Bruno

THE PSYCHOLOGY OF POLIO AS PRELUDE TO POST POLIO SEQUELAE:

Behavior modification and psychotherapy

Richard L. Bruno, Ph.D.¹ and Nancy M. Frick, M.Div.²

Bruno RL, Frick NM. The psychology of polio as prelude to Post-Polio Sequelae: Behavior modification and psychotherapy. Orthopedics, 1991, 14(11) :1185-1193.

 

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

1. Post-Polio Rehabilitation and Research Service

Kessler Institute for Rehabilitation

East Orange, New Jersey

and

Department of Physical Medicine and Rehabilitation

New Jersey Medical School

University of Medicine and Dentistry of New Jersey

2. Harvest Center

Hackensack, New Jersey

Address correspondence to:

Nancy M. Frick, M.Div.,

Harvest Center,

151 Prospect Avenue,

Hackensack, New Jersey 07601

THE PSYCHOLOGY OF POLIO AS PRELUDE TO POST-POLIO SEQUELAE: Behavior modification and psychotherapy.

Richard L. Bruno, Ph.D.¹ and Nancy M. Frick, M.Div.²

ABSTRACT

Even as the physical causes and treatments for Post-Polio Sequelae (PPS) are being identified, psychological symptoms – chronic stress, anxiety, depression and compulsive, Type A behavior – are becoming evident in polio survivors. Importantly, these symptoms are not only themselves causing marked distress but also are preventing patients from making the lifestyle changes necessary to treat their PPS. Neither clinicians nor polio survivors have paid sufficient attention to the acute polio experience, its conditioning of life-long patterns of behavior, its relationship to the development of PPS and its effect on the ability of individuals to cope with and treat their new symptoms. This paper describes the acute polio and post-polio experiences on the basis of patient histories, relates the experience of polio to the development of compulsive, Type A behavior, links these behaviors to the physical and psychological symptoms reported in the National Post-Polio Surveys and presents a multimodal behavioral approach to the treatment of PPS by describing patients who have been treated by this Post-Polio Service.

ACKNOWLEDGEMENTS

The authors would like to thank those who have volunteered as research subjects and our patients, whose experiences are the foundation of this report and who have allowed themselves to be quoted for this paper. We would also like to thank Dr. Thomas Galski for his criticism of the manuscript, Ms. Joan Kelly for her computer expertise and Mary Ann Solimine, R.N., M.L.S., without whom this work would not have been possible. This research was funded by grants from the Joel Leff and George Ohl Foundations.

THE PSYCHOLOGY OF POLIO AS PRELUDE TO POST-POLIO SEQUELAE: Behavior modification and psychotherapy in treating PPS

Richard L. Bruno, Ph.D. and Nancy M. Frick, M.Div.

“I’ll walk without crutches. I’ll walk into a room without scaring everybody half to death. I’ll stand easily enough in front of people so that they’ll forget I’m a cripple.” Franklin D. Roosevelt (1)

In the 1980’s, nearly half of the 1.63 million American polio survivors reported new and unexpected symptoms – fatigue, muscle weakness and pain – that have come to be called Post-Polio Sequelae (PPS) (2,3). While the medical community focused its attention on these new physical symptoms, polio survivors’ psychological reactions to PPS emerged. Fear, disbelief, denial and confusion were experienced first since PPS were completely unexpected (4). The 1985 National Survey of 738 polio survivors (5,6) revealed that 86% of the respondents never considered the possibility of developing new problems after having reached maximum recovery from polio and that 72% did not initially think that their new symptoms were related to their having had polio. Since polio survivors did not know the cause of their new symptoms they looked to the medical community for help.

Fear and confusion increased as medical ignorance and disinterest frustrated and angered polio survivors. Many physicians dismissed new symptoms as psychogenic, factitious or unrelated to polio. In the 1985 National Survey, 23% of respondents were told by physicians that the symptoms were “all in their minds” while 26% were told that the symptoms “could not possibly be related to having had polio.” Other patients received frightening and unfounded diagnoses, such as “some kind of ALS” or recrudescent polio (3). Many patients were told that they would have to accept new symptoms and loss of function since all polio survivors were “just getting old” (4).

Now, in the second decade of PPS, the medical community has accepted the reality of PPS and is identifying effective treatments for new fatigue, weakness and pain. Even with these advances, additional

psychological symptoms – chronic stress, anxiety, depression and compulsive, Type A behavior – are being identified in polio survivors. Importantly, these symptoms are not only themselves causing marked distress but also are preventing patients from making the lifestyle changes necessary to treat their PPS (6,7,8).

It appears that the factor central to the etiology of these psychological symptoms is that polio survivors are being forced to cope with new symptoms and disability when many have not yet dealt with the emotional reality of their having had polio over thirty years ago. Evidence for the postponement of coping with the acute polio experience was found in the 1985 National Survey, where 26% of respondents reported that they “did not think of themselves as having a disability” before developing PPS; with the onset of PPS 42% admitted to having a “second disability.” (6)

Neither clinicians nor polio survivors have paid sufficient attention to the acute polio experience, its conditioning of life-long patterns of behavior, its relationship to the development of PPS and its effect on individuals’ ability to cope with and treat their new symptoms. It is the purpose of this paper 1) to describe the acute polio and post-polio experiences on the basis of published reports and patient histories; 2) to relate the experience of polio to the development of compulsive, Type A behavior; 3) to link compulsive, Type A behavior to the physical and psychological symptoms reported in the National Post-Polio Surveys; 4) to present a multimodal behavioral approach to the treatment of PPS by describing patients who have been treated by this Post-Polio Service during the past year.

The Acute Polio Experience

Polio survivors frequently report that the onset of PPS have forced them, often for the first time, to recall and examine their acute polio experience (9). With the onset of polio, these individuals understood that they had been stricken by the “greatly feared disease,” believed that disability was likely and death was possible (10). These fears were amplified in those who knew others who had been disabled or even killed by polio (10). Polio survivors recall confusion and terror at being wrenched from their homes and being given by their parents into the care of strangers with whom they were required to live for weeks, months or even years (11). Even children too young to understand what was happening likely sensed their parents’ fear and panic; they certainly felt the pain of their own illness and the desolation of being abandoned by their parents (11,12,13,14).

Individuals admitted to hospital quickly became aware of their complete loss of control and total dependency on the hospital staff. Many could not feed or toilet themselves; those with the most severe paralysis could not even move. Patients’ were forced to rely totally on hospital staff if their most basic survival needs were to be met. If they were to survive, patients had little choice but to suppress their fears and follow their parents’ parting instruction: “Be good, don’t make trouble and do everything you’re told” (11).

Separation from parents and loss of control were not the only sources of fear. Patients describe a hospital regimen of painful and often frightening therapies including hot packs, splinting, bracing, stretching and exercise (15). Patients also report undergoing multiple surgeries, including muscle transplants, tendon lengthenings and osteotomies. One especially brutal procedure that many parents attempted to obtain for their children, pulverized weakened muscles and their motor nerves with an electric riveter to promote axonal sprouting – a uniquely medieval attempt to prevent permanent visible disability (16,17). These procedures were administered usually without explanation and certainly without consent since, especially at the height of the epidemics, hospitals were overwhelmed by the sheer number of polio patients needing care (10,11,12,13,15,18,19). Excluding young patients from treatment decisions and the failure to

communicate information about their illness magnified the severity of the illness to the patients and permitted their inherent fear of death to emerge (14).

Questions or complaints about therapies were not infrequently met by staff anger or punishment (13,18). Staff anger was viewed as a very real threat to survival by patients who were isolated from parents and totally dependent on the staff. One patient described this situation as placing her in “mortal danger” (15); she believed that the staff had to be placated if she were to survive. Patients discovered that staff anger could sometimes be prevented if they complied with hospital regimen without questions. Full participation in therapies, without regard to pain or exhaustion, would sometimes garner brief attention or even praise. Thus, the expression of personal needs and emotion were punished by the staff while unquestioning compliance with and performance for the staff were reinforced. Unfortunately, protection from punishment could not always be assured. Patients describe staff who acted with unnecessary cruelty in maintaining order and control on the ward. Family visits were restricted to only a few hours per week, reinforcing patients’ feelings of parental abandonment and dependency upon staff (12,15,20). Normal, child-like behaviors were punished excessively. Several patients reported having been locked in a completely dark closet overnight when they spoke or cried after “lights out.” Even appropriate and necessary nursing care could be withheld for no apparent reason (15,18).

Many of the patients treated by this Service have related other instances of psychological, physical and even sexual abuse at the hands of hospital staff. Instead of causing patients to complain, such abuse further reinforced patients’ belief that survival depended upon the suppression of personal needs and emotion and unquestioning compliance with those in authority. This belief is evidenced in one patient’s rationale for not reporting repeated sexual abuse: “I would have made my parents angry because I was not doing what I was told and the nurses, who I depended on for everything, would have punished me for making trouble for them. All I could do was stop feeling bad about it and smile.”

Under these circumstances, some patients report having lost their sense of “identity” (18,20). Some report over-identifying with the staff and even expressed the desire to become “orthopedic surgeons when they grew up” (21). Others apparently submitted so completely to the hospital staff that they did not wish to go home or even asked to return to the hospital following discharge (22,23). It appears that many polio survivors learned to deal with their abandonment, loss of control, fear, pain and abuse by submitting to those in authority, complying fully with external expectations and denying personal needs, physical and emotional pain and even their own individuality (24). These behaviors are evident in this summary of the “Good Chart” written by polio wardmates at Baltimore’s City Hospital (22):

LISTEN TO THE DOCTORS

OBEY THE NURSES

DO NOT FIGHT

DO NOT BE BAD

BE GOOD IN SCHOOL

DO YOUR HOMEWORK

DO NOT TALK AT DINNER or IN SCHOOL

LITTLE FOLKS SHOULD BE SEEN AND NOT HEARD

When polio patients returned home as “polio survivors,” the “Good Chart” became their prescription for proper behavior. They had learned very young and very well that submission, compliance and suppression of emotion were required if they were to escape “mortal danger”(15).

Polio Survivors in the Community

When polio survivors re-entered their communities, any special attention that may have been provided to them and their families during the acute polio often quickly stopped (22). Friends of polio survivors’ families, unable to deal with the reality of polio, often disappeared (22). It was not uncommon for neighborhood children to be prohibited from playing with “polio victims” for fear that the “crippling” was contagious (23). Polio survivors, no longer physically able to participate easily or fully in social activities, became isolated (10,25). Such alienation and isolation made polio survivors pariahs (10,14,22,25).

However, there was “hope.” Individuals had been imbued with the “unqualified progress ideology” of physical therapy following their acute illness and had often been given prognoses upon discharge that were unrealistically optimistic (26). One paraplegic woman was told, “You’ll walk out of your braces

before you start dating.” Such prognoses were seen as a call to normalcy. The use of braces, crutches and wheelchairs, that had made mobility possible and had been symbols of triumph over paralysis in the hospital, became stigmata of “the crippled” and symbols of one’s failure to have recovered completely. Necessary assistive devices were quickly discarded, regardless of the discomfort, fatigue, awkwardness or pain that resulted, as individuals strove for the “appearance of complete physical normalcy” (21,27). Painful and exhausting physical therapies were resumed upon patients’ return home from hospital or initiated in those who were not hospitalized. Physical therapy often continued for more than a decade after the acute polio with the only goal being “complete recovery” (11). One patient, who became triplegic at age six and has consistently thereafter used a wheelchair, continued daily physical therapy until she left home for college, “Everyone believed that physical therapy would make me walk – eventually.” As motivation to walk without braces, another patient was repeatedly beaten by his father who said, “I’d rather see you dead than a cripple.”

The abuse of polio survivors by parents was usually less overt but was not infrequent, being motivated by a denial of polio and revulsion by disability (13,14,27). Some polio survivors report being physically trapped by their parents’ refusal to make accommodations for their physical limitations. Homes frequently remained inaccessible, making activities of daily living impossible without assistance from parents – assistance that could be inconsistently provided or consistently withheld (28). One patient was regularly carried outside in the early morning, regardless of the weather, where she remained until dark. Another patient reported that he was locked in a car with windows closed in the heat of midsummer so that his family could tour Washington’s then inaccessible national monuments. Polio survivors learned that physical abilities needed to be maximized if they were to survive in a “barrier-full” society.

Other parents strove to reincorporate the child into the family routine and “forget” about polio by requiring children to equal or exceed the level of physical performance they exhibited before their illness (11). One patient’s family took up hiking when their daughter returned home from the hospital, expecting her to “learn to keep up with the others” in spite of crutches and braces. Patients also report that they were expected to out-perform their peers academically. Several patients reported that they would be severely punished if they received “anything less than an “A”.” Such academic expectations reinforced the premature adult-like behaviors that were described in children who had been hospitalized following the acute polio (22).

Still other parents experienced both “shame and estrangement” at having been “singled out” by polio (22). Unable to deny the reality of their childs’ disability, they isolated the family from society and severely restricted their child’s physical activities (11,14,22). One patient, who had a barely noticeable limp following polio at 18 months, was prevented from leaving his yard or playing with other children until he attended high school. Such isolation impaired the development of social relationships (25,29,30) and parental shame of disability was learned by the child (14). Unfortunately, many patients report that their parents have consistently refused through the years to discuss anything having to do with the physical or

emotional reality of the polio experience (14,20). An attempt at discussion with one 45-year old patient’s mother was stopped by the angry statement, “It’s all too painful for me. Don’t ever mention it again.”

Whether unrealistically demanding or overly restrictive, parents’ powerful expectations, combined with those of physicians, nurses and therapists, dictated the behaviors that were required of polio survivors if they were to escape “mortal danger” (15) and be accepted by parents and society. Yet, despite the fulfillment of these expectations by the achievement of a remarkable degree of functional recovery in most individuals and even the appearance of “normalcy” in many, polio survivors were told that they were still not acceptable (31). They report being told by teachers that no one would ever hire them, by parents that no one would ever marry them, and by physicians that they should never have children (28,32,33). Thus, as polio survivors entered adulthood they had internalized the expectations of those in authority, which led to self-alienation and actual participation in the “execution of the self” (24). Polio survivors continued to follow the prescription for proper behavior set forth in the “Good Chart” even though they had been told that fulfilling the expectations of others would not result in acceptance by society (34).

Type A Behavior as a Post-Polio Sequelae

The majority of polio survivors have succeeded in minimizing the appearance of disability, maximizing independence and “disappearing” into society (28). Most polio survivors have learned to walk after discarding nearly all of their original assistive devices (6). Despite society’s negative expectations, polio survivors have had more years of formal education and a larger proportion of them are married as compared to the general disabled and non-disabled populations (Table 1)(35). Polio survivors also have a higher level of employment as compared to the general disabled population (36).

These data suggested the hypothesis that polio survivors have not merely achieved “normalcy” but have actually surpassed it by learning physically and emotionally stressful Type A behavior as a response to their experience of polio (5,28,37). The polio experience should have provided an ideal environment for the conditioning of Type A behavior. Type A behaviors are thought to learned in service of the “active avoidance of punishment” (38) by individuals who are engaged in a chronic “struggle to overcome environmental barriers” (39) “against the opposing efforts of other things or persons” (40). Further, Powell, et al (41) found that a “consistent set of beliefs and attitudes about the self, others, and life in general lay beneath the overt (Type A) behavior,” including “low social support,” “low self-esteem” and loss of control. Such beliefs would be the likely consequence of the above-described experience of polio.

To test the hypothesis that polio survivors are Type A, the Brief Type A Questionnaire (42) was administered to 738 polio survivors as part of the 1985 National Post-Polio Survey (5). Polio survivors’ mean Type A score of 53±22 was significantly higher than that of non-disabled controls (36±14). This finding was replicated by the 1990 National Survey of 373 polio survivors (6) who had a significantly elevated mean Type A score of 59±22 as compared to non-disabled controls (45±20) and adults with spina bifida (48±22)(Table 2).

Since it had been suggested that Type A individuals are hypersensitive to criticism (41), the 1990 National Survey also included the Reinforcement Motivation Survey (RMS) (43) that contains a Sensitivity to Criticism and Failure Scale. Polio survivors had a significantly elevated mean Sensitivity to Criticism and Failure score (68±28) as compared to non-disabled controls (59±27) that was significantly correlated with the Type A score (r=0.44)(5). This correlation suggests that Type A behavior in polio survivors serves in part to prevent criticism and to protect against feelings of failure (21,44).

Compulsive Behavior as a Post-Polio Sequelae

The majority of patients treated by this Service not only demonstrate Type A behavior but also evidence behaviors that are not typically Type A. Patients describe an inability to express emotion or admit having physical pain (“I can’t complain because people expect the handicapped to complain”). They inflexibly and punitively judge their own behavior on the basis of what is “normal” and on unachievable ideals of perfection. One patient stated, “If I fail at anything, I might as well die. It boils down to either using every ounce of my energy to lead a normal, successful life or giving in to my weaknesses and being inferior.”

Patients also demonstrate a strong need to be in control (15) and report marked anxiety with and nearly phobic avoidance of any change (“I am constantly moving and in a constant state of fear. I feel if I slow down, I’ll never get started again”) or decrease in the number or extent of their activities (“I can’t just sit and do nothing”). These patients refuse to slow down, delegate responsibilities or allow others to assist them even when they experience fatigue, weakness and pain. They report a saw-tooth pattern of activity,

characterized by working until physical symptoms prevent them from continuing. Exhausted, anxious and fearful of criticism, they rest until activity is again possible and then work until symptoms force them to halt. As one polio survivor stated, “I have spent my whole life pushing to keep going and I still push myself even though I know I shouldn’t. I keep going until I can no longer walk or stand the pain. I cannot be stopped. I work until I fall.”

Patients also report that they “must” satisfy the real or perceived needs of others (“I can’t ever say ‘No'”) and some describe a compulsion to perform nearly ritualistic behaviors in order to escape “mortal danger” (15). One patient stated, “I know it’s ridiculous, but I believe that if I make the bed every day I’ll never die; I will earn the right to continue to live.”

These behaviors are reminiscent of the Obsessive Compulsive Personality Disorder (OCPD)(45). However, polio survivors’ compulsive behavior differs from OCPD in important ways. Their compulsivity does not interfere with task completion or promote indecisiveness, nor do polio survivors lack generosity in giving to others. On the contrary, compulsive behaviors are goal-oriented and are performed in the service of others. Importantly, these behaviors are associated with the exacerbation and maintenance of PPS symptoms. Because of these differences with OCPD, a separate diagnostic category, Compulsive Psychophysiological Disorder (CPD), is suggested to highlight the relationship between goal-oriented, compulsive behaviors and the occurrence of physical symptoms (Table 3).

Fifty percent of the patients evaluated by this service met the criteria for CPD. Further, the diagnosis of CPD is significantly correlated with patients’ elevated Type A (r=.46) and Sensitivity to Criticism and Failure scores (r=.47) suggesting that compulsive behaviors are related to Type A behavior and may also serve in part to prevent criticism and to protect against feelings of failure. These findings support the theoretical literature on compulsive behavior, which suggests that compulsivity protects against loss of control and failure (46) and is used to “avoid or overcome distressful feelings of helplessness” (47)(see “flashbacks,” below). Millon and Everly (48) state that compulsive behavior occurs when the “child never develops a separate identity, and functions in the world by conforming to strict, internalized parental standards and to the standards around him or her” – conditions that are described above as central to the experience of polio. And, compulsive behavior has been seen to develop in children hospitalized even for brief periods (20).

Compulsive, Type A Behavior and the Etiology of PPS

Unfortunately, polio survivors appear to have paid a high price for learning compulsive, Type A behavior in an effort to achieve “normalcy,” escape “mortal danger” (15) and protect against the emotional pain generated by the polio experience. That price is Post-Polio Sequelae (28). Physical overexertion and

emotional stress were reported to be the first and second leading causes of PPS on the 1985 National Survey (5). The majority of respondents to the 1990 National Survey reported symptoms of chronic stress (frequent feelings of anxiety, low mood and difficulty falling asleep because the “mind is racing”)(6,7,8). These symptoms, along with the Type A and Sensitivity to Criticism and Failure scores, were significantly correlated with PPS symptom severity (Table 2)(5,6,49).

These data indicate that PPS are psychophysiological in nature and that clinicians must address both physical and psychological symptoms when polio survivors present for treatment of PPS. Thus, the treatment of PPS requires behavior modification to reduce compulsive, Type A behavior and thereby decrease PPS symptoms. Psychotherapy is also necessary to address the dysfunctional beliefs and powerful suppressed emotions resulting from the experience of polio that motivate compulsive, Type A behavior, produce psychological distress and prevent compliance with therapy.

Behavioral Modification and Psychotherapy in Treating PPS

All patients who present for treatment by the Post-Polio Service undergo complete physiatric and psychological evaluations, the latter beginning with administration of the Reinforcement Motivation Survey (RMS) (43) and Beck Depression Inventory (BDI) (50). Of the 32 patients evaluated during the past year, the majority had elevated Type A (mean = 57 ± 28 ) and Sensitivity to Criticism and Failure (mean = 75 ± 28) scores on the RMS but had insignificantly elevated BDI scores (mean = 14 ± 9).

Although 57% of patients reported “low mood,” only 31% met DSM-IIIR criteria for Major Depressive Episode (MDE), a percentage similar to the 32% reported by Freidenberg, et al. (51) but higher than the prevalence of MDE in the general population (3% to 7%) (52,53) and in those with medical illness (20%) (54). Friedenberg, et al. (51) suggested that damage to monoaminergic neurons in the brain stem may predispose polio survivors to MDE (see 49). However, the report of “treated depressions” in 38% of adults with spina bifida suggests that childhood-onset orthopedic disability itself may predispose to MDE in adults (55).

The presence of MDE in our patients is important, not only because it requires treatment, but also because MDE is significantly correlated with treatment non-compliance. MDE was diagnosed in 63% of patients who refused further treatment after the initial evaluation and in 50% of those who were discharged for therapeutic non-compliance. Only 11% of patients who were fully compliant and 29% of those who were partially compliant with therapy were diagnosed as having a MDE. MDE is always treated with psychotherapy, although depression was sufficiently severe in 70% of patients that an antidepressant was recommended.

Following psychometric testing, psychological, medical and psycho social histories are taken that include detailed descriptions of the new symptoms and their causes, the acute polio experience, post-polio rehabilitation and patients’ personal experience of disability. Information about the response of family, friends and community to the acute polio, to patients’ original disability and to PPS is elicited along with a chronology of school and vocational achievements noting the onset and development of compulsive, Type A behavior.

Treatment. The treatment of PPS is approached by all members of the treatment team – physician, psychologist and occupational and physical therapists – from a psychophysiological perspective using the techniques of multimodal behavior therapy (56). Therapy begins by asking patients to list their treatment goals and keep a daily log of activities, perceived exertion, fatigue, muscle weakness, pain, emotional stress, thoughts and emotions. This log is used to document physical and emotional symptoms and

demonstrate their relationship to thoughts, affect and compulsive, Type A behavior (56). Outpatients are then evaluated by occupational and physical therapists during twice-weekly sessions. Inpatients receive all therapies daily. After two weeks, log data and evaluation results are used by the treatment team to formulate a behavioral plan designed to decrease behaviors that cause physical symptoms, initiate self care activities and incorporate stress and time management, energy conservation, work simplification and a program of relaxation, stretching and non-fatiguing progressive resistance exercises (57) into patients’ daily routine. The behavioral plan is then presented to patients and their significant others at a meeting with the treatment team. Also discussed are the hypothesized causes of PPS, the need to modify behavior to treat PPS and the team’s expectation of total compliance with all aspects of the behavioral plan. Patients continue to keep logs throughout their treatment to document symptoms, the progression of exercises and modification of behaviors. Logs are reviewed with patients by each therapist at every treatment session. Goals are reviewed by the entire treatment team at twice-monthly behavioral rounds. Problems with the behavioral plan, especially those having to do with compliance, are communicated immediately to all members of the team. Significant problems with compliance may require that patients be called in to meet with the team. If no significant problems develop, patients meet with the team once again at the end of the eight to twelve week treatment program.

Compliance problems often arise with the daily log and the behavioral plan. Patients have difficulty keeping the log because it interferes with the compulsive performance of their scheduled activities and forces them to recognize the severity and pervasiveness of their symptoms. Invariably, patients have difficulty in complying with the behavioral plan. They will ‘forget’ to alter their schedules and refuse lifestyle modification because such changes directly conflict with their compulsive, Type A behavior (46). As one patient who was discharged for non-complince angrily stated, “I don’t want to change. I just want to be the way I was.”

Unfortunately, such problems with compliance are typical of post-polio patients. One clinic reported that only 41% of patients sporadically used a newly prescribed brace while 70% refused to use a new cane or crutch simply because they “didn’t want to” (58). Another clinic concluded that “the major reason for failure (of PPS symptoms) to improve was the unwillingness of the patient to follow any of the recommendations made” (59). Peach (60) has documented the importance of compliance with regard to the treatment of PPS muscle weakness, showing that muscle strength decreases over time only in patients who fail to fully comply with therapeutic recommendations.

During the past year, 47% of the patients treated by this service were judged to be fully compliant, 34% only partially compliant while 19% were discharged from therapy for not complying with the behavioral plan . Of all patients who received an initial evaluation, 28% refused any treatment for their PPS. It appears that inadequate compliance results in part from patients’ fear of relinquishing compulsive, Type A behavior that is believed by them to protect against “mortal danger” (15). Some non-compliant patients fear that PPS will return them to the same position of helplessness that they experienced following acute polio (cf. 47). Most report an even greater fear of criticism and a sense of failure when they merely contemplate lifestyle changes or the use of new assistive devices, even though without them PPS symptoms persist, progress and functional ability deteriorates. Not unexpectedly, inadequately compliant patients have an elevated mean Sensitivity to Criticism and Failure score (81 ± 2 0) as compared to fully compliant patients (74 ± 35).

Patients’ ability to change their behavior is directly related to their ability to challenge long-held beliefs about self-worth and survival and tolerate the emergence of the powerful fears and long-suppressed emotions generated by the polio experience (41). To promote compliance with the behavioral plan, cognitive therapy techniques are employed through weekly individual psychotherapy to identify and

modify dysfunctional beliefs, fears and emotions (46). Learning theory is also employed to explain how compulsive, Type A behavior was learned to protect against fear, emotional pain and “mortal danger” (15), while behavior modification techniques are used to decrease and eliminate these overlearned behaviors (41,46).

As these techniques assist patients to decrease compulsive, Type A behavior, increased anxiety and fear invariably result. Some patients report insomnia, panic attacks, intrusive and emotionally-charged flashbacks about their early polio experience and even fear of impending death (47). The psychotherapist then helps patients understand the origin of these most disturbing experiences and learn to tolerate them, reinforces the new self-care behaviors and supports patients through what can be terrifying days and nights.

As therapy continues, patients discover that their compulsive, Type A behavior has been more habitual than necessary. They begin to discard the “Good List,” become better able to care for themselves and begin to believe that they can have both self-worth and a disability. As the behaviors and emotions conditioned by the polio experience decrease, the focus of psychotherapy shifts to the experience of PPS. Depressed mood is replaced by sadness and anger as the trauma of a “second disability” is experienced (4). Twice-monthly group psychotherapy provides peer support to assist patients in dealing emotionally with their PPS and practically with the lifestyle changes necessary to treat them. The realization by patients in the group that they are not alone in their painful physical and emotional experiences is itself powerfully therapeutic. Couples and family sessions are also employed for both education about PPS and psychotherapy. It is noteworthy that nearly all spouses, with some striking exceptions, respond favorably to patients’ lifestyle changes with the most frequently heard comment being, “I’ve been wanting you to slow down for years.”

All patients treated by this Service, whether fully or even partially compliant, report meaningful reductions in physical and affective symptoms and increased function (cf. 60). But, as fatigue, muscle weakness and pain decrease, patients frequently increase activity and have a tendency to resume compulsive, Type A behavior (46). To prevent this, patients are instructed to strictly adhere to the modified daily schedule as set forth in their behavioral plan, especially after they are discharged from physical and occupational therapies and individual psychotherapy. Patients continue in group psychotherapy for at least six months after discharge, are seen at three-month intervals for medical and psychological follow-up and are encouraged to call their therapists at any time with questions or for support.

Conclusion

It is possible that the large proportion of patients treated by this Service who have experienced severe psychological trauma as a result of their polio experience may not be representative of all polio survivors or even of all individuals who present for treatment of PPS. However, there is something unique about the experience of polio that predisposes to the development of compulsive, Type A behavior, sensitivity to criticism and failure and chronic stress, anxiety and depression: the early onset of a universally feared, potentially life-threatening illness that presented in epidemic proportions; the experience of severe paralysis that could be forced to recede with the application of years of strenuous physical therapy; the need to maximize physical abilities in order to function in a “barrier-full” society that was repelled by disability; the sparing of intellectual abilities that were used to overcompensate for residual physical limitations (61,62). These factors must be studied if we are to understand the psychology of the polio survivor, maximize treatment compliance and effectively treat both the physical and psychological symptoms of PPS.

TABLES

Table 1 [below]

EDUCATIONAL, MARITAL, AND EMPLOYMENT STATUS*
	Post-Polio	Disabled	Non-Disabled
Marital Status
Single	11%	13%§	22%§
Married	71%	56%	58%
Other¤	18%	31%	20%
Education
< High school	29%	71%§	52%§
> High school	71%	29%	48%
Employment Status
Working
All positions¥	53%	38%§	79%**
Full time	40%	24%	59%
Unemployed	3%	62%	6%
Disability payments	28%	36%	1%
* Respondents to the 1990 National Post-Polio Survey compared with disabled and non-disabled Americans. ¤ Divorced, widowed, or separated. ¥ Full-time, part-time, or homemaker. Data from reference 35§ and 36**.

 

 

Table 2 [below]

DEMOGRAPHIC DATA, NEW SYMPTOMS, AND PSYCHOLOGICAL MEASURES*
	Post-Polio Subjects (n = 373)	Non-Disabled Controls (n = 145)
Age	54 ( ±11 )¤	47 ( ± 14)
Female/Male ratio	1.5	1.5
Years of education	14.5 ( ± 3)	15 ( ± 3)
Reinforcement Motivation Survey Scores:
Type A	59 ( ± 22)¤	45 ( ± 20)
Sensitivity to Criticism and Failure	68 ( ± 28)¤	59 ( ± 27)
New Symptoms
Fatigue	83%¥	15%
Muscle weakness	75%¥	9%
Pain	72%¥	15%

 

 

Emotional stress increases symptoms	75%¥	53%
Psychological Symptoms
Frequent feelings of anxiety	69%¥	36%
Chronic back/neck pain	66%¥	35%
Frequent trouble falling asleep because “mind is racing”	65%¥	33%
Frequent “low mood”	57%§	41%
Diagnosed depression	27%§	15%
* From the 1990 National Post-Polio Survey Significantly different from control values using independent groups t-test (¤P<.001) or chi-square statistics (§P<.002; ¥P<.0001).

 

 

Table 3 [below]

DIAGNOSTIC CRITERIA FOR COMPULSIVE PSYCHOPHYSIOLOGICAL DISORDER A. A pervasive pattern of continuous and excessive goal-oriented behaviour beginning in childhood or adolescence as indicated by at least four of the following:

1. Marked anxiety generated by and avoidance of any decrease in goal-oriented activities or changes in daily schedule
2. Refusal to delegate responsibilities or allow others to provide assistance associated with an unusually strong need to be in control
3. Rigidly and inflexibly judges behaviour on the basis of ideals of perfection or “normality” 4. Excessive time-consciousness and Type A behaviour
4. Extreme sensitivity to criticism with the constant expectation of failure and rejection 6. Inability to identify or express emotion, with the exception of anxiety, anger, and sadness. B. The association of these behaviours with the exacerbation or maintenance of physical symptoms, such as muscle spasm-induced pain, fatigue, or muscle weakness.

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1. Gallagher HG: FDR’s Splendid Deception. New York, Dodd, Mead, 1985.
2. Parsons PE: Data on polio survivors from the National Health Interview Survey. Washington, D.C., National Center for Health Statistics, 1989.
3. Frick NM: Post-Polio Sequelae: Physiological and Psychological Overview. Rehabilitation Literature 1986: 47:106-111. [Lincolnshire Library Full Text]
4. Frick NM: Post-Polio Sequelae and the psychology of second disability. Orthopedics 1985; 8: 851- 853. [PubMed Abstract]
5. Bruno RL, Frick NM: Stress and “Type A” behavior as precipitants of Post-Polio Sequelae, in Halstead LS and Wiechers DO (eds): Research and CLinical Aspects of the Late Effects of Poliomyelitis. White Plains, March of Dimes, 1987. [Lincolnshire Library Full Text]
6. Bruno RL, Frick NM: Psychological profile of polio survivors. Arch Phys Med Rehabil 1990; 10: 889.
7. Bruno RL, Frick NM: Five year follow-up to the 1985 National Post-Polio Survey. Arch Phys Med Rehabil 1990; 10: 886.
8. Conrady LJ, Wish JR, Agre JC, Rodriquez, AA, Sperling, KB: Psychological characteristics of polio survivors. Arch Phys Med Rehabil 1989; 70: 458-463. [PubMed Abstract] 9. Bozarth CH: Unfinished Business: Support Group Members Confront Feelings Surrounding the Late Effects of Polio. Lansing, Polio Survivors Support Group, 1987.
9. Copellman FS: Follow-up of one hundred children with poliomyelitis. The Family 1944; December: 189-196.
10. Meyer E: Psychological considerations in a group of children with poliomyelitis. J Pediatrics 1947; 31: 34-48.
11. Moustakas CE: Loneliness. Englewood Cliffs, Prentice Hall, 1961.
12. Seidenfeld MA: The psychological sequelae of poliomyelitis in children. The Nervous Child 1948; 7: 14-28.
13. Wright BA: Physical Disability – A psychosocial approach. New York, Harper and Row, 1983. 15. Register C: Living with Chronic Illness. New York, Free Press, 1987.
14. Berg RH: Polio and its Problems. Philadelphia, Lippincott, 1948.
15. Billig HE, van Harreveld A, Wiersma CAG: On re-innervation of paretic muscles by the use of their residual nerve supply. J Neuropath Exp Neurol 1946; 5:1-23.
16. Kerr N: Staff expectations for disabled persons. Rehabilitation Counseling Bulletin 1970; 14: 85-94. 19. Hawke WA, Auerbach A: Multidiscipline experience: A fresh approach to aid the multihandicapped child. Journal of Rehabilitation 1975: 41:22-24. [PubMed Abstract]
17. Rowlands P: Children Apart. New York, Pantheon, 1973.
18. Schechter MD: The orthopedically handicapped child. Arch Gen Psychiatry 1961; 4: 53-59. 22. Davis F: Passage Through Crisis. Indianapolis, Bobbs-Merrill,1963.
19. Rice EP: The families of children with poliomyelitis, in Poliomyelitis. Philadelphia, Lippincott, 1949.
20. Moustakas CE: Loneliness and Love. Englewood Cliffs, Prentice Hall, 1972. 25. Ladieu-Leviton G, Adler DL, Dembo T: Studies in adjustment to visible injuries: Social acceptance of the injured. Journal of Social Issues 1948; 4: 55-62.
21. Davis F: Illness, Interaction and the Self. Belmont, California, Wadsworth, 1972. 27. Long L, Roth C: The psychological aspects of rehabilitation, in Soden WH (ed) Rehabilitation of the Handicapped. New York, Ronald, 1949.
22. Trieschmann RB: Aging with a Disability. New York, Demos, 1987.
23. Battle CU: Disruptions in the socialization of a young, severely handicapped child. Rehabilitation Literature 1974; 35:130-140. [PubMed Abstract]
24. Anthony WA: Societal rehabilitation: Changing society’s attitudes toward the physically and mentally disabled. Rehabilitation Psychology 1972; 19: 117-126.
25. Wright BA: Issues in overcoming emotional barriers to adjustment in the handicapped. Rehabilitation Counseling Bulletin 1967; 11: 53-59.
26. Bloom JL: Sex education for the physically handicapped. Sexology 1970; 36: 62-65. 33. Hohmann GW: Reactions of the individual with a disability complicated by a sexual problem. Arch Phys Med Rehabil 1975; 56: 8-13. [PubMed Abstract]
27. Ladieu G, Hanfmann E, Dembo T: Studies in adjustment to visible disability. The Journal of Abnormal Social Psychology 1947; 42: 169-192.
28. Taylor H, Kagay MR, Leichenko S: The ICD Survey of Disabled Americans. New York, Louis Harris, 1986.
29. Statistical Abstract of the United States: 1990 (110th edition). U.S. Bureau of the Census, Washington, D.C., 1990.
30. Bruno RL, Frick NM: The origins of “Type A” behavior and stress induced symptoms in polio survivors. Proceedings of the Society of Behavioral Medicine 1989; 10: 85.
31. Perkins KA: Heart rate change in Type A and Type B males as a function of response cost and task difficulty. Psychophysiology 1984; 21: 14-21. [PubMed Abstract]
32. Rosenman RH, Friedman M, Straus R, et al: A predictive study of coronary heart disease. JAMA 1964; 189: 113-124.
33. Friedman M, Thoresen CE, Gill JJ, et al: Feasibility of altering Type A behavior pattern. Circulation 1982; 66: 83-92. [PubMed Abstract]
34. Powell LH, Friedman M, Thoresen CE, et al: Can the Type A behavior pattern be altered after myocardial infarction? Psychosom Med 1984; 46: 293-313. [PubMed Abstract] 42. 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 1982; 55: 1039-1042. [PubMed Abstract] 43. Bruno RL: Predicting pain management program outcome: The Reinforcement Motivation Survey. Arch Phys Med Rehabil 1990; 10: 785.
35. Mandell AJ: Toward a psychobiology of transcendence, in Davidson JM, Davidson RJ (eds): The Psychobiology of Consciousness, New York, Plenum, 1 980.
36. Diagnostic and Statistical Manual of Mental Disorders (ed 3, revised). Washington, D.C., American Psychiatric Association, 1987.
37. Beck AT, Freeman A: Cognitive Therapy of Personality Disorders. New York, Guilford, 1990. 47. Salzman L: The Obsessive Personality. New York, Jason Aronson, 1973.
38. Millon T, Everly G: Personality and its Disorders. New York, Wiley, 1985. 49. Bruno RL, Frick NM, Cohen J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae. Orthopedics 1991; (this issue). [Lincolnshire Library Full Text]
39. Beck AT, Ward CH, Mendelson M, et al: An inventory for measuring depression. Arch Gen Psychiatry 1961; 4: 561-571.
40. Freidenberg DL, Freeman D, Huber SJ, Perry, J, Fischer, A, Van Gorp, W G, et al: Postpoliomyelitis Syndrome: Behavioral Features. Neuropsychiatry, Neuropsychology and Behavioral Neurology 1989; 2: 272-281. [Lincolnshire Library Full Text]
41. Myers JK, Weissman MM, Tischler OL, et al: Six month prevalence of psychiatric disorders in three communities. Arch Gen Psychiatry 1984; 41: 959-967. [PubMed Abstract]
42. Roberts RE, Vernon SW: Depression in the community: Prevalence and treatment. Arch Gen Psychiatry 1982; 39: 1407-1409. [PubMed Abstract]
43. Schleifer SJ, Macari M, Slater W, et al: Predictors of outcome after myocardial infarction: Role of depression. Circulation 1987; 74 (supplement II): 38.
44. Dunne KB, Gingher N, Olsen L, et al: The Adult Spina Bifida Network Survey. Insights 1986; 14: 28-32.
45. Lazarus A: Multimodal Behavior Therapy. New York, Springer Verlag, 1976. 57. Fillyaw, et al. Orthopedics December, 1991.
46. Waring WP, Maynard F, Grady W et al: Influence of appropirate lower extremity orthotic management on ambulation, pain and fatigue in a postpolio population. Arch Phys Med Rehabil 1989; 70: 371-375. [PubMed Abstract]
47. Agre JC, Rodriquez AA, Sperling KB: Symptoms and clinical impressions of patients seen in a postpolio clinic. Arch Phys Med Rehabil 1989; 70: 367-370. [PubMed Abstract] 60. Peach PE, Olejnik S: Effects of treatment and noncompliance on post-polio sequelae symptoms and muscle strength. Orthopedics 1991; (this issue). [Lincolnshire Library Full Text] 61. Scheer J, Luborsky M: Culture and polio. Orthopedics 1991; (this issue). [PubMed Abstract] 62. Not present in source document.

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Post Polio Sequelae Research and Treatment in the Second Decade

Richard L Bruno

POLIOENCEPHALITIS, STRESS AND THE ETIOLOGY OF POST-POLIO SEQUELAE

 

Richard L. Bruno, Ph.D.¹, Nancy M. Frick, M.Div.², and Jesse Cohen, M.D.³

 

Orthopedics, November 1991 14; 11

 

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

 

1. Post-Polio Rehabilitation and Research Service

 

Kessler Institute for Rehabilitation East Orange, New Jersey

 

2. Harvest Center

 

Hackensack, New Jersey

 

3. Medical Imaging Center of the Oranges

 

West Orange, New Jersey

 

Address all correspondence and reprint requests to: Dr. Richard L. Bruno,

 

Kessler Institute for Rehabilitation, 240 Central Avenue,

 

East Orange, New Jersey 07018

 

ACKNOWLEDGEMENTS

 

This paper is dedicated to David Bodian, M.D. This research was supported by grants from the George Ohl Foundation. The authors grateful ly acknowledge the criticism of Dr. Robert Sapolsky and the expertise and tireless efforts of Mary Ann Solimine, R.N., M.L.S., without whom this work would not have been possible.

 

POLIOENCEPHALITIS, STRESS AND THE ETIOLOGY OF POST-POLIO

 

SEQUELAE Richard L. Bruno, Ph.D., Nancy M. Frick, M.Div., and Jesse Cohen, M.D.

 

ABSTRACT

 

Post-mortum neurohistopathology from 158 individuals who contracted polio before 1950 are reviewed that document polio virus-induced lesions in reticular formation, hypothalamic, thalamic, peptidergic and monoaminergic neurons in the brain. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present hypotheses that polioencephalitic damage 1) to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue and 2) to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. Hypotheses are also presented that the anti-metabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons is responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress. Suggestions for the treatment of Post-Polio Sequelae based on these hypotheses are also presented.

 

POLIOENCEPHALITIS, STRESS AND THE ETIOLOGY OF POST-POLIO SEQUELAE

 

Richard L. Bruno, Ph.D., Nancy M. Frick, M.Div., and Jesse Cohen, M.D.

 

“In spite of much evidence opposing such a view, there appears to be at least the popular impression that the only symptomatically important injury to the nervous system associated with poliomyelitis is the decrease in motor neuron collections” (1).

 

This statement made in 1948 is still an accurate description of the current thinking about the pathophysiology of poliomyelitis. Yet, as early as 1884, clinicians were relating the symptoms of polio not only to damage of spinal cord neurons but also to polio virus lesions in the brain (2). Today, we must look back to studies performed during the polio epidemics and look beyond the anterior horn if we are to fully understand the etiology of Post-Polio Sequelae (PPS).

 

The purpose of this paper is to 1) present neurohistopathological data collected during the polio epidemics and new radiographic findings to describe the location and extent of polio virus-induced damage in the brain, 2) use these findings in combination with data from the 1990 National Post-Polio Survey to formulate hypotheses about the etiology of post-polio fatigue, pain, muscle weakness and stress-induced symptoms and 3) suggest treatments for PPS based on these hypotheses.

 

POLIOENCEPHALITIS and CNS LESIONS

 

The most incisive and insightful studies of the supraspinal activities of the polio virus are those of David Bodian. Fifty years ago, Bodian performed histopathology on both lower primates experimentally inoculated with the polio virus and humans who came to autopsy following poliomyelitis to identify the portal of entry of the polio virus. In doing so, Bodian mapped the lesions caused by the virus from the spinal cord to the cortex. His findings revealed that the polio virus was not a ‘silver bullet’ aimed only at

 

anterior horn motor neurons, but that the virus caused widespread but consistently placed lesions throughout the central nervous system (CNS). He concluded that poliomyelitis was actually an afterthought of the polio virus, with the primary site of viral activity and neuronal lesions being the brain:

 

All the evidence available shows conclusively that every case of poliomyelitis, human or experimental, exhibits lesions of the brain. In the experimental animal this includes nonparalytic and abortive cases as well as paralytic cases. Some animals with non-paralytic poliomyelitis do not have lesions in the spinal cord but have a characteristic distribution of lesions in the brain (3).

 

It seems likely that in all cases of poliomyelitis…an encephalitis exists whether symptoms are present or not. Neuronal and inflammatory lesions may be regularly found in any susceptible center… in individuals who have never exhibited symptoms (4). As far as the pathologist is concerned, all cases of poliomyelitis are encephalitic (3).

 

Bodian and other researchers consistently documented polio virus-induced lesions in certain brain areas while noting the consistent absence of lesions in others: polioencephalitis “may be of greater or less severity (from case to case), but the distribution of lesions in susceptible centers varies but little” (5). Table 1 lists the frequency and severity of brain lesions seen in autopsies of 158 polio patients. Brain stem centers were found to be “involved in even mild cases” (3). As early as 1933, Guizetti (6) stated that “only one area is always severely altered, and that is the so-called substantia reticularis.” The substantia reticularis, or reticular formation, was found by Bodian to be “heavily peppered throughout” with lesions described as “very common and often severe” (4)(Figure 1). Other researchers also documented these frequent and severe lesions (1,7,8) with Luhan (9) describing the reticular formation as the “most uniformly involved” brain center following polio infection.

 

Moving dorsally in the brain stem, the vestibular (1,3) and cerebellar roof nuclei (5,7) were found to be the next most severely affected centers. It is important to note Bodian’s statement that in “patients who had no bulbar signs, and in whom death was due to… complications unrelated to poliomyelitis infection, we found…the only centers consistently showing severe damage were the reticular formation and the vestibular nuclei” (10). It appears that damage to these areas was a consequence of all polio infections regardless of their severity and occurred “whether (clinical) symptoms are present or not” (5).

 

Moving rostrally, the next most severely affected center was found to be the periaquiductal gray (1,3) whose neurons have more recently been identified as producing the opioid peptide enkephalin (11). Other brain stem neurons that produce neurotransmitters were also found to be damaged by the polio virus. The locus ceruleus, center of origin for noradrenergic fibers, was said by Luhan (9) to be the brain center “most hard hit” by the polio virus. Several studies found that the dopamine-producing substantia nigra was damaged (3,7,8,9) as were the serotonergic median raphe nuclei (8).

 

Hypothalamic nuclei were also found to be damaged, with “severe” lesions seen in some cases (4). Bodian (3) documented lesions in both the paraventricular nucleus and preoptic area while Luhan (9) documented hypothalamic lesions that he thought were more severe in posterior regions. Lesions were also noted in lateral, median and midline thalamic nuclei and the septal nuclei (5).

 

Cortical lesions, while frequent and severe, were limited only to the motor and pre-motor areas (4,5). It is important to note that all other cortical areas were completely unaffected by the polio virus. For example, even when directly inoculated into the occipital cortex, the polio virus was unable to infect these nonmotor neurons (12,13). The inability of the polio virus to damage other cortical regions left higher-order cognitive processes intact and allowed polio survivors to attain levels of social, educational and

 

professional achievement far higher than those of the general population (14).

 

Magnetic Resonance Imaging of Polioencephalitic Lesions

 

In an attempt to document polioencephalitic lesions in survivors of paralytic polio, magnetic resonance imaging (MRI) of the brain was performed on 12 subjects. White matter scarring characteristic of postencephalitic gliosis was seen in 92% (11) of the subjects. Similar lesions may be seen in only 1-2% of age-matched controls. Gray matter lesions in the reticular formation or lateral thalamic nuclei were seen in 17% (2) of those studied. The individual with reticular formation lesions reported the most severe fatigue in this series, fatigue that had forced her to stop working and accept Social Security disability payments. Additional data is being collected to document the relationship between the location of brain lesions and PPS fatigue. However, these preliminary data do support the histopathological findings of polioencephalitis in non-fatal paralytic polio and are evidence of polio virus-induced damage to reticular formation and thalamic neurons.

 

The relatively low incidence of detectable gray matter lesions and the high frequency of white matter lesions requires explanation. First, because of limited MRI resolution, the ability to visualize gray matter lesions is restricted to those cases in which there are clearly delineated areas of neuron destruction and gliosis greater than 1 mm. in diameter. Even the extensively damaged reticular formation was said to be “heavily peppered throughout (with lesions) rather than being predominately composed of large discrete areas of destruction” (4). Bodian (4) reported only a few cases in which lesions were seen “as large as 1 or 3 mm. in diameter.”

 

Second, not all neurons that evidence lesions in the acute and post-acute stages are destroyed by the polio virus. Bodian (15) suggests that approximately 50% of motor neurons infected by the polio virus are destroyed. Thus, because of the scattering and small size of the areas of neuronal destruction and the percentage of neurons destroyed by the polio virus, imaging of gray matter lesions with MRI may be infrequent.

 

With regard to the white matter lesions, the areas in which they were imaged contain cortico-spinal (cerebral peduncle, centrum semiovale) and thalamo-cortical radiations that have been implicated in the centrifugal spread of the polio virus (13). Lesions along these fiber tracts may result from the reaction of tissues other than gray neurons to the passage of the polio virus or the virus’ entrance into susceptible tissues.

 

For example, lymphocytic infiltrations following the acute polio infection were said to “sometimes reach massive proportions” and be “present diffusely in the tissue for two or three weeks, but persist as perivascular accumulations as late as the second month” (10). Bodian suggested that the combination of a “severe reaction to virus and local circulatory embarrassment” may be responsible for tissue damage seen in other than gray neurons following polio infection, just as it is thought to cause damage:

 

….frequently described in other viral encephalitides. It is also conceivable that in small areas an unusually high concentration of virus may produce a toxic effect apart from the ordinary pathologic effect (on gray) nerve cells. Such toxic effects of influenza virus have been described (4).

 

Thus, damage caused by the “cuffing” of blood vessels by lymphocytes, local ischemia and a tissue toxic effect of the polio virus may combine to produce the white matter lesions imaged. However, only the correlation of post-mortum histology with MRI findings will define the nature of these lesions.

 

This histological and MRI evidence of lesions indicates that the polio virus both damaged and destroyed neurons in CNS territories beyond the anterior horn. These findings allow hypotheses to be formulated about the etiology of PPS, especially those symptoms that cannot be explained by poliomyelitic-damage to the motor unit, such as fatigue and pain. Further, polioencephalitic lesions in the brain may help to explain the report of PPS symptoms by 28% of the more than 820,000 survivors of non-paralytic polio (16).

 

Polioencephalitis and Post-Polio Fatigue

 

Unaccustomed fatigue is the most commonly reported, most debilitating, least studied and most poorly understood PPS. In the 1985 National Survey of 676 polio survivors, 91% reported new or increased fatigue (17 ). Fatigue was also reported to be the most disabling of PPS, significantly interfering with social activities in 13% of respondents, with self-care in 25% and with completing or performing work in 41%. The 1990 National Survey of 373 polio survivors documented that 12% of respondents who reported new fatigue without new muscle weakness were not working or were on disability, as compared with only 2% of those reporting new muscle weakness without new fatigue.

 

Etiology of PPS Fatigue. The first study of the etiology of PPS fatigue was performed by Owen and Jones. (18) They assessed cardiopulmonary conditioning in post-polio subjects and found a “level of cardiac fitness comparable to (that in) patients after recent myocardial infarction .” They concluded that post-polio fatigue may be related to cardiopulmonary deconditioning since it is “quite similar to (fatigue) seen in deconditioned individuals with chronic pain syndrome.”

 

The subjects in this study were significantly different from the post-polio patients with fatigue treated on this service, many of whom report a severe and malignant fatigue and not just “moderate problems with fatigue and endurance” (18). Where Owen and Jones (18) subjects’ reported fatigue following “5 to 7 minutes of exercise,” our patients cannot exercise for one minute without experiencing fatigue. Further, post-polio fatigue is more than the physical ‘tiredness’ and shortness of breath that are reported by chronic pain patients. As one polio survivor described her fatigue, “I get a very drowsy feeling, as if I were drugged. When I have things to do, I get very flustered and stressed and then I can’t organize my mind to get the job done. Even when I sleep for ten hours, I never feel as if I’m rested. My brain always feels tired.”

 

Respondents to the 1990 National Post-Polio Survey reporting new fatigue also described “brain tiredness,” reporting difficulty with concentration (96%), memory (85%), attention (82%), word finding (80%), maintaining wakefulness (71%) and thinking clearly (70%). Seventy-seven percent of respondents reported moderate to severe difficulty with these cognitive functions.

 

Post-Polio Fatigue and the Reticular Activating System

 

These data suggest that post-polio fatigue is related less to physical deconditioning and more to an impairment in the brain’s ability to activate itself. This suggestion should not be surprising given the location and severity of polioencephalitic lesions in the reticular formation, posterior hypothalamus and thalamus – the brain’s Reticular Activating System (RAS)(Figure 2).

 

The reticular formation is a net-like arrangement of brain stem neurons that receive input from all sensory systems (19,20,21). Sensory stimuli enter the reticular formation and activate the thalamus, via the posterior hypothalamus, causing desynchronization of cortical neurons and ‘alerting’ the cortex to begin processing the ascending sensory information (22,23). Neurons in the locus ceruleus and substantia nigra

 

are thought to stimulate the RAS and promote cortical arousal through their respective release of norepinephrine (24) and dopamine (25,26,27).

 

It has long been known that severe damage to RAS centers can prevent cortical arousal and thereby cause irreversible coma (28). Polioencephalitic damage to the RAS following the acute polio infection was reported to cause coma, but also was associated with less severe symptoms such as “disorientation,” “drowsiness” (4) and “prolonged somnolence” (29). Meyer (30) noted that a “high percentage of children clinically recovered from poliomyelitis insofar as motor disability is concerned, reveal qualitative difficulties in mental functioning (such as) fatigability and fleeting attention” for months after the acute polio infection. She stated that these symptoms were “particularly evident” when there was acute “encephalitic involvement” and were present “to some degree (where) the medical history notes drowsiness, severe headache, and, in some instances, only nausea at the onset.”

 

These clinical observations suggest that polioencephalitic lesions impaired the ability of the RAS to maintain a state of alert wakefulness, activate the cortex and arouse the brain to effectively process information both acutely and for months after the acute polio infection. It is hypothesized that PPS fatigue, characterized by impairments in arousal, attention, cognition and memory, also results from polioencephalitic lesions in the RAS and associated brain-stem centers impairing the mechanism of cortical arousal. The emergence of PPS fatigue thirty or more years after the acute polio infection may result from age-related changes in and possibly the death of neurons that had been damaged by, but survived, the acute polio virus infection.

 

Machado-Salas, et al. (31) described reticular formation neurons in aged mice as having distorted cell bodies and a marked loss of dendritic shafts – abnormalities that the authors state resemble degenerative changes in the aging human cortex. Mann, et al. (32) documented age-related decreases in the number of locus ceruleus neurons in humans beginning at about age 35.

 

McGeer, et al. (33) documented age-related decreases in the number of substantia nigra neurons in the human brain, with a mean loss of 33% of nigral neurons by age 50. The age-related loss of substantia nigra neurons, combining with an already diminished neuron pool, is thought to be responsible for the emergence in mid-life of Parkinson’s Disease, another putatively postencephalitic disorder (34).

 

Age-related degeneration of reticular formation neurons and the attrition of locus ceruleus and substantia nigra neurons, combining with an already decreased number of neurons in these areas as a result of the original polio infection, are hypothesized to impair the RAS sufficiently to decrease cortical activation and produce symptoms of PPS fatigue as polio survivors reach mid-life (Figure 2).

 

Stress and Post-Polio Fatigue

 

Any hypothesis concerning the pathophysiology of PPS must explain the finding that emotional stress was reported to be the second leading cause of PPS (17). Sixty-one percent of respondents to the 1985 National Survey reported that emotional stress caused fatigue (17). In the 1990 National Survey, significantly more of the respondents who reported new fatigue also reported symptoms of anxiety (Table 2). Also, respondents who reported fatigue had significantly elevated Type A and Sensitivity to Criticism and Failure scores on the Reinforcement Motivation Survey (RMS) as compared to those without fatigue (35,36). Further, the severity of fatigue was significantly correlated with symptoms of anxiety, the two RMS scores and respondents’ reports that emotional stress increased PPS symptoms.

 

Emotional stress has been found to induce symptoms similar to those associated with PPS fatigue in nondisabled individuals. An emotional stressor (“dealing with difficult or helplessly ill patients”) was reported

 

to be the most frequent cause of fatigue in a survey of 1801 nurses (37). Empirically, subjects who described a sustained vigilance task as stressful reported an impaired ability to maintain attention, decreased energy, increased fatigue and drowsiness – symptoms that were correlated with the release of glucocorticoids (38). And, it is glucocorticoids that are thought to be the chemical link between emotional stress and symptoms of fatigue.

 

In humans, the administration of high-dose glucocorticoids has been shown to decrease attention, concentration and mental speed (39). Elevated glucocorticoid plasma levels have been associated with increased signal detection thresholds (40) while elevated but still physiological plasma levels of cortisol were associated with reduced cortical activation and decreased sensitivity to changing stimuli (41). Glucocorticoids are thought to produce these effects by generally inhibiting neuronal activity in the brain (40) and by specifically impairing the ability of RAS to focus attention (42) through the activation of glucocorticoid receptors on RAS neurons.

 

Glucocorticoid receptors have been demonstrated in animals on reticular formation (43), thalamic (44), locus ceruleus and substantia nigra neurons (45). Glucocorticoids have been shown in animals to specifically decrease the firing rate of reticular formation neurons (46,47) and decrease glucose utilization in locus ceruleus neurons by at least 25% (48 ). Glucocorticoids are thought to generally interfere with neuronal glucose uptake, inhibit protein synthesis and deplete neuronal energy reserves (43). Neurons that survived the original polio virus infection would be especially susceptible to disruption of their metabolism because of polio virus-induced damage to the metabolic apparatus (endoplasmic reticulum, Golginet), alterations in oxidative metabolism and protein synthesis (49) and the impairment of axonal transport of trophic factors in polio-damaged neurons (50).

 

Thus, it is hypothesized that the anti-metabolic effect of glucocorticoids secreted during stress inhibits the firing of the remaining, degenerated reticular formation neurons and remaining locus ceruleus and substantia nigra neurons. This decrease in neuronal firing inhibits the ability of the RAS to activate the cortex and thereby triggers or exacerbates symptoms of PPS fatigue. Since Type A behavior has been associated with a hyperactive and slowly recovering endocrine response to stress (51,52), the elevated Type A scores in polio survivors reporting PPS fatigue suggest that they may produce an accentuated and prolonged glucocorticoid response to stress. Such a response would amplify and prolong the antimetabolic action of glucocorticoids on CNS neurons and could explain patients’ reports that PPS fatigue often lasts for days after an emotionally stressful situation has ended.

 

Stress and Post-Polio Muscle Weakness

 

The hypothesized relationship between stress, glucocorticoids and PPS may also help to explain the report of stress-induced muscle weakness in 55% of respondents to the 1985 National Survey (17). Stress in animals has been shown to accelerate the onset of muscle fatigue (57) and augment age-related decreases in the number of terminal axon branches (58). Again, glucocorticoids may be the mediators of these stress-induced impairments. Glucocorticoid receptors have been demonstrated in animals on spinal motor neurons (59) and elevated glucocorticoid levels have been associated with the inhibition of axonal sprouting in motor neurons of aged animals with experimental motor nerve denervation (60). These effects may result from glucocorticoid-induced inhibition of glucose utilization and the impairment of neuronal metabolism.

 

Bodian suggested that post-polio motor neurons “may be vulnerable for life to metabolic factors such as changes of senescence” (15) since their metabolic apparatus was altered by the polio virus and because the axons of surviving damaged motor neurons are extensively sprouted (49). Wiechers (61) suggested that

 

PPS muscle weakness emerges as these “metabolically vulnerable” motor neurons fail to function, lose axonal sprouts and even die because they “are just not able to keep pace with the metabolic demands of innervating all of their muscle fibers.” Stress-induced muscle weakness may result from glucocorticoids further impairing metabolism and preventing already compromised neurons from activating their excessively sprouted but progressively decreasing motor unit territories.

 

Polioencephalitis and Pain

 

It was noted above that neurons in the periaquiductal gray were found by Barnhart, et al. (1) and Bodian (3) to have been damaged by the polio virus. Neurons in the substantia gelatinosa (lamina II of the spinal cord’s posterior horn) were also found by these researchers to be damaged following polio infection. Both periaquiductal gray and substantia gelatinosa neurons produce the opioid peptide enkephalin (11).

 

Polio virus-induced destruction of or damage to these neurons may explain the finding that polio subjects were approximately twice as sensitive to experimentally-induced pain as were non-disabled controls (Figure 3)(53). It is hypothesized that increased pain sensitivity in polio survivors may be the result of decreased production of enkephalins by a reduced number of polio-damaged enkephalinergic neurons in both spinal cord and brain.

 

Increased pain sensitivity is reported clinically by polio survivors and must be acknowledged by clinicians when administering therapies that are painful (e.g., stretching) and when treating acute pain. A number of patients have reported that they are not believed when describing their heightened sensitivity to pain, and have been refused adequate medication for acute (e.g., post-operative, post-traumatic) pain.

 

Conclusion

 

The ability of the polio virus to produce symptoms by its destruction of neurons outside of the anterior horn has been accepted for more than 100 years. It is only our recent experience with PPS that has forced us to recognize that both the people who survived the original viral onslaught and their central nervous systems have been operating for decades under extreme stress. This stress now may be combining with the aging of an extensively damaged but here-to-fore remarkably functional central nervous system to reveal the previously hidden symptoms of polioencephalitis.

 

The above-presented hypotheses concerning the etiology of PPS fatigue suggest that reductions in both emotional and physical stress will reduce PPS. This is the experience of post-polio clinics throughout the world (36,54,55,56). As PPS fatigue continues to be treated with stress-management, work simplification and energy conservation (see 36,56), the hypotheses are being tested by studying the neuroanatomy, neuroendocrinology and neuropsychology of PPS fatigue. In addition, pharmacological means for stimulating the RAS that do not further metabolically stress its remaining aging, polio-damaged neurons are being tested.

 

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Tables

 

Table 1. [below]

 

Polio virus lesions in the brain ranked in order of descending fequency and severity¹

 

Reticular formation1,3,5,7-9

Vestibular nuclei1,3

Cerebellar roof nuclei3,5,7

Periaquiductal gray1,3,5,8

Hypothalamic nuclei1,3,5,9

Substantia nigra3,7-9

Thalamic nuclei5,8

Preoptic area3,5

Locus ceruleus9

Median raphe nuclei8

¹ on the basis of 158 human autopsies

Table 2. [below]

 

Measures of stress, new symptoms, polio severity, and their correlation with fatigue severity rating¹

 

Correlation

 

With

 

Fatigue

 

Severity

 

Respendents

 

With

 

Fatigue

 

Respendents

 

Without

 

Fatigue

 

Emotional stress increases PPS symptoms .43* 82%§ 45%

 

Frequent feelings of anxiety .36* 74%§ 46%

 

Diagnosed as having depression .33* 28% 23%

 

Breathing problems today .29* 44% 39%

 

Frequent difficulty falling asleep because “mind is racing” .27* 69%§ 41%

 

Mean Sensitivity to Criticism and Failure Score .24** 70¥ 52

 

Mean Type A Score .20** 62¥ 51

 

Mean number of limbs paralyzed by polio .14 2.3 2.1

 

Breathing problems with acute polio .14 37% 40%

 

Hospitalized with acute polio .10 79% 77%

 

¹ From the 1990 National Post-Polio Survey. See references 17 and 35 for survey methods.

 

* Cramer’s V significant at P<.001: ** Pearson product-moment correlation significant at P<.01: § Number of respondents reporting symptom (Chi squared, P<.0001) significantly elevated compared to respondents not reporting fatigue (n = 230). ¥ Mean score (independent groups t-test, P<.001).

 

Figures

 

Figure 1. [below]

 

Brain areas lesioned by the polio virus as seen in 158 human autopsies. Severe lesions: Reticular formation (RF), vestibular nuclei (V), cerebellar roof nuclei (R), periaquiductal gray (PG). Moderate lesions: Posterior hypothalamic nuclei (P), paraventricular hypothalamic nucleus (PV), substantia nigra (SN). Mild lesions: Thalamic nuclei (T), preoptic hypothalamic nuclei (PO), locus ceruleus (LC), median raphe nuclei (MR) .

 

Figure 2. [below]

 

Polio-virus lesions within the reticular activating system (arrows): Thalamic nuclei (T), posterior hypothalamus (P), substantia nigra (S), reticular formation (RF), and locus ceruleus (LC).

 

Figure 3. [below]

 

Linear regression of subjective rating of stimulus intensity on applied stimulating current as a

 

measure of pain sensitivity in post-polio and non-disabled control subjects (see 51).

 

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Polioencephalitis Explaining Post Polio Fatigue, Type A Behavior or Pain

Richard L Bruno

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]

 

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