific hormonal concomitant of stress, has been shown to have deleterious effect that directly related to the hypothesized pathophysiology of PPS. Elevated corticosterone levels have been associated with the inhibition of axonal sprouting in aged animals with motor neuron denervation (15). Further, stress-induced hypersecretion of corticosterone has been shown to accelerate age-related losses of hippocampal neurons (16). This effect is thought to result from corticosteroneinduced inhibition of neuronal glucose uptake and the impairment of neuronal energy metabolism in these “metabolically vulnerable” neurons (7). It has been suggested that polio-damaged and extensively sprouted anterior horn cells are also metabolically vulnerable, and that post-polio muscle weakness might occur as these neurons fail to function and even die because they “are just not able to keep pace with the metabolic demands of innervating all of their muscle fibers” (13). Clearly, research needs to be conducted to document the relationship between the physiologic effects of stress and the pathophysiology of PPS.

Clinical Implications

This survey has documented the deleterious effects of physical overexertion and exposure to extremes in temperature in persons who were less severely affected by the original polio infection than were subjects in other studies (10,11,17). It has also documented that GRM occurred in nearly two-thirds of this postpolio sample. However, the failure to find a relationship between GRM-induced sleep disturbance and daytime fatigue suggests that GRM may not be a contributor to new and unaccustomed fatigue. Most importantly, this survey demonstrated that emotional stress is a precipitant of PPS. Fortunately, it is a precipitant that can be treated. Post-polio clinics and support groups should include stress management as an integral part of both therapeutic and wellness programs. We are presently studying combinations of cognitive and autonomic stress management techniques designed to reduce “Type A” behavior, counter the psychophysiologic symptoms of stress, and thereby decrease the symptoms of post-polio sequelae.

ACKNOWLEDGMENTS

The authors thank the survey participants, John H. Zuck, Jr. for his labors in preparing and distributing the survey, Dr. Charles Rooney for his motivating logic, and Drs. Larry Young, Julie Rosenheimer, Bob Sapolsky, and Nan Gingher for their generous assistance in the preparation of the manuscript.

REFERENCES

1. Codd MB, Mulder DW, Kurland LT et al: Poliomyelitis in Rochester, Minnesota, 1935-1955:

Epidemiology and long-term sequelae: A preliminary report. In Halstead LS, Wiechers DO (eds):

“Late Effects of Poliomyelitis.” Miami- Symposia Foundation, 1985. pp 121134.

2. Lando ME, Cutier RR, Garnber E: 1979 Survey of Disability and Work (Data Book). Washington.

DC: U.S. Department of Health and Human Services, 1982.

3. Frick NM: Demographic and psychological characteristics of the post-polio community. A paper

presented at the First Annual Conference on the Late-Effects of Poliomyelitis. Lansing. Michigan;

October, 1985.

4. Frick NM, Bruno RL: Post-polio sequelae: Physiological and psychological overview, Rehabil Lit

47:106-111, 1986. [Lincolnshire Library Full Text]

5. Parc WP: The effect of chronic environmental stress on premature aging in the rat. J Gerontol

20:78-84, 1965.

6. Rosenheimer JL: Effects of chronic stress and exercise on age-related changes in end-plate

architecture. J Neurophysiol 53:1582-1589, 1985. [PubMed Abstract]

7. Sapolsky RM, Krey LC. McEwen BS: The neuroendocrinology of stress and aging: The

glucocorticoid cascade hypothesis. Endocrin Rev (in press) [PubMed Abstract]

8. Young LD, Barboriak JJ: Reliability of a brief scale for assessment of coronary-prone behavior and

standard measures of Type A behavior. Percept Mot Skills 55:1039-1042. 1982. [PubMed Abstract]

9. Bruno RL: The autonomic psychophysiology of post-polio sequelae. A paper presented to the 152nd

National Meeting of the American Association for the Advancement of Science. Philadelphia,

Pennsylvania: May, 1986.

10. Halstead LS, Wiechers DO, Rossi CD: Late effects of poliomyelitis : A national survey. In Halstead

LS, Wiechers DO (eds): “Late Effects of Poliomyelitis.” Miami: Symposia Foundation, 1985, pp 11-

32.

11. Halstead LS, Rossi CD: New problems in old polio patients: Result of a survey of 539 polio

survivors. Orthopedics 8:845-850, 1985. [PubMed Abstract]

12. Wiechers DO, Hubbell S: Late changes in the motor unit after acute Poliomyelitis. Muscle Nerve

4:524-528, 1981. [PubMed Abstract]

13. Wiechers DO: Acute and latent effect of poliomyelitis on the motor unit as revealed by

electromyography. Orthopedics 8:870-872, 1985. [PubMed Abstract]

14. Dalakas MC, Elder G, Hallet M et al: A long-term follow-up study of patients with postpoliomyelitis neuromuscular symptoms. N Eng J Med 314:959-963, 1986. [PubMed Abstract]

15. Dekosky ST, Scheff SW, Cotman CW: Elevated corticosterone levels: A possible cause of reduced

axon sprouting in aged animals. Neuroendocrinology 38:33-38, 1984. [PubMed Abstract]

16. Sapolsky R M: G glucocorticoid toxicity in the hippocampus: Reversal by supplementation with

brain fuels. J Neurosci (in press) [PubMed Abstract]

17. Bruno RL, Johnson JC, Berman WS: Vasomotor abnormalities and post-polio sequelae: Functional

and clinical implications. Orthopedics 8:865-869, 1985. [PubMed Abstract]

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/typea.html> Created: 15th February 1997

Last modification: 29th January 2010.

Stress and Type A Behaviours as Precipitants of Post Polio Sequelae The Felician Columbia Survey Read More »

Mouth Intermittent Positive Pressure Ventilation in the Management of Postpolio Respiratory Insufficiency

John R. Bach, M.D.,Ť Augusta S. Alba, M.D.; George Bohatiuk, M.D., Lou Saporito, R.R.T.; and Mathew Lee, M.D.S

The use of mouth intermittent positive pressure ventilation alone or in combination with other noninvasive respiratory techniques as an alternative to tracheostomy in the home management of respirator-dependent postpolio persons was studied in 75 patients. The onset of polio was at an average age of 15 years. At that time, all were dependent on some form of respiratory assistance, most frequently, the iron lung. Fifty-nine percent of them remained respirator dependent from the onset. Forty-one percent became respi rator-dependent at an average of 18 years after onset of polio. Overall, they lost an average of 1.9 percent of vital

capacity per year. All used mouth intermittent positive pressure ventilation as their predominant mode of respira tory assistance for an average of 14.5 years. Four of them who had no measurable vital capacity used only mouth intermittent positive pressure ventilation 24 hours per day. Of the 66 who had no significant tolerance off 24 hours per day respiratory assistance, only six had tracheostomies. Despite severe physical disability and dependence on artificial ventilation, the majority of these persons have married, have been gainfully employed, and lead useful lives in society.

Poliomyelitis, which is caused by three antigenically

distinct types of enteroviruses, is a disease with a wide spectrum of clinical severity. As many as 99 percent of afflicted persons may be asymptomatic or suffer only minimal symptomatology. However, for the small percentage of persons who develop paralytic polio, the effects can be devastating with maximal muscle weakness occurring within days following the onset of weakness.

Although the last major polio epidemic in the United States was in 1955, over 200 cases have been reported since 1969 with 21 vaccine-associated cases in 1983 alone. Many of those afflicted during the polio epidemics of the 1940s and 1950s are now seeking medical attention for late postpolio complications of which the most life-threatening is insidious progres sive respiratory insufficiency.

The respiratory failure associated with polio occurs either by the destruction of anterior horn cells in the spinal cord innervating respiratory muscles or by neuronal destruction of the respiratory center in the medulla or both. Whatever the mechanism, this necessitates mechanical ventilatory assistance by means of the iron lung or tracheostomy tube with tracheostomy intermittent positive pressure ventilation (TIPPV) during the acute management of these patients. This acute stage had a 25 to 75 percent fatality rate.

Although many recover sufficient respiratory muscle control to free themselves from respiratory aid, the muscle weakness and loss of lung volume that occurs with age necessitates the reintroduction of artificial ventilation for some. This mechanical aid has usually been TIPPV despite the potentially serious complications of tracheostomy placement, poor cosmesis and patient reluctance.

Lassen et al’ reported 232 tracheostomized paralytic polio patients receiving TIPPV in 1956, but only 138 of them survived four years. In a more recent study of respiratory-dependent postpolio persons, the largest to date, TIPPV was the predominant method of assistance offered.” In that study, 23 used respiratory assistance full-time and 80 used it only overnight. Of these persons, 76 used TIPPV. Twenty-seven used the iron lung or the chest shell overnight, but as their margin of reserve diminished, the author stated that “the spectre of a tracheostomy” looms in their management.

To avoid the pitfalls associated with tracheostomy use, others have employed noninvasive means of nocturnal mechanical ventilation. Garay et alt reported the overnight use of the Emerson wrap negative pressure respirator by two severely hypercapnic post polio persons for 12 and 15 years. Nocturnal use alone allowed them to maintain “acceptable” blood gas levels during the day (Pco, 44 mm Hg, Pog, 58 to 63 mm Hg) and reversed marked pulmonary hypertension. However, their vital capacities (VC) were 1,000 and 1,060 ml, and thus, they did not require full time respiratory assistance. Likewise, Guilleminault and Motta reported the use of night-time chest shell ventilator in

*From the University of Medicine and Dentistry of New Jersey,

Newark, and New York University, New York. Assitant Professor of Rehabilitation Medicine.

Associate Clinical Professor of Rehabilitation Medicine. Professor of Rehabilitation Medicine. Manuscript received July 25; revision accepted October 30. five postpolio persons with symptomatic central and/or obstructive sleep apnea. These persons, too, had sufficient pulmonary reserve not to require full time respiratory aid.

We have studied the change in type and amount of respiratory assistance in severely involved postpolio persons with progressive decline in vital capacity Symptomatic hypoventilation, and in particular, severe nocturnal hypercapnia indicated the need for assistance. Mouth intermittent positive pressure ventilation (MIPPV) was used as the predominant method because of its effectiveness and its advantage in obviating the need for tracheostomy tube placement. Those persons not using overnight aid but employing MIPPV only during daytime hours were not included in this study since they generally had VC greater than 1 L and thus, were not vitally dependent on mechanical aid.

This is a study of the efficacy of MIPPV as an alternative to TIPPV in postpolio persons vitally dependent on respiratory assistance.

FIGURE 1. Patient with a vital capacity of 240 ml operating her motorized wheelchair by tongue control and receiving MIPPV via mouthpiece held in place by a gooseneck clamp.

maintain normal blood gas tensions while on body respirators. These patients switched to overnight MIPPV. Others, already using MIPPV during the daytime, preferred to continue employing it at night as well.

During periods of respiratory distress associated with URLs, patients are often hospitalized. They may be managed in the iron lung with or without supplementary MIPPV while compromised The effectiveness of ventilation is assessed by overnight capnograph (Pco, metabolic analyzer) monitoring correlated with blood gas determinations. Difficulty clearing secretions is managed by assisted coughing techniques. These include deep assisted breaths by mechanical aid or glossopharyngeal breathing (GPB) prior to a manually assisted cough, tracheal suctioning via the mouth or nose, and the use of a Coffiator. The Coffiator delivers a deep positive pressure breath via a face mask followed by a drop in pressure to – 100 cm Ho in 0.02 s to provide clearance of bronchial secretions. The GPB is an effective technique in aiding pulmonary toilet and normalizing the speech patterns of many patients. It can also provide from minutes to many hours of time free of ventilator assistance for many patients including some with vital capacities approaching 0

METHOD Seventy-five postpolio persons on MIPPV were studied. Seventy were managed in the home, five in chronic care institutions. Eight had complete quadriplegia, while 67 had varying degrees of quadriparesis. None had severe bulbar involvement. All were wheelchair dependent. The majority were mobile only in motorized wheelchairs. Twenty-seven had significant scoliosis. Sixty-sús were ventilator dependent 24 hours a day and could tolerate only seconds of time free of assisted ventilation unless capable of effective glossopharyngeal breathing.

All persons respirator dependent since onset of polio (1950 to 1956) were maintained on body respirators such as the iron lung and rocking bed until the late 1950s. They were trained in the use of MIPPV for daily periods of positive pressure ventilation as early as 1958. It allowed the postpolio survivor to “sigh” by taking two to three breaths before exhaling. It also provided him with additional ventilation during upper respiratory tract infections (URIS) and periods of respiratory distress. With the increasing availability of portable positive pressure ventilators over the next five years, the use of daytime MIPPV increased. Postpolio survivors no longer needed to be recumbent to receive aid. They left their body respirators each morning in favor of MIPPV with portable ventilators mounted onto motorized wheelchairs. A gooseneck holder maintained the mouthpiece adjacent to the mouth allowing an assisted breath as the person required (Fig 1).

It was soon appreciated that during sleep excessive loss of insufflated air with resulting poor ventilation could be prevented by holding the mouthpiece in the mouth with a lip seal (Fig 2) and by reflex contraction of the lip and jaw muscles. If needed, the cycling pressure or the tidal volume was increased on the respirator above daytime settings. It was rarely necessary to cover the nostrils to prevent leakage. As early as 1964, patients began to depend on MIPPV alone for their total ventilation 24 hours a day.

Postpolio persons with late onset respiratory insufficiency were also introduced to daytime MIPPV. As their restrictive pulmonary syndromes worsened and respiratory insufficiency progressed, the use of MIPPV gradually increased to full time. Thirty-one of these patients were ventilated adequately overnight for a decade or more with the chest shell and rocking bed. Some began to express symptoms of nocturnal hypoventilation and were found to no longer

When overnight MIPPV is ineffective, particularly in the presence of organic lung disease, severe complicating medical condition, or incompetent buccopharyngeal muscles, intubation or tracheostomy may be indicated.

FIGURE 2. Patient with a vital capacity less than 20 ml set up for overnight MIPPV with the mouthpiece supported firmly in the mouth by the Bennett lip seal.

Mouth PPV in Postpotion Respiratory f

ufficiency (Bach et at

Table 1 Forty Three Postpolio Persons with no Tolerance

off Respiratory Assistance on MIPPV Only

www

Average

Range

(21-66)

(0-1700)

47.8 yrs Age at onset of polio

14.9 yrs VC, most current

607 ml (supine)

17.4% pred Age at death (11 persons) 41.6 yrs

following MIPPY for

17.9 yrs *Includes one tracheostomized person.

(2-29)

RESULTS Seventy-five respirator dependent postpolio persons were separated into three categories as a function of the amount and method of respiratory assistance. Table 1 represents those persons with no tolerance off mechanical aid who used only MIPPV 24 hours per day. Twenty-three of these persons had been dependent on aid since the onset of polio. Twenty returned to at least overnight respiratory aid an average of 18.1 years (one to 37) postpolio and subsequently progressed to full-time respirator dependence. Twenty five of these persons had used the rocking bed, iron lung, or chest shell for an average of 14.7 years (two to 34) prior to switching to full-time MIPPV. Only four were recently tracheostomized and have used TIPPV an average of 2.5 years (1-4 years). + One of these is deceased.

Table 2 lists 23 persons dependent on full-time respirator assistance using MIPPV combined with some other technique compatible with home management. Twenty-one of these patients had been totally dependent on mechanical ventilation since onset of polio. Two became ventilator-dependent, three and 25 years postpolio, respectively. Twenty of the above persons had used a combination of the iron lung, chest shell, and/or rocking bed for an average of 14.2 years (two to 30) prior to employing MIPPV. The MIPPV was used overnight and the pneumobelt was used during the day in seven persons. Daytime MIPPV was used in conjunction with overnight use of the other body

respirators in the remaining 16 persons. Within the past two years, only two of these patients were tracheostomized and placed on TIPPV.

In these two groups of postpolio survivors (average VC 565 ml), only six have had tracheostomies after an average of nearly 30 years of dependence solely on noninvasive techniques of totally assisted ventilation.

Table 3 concerns postpolio survivors who became dependent on MIPPV overnight, and daytime as needed, an average of 26.6 years (17 to 35) after onset of polio. They were an average of six years younger than the patients on full time aid and had vital capacities an average of 450 ml greater. The Co, monitoring of these individuals off aid often revealed severe nocturnal hypercapnia to levels greater than 75 mm Hg with only moderate Pco, elevations (50 to 60 mm Hg) or even normal levels when they were awake. Oxygen saturation dropped from normal levels during waking hours to 70 to 80 percent and occasionally to 50 percent during sleep without aid.

Thirty-eight subjects were studied retrospectively over 150 patient years to determine the rate of loss of VC. There was an average loss of 18.6 ml per year or 1.9 percent (SD, 1.2). The average annual decrement in VC in the normal population is 1 to 1.2 percent. Our results are similar to those previously cited.’ Possible accelerated anterior horn cell drop out in postpolio survivors, scoliosis, and increased frequency of serious respiratory infections and atelectasis are probable factors related to the increased loss of VC.

The 75 persons in this study have used MIPPV as a major part of their aid regimen for a total of 1,028 patient-years or 14.8 years per person. Seventeen persons died while using MIPPV (one death per 60.5 patient-years of MIPPV). Sixteen of 32 men have married and 19 have been gainfully employed. Twenty six of the 42 women have married and 15 have been gainfully employed.

Table 2-Tucenty Three Postpolio Persons with no Tolerance off Respiratory Assistance on MIPPV and Body

Respirators

CASE 1 (Fig 2)

This person developed complete quadriplegia and respiratory insufficiency from paralytic polio in August 1955 at age 15 years. He has no vertebral deformity and has normal bulbar muscle control, but his VC has been Oml since onset. He used an iron lung for several months. He was then placed on a chest shell ventilator overnight. He also used MIPPV, as well as the Pneumobelt during the daytime from 1956 to 1981. In 1981, his Pco, rose to 45 to 50 mm Hg with a maximum tidal volume of 320 ml received by chest shell ventilator use. Because of suboptimal ventilation and the inability to “sigh” on Table 3.-.-Nine Postpolio Persons on MIPPY Overnight

Plus Daytime pra

Average

Range

48.0 yrs 17.6 yrs 485 ml 12.3% pred

(18-71) (3-37) (0-1600)

Average

Range

Age Age at onset of polio VC, most current

(supine) MIPPV+chest shell (nine pts),

the pneumnobelt (seven points), + rocking bed (six pts), to iron lung (two pts),

24 hours/day Age at death (seven persons)

following MIPPV for

16.7 yrs 44.3 yrs 14.6 yrs

Age Age at onset of polio VC, most current

(supine) MIPPV, overnight plus daytime prn

(31-54) (0-16) (G90-1720)

(2-30) (18-49)

42.3 yrs 8.3 yrs 1034 ml 29.9% pred 7.7 yrs

(2-14)

CHEST / 91 / 6 / JUNE, 1987

867

this device, he switched to overnight MIPPV which he continues to use. His tidal volume on MIPPV with the Bennett lipguard is 600 ml and his pcc, from 32 to 38 mm Hg with a respirator cycling pressure at 24 cm H.O. His O, saturation is 95 percent or better during continuous monitoring. He receives regular sighs of 1.85 L with the sigh pressure adjusted to 40 cm H.O.

Although occasionally using MIPPV 24 hours a day, he usually uses it during the daytime only while transferring into his pneuemobelt. He continues to receive tidal volumes of 380 to 400 ml with pneumobelt use. This is sufficient to maintain normal blood was levels during the daytime.

The GPB allows him four hours or more of time free from his respiratory assistance. Recently monitored on GPB alone, he demonstrated O, saturation greater than 95 percent and Pco, less than 12 mm Hg for the full four hours of the test while carrying on normal conversation. His maximum GPB assisted breath is 16 L.

CASE 2

This person developed paralytic polio at age nine years in 1934. It resulted in quadriparesis with severe involvement of the chest wall and the right upper extremity. He required iron lung assistance for several months but was effectively weaned from aid. Severe kyphoscoliosis, for which he eventually underwent two spinal fusion procedures, began at age IL In 1970, with a four-year history of shortness of breath, nightmares, lightheadedness, and cor pulmonale, he was tracheostomized and received TIPPV for several months. His VC was 1,600 ml. The tracheostomy site was allowed to close, but he was tracheostomized and received TIPPV on two other occasions, in 1971 and then in 1972 when he suffered respiratory arrest during sleep. The site was allowed to close for the last time when a phrenic pacemaker was implanted in December 1972. He was hospitalized several times in 1974 and 1975 and treated for symptomatic cor pulmonale. Oxygen administration markedly depressed his respiration. The pacemaker did not provide an adequate tidal volume. In April 1975, the chest shell ventilator was used in synchrony with the pacemaker, but this also was not effective in relieving his severe symptomatic nocturnal hypoventilation. Finally, in November 1976, with a VC of 1,281 ml, he was placed on overnight MIPPV which normalized his blood gas values. He reported: “The first morning after being on the Bantam was truly like a miracle. I woke up bright-eyed and bushy-tailed, like my old self. It has been five years since I bad woken up fully or even modestly alert.” He continues 24 hour a day MIPPV and has not been hospitalized since 1976.

ulcers. He also noted decreases in systemic hypertension, enhanced mental activity, and sustained improvement in blood gas values. Improvement in Pox with a decrease in the P(A-a)O, suggested improvement in ventilation/perfusion relationships probably from diminished atelectasis and better basal ventilation with deeper breaths (sighs”). Bergofsky et al reported sustained improvements in Po, and Pco, and normalization of pulmonary artery pressures, increased lung compliance and decreased work of breathing? following frequent short periods of mouth intermittent positive pressure breathing.

Rochester et al studied the respiratory muscle sparing effect of body respirator use for patients with both obstructive and restrictive respiratory insufficiency. The complete relief of dyspnea and EMG evidence of diaphragm rest observed were not associated with correction of blood gas values. Although Po, levels were mildly increased from 32 mm Hg to 64 mm Hg, Pco, levels averaging 34 mm Hg were not significantly changed by body respirator use. Carryover of the mildly improved Po, levels to periods while not using body respirators was not discussed. In a later work, Braun studied 35 patients, 18 with thoracic diseases, ie, COPD, and 17 with neuromuscular diseases. All were chronically hypercapnic. They received four to ten hours a day of positive or negative pressure respiratory assistance. Braun reported increases in VC, maximum inspiratory and expiratory pressures, and maximum voluntary ventilation as a result of the treatment. Arterial Pco, measured when off aid had decreased from 54 to 43 mm Hg and hospital admissions over a two-year period were significantly reduced in the treated group.

We have found complete relief of dyspnea, дor malization of O, saturations and Pco, levels in the majority of our postpolio persons while on MIPPV despite VC approaching O ml in many cases. Abnormal although improved blood gas tensions might be anticipated with MIPPV use in patients with significant intrinsic lung disease.

The long-term significance of the muscle-sparing effect of assisting ventilation is unknown. Despite the transiently improved off-aid blood gas values, vital capacities, and sense of well-being that we observe in our patients using daily periods of respiratory assistance, neuromuscular and pulmonary deterioration continue. The average yearly loss of 1.9 percent of VC which we observed appears to occur irrespective of the style of respiratory assistance employed. Twenty-two of our patients who had been using respiratory aid for periods only during the day progressed to 24-hour respirator dependency. The nine patients in Table 3 also continue to require increasing periods of respiratory aid.

Theoretic concerns regarding the mode of respira

DISCUSSION Respiratory insufficiency in the majority of postpolio patients is due primarily to respiratory muscle weakness. Most of our patients requiring full time aid have VCs below 1L. In others requiring full time aid, VCs as high as 1, 700 ml have been noted. These patients may have intrinsic lung disease, central hypoventilation, or a pattern of upper airway collapse and sleep apnea episodes as well as thoracic deformity. Symptoms of hypoventilation are insidious in onset but lead to cor pulmonale and progressive cardiopulmonary failure. Often, these persons with signs of right heart failure progress despite treatment with nasal O., and perhaps medroxyprogesterone.

With the use of MIPPV for only short periods several times a day, Lane et all have reported improved tolerance of and reduced frequency of URIs, loss of ankle edema, and sometimes healing of peripheral

862

Mouth IPPV in Postpalic Respiratory Insufficiency (Bach et a}

tory aid to be employed to provide optimal muscle sparing also has little bearing on these cases. Until the 1970s, portable ventilators were available with only control mode ventilation. Although most of our patients now employ assist-control ventilation, in the absence of conscious respiratory drive during sleep, the patient quickly becomes habituated to allowing the cycling respirator to breathe for him and he triggers few assisted breaths. As VC deteriorates, patients trigger fewer assisted breaths during the daytime as well. We cannot discern any clinical difference in the pulmonary function or sense of well-being of those who trigger some breaths during the day as opposed to those on MIPPV using only control ventilation in the past or present. We feel that normalization of blood gas values and adequate “sighing” by regular respirator assisted or glossopharyngeal breathing assisted breaths are more important considerations in preventing long. term cardiopulmonary sequellae in these patients.

The administration of nasal O, alone, even at low flow, to hypoventilating persons may partially relieve some symptoms and decrease pulmonary artery tensions, but it may also decrease the patient’s breathing effort and increase hypercapnia. In 1967, Bergofsky et al’s observed the importance of relieving both hypoxia and hypercapnia and preventing acidosis in normalizing pulmonary artery pressures with positive pressure ventilation. He noted that mechanical ventilatory assistance will also decrease the O, consumption and energy demands of respiratory muscles. Other work demonstrated the pulmonary artery pressor response to hypercapnia as well as hydrogen ion concentration.

Sleep apnea syndrome in polio patients was recognized as early as 1958.17 The MIPPV with correction of blood gas values provides an alternative to tracheostomy for many of these persons. Despite this, overnight nasal O, alone is still commonly recommended in severely hypercapneic persons (Pco,>60 cm H,0).18

We have observed 18 deaths; 17 patients died while receiving MIPPV or only one death for every 60.5 patient-years on MIPPV. Providing better ventilation protects against the development of right ventricular decompensation over a period of years. The deaths were related to pulmonary conditions in seven cases, noncardiopulmonary medical conditions in four cases, substance abuse in three cases, motor vehicle accident in one case, complications of tracheostomy in one case, and unknown causes in two cases. Of the seven deaths related to pulmonary causes, one patient died overnight while using the chest piece. She had been warned that it was no longer adequately ventilating her and was advised to change to using only MIPPV two years earlier. Two patients died when they fell asleep without using the lipguard. They lost the mouthpiece while sleeping. Two patients, one of whom was severely obese, died from complications of acute bronchopneumonia. One died from complications of recurrent massive atelectasis. He smoked heavily, had chronic bronchorrhea, frequent bouts of pneumonia, and died with advanced bullous emphysema. Finally, one patient died suddenly and was thought to have had acute obstruction due to a mucous plug probably during a URI.

Daytime use of negative pressure respiratory assistance does not permit normal daytime activities since persons have to be stationary and remain at least semirecumbent in these assistive devices. The chest shell, poncho, and rocking bed body respirators may provide optimal ventilation for years before loss of pulmonary compliance and complications of advancing respiratory insufficiency may render these methods ineffective. These body respirators are also ineffective in the presence of severe scoliosis and are not tolerated by many patients. Twenty-five of the persons noted in Table 1 switched to full time MIPPV because of this. Sixteen persons noted in Table 2 switched to daytime MIPPV and may follow with nighttime MIPPV in the future. The other seven employ MIPPV overnight and are still able to use the more cosmetic pneumobelt during the daytime. Only two of our patients on MIPPV have recently required supplemental O, because of intrinsic lung disease.

The Table 3 postpolio patients are at an “earlier stage,” requiring MIPPV only overnight at the present time. The use of MIPPV alone obviates the need for obtaining both positive pressure and negative pressure pumps. The MIPPV also provides optimal ventilation with tidal volumes exceeding those which can be delivered by the present models of the iron lung.

There are a number of patients who, although not – dependent on respiratory assistance when well, employ MIPPV overnight and for periods during the daytime when ill. Still other patients not included in these tables require aid for particular reasons. One man, a victim of polio in 1944, developed severe postural respiratory insufficiency and was unable to tolerate recumbency since 1962. He slept in the sitting position for 18 years. He was misdiagnosed and treated for narcolepsy because of severe somnolence due, in reality, to progressive respiratory insufficiency. The MIPPY has allowed him to sleep recumbent since – 1981.

In a few patients, including the one noted in the previous paragraph, there is a severe leak of insufflated air from the nose during sleep due to an incompetent soft palate. Sealing the nose or a mask covering the nose and mouth has been used. Substituting for the Bennett lipseal with molded dental appliances or other modifications are occasionally required.

We have observed no serious complications due to positive pressure ventilation such as pneumothorax or symptoms related to increased intrathoracic pressure. Aerophagia, although often a source of discomfort at first, is generally relieved by the passing of flatus during normal daily activities. Occasional severe abdominal distension and discomfort can be relieved with the use of a rectal tube. This has never been a reason to discontinue MIPPV.

The MIPPV should, therefore, be introduced early to postpolio survivors with slowly progressive restrictive pulmonary syndrome and signs or symptoms of hypoventilation. It can help prevent acute cardiopulmonary complications associated with chronic alveolar hypoventilation and allow optimal activities of daily living while providing an effective alternative to tracheostomy. Similar studies indicating its effectiveness in prolonging life for 31 patients with Duchenne muscular dystrophy and other neuromuscular conditions have been reported. It is the technique of choice for patients without severe intrinsic lung disease who require 24-hour respiratory aid or who are likely to progress to 24-hour respirator dependency and who have sufficiently intact oropharyngeal muscles to prevent excessive inspiratory leak.

sequela of poliomyelitis. Froc Foundation Series. New York Alan

Liss, Ine, 1978:308-15 6 Alba A, Solomon M, Trainor FS. Management of respiratory

insufficiency in spinal cord lesions. In: Proceedings of the 17th veterans administration spinal cord injury conference. Septera

ber 19, 30 and October 1, 1969 7 Bach JR, Alba AS, Bodofsky E, Curran FJ, Schultheiss M.

Glossopharyngeal breathing and non-invasive aids in the management of post-polio respiratory insufficiency. In: Paul N, ed. Proceedings of the 2nd research symposium on the late effects of poliomyelitis, September 1986. Warm Springs, GA: March of

Dimes Birth Defects Foundation, 1987 (in press) 8 Morris JE. Spirometry in the evaluation of pulmonary function.

West J Med 1976; 125:110-18 9 Mulder DW, Rosenbaum RA, Layton DD. Late progression of

poliomyelitis or forme fruste ALS? Mayo Clin Proc 1972;

47:756-61 10 Lane DJ, Hazleman B, Nichols PJR. Late onset respiratory

failure in patients with previous poliomyelitis. J Med 1974;

18:351-68 11 Bergofsky EH, Turino GM, Fishman AP. Cardiorespiratory

failure in kyphoscoliosis. Medicine 1959; 38:263-317 12 Sinha R, Bergofsky EH. Prolonged alteration of lung mechanics in kyphoscoliosis by positive pressure hyperinflation. Am Rev

Respit Dis 1972; 100:47-57 13 Rochester DF, Braun NMT, Laine 5. Diaphragmatic energy

expenditure in chronic respiratory failure. Am J Med 1977;

63:223-32 14 Brain NMT. Respiratory muscle dysfunction. Heart and Lung

1964; 13:327-32 15 Bergotsky EH. Cor pulmonale in the syndrome of alveolar hypoventilation. Prog Cardiovasc Dis 1967; 9:414-37 16 Bergofsky EH, Haas F. Porcelli R. Determination of the sensitive vascular sites from which hypoxia and hypercapnia elicit rises in

pulmonary artery pressure. Fed Proc 1968; 27:1420-25 17 Plum F, Swanson AG. Abnormalities in central regulation of respiration in acute and convalescent poliomyelitis. Arch Neurol

Psychiatr 1958; 80:267-85 18 Libby DM, Briscoe WA, Boyce B, Smith JP. Acute respiratory

failure in scoliosis or kyphosis: proloaged survival and treatment.

Am J Med 1982; 73:532-38 19 Bach J, O’Brien, Krotenberg R, Alba AS. Management of end

stage respiratory failure in Duchenne muscular dystrophy, Muscle and Nerve (in press) 20 Bach 1, Alba A, Lee M, Rideau Y. Long term respiratory

rehabilitation in the treatment of neuromuscular disease. Ain Readapt Med Phys 1983; 26:101-09

ACKNOWLEDGMENTS: The authors wish to thank Marie Schultheiss, B.A.C. C.P.T., Craig Senna, C.R.T.T., Enrique Conzalez, C.R.I.T., and Joan Adler, M.A., for their technical assistance. We also wish to thank Ms. Diane Perlman for her help in preparing this paper.

REFERENCES

I Paralytic poliomyelitis United States, 1982 and 1983. Current

Trends. MMWR 1984; 33:635-38 2 Lassen HCA. Traitement des troubles respiratoires et de la

paralysie bulbaire dans la poliomyelite. J Med Lyon 1956;

37:236-39 3 Fischer An. Poliomyelitis: late respiratory complications and

management. Orthopedics 1985; 8:891-94 4 Garay SM, Turino GM, Coldring RM. Sustained reversal of

chronic hypercapnia in patients with alveolar hypoventilation

syndromes. Am J Med 1981; 70:269-74 5 Guillenminault C, Motta ) Sleep apnea syndrome as a long-term

Mouth Intermittent Positive Pressure Ventilation in the Management of Postpolio Respiratory Insufficiency Read More »

Late Effects of Poliomyelitis

James L. Cosgrove, MD

University of Pittsburgh School of Medicine, Pittsburgh, PA 15213 Michael A. Alexander, MD, Ellen L. Kitts, MD, Barbara E. Swan, MD, Milton J. Klein, MD, Roberta E. Bauer, MD

D.T. Watson Rehabilitation Hospital, Sewickley, PA 15143

ABSTRACT. Cosgrove JL, Alexander MA, Kitts EL, Swan BE, Klein MJ, Bauer RE: Late effects of poliomyelitis. Arch Phys Med Rehabil 68:4-7, 1987.

Muscular atrophy and decreased functional abilities are recognized as late complications of poliomyelitis. This study sought to more clearly define late-onset, postpolio muscular weakness age of onset, symptoms, and severity and to determine whether people might benefit from environmental modification, respiratory aids, and orthoses. A total of 183 postpolio patients were examined by a physician and completed a questionnaire on their ambulatory status and related musculoskeletal and respiratory symptoms. Of those participating in the study, 154 claimed to be experiencing late deterioration in strength. The syndrome included decreased endurance, more limited ambulation, and increased weakness in the previously affected limb/s. For those describing late-onset weakness, average ages were determined for the onset of polio (8.3 years), the onset of postpolio muscular weakness (42.3 years), and the latent period of stable functioning (34.8 years). Patients claimed to have experienced a new, lower level of strength for an average of 4.7 years. All 33 patients who had undergone muscle transfer surgery were experiencing late-onset weakness in that extremity. Fasciculations (51%) and long-bone fractures secondary to falls (21%) were common sleep disturbances occurred frequently (31%) even in those without prior bulbar involvement. Upper motor neuron signs were present in only one case. Bracing was rare (16%) and the braces used were usually old. The subjects did not report a steadily or rapidly progressive decline, but rather described a steplike decrement with long plateaus. The population described losses … strength that had significant bearing on functional status and general health Many patients had not sought routine care in years, let alone special evaluation of neuromuscular function. Perhaps most alarming was the large number of sleep complaints. Clearly, many postpolio patients might benefit from closer medical supervision, with orthotic and pulmonary intervention being commonly required. On completion of this study, 31% of the patients had new orthoses prescribed and 18% were referred for further pulmonary evaluation.

KEY WORDS: Fasciculations; Fractures; Orthotic devices; Paralysis: Poliomyelitis

sleep studies have revealed the presence of central sleep apnea in patients with prior bulbar involvement who hai been weaned from respiratory support.7.11.18 An increase in the use of respiratory support of between 25% and 80% from the time of optimal neurologic functioning has been reported.

Despite the number of case reports and descriptions of late onset, postpolio muscular weakness (LOPPMW) in literature, no large series of patients has been examined systematically. The intent of this study is w describe the syndrome of LOPPMW more completely and the sold of continuing medical intervention.

The syndrome of late muscular weakness with antecedent poliomyelitis was first documented by Charcot in 1875. Since that time many authors have commented upon this phenomenon. 1-4,8,11,14,16,18,22 Though epidemiologic data is not abundant, a prevalence rate of 22% has estimated.”

Myriad neuromusculoskeletal complications have been associated with the late effects of poliomyelitis. Late-onset weakness usually has been described in muscles that were previously atrophied.9,14,16,18 However, the contralateral, un involved limb has often been involved as well.2,8,16 Weakness has been generally perceived as simply progressive. However, it has been suggested that a subgroup of more active individuals may have a more rapidly progressive form of the syndrome. Two deaths have been noted secondary to late motor neuron dysfunction. 16,18 Joint pain, joint instability, and long bone fractures are also identified as late complications of polio.1,2,8,11,16 Yet the relative frequency of these conditions is not known. Muscle fasciculations have been de scribed,1,2,9,11,15,16,22,23 but often no distinction is made be tween true fabulations and contraction fasciculations.” Muscle cramps, myalgia and fasciculations, commonly associated with late muscular weakness, are often found in, via polio patients without new weakness. 6,11 Upper motor neuron signs have been rare, having been present in only six of the 100 cases reported between 1969 and 1984.,2,5,11,14,16,18

Respiratory insufficiency also has been implicated as a late complication of previous bulbar poliomyelitis.

MATERIALS AND METHODS The subjects were 183 volunteers void previous paralytic poliomyelitis who attended one of four conferences on the late effects of poliomyelitis. They were canned by one of the six authors. At that time a detailed questionnaire was completed regarding demographic data, initial presentation of polio, extent of current involvement, functional areas of impairment, use of orthotics, previous orthopedic procedures, frequency and severity of falling, fasciculations, initial bulbar … involvement, respiratory difficulties, use of ventilatory and the quality of sleep.

Late-onset weakness and decreased endurance were ascertained through the questionnaire and the physician’s history.

Submitted for publication March 1, 1985. Accepted in revised form April 30, 1986

Årch Phys Med Rehabil Vol 68, January 1987

POSTPOLIO SYNDROME, Cosgrove

In both the questionnaire and the examination an effort was made to separate true fasciculations from contraction fasciculations. True fasciculations were accepted as muscle twitching that occurs at rest, not under voluntary control. Bulbar involvement was defined as having difficulty swallowing or requiring respiratory assistance of a rocking bed, supplemental oxygen, iron lung, or any artificial means of ventilation. Sleep disturbances were determined by patient report. Prolonged apneic spells during sleep, loud snoring, excessive daytime sleepiness, early morning headache, and waking at night gasping for air were considered significant sleep disturbances.

reported increased fasciculation after exhaustive exercise (usu ally stair climbing). Fasciculations were noted quite often in muscles that were not clinically weak. In fact, eight of the 29 patients without LOPPMW were noted on physical examination to have fasciculations.

In one patient, there was noted a positive Babinski sign. In all others, no upper motor neuron signs of Babinski, Hoffmann or clonus were found on examination. In that patient, the diagnosis of multiple sclerosis has not been ruled out.

Nineteen patients complained of bulbar involvement as a part of the initial presentation of poliomyelitis. Twenty-six patients complained of late occurrence of dysphagia and dysphonia. Only one patient was on supplemental oxygen or respiratory assistance (chest cuirass at night time only). None required positive pressure ventilatory support.

However, 47 of 154 patients with weakness and one of 29 patients without weakness complained of a sleep disturbance. Nine of the 19 patients with previous bulbar involvement had sleep complaints. Four patients complained of apneic spells, six complained of loud snoring, ten complained of gasping for air, and 27 complained of early morning headache. Interestingly, 19 of the patients with headache had sought the advice of a physician and had been diagnosed as having allergies.” None had experienced relief with decongestants or antihistamines. Sleep apnea had been diagnosed in the one patient mentioned above, utilizing the chest cuirass. All 19 patients with bulbar involvement initially were advised to undergo baseline pulmonary function tests. Those patients with reports of apnea or gasping were referred to a pulmonologist for further evaluation.

RESULTS Of the 183 patients in the study group, 29 were experiencing no late effects of poliomyelitis. Of the 154 with late weakness there were 58 men and 96 women. The following data concerns those 154 patients, who were experiencing the syndrome of LOPPMW.

The initial onset of paralytic polio occurred between 2 months and 33 years of age (mean, 8.3 years). All 154 patients reported stable neurologic status during adulthood. Late-onset weakness began between years 18 and 74 (mean, 42.3 years). The latent period ranged from 15 to 72 years (mean, 34.8 years). The patients had noticed diminished function for I to 40 years (mean, 4.7 years). Most patients had difficulty giving an exact date for the late onset of weakness. Those with minor residual deficits and very active lifestyles could, however, pinpoint an exact month and year of the onset of their late symptoms. In no subgroup was a steady downhill progression seen. All 154 were experiencing late-onset weakness in mascles that were clinically affected by polio previously. Seventy patients (45%) noted weakness in muscles that previously they had considered normal. The single most common complaint was decreased endurance (153 of 154). Increased difficulty with stair climbing was almost universal (152 of 154). Bracing was found only in 25 of 154 (16%). Most braces were old; with an average age of 15.3 years. Only 30 patients (19%) had begun to use a cane or crutches. Six patients (4%) had ceased to ambulate and were wheelchair bound. The use of braces alteration in bracing was recommended for 56 of the 183 patients examined. Fifty of these 56 were complaining of late weakness. Also, 13 of the 154 patients were advised to begin using either a cane or crutches. Though scoliosis was present in 30 of 154 patients, only three were braced because of deformity. No changes in orthoses were made with these patients. Seventy-eight patients had undergone orthopedic surgery for the treatment of polio-related disorders. Of those, 33 nad muscle transfer surgery. All of those patients experienced late onset weakness in that extremity. Failing was a frequent complaint. Falls had caused long bone fractures in 32 patients. Only eight of these 30 patients with fractures had begun to use ambulatory aids or bracing. Though 76 patients claimed on the questionnaire to have had fasciculations, only 35 could be verified on physical examination. Three other patients denied having fasciculations, which were nevertheless noted on physical examination. In total there were 79 of the 154 patients who either complained of or who were observed to have fasciculations. All patients

DISCUSSION

Remarkably consistent throughout previous literature and corroborated by this study is the time course of stable neuro logic functioning. Between the third and fourth decade after onset of poliomyelitis is the average age of presentation for LOPPMW.1,2,3,8,11,14,16,18.22 Our findings indicate a mean latent period of 34.8 years. Yet the wide range in years of stable functioning (15 to 72 years) indicates that the time course of the onset of the weakness can be extremely variable. The progressive nature of this syndrome is difficult to assess. Often interpretation of the data is complicated by in creased age and weight. The patients in this study had noted the presence of late-onset weakness, as evidenced by diminished ability o ambulate, for an average of almost five years. As in the previous literature, they described a slow, sporadic decline in strength, with plateaus of stability, 1.2,14,15,16.22 Daiakas and colleagues were able to distinguish between two separate subgroups of LOPPMW (ie, progressive and nonprogressive), but we were unable to do so. Patients with more minor residual deficits, however, were much more exact in describing the onset of their late symptoms.

Systematic evaluation of orthoses in postpolio patients had not been performed by previous investigators. Few of our patients used braces or walking aids and those that were used were quite old. Few had sought medical advice concerning ambulation, despite the universal impression of increased weakness (100%) and the high incidence of falls resulting in broken cones (21%). The explanation of this behavior appears hypercarbia and hypoxemia. Hypercarbia and hypoxemia due to central sleep apnea have been well-described as late com plications of poliomyelitis.7.12.20 Unlike previous cases cited in the literature, however, most of our patients denied having previous bulbar polio. One might argue that sleep complaints are quite common in the general population and that our results represent an unrelated epiphenomenon. However, the findings in 19 of 27 patients who had headache severe enough to seek medical care, none of whom experienced relief with medical management, is certainly suggestive of a central process.

to lie deeper than simple denial of disability. More likely it is attributable to the medical, family, and social influences of their early rehabilitation experience. The patients reported that their ritual of exercise was religiously followed, with the goal being to rid themselves of braces. A return to braces, then, may be viewed as failure. Although initial resistance to bracing was almost unanimous, with the aid of counseling and through demonstration, prescriptions were written for 56 orthoses. Another 13 patients were advised to use walking aids.

Scoliosis, though present in 20% of our population, did not represent a major clinical problem. Only three of the patients were braced and none appreciated any progression of their curvatures. Furthermore, no new spinal orthoses were pre scribed. The absence of progression in scoliotic curves is difficult to reconcile with more marked changes in the appendicular musculature. Perhaps the decrease in muscle strength tended to be symmetrical, thus causing no dramatic spinal changes. Since routine pulmonary function tests were not done, we cannot comment on the presence or absence of restrictive lung disease. The long-term results of muscle transfer surgery have not been studied by previous authors. In our population, 21% had undergone muscle transfer surgery, all of whom were experiencing LOPPMW in the muscle group/s that had been transferred. It thus appears that muscle transfers are best considered as a temporizing measure. Fasciculations in LOPPMW have been noted by many investigators. We attempted to separate con traction fasciculations from true fasciculations, both in the questionnaire and during the examination. Only fasciculations at rest were counted as true fasciculations. Fasciculations were quite common, being observed in 38 patients and reported by 41 others. However, fasciculations frequently occurred in muscles that were not clinically weak. Furthermore, many patients reported having fasciculations in my life.” Though the significance of fasciculations remains unclear, they do not appear to portend imminent decline in muscle strength. In only one patient was an upper motor neuron sign of Babinski, Hoffmann or clonus elicited. In other studies upper motor neuron signs have also been rare, having been present in only six of 100 cases reported.-4.8.11.14,16,18,22,23 Their presence, however, has led some authors to suggest that LOPPMW rnay represent a forme fruste of amyotrophic lateral sclerosis (ALS). 16.18 The clinical data presented above, spe cilica.ly the rate of progression and the relatively limited areas of involvement, clearly indicate that ALS and LOPPMW rep resent different syndromes. Bulbar involvement during the initial illness was not as prevalent in our population (19 of 154) as in other studies. ,3 Furthermore, only one patient was requiring any type of ventilatory support (<1%), compared with 5% and 9% as noted by Halstead and Roosis and Codd and colleagues, respectively. Despite the apparent good respiratory health of this study population, 26 complained of dysphagiaidyspnonia and 47 complained of sleep disturbances. In contrast to the findings of Hamilton and coworkers, none of the patients in our study complained of increased sensitivity to cold, decreased memory, or decreased capacity or intellectual work. The most common sleep complaint, headache (27 of 17), is recognized as a symbiomoi

CONCLUSION Our examinations demonstrate that the late symptoms of poliomyelitis are not restricted to areas previously considered weakened by polio. Seventy of 154 patients experienced weakness in limbs that they did not feel were involved originally. Thirty-eight patients described sleep disturbances but denied ever having bulbar polio. The etiology of these symptoms remains unknown. Congenital predisposition,5,14,17,18 recrudescence of a latent virus,3,13,15,18 impaired cell-mediated immunity, premature anterior cell death,”1.22 and failure of the neuromuscular junctional have been proposed as theories. It has also been found in postmortem examinations that subclinical diminution of the number of anterior horn cells can occur without apparent weakness.”9 Furthermore, electromyographic (EMG) results indicate that complete reinnervation can occur if more than 50% of the anterior hom cells remain, thus obscuring the extent of involvement of poliomyelitis. 10,11 Therefore, we suggest that individuals with a decreased number of viable anterior hom cells ater undergo premature failure of the (remaining) motor units due to one of the mechanisms mentioned above. The result is late-onset weakness of the skeletal and bulbar musculature previously considered normal. The treating physician must therefore perform a complete physical examination, Being alert for subtle evidence of weakness, and not limiting the examination to areas of obvious clinical atrophy.

ADDRESS REPRINT REQUESTS TO: Michael Alexander, MD D.T. Watson Rehabilitation Center Sewickley, PA 15143

References 1. Anderson AD, Levine SA, Gellert H: Loss of ambulatory ability

in patients with old anterior poliomyelitis. Lancet 2: 1061-1063,

1972 2. Campbell AMG, Williams ER, Pearce J: Lower motor neuron

degenerasion following poliomyelitis. Neurology(Minneap)

19:1101-1106, 1969 3. Codd MB, Muider DW, Kurland LT, Beard CM, O’Fallon WM:

Poliomyelitis in Rochester, MN, 1935-1955: Epidemiology and Longterm Sequela: a preliminary report. In Halstead LS, Weich ers DO (eds): Late Effects of Poliomyelitis. Miami. Symposia

Foundation, Inc., 1985, pp 121-134 4. Dalakas MC, Sever JL, Madden DL, Papadopoulos NM, Shekarchi

IC, Albrecht P, Kreziewicz A: Late postpoliomyelitis muscular atropny: clinical, virologic, and immunologic studies. Rev Infect

Dis 6 (Suppl 2); S562-5567, 1984 5. Dausset J, Degos L, Hors J: Association of HL-A antigens with

Diseases. Clin Immunoi Immunopathol 3:127-19, 1974 6. Feteil MR, Smailberg G, Lewis LD, Lovelace RE, Havs ip.

Rowiana ir: Benign motor neuron discruer: Courtos und

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POSTPOLIO SYNDROME, Cosgrove

fasciculation after poliomyelitis and myelitis. Ann Neurol 11:+23–

427, 1982 7. Guilleminault C, Motta J: Sleep apnea syndrome as long-term

sequela of poliomyelitis. In Guilleminault C, Dement WC (eds): Sleep Apnea Syndromes. New York, Alan R Liss, 1978, pp 309

315

Halstead LS, Rossi CD: New problems in old polio patients:

results of survey of 539 polio survivors. Orthopedics 8:845-850,

1985 9. Hamilton EA, Nicholas PJR, Tait GBW: Late onset of respira

tory insufficiency after poliomyelitis: preliminary communica

tion. Ann Phys Med 10:223-229, 1970 10. Hansen S, Ballantyne JP: Quantitative electrophysiological study

of motor neurone disease. J Neurol Neurosurg Psychiatry 41:773

783, 1978 11. Hayward M, Seaton D: Late sequeiae of paralytic poliomyelitis:

clinical and electromyographic study. J Neurol Neurosurg Psy

chiatry 42:117-122, 1979 12. Hill R, Robbins AW, Messing R, Arora NS: Sleep apnea syn

drome after poliomyelitis. Am Rev Respir Dis 127:129–131,

1983 13. Jokelainen M, Tiilikainen A, Lapinleimu R: Polio antibodies and

HLA antigens in amyotrophic lateral sclerosis. Tissue Antigens 10:259–266, 1977

Kayser-Gatchalian MC: Late muscular atrophy after poliomye.

litis. Eur Neurol 10:371-380, 1973 15. Kurent JE, Brooks BR. Madden DL, Sever JL, Engel WK: CSF

viral antibodies: evaluation in amyotrophic lateral sclerosis and late-onset postpoliomyelitis progressive inuscular atrophy. Arch

Neurol 36:269–273, 1979 16. Mulder DW, Rosenbaum RA, Layton DD Jr: Latc progression

of poliomyelitis or forme fruste amyotrophic lateral sclerosis:

Mayo Clin Proc 47:756–761, 1972 17. Pietsch MC, Morris PJ: Association of HL-A3 and HL-A7 with

paralytic poliomyelitis. Tissue Antigens 4:50–55, 1974 18. Roos RP, Viola MV, Wollmann R, Hatch MH, Antel JP: Amy.

otrophic lateral sclerosis with antecedent poliomyelitis. Arch Neuro

37:312–313, 1980 19. Sharrard WJW: Correlation between changes in spinal cord and

muscle paralysis in poliomyelitis: preliminary report. Proc R Sac

Med 46:346-349, 1953 20. Solliday NH, Gaensler EA, Schwaber JR, Parker TF: Impaired

central chemoreceptor function and chronic hypoventilation mans

years following poliomyelitis. Respiration 31:177-192, 1974 21. Wiechers DO, Hubbel SL: Late changes in motor unit after poliomyelitis. Muscle Nerve 4:524–528, 1981 22. Zilkha KJ: Discussion on motor neurone disease. Proc R Soc Mei

55:1028-1029, 1962

Index to Volume 67 to be Republished

Computer malfunction resuited in elimination from the Subject and Author Index to Volume 67 (1986) of the Archives of Physical Medicine and Rehabil itation of all articles and their authors published in the November and December issues. The Archives regrets the omission and as a service to its subscribers will publish the Index to volume 07 in its entirety as a supplement to the February 1987 issue.

Arch Phys Med Renabil Vol 68, January

Late Effects of Poliomyelitis Read More »

Physical Therapy Management of the Patient with Post Polio Syndrome

Physical Therapy Management of the Patient with Post-Polio Syndrome

A Case Report

Donna J. Twist and Dong M. Ma

This case report documents the treatment of a patient who experienced progressive muscle weakness and a decrease in function over time that did not appear to be related to any secondary neuromuscular disease. We discuss the relationship between age and maximal muscle function in addition to some general guidelines for rehabilitation. This type of patient can represent a challenge for the physical therapist. This case report however, illustrates the degree of muscular and functional recovery that can result with a physical therapy program aimed at reducing levels and intensity of exercise, daily activity, and stress. Such a combination of short-term goals appears to be essential to the successful management of a patient with post-polio syndrome.

Paralytic poliomyelitis, caused by a small ribonucleic acid virus that invades the central nervous system, has ceased to be a common catastrophe in areas where vaccinations are administered routinely. Evidence of the crippling effects of this disease, nevertheless, is widespread. According to data from the Center for Disease Control, Atlanta, GA, an estimated 250.000 survivors of paralytic poliomyelitis are in the United States at present.

Until recently, poliomyelitis typically had been considered a stable. chronic disease. Many poliomyelitis survivors, however, experience new health problems years after contracting the disease. Based on data from a preliminary study by Codd et al at the Mayo Clinic, about 25% of poliomyelitis survivors seek medical assistance 25 to 30 years after the abrupt onset of the disease. A variety of complaints, such as fatigue, increased weakness in either the muscles affected by or presumably unaffected by the poliomyelitis virus, pain, and a change in functional status, appear to represent new symptom developments in patients with a history of poliomyelitis. This group of apparently poliomyelitis-related symptoms has been referred to as the “post-polio syndrome.”

The mechanisms for these poliomyelitis-related symptoms are unknown. Possible explanations include reactivation of the poliomyelitis virus, genetic predisposition allowing the previously affected motoneurons to deteriorate further, normal aging of a previously diseased motor unit and reduced ability to compensate for poliomyelitis-weakened muscles, immunopathologic mechanisms, or simply mere coincidence. The purposes of this report are to discuss a case of post-polio syndrome, characterized by a desinite. quantifiable increase in muscular weakness over time, and to describe the course of management for this condition.

PATIENT DATA AND TREATMENT

A 42-vear-old white woman contracted poliomyelitis in 1955 at the age of 13 years. The initial paralysis involved the muscles of the lower trunk and the lower extremities. The upper trunk and upper extremities were not involved. The patient underwent physical therapy that, according to the patient, involved “painful” stretching and strengthening exercises conducted three times a week by a physical therapist and on alternating days by a family member. The patient completed her rehabilitation program two years after the onset of poliomyelitis and was able to ambulate independently without the use of assistive devices. Results of a manual muscle test conducted after the completion of her initial two-year rehabilitation program revealed a muscle strength grade of Normal in the right leg, but Fair plus in the left quadriceps femoris and lower abdominal muscles, Good plus in the left ankle dorsiflexor and hip flexor muscles, and Fair minus in the hip abductor muscles (Table). The patient subsequently reported difficulty ascending and descending stairs and standing from a low seated position.

At the age of 35 years, the patient began to notice increased muscular weakness in the left leg that caused her to drag her leg during the swing phase of gait. She also recalled being awakened during the night with “muscle cramps” in her legs after a day of excessive walking. The muscular weakness and fatigue progressively worsened until she began falling outdoors a

exercise manual muscle test results revealed a muscle strength grade of Poor minus in the left quadriceps femoris muscles and Fair in the left ankle dorsiflexor, hip flexor, and hip abductor muscles (Table). A progressive resistive exercise strengthening program performed three times a week on alternating days was prescribed by her physician. Three months later at discharge from the program. she was able to ambulate safely with a straight cane. Manual muscle test results after the three months of physical therapy showed improved muscle strength in the left quadriceps femoris to a grade of Fair and in the ankle dorsiflexors to a grade of Fair plus Hip flexor and hip abductor muscle strength improved to a grade of Good (Table). Despite these improvements in muscle strength. the patient reported that she had not regained her former level of endurance in ambulation. She independently continued the muscle stretching and strengthening exercises with leg weights at home.

TABLE

Pre-exercise Muscle Strength in the Left Lower Extremity

At 40 years of age, the patient was unable to ambulate out of doors the distance to her job even with the aid of a straight cane. A pair of Lofstrand crutches was prescribed by her former physical therapist. No formal gait training in the proper use of the crutches was provided. The patient independently used a three-point gait pattern with the bilateral use of Lofstrand crutches.

At the age of 42 years, the patient reported a feeling of generalized fatigue and muscle weakness. She began falling indoors and, thus, became fearful of ambulating. She was referred for physical therapy with the diagnosis of post-polio syndrome. The patient participated three times a week in a home physical therapy program that included therapeutic exercise, postural exercises, and gait training. After six to eight weeks of rehabilitation, the patient was to be reevaluated for signs of muscle strengthening. for functional and gait improvements, and for subjective improvement in fatigue and endurance. If no improvement occurred, electromyographic testing to identify a possible electrophysiologic basis of further neurogenic involvement was to be administered.

Preexercise manual muscle test results revealed a muscle strength grade of Good plus in the right leg with the exception of a grade of Fair plus in the muscles surrounding the right hip. In her left leg. she had grades of Poor minus in the quadriceps fernoris muscle, Good plus in the ankle dorsiflexor muscles, and Fair minus in the hip flexor and hip abductor muscles. Her abdominal muscles had a grade of Fair minus. Her passive range of motion in the lower extremities was essentially within normal limits except for limitations in dorsiflexion (0″ – 60″) and straight leg raising (0″ – 60″) bilaterally. Passive range of motion in both upper extremities was within normal limits except for shoulder flexion (0″ – 60″). shoulder abduction (0″-60″). and neck rotation to the right (0″ – 75″). Her posture showed a kyphoscoliotic thoracic spine. She assumed a hyperextended or forward head position with her neck,

The patient’s ambulation was guarded. She attempted to use a three-point gait pattern with the bilateral use of Lofstrand crutches for ambulating outdoors, but her balance was poor. Her gait was characterized by excessive plantar flexion at heel-strike. decreased hip and knee flexion during the swing phase of gait. short steppage and stride length, and a mild gluteus medius limp on the left side. Her pelvis and trunk were held rigid and lacked rotation. Indoors, she preferred to use a cosmetically pleasing, although unstable, straight cane for ambulation, Her gait deviations were more exaggerated with the use of a straight cane. She was able to ambulate about 20 ft. without grasping an item of furniture for additional support. Her newly acquired fear of falling prevented her from independently ambulating outside of her apartment. She preferred to remain inside her apartment and to ambulate as little as possible. Functionally, the patient could not stand from a low or cushioned chair, nor ascend or descend a curb or a flight of stairs.

After the assessment of the patient’s passive range of motion, we recorded manual muscle strength, gait, posture, and functional abilities, pertinent details about her life style, her perceived physical discomfort and muscle fatigue, and changes in her functional status. The

tient lived alone in a building having an elevator and drove to and from her full-time job three times a week. Her physical complaints included neck and left upper arm pain that had persisted during the previous six months and a feeling of increased muscle weakness and fatigue after excessive energy expenditure (physical or emotional). She feared rapid deterioration and immobility.

An initial treatment goal was to provide the patient with education and counseling about the causes of the post-polio syndrome and the problems associated with it. The most widely accepted causational theory attributes the loss of muscular strength to the slow, progressive loss of anterior horn cells, which superimposes new strength losses on already weakened muscles. A decrease in muscular contraction force occurs with aging and with exercise-induced damage of the motor unit. The patient, thus, was told that her remaining anterior horn cells had been overstressed for years because they had been made to function like a normal group of motor cells. Normal aging causes some of the cells to weaken or die more rapidly. The old principle of “no pain, no gain” that has been promoted by physical therapists in the past no longer is appropriate for poliomyelitis patients with new complaints of muscle weakness and fatigue. The appropriate treatment for such patients, however, is to prevent exercise-induced muscle weakness by promoting a plan of moderate exercise with frequent rest periods. Instructions for a safe and efficient gait to minimize energy expenditure and plans to decrease strenuous physical and stressful emotional situations also are recommended

The exercise protocol was as follows:

Mobility: Passive muscle stretching was directed primarily to the hamstring and calf muscles. Active assistive exercises were directed to the trunk. upper extremities, and neck.
Trunk muscle strengthening: Active isotonic exercises were administered to the back extensor and abdominal muscles. Active resistive exercises were administered 10 the lower trunk rotator muscles.
Lower extremity muscle strengthening: Brief (10-second holding) isometric and isotonic exercises were performed by the patient daily. The patient performed 10 repetitions of each exercise, and each exercise was preceded by and ended with a rest period. The group of active exercises took the patient about 20 minutes to complete. She was instructed to cease exercising if she experienced muscle twitching, pain, or fatigue.
Resistive exercises: The physical therapist conducted resistive proprioceptive neuromuscular facilitation exercises three times a week. Each exercise was administered 10 times and was followed by a 3-to 5-minute rest period. Initially, a total of 20 minutes of resistive exercise was provided. The patient then would rest for 5 to 10 minutes before proceeding to gait training. The patient was instructed not to use free weights independently (to guard against over-exercise effects).

5 Gait training: The purpose of gait training was to promote comfort and minimal energy expenditure. The patient was taught a four-point gait pattern using Lofstrand crutches. Training began in her apartment hallway to prepare for outdoor training. The four-point gait pattern was chosen to provide the patient with a means of stable outdoor and long-distance ambulation. One Lofstrand crutch instead of the cane was used indoors to improve the patient’s balance and quality of gait. 6. Review and follow-up: During each physical therapy session, the theory underlying the program of moderate exercise with rest was reviewed. The patient was questioned about subjective improvement. exercise compliance, and fatigue. The physical therapist provided the patient with feedback after each review.

After eight weeks of this program, the physical therapy evaluation revealed that passive range of motion was within normal limits in all extremities. Manual muscle test results showed muscle strength grades of Good plus in the patient’s right lower extremity: the left lower extremity showed grades of Fair plus in the quadriceps femoris muscle, Good plus in th dorsiflexor and hip flexor muscles, and Good in the hip abductor muscles (Table). She ambulated short distances with one Lofstrand crutch both indoors and outdoors with increased hip and knee flexion, increased dorsiflexion at heel-strike, and increased pelvic rotation. Marked improvement in her posture was noted during ambulation. Physical therapy continued three times a week for additional therapeutic exercise and gait and postural training.

Currently, the patient ambulates with a straight cane indoors and with the bilateral use of straight canes (using a four-point gait pattern) outdoors for short distances. For long-distance ambulation or ambulation during the patient’s working hours (which requires her to carry a heavily weighted backpack). she independently uses either Lofstrand crutches bilaterally or one Lofstrand crutch and one short cane in a four-point gait pattern.

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Subjectively. the patient reports the absence of neck and arm pain, increased stability and confidence during ambulation, and increased muscle strength and endurance. She, nevertheless, reports experiencing fatigue during the late after-noon as a result of a long day’s work or excessive ambulation. Generalized fatigue and instability during ambulation appear to result after periods of increased stress or anxiety. Brief rest periods of 30 to 60 minutes help to relieve the physical fatigue. Reorganizing busy work schedules (a suggestion made by the physical therapist) helps to decrease stress and thus, relieve the generalized fatigue attributed to anxiety.

DISCUSSION

Many of the complaints of patients with post-polio syndrome such as muscle weakness, pain, and fatigue are ailments commonly associated with patients having a long-standing medical history of a musculoskeletal or neuromuscular disease. Therefore, eliminating common medical problems when evaluating the patient with post-polio syndrome presents a dilemma for the physician. Deciding which cases represent a quantifiable increase in gradual muscle weakness over time and providing the diagnosis of post-polio syndrome represents a complicated diagnostic issue.

After the diagnosis of post-polio syndrome is made, the clinical management program then should be adapted specifically to each patient’s needs. We have presented the exercise protocol for this particular case. Some general guidelines that may be helpful for physical therapists in the rehabilitation of patients with a similar diagnosis are as follows:

Education and counseling are critical components in the management of all progressively disabling diseases. The emotional impact of such disabilities may be especially devastating to the patient with post-polio syndrome, however, because the patient typically is middle-aged, and the onset of progressive weakness often is compounded by psychosocial and vocational problems associated with aging.
A combination of moderate exercise with rest should be monitored appropriately. Therapists may encounter resistance from patients to a new approach for an old disease. After years of living with the knowledge that tough and exhaustive exercise originally helped these patients to “conquer” their disease, they now are being asked to change their cognitive style of coping and possibly to change their life style. A method that was helpful in this particular case was the idea of implementing a six- to eight-week trial program of this ‘-new” treatment approach of moderate exercise, rest, and recovery. In this way. the patient believed that she had control over the program. The patient’s immediate family also was informed about the causes of post-polio syndrome and the effects of aging and exercise, and the new approach for rehabilitation was discussed.
Multiple options for ambulation should be provided for the patient. Depending on the patient’s status, these options may include adaptive equipment. bracing, or transportation. The goal for each patient should be efficient energetics. The patient’s level of fatigue and endurance and the functional setting should determine the type or types of assistive devices necessary for safety, efficiency, and comfort. These options provide the patient with a sense of security and not with a sense of failure.
Psychological counseling and post-polio syndrome support groups should be made available to the patient. The patient’s realization that a change in life style may be necessary to reverse functional losses and to prevent further losses from occurring may be traumatic.

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Physiological and Psychological

POST-POLIO SEQUELAE: Physiological and Psychological Overview

Nancy M. Frick, M. Div. and Richard L. Bruno, Ph.D. Rehabilitation Literature, 1986; 47 (5-6): 106-111.

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation’s consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely

unpleasant to severely debilitating:

unaccustomed fatigue, so severe in some individuals that they must must lie down to rest in the middle of the day – a phenomenon called the “Polio Wall”; [1]

new joint and muscle pain;

new weakness in muscles affected and in those apparently unaffected by the polio;

new respiratory difficulties in those who had bulbar or upper spinal polio that require some to return to using a respirator;

cold intolerance, where exposure to even mild cold temperatures results in noticeable muscle weakness, marked coldness and discoloration of the limbs and burning pain – symptoms that increase with aging. [1,2,3,4,5,6,7]

INCIDENCE AND PREDICTORS OF NEW SYMPTOMS

Of the hundreds of thousands of persons who contracted poliomyelitis during the past fifty years, how many are now experiencing these new symptoms, when do new symptoms occur and are there any factors

that predict their occurrence? At this time, there are only preliminary studies that speak to these questions. The only population-based study of incidence was conducted by researchers at the Mayo Clinic.[8] This initial study determined that at least 22% of the 201 persons who contracted paralytic poliomyelitis in Rochester, Minnesota between 1935 and 1955 developed new symptoms. The National Health Interview Survey of 1977 reported that there were 254,000 persons alive in the United States who had been paralyzed by poliomyelitis.[9] If one combines the data from these two sources, it appears that there are at least 55,880 persons who were paralyzed by poliomyelitis and are experiencing new symptoms.

However, it has become clear that individuals who were diagnosed as having had non-paralytic poliomyelitis also can develop new symptoms. Between 1933 and 1983, a total of 484,862 cases of paralytic and non-paralytic poliomyelitis were reported to the Centers for Disease Control (R. Bruce, personal communication, 1984). The CDC admits that this number is an underestimate of the true incidence of polio because reporting requirements were not instituted until the late 1950’s. Mindful of this caveat, if the case fatality ratio from the Mayo Clinic study (14.5%) is applied to the CDC total, there are at least 414,557 persons alive today who are at risk for developing new symptoms.

With regard to the chronology and predictors of the new symptoms, a questionnaire study of over 500 persons who had polio documented that the occurrence of new symptoms is related to the number of years since the initial polio infection, and not to chronological age.[1] New symptoms were found to appear approximately thirty years after the acute infection. This study also identified four factors which, if present during the acute phase of polio, predict an increased likelihood of developing new symptoms: having been hospitalized, having been 10 years of age or older at the time of the acute infection, having had paralytic involvement of all four limbs or having used a ventilator. [1]

TERMINOLOGY AND ETIOLOGY

The new symptoms reported by persons who had polio have been given a variety of names by the popular press, including “Post-Polio Syndrome ” and Post-Poliomyelitis Progressive Muscular Atrophy (PPMA). The initial magazine articles that named the new symptoms also proffered their probable cause. It was suggested that the polio virus, lying dormant in the spinal cord for thirty years, had become reactivated and was giving people polio for a second time. It was also suggested that new weakness was the result of “some kind of amyotrophic lateral sclerosis.” These two suggested etiologies are now regarded as incorrect. The researchers and clinicians who gathered at Warm Springs, Georgia for the First International Symposium on the Late Effects of Poliomyelitis in May, 1984 rejected both explanations because neither has been supported by empirical evidence. The name “Post-Polio Syndrome” was rejected since most persons report some but not all of the five major symptoms listed above. The term Post Poliomyelitis Progressive Muscular Atrophy (PPMA) was also rejected as a general descriptor of the new symptoms since the over-whelming majority of persons reporting new symptoms are not experiencing progressive muscular atrophy.[10] The Post-Polio Task Force, meeting again in Boston in October, 1984, adopted the term Post-Polio Sequelae as the general descriptor of the new symptoms.

PATHOPHYSIOLOGY OF POST-POLIO SEQUELAE

If Post-Polio Sequelae result neither from reactivation of the polio virus nor from ALS, what is their cause? Once medical conditions unrelated to polio have been ruled out as causes of new symptoms, the symptoms can be grouped into two categories: Post-Polio Sequelae with explainable causes and those with unknown etiologies. Many instances of pain, muscle weakness and fatigue can be attributed to readily diagnosible causes that are in some way related to the original paralysis caused by the poliomyelitis. For example, carpal tunnel syndrome frequently arises in persons having lower extremity

paralysis who have ambulated on crutches for thirty years.[1] The original muscle paralysis can also predispose to scoliosis and its related respiratory difficulties, as well as knee instability and deformities that produce joint and muscle pain.[2,3,4,11]

The second category of Post-Polio Sequelae includes those symptoms that cannot be attributed to any other diagnosis. Testable hypotheses are just being proposed to explain the cause of these symptoms. Although hypotheses to explain fatigue and pain have not yet been clearly outlined, there are several hypotheses describing the cause of muscle weakness.

The most parsimonious hypothesis suggests that normal age-related losses of anterior horn motor neurons reduces the size of the pool of functioning anterior horn cells – a pool that was markedly diminished by the original poliomyelitis – and that these reductions lead to new muscle weakness. It is a remarkable fact that

persons who had spinal poliomyelitis were shown to have normal muscle function even after the loss of 60% of their anterior horn cells.[12] Since normal anterior horn cell loss is suggested to occur at a rate of 5% per decade after age twenty, aging-related losses of spinal motor neurons could reduce the total number of anterior horn cells to below 40% and thereby cause decreased muscle functioning.[13]

A second hypothesis relates to the process of functional recovery following poliomyelitis. One mechanism by which muscle function is restored following the acute infection is by surviving motor neurons producing axonal “sprouts.” Through the growth of axonal sprouts, remaining motor neurons innervate not only their own muscle fibers but also thousands of additional muscle fibers “orphaned” when surrounding motor neurons died.[14] Electromyographic studies indicate that the number of sprouts and their ability to function are decreased in persons who had polio – decreases that correlate with the number of years since polio onset and not chronological age. Further, recent animal studies indicate that exercise and stress cause the destruction of axonal sprouts and that sprouts are not replaced in aged animals (Smith and Rosenheimer, personal communication, 1984). While clinical reports indicate that exercise and stress do precipitate decreases in muscle function in persons who had polio, a relationship between failed axonal sprouts and muscle weakness has yet to be empirically documented.[11,15,16]

Finally, it has been suggested that the transient muscle weakness that follows exercise may be caused by the metabolic fatigue of polio virus-damaged or extensively sprouted anterior horn cells.[14] These motor neurons may become unable to meet the metabolic demands of functioning after years of operating in a damaged state or supplying more muscle fibers than they were “designed” to innervate.

TREATMENT OF POST-POLIO SEQUELAE

Although research into the cause of Post-Polio Sequelae is in its infancy, a number of important facts have come to light regarding treatment for the symptoms of unknown etiology. The “use it or lose it” dictum of polio rehabilitation during the 1940’s and 1950’s appears not to apply to the post-polio population today. Many persons report that strenuous and fatiguing activities result in decreased muscle strength, generalized fatigue and pain.[4,15,16] Continuing to walk without assistive devices or with originally prescribed braces, canes or crutches has become too exhausting or painful for many. The use of new or additional assistive devices and the appropriate bracing of weak and abused joints have been very successful in reducing symptoms and maintaining function.[1,2,15] Also, the combination of a reduction in strenuous activities with carefully prescribed and monitored progressive resistance exercises and cardiopulmonary conditioning has been shown both to reverse weakness and reduce fatigue in a number of individuals.[7,16,18] However, it is crucial that even therapeutic exercise be stopped at the first indication of fatigue.[16]

It is becoming clear that for the majority of persons experiencing Post-Polio Sequelae changes in daily schedule, decreasing strenuous and fatiguing activities and new physical therapies or assistive devices will be required to treat the new symptoms. However, to the persons experiencing them, such changes in lifestyle often feel as if they are becoming disabled for a second time by the same disease.

THE PSYCHOLOGY OF POST-POLIO SEQUELAE

Many medical conditions cause more than one disability. Atherosclerosis, causing a function-limiting heart attack, can progress to also cause a disabling stroke. Diabetes can cause late onset blindness and peripheral vascular disease that not infrequently necessitates multiple amputations. Usher’s syndrome is a hereditary condition that causes profound congenital deafness and progressive blindness.[19,20] In all of these instances affected persons know that additional symptoms are possible and know their cause. It is unique to Post-Polio Sequelae that neither the persons who had polio nor their physicians knew there was any potential for additional symptoms to occur or knew their cause when they appeared.

When persons who had polio first began reporting new symptoms to their physicians, almost no one was told that their problems might be related to a bout with polio thirty years earlier. Thousands across the country have undergone extensive medical testing that was unable to establish a diagnosis. As a result, many persons experiencing new symptoms have become frustrated and desperate after being repeatedly told there was nothing wrong or that they would “get used to” pain, fatigue, weakness, etc. Some individuals have even been admitted to hospital with a diagnosis of depression because no physical cause could be found for their complaints. On the opposite end of the spectrum, many others have been diagnosed as having “some kind of ALS” or were told that they were actually having polio again.[17] Most did not even know that others were experiencing similar symptoms and difficulties since persons who had polio have not traditionally affiliated with each other as a disability group. These persons felt completely alone, without medical or social support. Further, individuals who had polio regained function through great physical and emotional effort. Now, after thirty or more years, they were being forced to deal with disabling symptoms that felt very much like the symptoms they overcame and supposedly left behind following the initial polio infection.

The occurrence of a second disability as the result of Post-Polio Sequelae is psychologically devastating because of the combined effects of the occurrence of unexpected symptoms whose cause is unknown, a lack of knowledge and understanding on the part of the medical community, feelings of isolation and the loss of hard-won physical abilities whose recovery was thought to be permanent. Although clinical and research emphasis has thus far been placed on the physical symptoms reported by persons who had polio, in many cases the psychological trauma connected with Post-Polio Sequelae may be more damaging than the physical problems.

PSYCHOLOGICAL SEQUELAE OF SECOND DISABILITY

One individual who had polio has walked with a limp using a long-leg brace since he attained maximum function twenty-eight years ago. His physiatrist recently recommended that he begin using a cane to reduce energy expenditure and decrease pain. Later he told a friend, “I would not want to live like that!”

This statement expresses the attitude of most of society toward the disabled. Most people would not want to live “like that” since society devalues the disabled because they have lost something of value – full use of their bodies. [21,22,23,24,25,26] However, since disabled persons live in this society and are as subject to its beliefs and mythologies as anyone, they devalue themselves as the rest of society does.[27,28] Thus, when Post-Polio Sequelae affect persons who were disabled by polio with what is perceived as a second

disability, they are psychologically traumatized by the devaluing attitudes always associated with becoming disabled.

Society’s devaluing and generally negative attitude toward the disabled can readily be observed in the ways the disabled are usually portrayed in the media. Television and print coverage of the “story” of Post Polio Sequelae has relentlessly referred to persons who had polio as “polio victims” or as “suffering from polio.” Neither of these phrases is acceptable because each gives the impression that the lives of persons who had polio are primarily the result of a polio virus. The terms “Polio Survivor” and “Post-Polio” are also objectionable to many persons in the post-polio community because the former is felt to make unjustifiable reference to the Nazi Holocaust and the latter refers to those who had polio as if they were a diagnosis, not individuals.

THE PROCESS OF ACCEPTANCE OF SECOND DISABILITY

How does one adjust to the fact of a second disability in the face of negative societal attitudes and self devaluation? The following processes have been described for disabled person to work toward acceptance of disability.[21,22,23,24,28] Here, these processes have been modified to apply to the Post-Polio experience:

A period of mourning is usually needed for the loss of physical prowess. Such mourning is appropriate and usually occurs no matter how often disability occurs in a person’s life. In this phase, persons experiencing Post-Polio Sequelae might reject new assistive devices because those devices are symbols for the loss of physical ability earned through great effort.

Devaluing physique is the process whereby physical achievement and appearance become less important than personal competencies and effort. This process diminishes the devaluing of the individual and leads to coping with the disability rather than succumbing to its difficulties. This process will allow persons with Post-Polio Sequelae to be better prepared psychologically to deal with using new or additional assistive devices and with other changes in life style.

Enlarging the scope of values precludes allowing the single value of “the body” to become decisive in determining a person’s worth. An individual can become interested in work, study, politics, leisure activities, etc. as means to judge his worth rather than focusing on what his body can or cannot do. The Mayo Clinic study found that 78% of the persons in their group were employed full time.[8] This statistic is staggering when one considers that among the gamut of severely disabled persons, only 15% to 20% of the men and 7% to 8% of the women are employed full time .[29] Information from the Sister Kenny Institute indicates that persons who had polio marry at about the same rate as the general population (M. Knapp, personal communication, 1984). These data suggest that many persons who had polio already have an enlarged scope of values and, therefore, may be better equipped to deal with a second physical disability.

Upholding asset evaluation entails emphasizing and learning to value what a person can do within the limitations of his disability instead of judging that person by so-called “normal” standards that are inappropriate for him. If it is best for persons who had polio to reduce their schedules or to start using assistive devices, then such sensible alterations in behavior should be made based on the needs and abilities of the individual. When persons experiencing Post-Polio Sequelae see such required changes as safeguarding their lifestyle, rather than eroding it, they are upholding asset evaluation.

THE POST-POLIO SUPPORT GROUP

A valuable technique for facilitating these processes and for dealing with the psychological aspects of Post-Polio Sequelae is the post-polio support group. Such groups have been established across the country and have been found to be helpful for a number of reasons: 1) individuals come together because they share a common problem; 2) peers come together with the expectation of helping themselves and each other through changing attitudes and behavior; 3) peers make themselves available to each other between regularly scheduled meetings; 4) the group process consists of receiving and giving feedback, sharing hope, experiences, coping strategies and encouragement.[30] In addition, social support has been found to be protective against the negative effects of stress.[31]

STRATEGY FOR THE FUTURE

Since research on the causes of and treatments for Post-Polio Sequelae is just beginning, it will be some time before all of the answers are found. In the interim, the Post-Polio Task Force suggests that persons who are experiencing new symptoms should first consult a physiatrist (a specialist in physical medicine and rehabilitation) to obtain a complete physical examination, advice on reducing strenuous activities,

assessment of assistive devices and the prescription of carefully monitored physical therapy and new assistive devices when appropriate.

Second, all persons who had polio should become knowledgeable about Post-Polio Sequelae in order to be their own best advocate for appropriate medical care and needed community services. Post-polio support groups will gather information concerning local resources (e.g. names of informed and interested physicians, hospital-based rehabilitation clinics, independent living centers) as well as access the national information network that these groups are creating. In addition, the book Late Effects of Poliomyelitis (the proceedings of the Warm Springs Symposium on the Late Effects of Poliomyelitis) and the July, 1985 issue of the journal ORTHOPEDICS, which is devoted to Post-Polio Sequelae, are available. The papers included in these volumes will enable persons who had polio to become familiar with the latest information on Post-Polio Sequelae and to inform and educate their own physicians.

Third, state and federal officials must be apprised of the problems and needs of the post-polio community. Members of Congress must be asked to hold hearings on Post-Polio Sequelae so that federal funds will be made available for treatment programs and research grants.

CONCLUSION

It is now clear that persons who had polio are in fact experiencing new and unexpected symptoms. Ours is the first generation of persons to document what happens to bodies that have been working for thirty or more years under the special stresses caused by having had polio. Surviving the acute polio infection, overcoming its paralytic sequelae and attaining quality of life required determination and tremendous effort. No less determination and effort will be required of both the post-polio and rehabilitation communities to overcome Post-Polio Sequelae and maintain that quality of life.

REFERENCES

Halstead LS and Rossi CD: New problems in old polio patients: Results of a survey of 539 polio survivors. Orthopedics; 8(7), 1985:845-850. [PubMed Abstract]
Perry J: Orthopedic management of Post-Polio Sequelae. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985.
Fischer DA: Poliomyelitis: Late pulmonary complications and management. In: Halstead LS and

Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985. 4. Maynard FM: Differential diagnosis of pain and weakness in post-polio patients. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985. 5. Bruno RL, Johnson JC, Berman WS: Motor and sensory functioning with changing ambient temperature in post-polio subjects. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis . Miami, Symposia Foundation, 1985.

Bruno RL, Johnson JC, Berman WS: Vasomotor abnormalities as Post-Polio Sequelae: Functional and clinical implications. Orthopedics; 8(7),1985:865-869. [PubMed Abstract] 7. Owen RR: Polio residuals clinic and exercise protocol: Research implications. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985. 8. Codd MB, Mulder DW, Kurland LT, et al: Poliomyelitis in Rochester, Minnesota, 1935-1955: Epidemiology and long-term sequelae: A preliminary report. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985.
National Health Survey: Prevalence of selected impairments (series 10, number 134). U.S. Department of Health and Human Services publication [(PHS) 81-1562], 1981, p 15. 10. Dalakas MC, Sever JL, Fletcher M, et al. Neuromuscular symptoms in patients with old poliomyelitis: Clinical, virological and immunological studies. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985.
Wiechers DO: Late effects of poliomyelitis: Case reports. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis . Miami, Symposia Foundation, 1985.
Sharrard WJ: The distribution of permanent paralysis in the lower limbs in poliomyelitis. Journal of Bone and Joint Surgery, 37B, 1955: 540-558. [Lincolnshire Library Full Text] 13. Tomlinson BE, Irving D: Changes in spinal cord motor neurons of possible relevance to the late effects of poliomyelitis. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985.
Wiechers DO: Pathophysiology and late changes of the motor unit after poliomyelitis. In: Halstead LS and Wiechers DO (eds.): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985. 15. Maynard FM: Post-Polio Sequelae: Differential diagnosis and management. Orthopedics; 8(7),1985:857-861. [PubMed Abstract]
Feldman RM: The use of strengthening exercises in Post-Polio Sequelae. Orthopedics; 8(7),1985:889-890. [PubMed Abstract]
Mulder DW, Rosenbaum RA, Layton DD: Late progression of polio-myelitis or forme fruste amyotrophic lateral sclerosis? Mayo Clinic Proceedings 1972; 47:756-761. [PubMed Abstract] 18. Owen RR and Jones D: Polio residuals clinic: Conditioning exercise program. Orthopedics; 8(7),1985:882-883. [PubMed Abstract]
Vernon M: Usher’s Syndrome – deafness and progressive blindness. J. chron. Dis. 1969; 22:133-151. [PubMed Abstract]
Rainer JD: Psychiatric aspects of Usher Syndrome. In: Tully W(ed): Papers Presented at Workshop on Usher’s Syndrome. New York, Helen Keller National Center, 1977.
Wright BA: Physical Disability – A Psychosocial Approach. New York, Harper and Row, 1983. 22. Meyerson L: Somatopsychology of physical disability. In: Cruickshank WM (ed): Psychology of Exceptional Children and Youth. Englewood Cliffs, Prentice-Hall, 1955.
Barker RG, Wright BA, Meyerson L, et al: Adjustment to Physical Handicap and Illness: A Survey of the Social Psychology of Physique and Disability. New York, Soc. Sci. Res. Council, Bull. 55, 1953.
Meyerson L: Special disabilities. In Farnsworth PR (ed): Annu. Rev. Psychol., Vol. 8 . Palo Alto, Annual Reviews, 1957.
Siller J: Attitudes toward disability. In: Rusalen H, Malikin D (eds): Contemporary Vocational

Rehabilitation. New York, New York Univ. Press, 1976.

Siller J: Psychological aspects of physical disability. In: Meislen J (ed): Rehabilitation Medicine and Psychiatry. Springfield, C.C. Thomas, 1976.
Finkelstein V: Attitudes and Disabled People: Issues for Discussion. New York, World Rehabilitation Fund, 1980.
Wright BA: Issues in overcoming emotional barriers to adjustment in the handicapped. Rehabil. Counsel. Bull 1967; 11:53-5.
Lando ME, Cutler RR, Gamber E: 1978 Survey of Disability and Work (Data Book) Washington, U.S. Department of Health and Human Services, 1982.
Jaques ME , Patterson KM: The self-help group model: A review. Rehabilitation Counseling Bulletin 1974; 18:48-58.
Cobb S: Social support as a moderator of life stress. Psychosom. Med 1976; 38:300-314

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom

Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk

Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/lit.html> Created: 23rd January 1997

Last modification: 29th January 2010.

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