Bruno – Page 2 – Polio Survivors Network

Ultimate Burnout Post-Polio Basics

Ultimate Burnout: Post-Polio Sequelae Basics

Dr. Richard Louis Bruno

New Mobility, 1996; 7: 50-59

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

It’s 8:00 p.m. and only one light is burning at corporate headquarters. Mr. Gray, MBA, the 55-year-old CEO, is awakened by the phone. He lifts his head from the desk to answer and hears his wife asking when he’s coming home. Feeling as if he weighs a ton, his muscles burning, Mr. Gray wheels himself to the car and, with barely enough strength to pull his chair in behind him, drives home. He greets his kids, rolls into the bedroom, and throws himself on the bed. It’s the third night this week he has gone to bed without dinner and slept in his clothes.

The alarm rings at 5 a.m. A clean suit and a cup of coffee later, Mr. Gray is on the road again. Driving to work, he thinks he hears the same faint “sizzling” sound he hears every morning–a sound like electricity arcing between two frayed wires. But on this and every other morning, he ignores the sizzling, ignores his increasing fatigue and muscle weakness and pain. He has a corporation to run. There’s no time to “give in” to his body. But he has to wonder: “What is that sizzling sound?”

Well, Mr. Gray, that metaphoric sizzling is the sound of your nerves burning out. Nearly 80 percent of America’s 1.63 million polio survivors hear the same sound as they, too, experience Post-Polio Sequelae (PPS). PPS are the unexpected and often disabling symptoms–new fatigue, muscle weakness, joint pain, cold intolerance–that occur about 40 years after the original bout with polio. Unfortunately, most survivors of the polio epidemics that terrorized America during the middle of this century are just like Mr. Gray: hard working, time-conscious, perfectionistic super-achievers who, as a way of life, push themselves beyond their physical limits. Like him, they ignore the sizzling sound, refuse to give up control, refuse to “give in” to new fatigue and weakness and pain even though their bodies are begging them to stop.

Post-Polio Past

Why won’t polio survivors listen to their bodies and slow down? When you understand what it was like to

have had polio, you can appreciate why they feel that “giving in” to PPS is the same as giving up their lives. “During the epidemics,” says researcher Nancy Frick, a polio survivor herself, “polio was America’s summer terror. The adorable March of Dimes poster children–clad in metal braces, leaning on their crutches–were everywhere saying, ‘Give money to find the polio vaccine. Don’t let your child become crippled like me.'”

When the polio vaccine arrived in 1954, the poster kids were needed no more. Their braces, crutches and wheelchairs were evidence of a horror that Americans wanted to forget. “So polio was eagerly forgotten by everyone,” Frick says, “including those disabled by it. To be accepted back into society, polio survivors had to look ‘normal’ again. And since buildings were totally inaccessible, even paraplegic polio survivors had to be able to walk if they wanted to go to school or get a job.”

Polio survivors were ripped away from their families for months or years and admitted to rehabilitation hospitals where they underwent multiple surgeries and draconian physical therapy. Many were verbally abused, slapped or even beaten with rubber truncheons by therapists or family members to “motivate” them to walk. “Is it any wonder that polio survivors discarded their braces and crutches when they came home from the hospital,” asks Frick. “They were eager to do anything to look ‘normal’ and stop the abuse.”

Unfortunately, polio survivors went beyond mere normalcy. Two surveys show that, regardless of the severity of their paralysis, more polio survivors marry and go to college than nondisabled Americans. They work more hours of overtime and take fewer sick days. They became the leaders of their communities and the chief executives of the world’s largest corporations. That’s why their nerves have started to sizzle.

Post-Polio Present

PPS are not caused by the return of the poliovirus that’s been hiding in the body for decades. Nor are they the result of some new disease. The simplest explanation is that polio-damaged neurons are mad as hell and are not going to take it anymore.

When polio struck, large numbers of neurons in the brain stem were damaged by the poliovirus. For those who had paralysis, the poliovirus damaged 90 percent of the neurons in the spinal cord that run the muscles; nearly half of those neurons died. The surviving but damaged neurons are less able to manufacture acetylcholine, the chemical that neurons release to make muscles contract. Yet the damaged neurons sent out sprouts–like extra telephone wires–to turn on muscle fibers orphaned when their motor neurons were killed by the poliovirus. This sprouting allowed many people who were totally paralyzed during the initial polio attack to actually walk out of the hospital some months later.

But since these polio-damaged and over-sprouted motor neurons have been doing double and triple duty for 40 years, even polio survivors with minimal loss of function have been heading for a fall. “Sprouted motor neurons can no longer make muscles contract as forcefully as they once did, or for as long as they once could, because their damaged internal chemical factory is failing after years of overworking,” says Kessler Institute for Rehabilitation physiatrist Jerald Zimmerman. “Muscles become weaker and hurt as they try to do more work than they are able to do. And because weakened muscles can no longer support the joints, polio survivors’ shoulders and elbows ache and their knees start to bend backward.” Standing,

walking, lifting, pushing a wheelchair–sometimes swallowing and breathing, especially in those who had respiratory polio–become more difficult and more painful.

While new muscle weakness and pain are reported by more than 75 percent of polio survivors, the most common PPS symptom is fatigue. Fully 91 percent of polio survivors report two kinds: “body fatigue” and

“brain fatigue .” Body fatigue is the feeling that you weigh a ton and that the longer you use your muscles, the more they lose strength. “Because of new muscle weakness, many polio survivors expend as much energy just getting up and dressed and out to work in the morning as does a marathon runner,” says Lauro Halstead, Post-Polio Program director at Washington’s National Rehabilitation Hospital. “Why wouldn’t they feel fatigued?”

Brain fatigue is the feeling that you can’t focus your attention, can’t concentrate on what you’re reading and can’t stay awake as the day goes on. Researchers at Kessler Institute have recently found that polio survivors with brain fatigue do in fact have significantly impaired attention and are not able to release the brain-stimulating hormone ACTH in response to stress. MRI scans of fatigued polio survivors have found damage in the brain areas that control wakefulness and attention, the same areas that were damaged by the original poliovirus infection.

Post-Polio Future

With the coming of PPS, the future might sound bleak for polio survivors. Both the March of Dimes Birth Defects Foundation and the U.S. Congress have refused to set aside funds for PPS research. “In spite of this, much has been learned about the cause and treatment of PPS,” says Paul Peach, medical director of Georgia’s Roosevelt Warm Springs Institute for Rehabilitation. “The future is much more optimistic than had been expected.” Two recent studies have identified drugs that may decrease PPS fatigue. Neil Cashman of the Montreal Neurological Institute found that Mestinon, a drug that prevents the breakdown of acetylcholine in the muscle, may reduce body fatigue in some polio survivors. A just-completed study from Kessler Institute found that Parlodel, an anti-Parkinson’s disease drug, helped to increase attention and wakefulness and reduce post-polio brain fatigue.

But Peach warns that using drugs to treat PPS may be dangerous. “Weakness and fatigue are the body’s way of telling polio survivors to rest,” he says, “like a fuse that blows when their nerves are overworked.” Peach worries that using these drugs will be like putting a penny in the fuse box , allowing polio survivors to do even more harm to polio-damaged neurons.

But, neither Mestinon nor Parlodel is effective if polio survivors don’t follow The Golden Rule for the treatment of PPS: “If what you do causes pain, fatigue or weakness, don’t do it!”

The Golden Rule does not mean that polio survivors should sit home and be come couch potatoes. The Golden Rule does mean that polio survivors should stop exhausting themselves, especially with regard to exercise. Many survivors are prescribed–or actually ask for–the kind of physical therapy they had right after polio: exercising to the point of exhaustion. But several studies show that pumping iron will not increase the strength of muscles that are becoming weaker, and can actually cause an irreversible loss of strength. No free weights, exercise bikes, tread mills, NordicTracs or Thighmasters for polio survivors. “Feeling the burn” means nerves are burning out.

Stretching, however, helps decrease pain and increase range of motion. And gentle, non-fatiguing exercise can be useful for some survivors to help maintain muscle strength and tone, especially after getting a new brace.

Many studies show that changing survivors’ physically and emotionally stressful lifestyle is the best way to reduce weakness, fatigue and pain. On[e study] by James Agree at the University of Wisconsin shows that polio survivors who pace activity–that is work and then rest for an equal amount of time–can do 240 percent more work than if they push straight through the task. Another study shows that survivors use three times less energy in walking if they use a short brace on a weakened leg. And the follow-up study of

Kessler’s six-week program of behavior modification and physical and occupational therapy shows that polio survivors who comply with treatment –pace activities, conserve energy, take two 15-minute rest breaks a day, and use assistive devices (a brace, cane, crutches, wheelchair or scooter)–have up to 22 percent less pain, weakness and fatigue 18 months after therapy ends. But survivors who quit therapy have 21 percent more fatigue and 76 percent more weakness 18 months after leaving the program.

And there’s the rub. It’s only the refusal to listen to that sizzling sound that causes PPS to continue or progress. That’s why behavior modification and psychotherapy are required to help survivors accept their second bout with polio, change their super-achieving lifestyles and deal with the fear of looking disabled. “There will be no magic pill to cure PPS,” says Paul Peach. “But by making lifestyle changes and eliminating ‘overuse abuse,’ progressive fatigue, weakness and pain can be substantially reduced or eliminated.”

As Mr. Gray knows well, this whole PPS business stinks. But isn’t it far better to accommodate to PPS now than to be forced to give up your entire life because you are too weak and fatigued to function? I know, you’ll slow down and take care of yourself “when you’re ready.” You’ll use a wheelchair “when

there’s no other choice.” Well, you don’t drive your car until it’s out of gas. Why drive your body until it’s out of neurons? Isn’t it time to listen to the sizzling sound? Isn’t it time to take care of yourself? To paraphrase one famous paraplegic polio survivor who refused to take care of himself, “You have nothing to fear but fear itself.”

Red Flags for Polio Survivors

[Mis]Diagnosis: Many polio survivors have been incorrectly told they cannot have PPS because they didn’t have paralytic polio. The National Institute of Health has just studied pairs of identical twins, one who had paralytic polio and one who had non-paralytic polio. Seventy-one percent of those who had paralytic polio have PPS. The surprise is that 42 percent of those who had non-paralytic polio also have PPS. The lesson: Don’t let a doctor tell you it’s all in your head just because you didn’t have paralytic polio.

Anesthesia: Polio survivors are anesthetized very easily because the part of the brain that keeps you awake was damaged by the poliovirus. They also stay anesthetized longer and can have significant complications from anesthesia. Even nerve blocks with local anesthetics can cause problems.

All polio survivors should have lung function tests before having a general anesthetic. “Polio survivors with a lung capacity below 70 percent may need a respirator or respiratory therapy after surgery,” says Augusta Alba of New York’s Goldwater Memorial Hospital. Your complete polio history and any new problems with breathing, coughing or swallowing should be brought to the attention of your surgeon or dentist–and especially your anesthesiologist–long before you go under the knife. Also, polio survivors should not have same-day surgery.

Pain: Two studies have shown that polio survivors are twice as sensitive to pain as everyone else and need more pain medication for a longer time after surgery or an injury. However, survivors have had to develop an extremely high pain tolerance to deal with their increased pain sensitivity and are not likely to become addicted to pain medications.

Sleep: The majority of polio survivors have trouble sleeping due to pain, anxiety or sleep disorders, such as sleep apnea or generalized random myoclonus. GRM occurs when muscles in different parts of the body twitch and jump throughout the night. Amazingly, most polio survivors aren’t aware that they stop breathing or twitch. If you awaken with a headache, with your heart pounding, short of breath or don’t feel

rested in the morning, call a local sleep disorders center for a sleep study.

Resources

Orthopedics, November and December, 1991.

These special issues provide all the information needed to start a PPS clinic. A must read for anyone who treats polio survivors.

Harvest Center, Inc., 151 Prospect Ave., Hackensack, NJ 07601; (201)342-6777. Provides the 1995 Audio Symposium for PPS, four audio taped lectures on the cause and treatment of PPS and the psychology of polio. Also available is the Computerized Assessment Program for PPS. After a brief questionnaire, the computer suggests possible causes, tests and therapies for new symptoms and lists the world’s medical literature on PPS.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

The Secretary, Lincolnshire Post-Polio Network

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

Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk

Web Site: www.lincolnshirepostpolio.org.uk

The Lincolnshire Post-Polio Network takes great care in the transcription of all information that appears at this site. However, we do not accept liability for any damage resulting directly or otherwise from any errors introduced in the transcription. Neither do we accept liability for any damage resulting directly or otherwise from the information available at this site. The opinions expressed in the documents available at this site are those of the individual authors and do not necessarily constitute endorsement or approval by the Lincolnshire Post-Polio Network.

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/burnout.html>

Created: January 1997

Last modification: 29th January 2010.

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Fainting and Fatigue Causation or Coincidence

FAINTING AND FATIGUE: Causation or Coincidence Richard L. Bruno, Ph.D.

Bruno RL. Fainting and Fatigue: Causation or Coincidence? CFIDS Chronicle, 1996; 9(2): 37-39.

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

Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation,

Saddle Brook, New Jersey USA

As the former autonomic nervous system fellow at New York’s Columbia-Presbyterian Medical Center, and in my current incarnation studying chronic fatigue in polio survivors, I have read with special interest the reports from Johns Hopkins University describing neurally mediated hypotension (NMH) in adults and adolescents with CFIDS.[1] In June 1995, we presented a paper to the American Congress of Rehabilitation Medicine describing several of our post-polio patients who have had episodes of vasovagal syncope.[2] One patient with a 10-year history of severe, chronic and disabling post-polio fatigue had a history of frequent fainting 35 years before she ever experienced fatigue.

The 1995 North American Post-Polio Survey

Because of fainting episodes in our patients, we included questions about fainting and late-onset fatigue in the 1995 North American Survey of Polio Survivors conducted last Fall. We have recently analyzed data from the first 1,000 respondents to the survey. As was seen in our two previous National Post-Polio Surveys, chronic fatigue was the most frequent symptom in polio survivors; the daily fatigue rating was reported as “moderate” or higher in 76 percent of the 665 polio survivors, versus 29 percent of the 406 non-disabled controls.

An equal number of polio survivors and controls (44%) reported at least one fainting episode during their lifetimes. The causes of fainting – from physical trauma to emotional stress – were identical in polio survivors and controls. However, the occurrence of even one fainting episode was significantly related to having a daily fatigue rating of moderate or greater in both polio survivors (X2=7.46; p=.0063) and in

controls (X2=7.74; p=.005). Polio survivors who passed out at least twice in their lives had a 14 percent higher daily fatigue rating (p=0.0014) and controls had a 31 percent higher daily fatigue rating (p=0.019) as compared to those who never had fainted. These finding suggest that there may be a relationship between fainting and fatigue in polio survivors and the general population, as well as in people with CFIDS.

Physiology of Fainting and NMH

The question raised by our data and those from Johns Hopkins is: why do fainting, NMH and fatigue appear to go together? The answer may lie in the physiological mechanisms that regulate blood pressure and actually trigger NMH.

NMH has been said to result from a “miscommunication between the heart and the brain which causes blood pressure to lower when it should rise.”[3] Actually, NMH is a survival mechanism, hard-wired into the brain, that turns on when the autonomic nervous system (ANS) is unable to stop blood pressure (BP) from falling. If BP falls for any reason (e.g., loss of blood from a cut artery) pressure detectors in the carotid arteries of the neck (the baroreceptors)stop firing.[4] (Figure 1) The silence of the baroreceptors is “heard” by BP regulation centers in the solitary tract and dorsal vagal nuclei, which release the brake on heart rate that is normally provided by the cardiodepressor center of the reticular formation. The BP regulation centers also trigger the release of vasopressin and norepinephrine, neurochemicals that constrict blood vessels and make the heart beat harder and faster, and should help to increase BP.

However, if these measures cannot stop BP from falling, the amount of blood returning to the heart will decrease. If the heart continues beating hard and fast even though it is nearly empty, the heart muscle will be damaged. Stretch receptors in the heart detect that it is “beating on empty,” and send a signal to the dorsal vagal nucleus to protect the heart by stopping its beating for a few seconds. With the heart stopped, BP falls to zero and causes the faint of NMH.

But, even fainting has a protective function (if you don’t knock yourself out falling to the ground). Lying flat, your brain will be at the same level as your heart. Without gravity pulling blood toward your feet, the heart will have an easier time increasing BP, pumping your remaining blood to your brain and (hopefully) waking you up again.

A Physiological Link Between Fainting and Fatigue.

If we assume that the heart itself is normal, there must be something wrong with the BP regulation centers in the brainstem that causes fainting in our post-polio patients and NMH in people with CFIDS. In the case of polio survivors, damage done by the poliovirus to the brain suggests that fainting should be expected.

The poliovirus frequently and severely damaged the brainstem, especially the reticular formation (RF).[5] The RF contains the cardiodepressor center that is responsible for “putting the brake” on heart rate. Of those with acute “bulbar” polio (“bulbar” meaning associated with the brainstem) 73 percent had hypertension and rapid heart rate and five percent died of cardiovascular collapse.[6]

Importantly, the RF is not only responsible for heart regulation, but also brain activation, keeping us awake and allowing us to focus attention. Using MRI of the brain, we have found lesions of the RF and its connections to the cortex, as well as clinically impaired attention on neuropsychological tests, in polio survivors with chronic fatigue.[7] Recently, Costa, et al. found that decreased perfusion of the brainstem

on SPECT was the only physiological finding that differentiated people with CFIDS from patients with depression, neurological disease and controls.[8] These data support our hypothesis that damage to the brainstem “reticular activating system” may be a cause of chronic fatigue in polio survivors and in patients with post-viral fatigue.[7]

Outside of the RF, but in the same area of the brainstem, lie the other cardiovascular control centers. The dorsal vagal nucleus, responsible for activating the stomach and slowing the heart, was damaged in 55 percent of those who had bulbar polio, as were the vestibular nuclei. Nausea, reported by 70 percent of those who have NMH, was seen in 27 percent of those with acute polio and attributed to poliovirus lesions in the vestibular nuclei.[1,5,6]

Also outside the RF but damaged by the poliovirus, were the nucleus ambiguus and solitary tract nuclei, the main centers for blood pressure regulation. These nuclei “communicate” with the heart via the vagus nerve, regulating blood pressure by sensing and altering the force and rate of the heart’s contraction.[4]

The Chemistry of Fatigue, Fainting and NMH.

Recent research has also found chemical abnormalities that may be related to fatigue and fainting in polio survivors and CFIDS and NMH. We found a marked blunting of adrenocorticotropic hormone (ACTH) release in polio survivors that was correlated with daily fatigue severity.[7] The secretion of corticotropin releasing hormone (CRH), which stimulates ACTH release, had already been found to be decreased in patients with CFIDS.[9] CRH is secreted by the paraventricular nucleus (PVN) of the hypothalamus, which is known to be damaged by the poliovirus.[5] Both ACTH and CRH are brain activating hormones, and their decreased secretion could cause symptoms of fatigue.[7]

The PVN of the hypothalamus also produces the vasoconstrictor neurochemical vasopressin, the secretion of which is also impaired in people with CFIDS.[10] Thus, the PVN may be damaged in polio survivors and people with CFIDS, causing impaired brain activation and faulty BP regulation in both groups.

The Coincidence of Fainting and Fatigue

Taken together, these findings suggest that polio survivors may be predisposed to fainting because of poliovirus damage to their brainstem cardiodepressor and blood pressure regulation centers and PVN. However, it is important to note that many viruses besides polioviruses (e.g., the Coxsackie viruses) are also known to frequently and preferentially damage the brainstem, especially the reticular formation. [7,11] People with post-viral fatigue may have similar brainstem and hypothalamic damage as is seen following poliovirus infection, as Costa’s finding of decreased brainstem perfusion in CFIDS suggests. Thus, damage to cardioregulatory centers could be responsible for NMH in people with CFIDS. But what of the coincidence of fainting and fatigue?

Since the cardioregulatory centers are either part of or lie close to the reticular activating system, it would be surprising if fainting and fatigue did not occur together. Damage to the RF alone could both decrease brain activation and impair BP regulation. The question that remains is whether NMH is the “cause” of chronic fatigue or if it is just coincidental in those who have chronic fatigue. At this early stage in the research, the answer to both of these questions appears to be “yes.”

The Johns Hopkins team reported that 47 percent of their adult CIFDS patients and 57 percent of their adolescent patients with NMH had “complete or near complete resolution of all symptoms” after receiving blood pressure elevating therapies. Could the resolution of fatigue symptoms in CFIDS patients whose

NMH was treated have resulted from elevated BP increasing brainstem perfusion, thereby enhancing the functioning of the reticular activating system, or by elevated BP directly increasing perfusion of the brain? SPECT scans in people with CFIDS whose symptoms disappear when their BP is increased could answer these question.

But what of the nearly 50 percent of patients whose CFIDS symptoms did not respond or were only “somewhat better” after NMH treatment? Could NMH in these individuals just be a coincidence of generalized damage to the brainstem, including the BP regulation centers? Could fatigue symptoms in these individuals actually result from damage to the brainstem’s reticular activating system, or the hypothalamus or the immune system? Only blinded, placebo-controlled trials of treatments for NMH, combined with SPECT scans of the brainstem, measurement of CRH, norepinephrine, and vasopressin, and assessment of the individual blood pressure and heart rate regulation pathways to and from the brain stem will provide answers to these important questions.

Acknowledgements:

Many thanks to Rebecca Moore for her stimulating questions and suggestions.

REFERENCES

1. Bou-Holaigah, et al.: The relationship between neurally mediated hypotension and the chronic

fatigue syndrome. JAMA 1995;274:961-967. [PubMed Abstract]

2. Bruno RL, Frick NM: Parasympathetic abnormalities as post-polio sequelae. Arch Phys Med

Rehab 1995;76:594.

3. Carpman V: News, knowledge & networking. CFIDS Chron 1995;8(4):22.

4. van Lieshout JJ, et al.: The vasovagal response. Clin Sci 1988;81:575-586. [PubMed Abstract]

5. Bodian D: Histopathological basis of clinical findings in poliomyelitis. Am J Med 1949;6:563-578.

6. Baker AB: Neurologic signs of bulbar poliomyelitis. Poliomyelitis Philadelphia: Lippincott, 1949.

7. Bruno RL, et al.: The pathophysiology of a central cause of post-polio fatigue. Ann NY Acad Sci

1995;753:257-275. [Lincolnshire Library Full Text]

8. Costa DC, et al.: Brainstem perfusion is impaired in chronic fatigue syndrome. Q J Med

1995;88:767-773. [PubMed Abstract]

9. Demitrack MA, et al.: Evidence for impaired activation of the HPA axis in patients with chronic

fatigue syndrome. J Clin Endocrin Metab 1991;73:1124-1234. [PubMed Abstract]

10. Bakheit A, et al.: Abnormal arginine-vasopressin secretion and water metabolism in patients with

postviral fatigue syndrome. Acta Neurol Scand 1993;87:234-238. [PubMed Abstract]

11. Bruno, et al.: The physiology of post-polio fatigue: A model for post-viral fatigue syndromes and a

brain fatigue generator. CFIDS Chron1994;7(4):36-42.

Figure 1.

Brain stem centers damaged by the poliovirus involved in brain activation and blood pressure and heart rate regulation.

Figure not available

Figure 2.

Brain areas damaged by the poliovirus involved in brain activation and blood pressure and heart rate regulation. PVN: Paraventricular nucleus of the hypothalamus; ACTH: Adrenocorticotropic hormone produced by the pituitary; CRH: Corticotropin releasing hormone, produced by PVN, that

releases ACTH; VP: Vasopressin, produced by PVN, and released from the pituitary.

Figure not available

Figure 3.

Possible mechanisms of fatigue symptom generation. Damaged reticular formation neurons may decrease reticular activating system (RAS) stimulation of the brain, in combination with reduced CRH and ACTH secretion. Damaged blood pressure regulation centers, in combination with reduced vasopressin secretion, may produce NMH, decrease blood pressure, and reduce the perfusion of the RAS and brain, resulting in decreased brain activation

Figure not available

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

The Secretary, Lincolnshire Post-Polio Network

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

Facsimile: +44 (0)870 1600840

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

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/faint.html>

Created: 20th January 1997

Last modification: 29th January 2010.

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Polio Cold Feet

Polio Feet

There’s a reason you have cold feet – but you can keep warm and stay cool

Richard Bruno, Ph.D.

New Mobility, March 1996

The process that cause “Polio Feet” to turn blue and cold and become difficult to move when it’s only cool is the same process that caused paralysis after the original polio.

The Polio virus got into the spinal chord and either destroyed or damaged the anterior horn cell motor neurons that transmit the message to move from the brain to a muscle. When those neurons were damaged, or especially when they died, they disintergrated and the muscle fibers that used to be turned on by those cells no longer were.

There is another kind of motor neuron that was affected by the virus – the motor nerve that controls the muscle around your blood vessels. When these muscles died, there were no motor nerves to tell the blood vessel to contract; if the blood vessel cannot contract, blood ‘pools,’ especially in the veins. When the blood pools in the veins, it is going to be blue, because venous blood is not oxygenated.

Polio feet are caused by warm blood that should be in the center of your body, flowing out into the hands, arms, and especially the legs (since gravity is pulling the blood down). The warm blood pools in the surface of your skin, and because the blood vessels cannot contract, the result is “polio feet.” The venous pooling causes your blood to radiate heat into the environment. People who had polio keep the world warm, unfortunately at their own expense. The price of this is a thorough cooling of the limbs and all tissues of the limbs.

When heat leaves the veins, the motor nerves that lie near the surface of the skin start to cool. The muscles that lies just a bit below the surface starts to cool. The connective tissue that connects muscle to muscle, and muscle to bone starts to cool and stops being elastic so it is harder for it to move.

When the motor nerves aren’t functioning well, the muscles aren’t going to function well; if the muscles don’t function well, there is going to be muscle weakness. We think that muscle weakness and the loss of body heat are causing fatigue; and we think that people who lose all their body heat into the environment

are burning calories to maintain their body temperature, so there are fewer calories to keep moving.

People who had polio should dress as if it is 20 degrees colder than it actually is, but you should dress in layers so you can control your body temperature and not pass out from a rapid flow of blood away from your head as your arteries warm

The bottom line is to keep warm, stay cool and:

1. Use polypropylene socks and underwear by Gortex Thinsulate.

2. Dress in layers.

3. Never wear a skirt after Labor Day (first Monday in September) or before Memorial Day (last

Monday in May).

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to The Secretary, Lincolnshire Post-Polio Network PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601 Facsimile: +44 (0)870 1600840 Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/kessler/feet.html> Created: 20th February 1997

Last modification: 30th January 2010.

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Cause and Treatment of Post-Polio Fatigue

THE CAUSE AND TREATMENT OF POST-POLIO FATIGUE

Richard L. Bruno, Ph.D., Nancy M. Frick, Lh.D., Susan J. Creange, M.A., Todd Lewis, Ph.D., and Terry Molzen, M.S.

From: Healthy Partnerships. Ontario: March of Dimes, 1995

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

Fatigue is the most commonly reported, most debilitating and least studied Post-Polio Sequelae (PPS) affecting the nearly 2 million North American polio survivors. Among polio survivors, 91% reported new or in creased fatigue, 41% reported fatigue significantly interfering with performing or completing work and 25% reported fatigue interfering with self-care activities (1,2). Fatigue was reported to be triggered or increased by physical overexertion in 92% and by emotional stress in 61%. Importantly, polio survivors distinguish between the physical tiredness and decreased endurance they associate with new muscles weakness, and a ‘brain fatigue’ that is characterized by problems with attention and thinking. Between 70% and 96% of polio survivors reporting fatigue complained of problems with concentration, memory, attention, word-finding, maintaining wakefulness and thinking clearly, with 77% percent reporting moderate to severe difficulty with these functions (3).

Problems with attention, memory and thinking suggest that the symptoms of post-polio fatigue cannot be explained merely by the poliovirus damaging anterior horn motor neurons (4). Autopsies performed fifty years ago on people who died after having had polio, whether they had paralysis or not, showed that the poliovirus almost always damaged specific areas in the brain (Figure 1). These damaged areas include the brain’s activating system that keeps you awake and allows you to focus your attention. The poliovirus also damaged neurons that produce neurotransmitters, including the enkephalins and endorphins (called the “body’s own morphine”) as well as dopamine and ACTH which activate the brain.

With poliovirus damaging the brain’s activating system, you would expect that the original polio infection should cause brain activating problems. And, reports written during the polio epidemics did describe “drowsiness,” lethargy, prolonged sleeping and even coma during the acute polio infection (7,12,21,22). One-third of patients with acute spinal, spinal and bulbar and even non-paralytic polio showed “disorientation, apathy, pronounced sleep disorder (and) irritability” (4). These mental changes were associated with the abnormal slowing of brain wave activity on the electroencephalogram (EEG). Further,

a high percentage of children clinically recovered from poliomyelitis insofar as motor disability is concerned, had qualitative difficulties in mental functioning such as “fatiguability and fleeting attention” for months after the acute polio (5).

These reports of persistent drowsiness, fatigue and fleeting attention following the acute poliovirus infection are similar to polio survivors’ recent complaints of late-onset fatigue and impaired attention (25). And, both acute and late-onset post-polio fatigue are reminiscent of nearly two dozen outbreaks during this century of post-viral fatigue syndromes (PFS) that are related clinically, historically or anatomically to poliovirus infections (26-28). These relationships and recent studies comparing post-polio fatigue and chronic fatigue syndrome will be described in an attempt to understand the cause and treatment of postpolio fatigue.

CAN THE POLIOVIRUS CAUSE FATIGUE?

Type II Poliovirus and Decreased Brain Activation. During the polio epidemics of the 1950’s, there were several small outbreaks of patients having drowsiness, prolonged sleeping, slowing of brain waves, as well as some of the symptoms of both bulbar polio and Parkinson’s disease (e.g., tremor and rigidity) (29- 30). In 1952, Type II poliovirus was isolated from one group of patients having these symptoms and it was found that the neurons in their brain activating system had been damaged.

The association of decreased brain activation and Parkinson’s disease symptoms remind Dr. Oliver Sacks of the “sleeping sickness” patients with Parkinson’s disease he described in his book Awakenings. The relationship between “sleeping sickness,” Parkinson’s disease and polio may be important for understanding post-polio fatigue, since all of these have conditions are associated with damage to a part of the brain activating system called the basal ganglia. For example, Parkinson’s disease (PD) patients have severe damage to one of the basal ganglia, the substantia nigra (sub-STAN-sha NYE-gra), which produces the neurotransmitter dopamine (doe-PAH-mean). PD patients often describe fatigue. “Excessive fatigue” was reported by 48% of PD patients in one study (40) while nearly one-third of PD patients reported that fatigue was their “most disabling symptom” (39). As a matter of fact, one of the first descriptions of Parkinson’ s disease (41) could serve as a definition of post-polio fatigue, i.e., a syndrome “characterized by a diminution of voluntary attention, spontaneous interest, initiative and the capacity for effort and work, with significant and objective fatiguability, and a slight diminution of memory” (38).

FIGURE UNAVAILABLE

Figure 1.

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: Paraventricular hypothalamic nucleus (PV); posterior hypothalamic nuclei (P); substantia nigra (SN). Mild lesions: Globus pallidus and putamen (GP); locus ceruleus (LC); median raphe nuclei (MR); preoptic hypothalamic nuclei (PO); thalamic nuclei (T).

“Atypical Poliomyelitis” and Chronic Fatigue. Beginning in Los Angeles in 1934 and continuing for more than twenty years, there were over a dozen outbreaks of a disease that was at first thought to be poliomyelitis, was then called “abortive” or “atypical” poliomyelitis and finally named “Myalgic Encephalomyelitis” (ME) (6). Like poliomyelitis, initial symptoms of ME included headache, neck pain, low-grade fever and muscle pain that were often followed by muscle weakness. Patients were excessively sleepy and had “conspicuous changes in their levels of concentration” that lasted for months after the initial illness. Slowing of the EEG similar to that seen in acute polio was also noted.

Unlike poliomyelitis, there were frequent complaints of numbness or tingling, usually no breathing problems, paralysis or muscle wasting and almost invariably no deaths. Also unlike poliomyelitis, recovery from the initial symptoms of ME sometimes required months with most patients being left with a marked “exhaustion and fatiguability” that were “always made worse by exercise (and) emotional stress.” Patients continued to have fatigue, excessive sleepiness, trouble concentrating, difficulty with word finding, memory and thinking for years after the acute episode.

Despite the differences between poliomyelitis and ME, an association with the poliovirus was suggested by the fact that, of the more than one dozen ME outbreaks before the introduction of the Salk vaccine, nine occurred during or immediately after outbreaks of polio and several involved hospital staff who cared for polio patients (7).

Type III Poliovirus and Chronic Fatigue in Iceland. A more direct association between the poliovirus and ME was seen in 1948 in Akureyri, Iceland. Patients there presented with fever, muscle pain and weakness and were at first diagnosed as having poliomyelitis. After about a month , this diagnosis was discarded as patients reported additional symptoms not typical of polio, including tingling, numbness, “nervousness” and “general tiredness.” Also unlike poliomyelitis, no deaths were reported and poliovirus was never isolated from any of these patients. When patients were reexamined six years after their original illness, 72% still had chronic “nervousness and general tiredness” and 21% reported a of “loss of memory.”

It was suggested that either an “unusual” and mild poliovirus or some unknown virus caused these symptoms that were called “Akureyri Disease” but are more commonly referred to as “Iceland Disease” (ID). Support for an “unusual” poliovirus as the cause came in 1955 (10). There was an extensive epidemic of poliomyelitis in Iceland caused by Type I poliovirus that coincided with and was followed by outbreaks of ID. Remarkably, two cities in which ID outbreaks were reported in 1955, as well as the area affected by the 1948 “Akureyri Disease” epidemic, were untouched by poliomyelitis. None of the children tested in the two ID-affected cities and only 13% of the children in Akureyri had antibodies to Type I poliovirus as opposed to 86% of the children tested in the polio epidemic areas. Further, following poliovirus immunization, children in one of the ID-affected cities demonstrated antibody titres to Type II and Type III polio virus that were four and twenty-five times higher than titres in a city where ID had not been reported. It was concluded that Type I poliovirus was not the cause of ID, but the citizens of the IDaffected areas had previously been exposed to something that was immunologically similar to Type III poliovirus.

An interesting coda to these findings is the report that when an American airman who had contracted polio in the 1955 Iceland epidemic returned to Massachusetts, a small outbreak of ID and polio occurred (11). More recent support for a relationship between poliovirus and ME came in 1989 when a “dangerously rising titre” to Type III poliovirus was documented in a patient who did not have polio but had been diagnosed with ME (12).

Post-Polio Fatigue and Chronic Fatigue Syndrome. A group of symptoms resembling ME was termed “Chronic Fatigue Syndrome” (CFS) following a Nevada outbreak in 1984 (13). Like ME and post-polio fatigue, CFS is characterized by complaints of chronic fatigue and trouble with concentration, memory and word finding that are triggered or exacerbated by physical exertion and emotional stress. And, although polio survivors are on average at least ten years older than patients with CFS, the years of education, sex distribution, frequency of difficulty with concentration and psychological symptoms are nearly identical in the two groups (Table 1)(17,18,19). However, unlike ME and PPS, CFS patients report recurring sore throat, swollen glands and fever, suggesting to some that CFS is caused by a recurring or chronic viral infection. It is important to keep in mind that there is no evidence that PPS is caused by a

persistent infection by any virus, including poliovirus (14,15).

Polio Survivors	CFS Patients
Sample Size	276	²	259
Age 54 (±11)	² 39 (±12)
College Graduates 40% ² 40%
% Female 73% ² 68%
I.Q. 111 (± 2) ³ 111 (± 4)
Difficulty with:
Concentration 85% ² 82%
Word Finding 82% ²
Fatigue
does not impair functioning 21% ¹ 0%
limits only social activities 13% ¹ 43%
impairs work or self-care ability 66% ¹ 57%
Depressive Symptoms 61% ² 68%
Anxiety 74% ² 71%

Table 1.

Comparison of demographic data and symptom frequency in polio survivors reporting fatigue and in patients with Chronic Fatigue Syndrome (CFS). ¹ : Bruno and Frick, 1987; ² : Bruno, et al., 1991; ³: Bruno, et al., 1993; : Buchwald, et al., 1992.

The recent occurrence of CFS has allowed it to be studied using techniques that were not available during the polio, ME and ID epidemics and now allow neuropsychologic, neuroanatomic and neuroendocrine comparisons between this newest PFS and post-polio fatigue.

COMPARISONS OF POST-POLIO FATIGUE AND CFS

Neuropsychologic Studies. Some of the subjective difficulties with attention and cognition in CFS patients and polio survivors have been confirmed with neuropsychologic testing. CFS patients and polio survivors with severe fatigue have been shown to have clinical impairments of attention and information processing speed (Table 1)(16,19). Polio survivors reporting severe fatigue required 23% to 67% more time to complete tasks requiring sustained attention and vigilance than did polio survivors with no or mild fatigue. In spite of these marked impairments of attention, CFS patients and polio survivors have been shown to have I.Q.s within the high normal or superior range and have higher than average levels of educational and professional achievement (Table 1)(17). Further, despite the high frequency of subjective complaints of memory impairment in CFS patients and in 87% of polio survivors reporting fatigue, verbal memory has been shown to be intact on testing in both groups (16,19,20). However, polio survivors have twice been shown to have trouble recalling visual information whether or not they report fatigue (7,16).

These findings indicate that fatigue in CFS patients and polio survivors is associated with impairment of attention and information processing speed but not of memory or thinking ability. Given the findings of frequent and severe poliovirus lesions in the brain’s activating system, it was hypothesized that damage to

the brain’s activating system is responsible for both fatigue and impaired attention in polio survivors.

Brain Scan Studies. To test this hypothesis, magnetic resonance imaging (MRI) of the brain was performed to look for evidence of poliovirus lesions in the brain’s activating system. In a first study, small areas of hyperintense signal (which look like white spots) on MRI were seen in the brain’s activating system and in the myelinated (insulated) neurons that connect the brain stem (at the bottom of the brain) to the cortex (the ‘supercomputer’ at the very top of the brain) in eleven of twelve polio survivors (1). In a second study, white spots were seen in 55% of polio survivors with fatigue but were not seen in any of the subjects without fatigue (Figure 2)(21). The presence of the white spots were not only related to increased fatigue severity, but also to problems with memory, thinking clearly, mind wandering, attention and concentration.

FIGURE UNAVAILABLE

Figure 2.

A 24 mm² focus of hyperintense signal (arrow) in the centrum semiovale in a 50 year old female polio survivor reporting moderate daily fatigue and frequent problems with concentration, thinking clearly, short term memory and staying awake (putamen lesion not seen in this view).

Hormonal Studies. The association of white spots in the brain activating system with the symptoms of post-polio fatigue suggested that the effects of poliovirus on other brain areas might also be evident in polio survivors. For example, poliovirus lesions were often seen on autopsy in the hypothalamus (hypoTHAL-ah-mus), the brain area that automatically controls the body’s internal environment and its response to stress.

To test the functioning of the hypothalamus, we measured polio survivors’ blood concentrations of ACTH (a-DRE-no cor-ti-co-TRO-pick hormone), one of the body’s stress hormones whose release is triggered by the hypothalamus. ACTH was measured following an an overnight fast, which is a mild stress known to cause the release of ACTH (7). ACTH was increased outside of the normal range (as it should be following stress) in polio survivors who reported mild fatigue. However, there was no ACTH increase in subjects reporting severe daily fatigue. Further, the higher the ACTH level, the lower the subjects’ reported fatigue and the less the difficulty with memory, word finding, muscle weakness and staying awake during the day.

These findings indicate that the hypothalamus had not been activated in the subjects with post-polio fatigue and that ACTH production is reduced in these individuals. This conclusion is interesting for two reasons. First, ACTH has been found in humans to promote alertness, increase attention and decrease fatigue by directly stimulating the brain activating system. Thus, a decrease in ACTH production may prevent brain activation and contribute to the symptoms of post-polio fatigue. Decreased activation of the hypothalamus has already been found in patients with CFS and a decrease in ACTH stimulation of the brain has been suggested as a cause of CFS (23).

Second, a decrease in ACTH production may be caused by a decrease in production of its parent molecule, POMC. POMC also produces beta-endorphin (BAY-ta en-DOOR-fin) which along with the enkephalins (en-KEF-ah-lins) are ‘the body’s own morphine.’ Since poliovirus also damaged the brain area that produces enkephalins, both beta-endorphin and enkephalin production may be reduced in polio survivors. A reduction in the body’s own morphine would help to explain why polio survivors have a nearly doubled sensitivity to pain (1).

A MODEL FOR POST-POLIO AND CHRONIC FATIGUE

Taken together, these findings suggest a model for the cause of post-polio fatigue:

Poliovirus damaged the brain activating system;

MRI and hormonal findings suggest that damage to the brain activating system is present today in polio survivors;

Neuropsychological testing shows impaired attention in patients with post-polio fatigue; Therefore, poliovirus damage to the brain’s activating system may cause decreased brain activation, impair attention and generate the symptoms of post-polio fatigue.

While poliovirus damage to the brain activating system would be expected to cause the sleepiness, inattention and fatigue reported during the original polio infection, it is the recurrence of these symptoms, or their appearance decades after the acute infection, that are more difficult to explain. The emergence of fatigue decades after the acute polio may result from normal age-related changes in and loss of brain activating system neurons that had survived the acute polio infection, combined with an already decreased number of neurons as a result of the original poliovirus infection. Eventually, the loss of brain activating system neurons would decrease cortical activation, reduce attention and produce the symptoms of fatigue as polio survivors reach mid-life (1). The occurrence of these symptoms during physical or emotional stress in polio survivors may reflect the ability of stressors to uncover otherwise unseen damage in the brain activating system.

IS FATIGUE ‘HARD WIRED’ INTO THE BRAIN?

The findings presented above describe an intimate relationship between impaired attention and fatigue. However, difficulty with attention is not fatigue’s only symptom. Even more disabling is the physical experience of fatigue: feelings of exhaustion, ‘passivity and an aversion to continued effort’ that generate an aversion to both mental and physical activity. However unpleasant these feelings are in man, passivity and aversion to activity have clear survival value, especially in organisms without conscious awareness that their attention and thinking speed are impaired. For example, an animal that continues to explore its environment even though its attention is impaired would be less able to direct attention on the goal of its exploration (e.g., searching for food) and would thereby waste already diminishing energy stores. More importantly, impaired attention could also render the animal unaware of dangers in its environment (e.g., a predator stalking the animal in search of its food). Thus , there would be survival value in a brain mechanism that monitors cortical activation, biases an animal toward stopping motor behavior and promotes rest when attention and thinking speed are impaired.

The Brain ‘Listens’ to Itself. Groups of neurons near the bottom of brain called the basal ganglia are in an ideal location to monitor the level of brain activation and stop an animal when it has too little attention to allow efficient and safe activity in its environment. All parts of the cortex connect to one of the basal ganglia, called the putamen (pew-TAY-men), which ‘listens’ to the activity level of the brain (24). If the brain is awake enough, the putamen allows us to focus attention, to move and to act. When the brain activating system turns down, the putamen stops the cortex from allowing us to move. Damage to the putamen in animals has been shown to slow movement, while damage to another of the basal ganglia, the substantia nigra, decreases or even stops movement and prevents us from focusing attention (7).

The importance of the basal ganglia – and especially the neurotransmitter dopamine – in focusing attention and allowing us to move is most evident in patients with Parkinson’s disease (PD). PD patients, whose damaged substantia nigra neurons produce too little dopamine, show not only slowed movement and an inability to focus attention but also excessive and disabling fatigue (7,38-41). And, remember Oliver Sacks’ Awakenings patients whose damaged basal ganglia caused both Parkinson’s disease and ‘sleeping

sickness.’

The Brain Fatigue Generator. It appears that the basal ganglia could produce the mental and physical symptoms of both normal and pathological fatigue. In normal fatigue, a long and hard day of work would slow the firing of brain activating system neurons. This decreased activity would impair attention and information processing ability (recognized by humans as symptoms of fatigue) and produce a decrease in cortical activation that would slow the firing of putamen neurons, prevent the release learned motor behaviors and slow or stop activity (Figure 3). Humans would notice problems with focusing attention, feel an aversion to activity and would be able to move only with significant conscious effort. Animals would slow or even stop their activity. In both man and animals, rest or sleep would increase the firing of brain activating system neurons, restore cortical activation, increase the firing of putamen neurons and once again allow the release of motor behavior.

FIGURE UNAVAILABLE

Figure 3.

A model for the brain fatigue generator. Fatigue would be produced by a reduction in reticular activating system (RAS) activity that would directly decrease cortical activation, impair attention and cognition and prevent the firing of putamen neurons (dark lines). The reduction in putamen activity would then inhibit the release of motor behavior, further decrease attention and produce the visceral feelings of ‘exhaustion’ and aversion to effort that accompany fatigue (stippled lines).

Pathological states such as chronic fatigue syndromes could be produced by viral damage to the brain activating system, putamen and/or dopamine-producing neurons. This damage would chronically reduce the firing of brain activating system and putamen neurons, decrease cortical activation and produce the symptoms of fatigue. Poliovirus would be expected to cause fatigue, impaired cortical activation and decreased attention since it damages all of these brain areas.

CLINICAL IMPLICATIONS

This description of the basal ganglia as the brain fatigue generator suggests that increasing brain levels of dopamine (the neurotransmitter that stimulates the basal ganglia) might ‘turn on’ the brain activating system, increase cortical activation and attention, release motor behaviors and reduce the symptoms of chronic fatigue. We are currently studying the use of a drug that stimulates dopamine receptors on brain neurons to treat post-polio patients whose fatigue has not responded to the current treatments of choice, i.e., adequate rest, energy conservation, the pacing of activities and reducing physical and emotional stress (2,17,27,28). Preliminary results show that fatigue, impaired attention and difficulty staying awake during the day decrease as the dose of the drug increases.

However, there is the very real danger that taking a drug that reduces fatigue will allow polio survivors to resume their hyperactive, Type A lifestyles (as they do now when they feel better following physical, occupational and psychological therapy for PPS) and further stress poliovirus-damaged, ‘metabolically vulnerable’ neurons in the brain and spinal cord. Decreasing ‘overuse abuse’ will always be necessary to treat PPS, regardless of whether a drug is found that decreases the symptoms of fatigue.

It is also possible that damage to the basal ganglia and a lack of dopamine may be related to other PPS symptoms. Word finding difficulties, reported by 82% of polio survivors with fatigue, appear similar both to word finding problems reported by CFS patients and the ‘tip-of-the-tongue’ phenomena seen in PD patients (Table 1)(1,7). And, in a study we have just completed, polio survivors with severe fatigue had low scores on a test of word finding ability – scores that were identical to those in Parkinson’ patients.

In addition, 63% of polio survivors report Generalized Random Myoclonus (GRM), the slow contraction or rapid twitching of hand, arm, trunk and leg muscles at night that disturb sleep in 33% of polio survivors (2). GRM may provide more evidence that polio survivors have a brain dopamine shortage, since GRM are similar to “periodic movements in sleep” seen in PD patients.

We continue to examine the possible role of the basal ganglia and dopamine in PPS to help identify the cause and treatment of not only post-polio fatigue, but also other PPS, CFS and to understand the neurophysiology of fatigue itself.

ACKNOWLEDGEMENTS

The authors gratefully acknowledge the participation of the subjects, the support of the Joel Leff Charitable Trust and Deluxe Corporation , the continued support of the George Ohl, Jr. Foundation, and the efforts and expertise of Mary Ann Solimine, R.N., M.L.S., without whom this work would not have been possible. We also thank Drs. Leonard Kurland and Oliver Sacks for their criticisms of the manuscript and Esther Carlton of Nichols Institute who provided the 11:00 AM norms for cortisol and ACTH.

REFERENCES

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4. Holmgren, B.E. 1952. Electro-encephalography in poliomyelitis. In Poliomyelitis. Lippincott.

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6. Gilliam, A.G. 1938. Epidemiological study of an epidemic, diagnosed as poliomyelitis, occurring

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7. Bruno, R.L., Sapolsky, R., Zimmerman, J.R., & N.M. Frick. 1995. The pathophysiology of central

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8. Sigurdsson, B., J. Sigurjonsson, H.J. Sigurdsson, et al. 1950. A disease epidemic in Iceland

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fatigue, neurologic and immunologic disorders and active human herpesvirus type 6 infection. Ann.

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References 29, 38-41 not listed in source document.

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

Director Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation,

300 Market Street,

Saddle Brook, New Jersey 07663.

1 (800) 648-0296 (x6057).

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/fat.html> Created: 8th February 1997

Last modification: 29th January 2010.

Cause and Treatment of Post-Polio Fatigue Read More »

Predicting Hyperactive Behavior as a Cause of Non-Compliance with Rehabilitation

Predicting Hyperactive Behavior as a Cause of Non-Compliance with Rehabilitation: The Reinforcement Motivation Survey Dr. Richard L. Bruno

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

Post-Polio Rehabilitation and Research Service Kessler Institute for Rehabilitation

Saddle Brook, New Jersey

Running Head: Hyperactive Non-Compliance

Descriptors: Non-compliance; Chronic Pain; Type A Behavior; Post-Polio Sequelae.

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

Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation,

300 Market Street,

Saddle Brook, New Jersey 07662.

(201) 368-6057

ABSTRACT

Non-compliance with therapy is a significant problem in vocational rehabilitation. Significant amounts of professional time and money are wasted treating patients who are unwilling or unable to participate in their own rehabilitation. The client with chronic musculoskeletal pain – depressed, without energy (i.e., “anergic”) and refusing to attend therapy – is the exemplar of non-compliance. However, clients with chronic pain and other disabilities demonstrate a different type of non-compliance, characterized by chronic hyperactivity and refusal to decrease behaviors that are known to maintain or increase symptoms. To document the occurrence of hyperactive non-compliance, 80 clients treated for chronic musculo

skeletal pain (CMP) and 41 clients treated for Post-Polio Sequelae (PPS) were studied prospectively and administered the Beck Depression Inventory (BDI) and the Reinforcement Motivation Survey (RMS). Forty percent of the CMP clients and 79% of the PPS clients who were discharged from therapy demonstrated hyperactive non-compliance. CMP clients as a group had significantly elevated BDI and RMS Type A behavior and Negative Reinforcement Motivation scores, while PPS clients as a group had elevated Sensitivity to Criticism and Failure scores, as compared to controls. Significantly elevated Type A behavior and Sensitivity to Criticism and Failure scores were associated with hyperactive noncompliance as well as completion of therapy. These findings indicate that hyperactive non-compliance is an frequent cause of treatment failure in rehabilitation clients and that the RMS may be of use in identifying potentially non-compliant clients and the form non-compliance will take. The design of individualized rehabilitation programs to manage non-compliance and maximize the probability of completing therapy is described.

Predicting Hyperactive Behavior as a Cause of Non-Compliance with Rehabilitation: The Reinforcement Motivation Survey.

Refusal to comply with therapeutic recommendations or prescribed therapies is a significant problem in medicine (Cameron and Gregor, 1987). Clients with conditions ranging from arthritis (Jette, 1982) to diabetes (Ekerling and Kohrs, 1984) often refuse to follow daily dietary and exercise regimens, while more than half of clients with hypertension or tuberculosis were found to refuse continued medical care

(Sackett and Snow, 1979)

Non-compliance is also a common problem in rehabilitation. Many clinicians point to the client with chronic musculoskeletal pain, especially low back pain, as the exemplar of non-compliance (Blumer and Heilbronn, 1981; King and Snow, 1989). Chronic musculoskeletal pain (CMP) clients are described as having a premorbid history of hyperactivity, characterized by their performing in “a slave-like manner, working and keeping active beyond average expectations” in order to provide for the needs of others (Blumer and Heilbronn, 1982). Such excessive caretaking is believed by these individuals to be required for their acceptance by family, friends and employers, and is said to protect against excessive sensitivity to criticism and profound feelings of “insecurity and guilt” (Blumer and He ilbronn, 1982).

Hyperactivity is said to persist until “a minor injury provide(s) a rational and socially acceptable” reason for ceasing excessive caretaking and becoming dependent on others (Gentry, et al., 1974). The cessation of hyperactive caretaking of others is thought to trigger a “depressive disorder” which causes both chronic pain and “a disabling degree of anergia” (i.e., a profound lack of energy and activity). This disabling anergia becomes evident when CMP clients “completely lose their initiative and zeal for work” (Blumer and Heilbronn, 1981). They become “helpless sufferers” waiting to be “spoiled and cared for continuously” and are disinterested in participating in a rehabilitation program designed to help them tolerate their pain and decrease their disability (Blumer and Heilbronn, 1982).

This description of the depressed, anergic CMP client as the exemplar of non-compliance is not consistent with our experience. While it does appear that many CMP clients are premorbidly hyperactive, not all chronic pain clients become depressed, anergic, helpless and dependent following injury. Even after pain onset, a large minority of CMP clients refuses to reduce their premorbid level of hyperactivity. They vigorously maintain their independence and continue to work excessively, caring for others, even though their hyperactivity maintains or exacerbates the pain. These clients often report that they are “overcome by guilt” if they reduce their caretaking of others. Further, it is persistent hyperactivity, and not depressive anergia, that prevents these clients from complying with treatment recommendations or even attending

therapy sessions and causes them to be discharged from the pain management program.

Recently, another group of rehabilitation clients who also demonstrate hyperactivity and therapeutic noncompliance has been described: the 1.63 million survivors of America’s polio epidemics (Frick and Bruno, 1986). Like hyperactive CMP clients, polio survivors vigorously strive to maintain their hard-won independence and work excessively, also performing in a slave-like manner, keeping active beyond average expectations in order to provide for the needs of others (Bruno and Frick, 1991). For example, polio survivors have been found to work significantly more overtime hours per week, regardless of the severity of their disability, than do their non-disabled peers (Lonnberg, 1993). Further, two national surveys have found that polio survivors on average demonstrate significantly more hard-driving, pressured, over-achieving Type A behaviors than do the general population (Bruno and Frick, 1987;1991; Rosenman, et al., 1964). The experience of polio has been suggested to provide the ideal environment for the development of the Type A personality, since hyperactive Type A behaviors are said to be learned in service of the “active avoidance of punishment” (Perkins, 1984) by individuals with “low social support and self-esteem” (Powell, et al., 1984) who are involved in a chronic “struggle to overcome environmental barriers” (Rosenman, 1964). And, polio survivors, like hyperactive CMP clients, persist in their hyperactivity even though it has been shown to cause and exacerbate Post-Polio Sequelae, the late-onset symptoms that include muscle and joint pain, overwhelming fatigue and muscle weakness (Bruno and Frick, 1986;1991).

Surprisingly, there have been few studies that document the frequency and examine causes of noncompliance in rehabilitation. Many studies of pain management program outcome have actually excluded non-compliers from their analyses (Aronoff and Evans, 1982; Chapman, et al., 1981; Gluck, et al., 1986: Maruta, et al., 1987; Painter, et al., 1980). King and Snow (1989) did compare CMP clients who “were discharged from or left” their inpatient pain program, but the behaviors that prompted discharge were not specified.

This paper describes

1. the frequency of anergic and hyperactive behaviors as causes of non-compliance with therapy in

rehabilitation,

2. the development of an instrument that measures the behaviors and motivational factors associated

with therapeutic non-compliance and helps to identify potentially non-compliant clients and

3. the creation of individualized rehabilitation plans that incorporate client management strategies to

minimize non-compliance and maximize the probability of clients completing rehabilitation and

returning to work.

METHODS

Subjects. Eighty consecutive clients admitted to the Pain Management Program over two years were studied. They had a history of 3.2 (+/- 3.5) years of musculoskeletal back and/or neck pain as a result of motor vehicle or work-related accidents. This chronic musculoskeletal pain (CMP) group consisted of 44 females and 36 males with a mean age of 39.8 (+/- 10.4) years having had 13.2 (+/- 2.6) years of education.

Forty-one polio survivors consecutively admitted over two years to the Post-Polio Service with a diagnosis of Post-Polio Sequelae (PPS) were also studied. The PPS group consisted of 32 females and 9 males with a mean age of 47.7 (+/- 11.4) years having 14.4 (+/- 2.1) years of education who reported new PPS symptom an average of 42 (+/- 8.0) years after having had polio.

Reinforcement Motivation Survey. The Reinforcement Motivation Survey (RMS) is a 36 item, forced choice questionnaire containing three scales designed to measure behaviors and motivational factors that have been noted in non-compliant clients: a Brief Type A behavior scale, a scale measuring negative reinforcement motivation and a scale measuring sensitivity to criticism and failure (Table 1).

Type A Behavior scale. Since the hyperactive behaviors seen in CMP and PPS clients appear similar to the behaviors characteristic of the Type A personality (Rosenman, et al., 1964), the 10 item brief Type A Behavior scale developed by Young and Barboriak (1982) was administered (Table 1). The TAB score was elevated when respondents agreed that they performed work in a excessive, driven, angry or competitive manner uninterrupted by rest or vacation.

Negative Reinforcement Motivation scale. Our CMP and PPS clients often report that their hyperactive behavior is not motivated by others’ praise or personal satisfaction but by a compelling noxious internal drive that ceases only when tasks performed for or required by others are successfully completed. Behavior motivated by an attempt to reduce a noxious stimulus is called negative reinforcement in learning theory, but is usually described by our hyperactive clients as “guilt.” Motivation resulting from negative reinforcement is consistent with the suggestion that hyperactivity protects against life-long feelings of guilt. (Blumer and Heilbronn, 1982; Bruno and Frick, 1991; Van Houdenhove, et al., 1987).

The 18 item RMS Negative Reinforcement Motivation scale (Table 1) was designed so that the scale score was elevated when respondents agreed that they were uninterested in others’ appreciation of or praise for their work, focused on only one task at a time and were motivated by their own internal sense of discomfort that decreased only when they worked continuously and completed tasks quickly.

Sensitivity to Criticism and Failure scale. Hyperactivity in CMP and PPS most often appears to serve the needs or expectations of others. This is consonant with the suggestion that hyperactivity is an attempt to protect against chronic insecurity and low self-esteem by preventing criticism or punishment by others and a sense of personal failure (Blumer and Heilbronn, 1982; Bruno and Frick, 1991; Perkins, 1984; Powell, et al., 1984; Van Houdenhove, et al., 1987)

The 8 item Sensitivity to Criticism and Failure scale (Table 1) was designed so that the scale score was elevated when respondents agreed that they were motivated by the need to please others, that they ruminated about past mistakes and that their self-image and affect were negatively impacted by a lack of success in their work or the critical comments of others.

Scoring. Items answered correctly on each scale (correct answer given in parenthesis in Table 1) were given a score of one, while those answered incorrectly were given a score of zero. The total scale scores were calculated by summing the individual item scores, dividing by the number of scale items and multiplying by 100.

RMS Normative Sample. The validity and reliability of the RMS were assessed on 125 individuals without CMP, polio or other disabilities. Clients were asked to provide their own controls by asking friends and relatives to complete the RMS. Item analysis was conducted by using product-moment correlations to relate responses on individual items with the total score for each scale. Correlations ranged from 0.18 to 0.59 for the Type A behavior items, from 0.17 to 0.49 for the Negative Reinforcement scale items and from 0.39 to 0.34 for the Sensitivity to Criticism and Failure scale items (Table 1).

The Type A behavior scale correlated 0.09 (p=N.S.) with the Negative Reinforcement scale and 0.29 (p=0.02) with the Sensitivity to Criticism and Failure scale. The Negative Reinforcement and Sensitivity to Criticism and Failure scales correlated -0.17 (p=N.S.) with each other.

Two pair of items with similar content were included in the Negative Reinforcement and Sensitivity to Criticism and Failure scales to assess reliability by measuring internal consistency. The Spearman-Brown formula, applied to phi correlations calculated for each pair of item scores, estimated full-scale reliabilities of 0.87 for the Negative Reinforcement scale and 0.79 for the Sensitivity to Criticism and Failure scale (Anastasi, 1988). The reliability of the Type A Behavior scale is given by Young and Barboriak (1982).

PROCEDURE

Clients were administered the Reinforcement Motivation Survey (RMS) and the Beck Depression Inventory (BDI) (Beck, et al., 1961) during the initial evaluation. Clients were directed to complete the RMS based on how they usually thought, felt or acted prior to the onset of pain or PPS symptoms and to answer the BDI questions on the basis of how they felt during the preceding week.

Clients in both groups were prescribed individualized rehabilitation programs including physical and occupational therapy, individual and group psychotherapy and EMG biofeedback. The CMP rehabilitation program was designed to eliminate the use of narcotic drugs and assistive devices, decrease anergic or hyperactive behaviors, prescribe stretching and endurance exercises and increase the level of personal and vocational functioning. The PPS rehabilitation program was designed to decrease pain, fatigue and muscle weakness by teaching energy conservation, work simplification and time management, prescribing nonfatiguing exercise and needed assistive devices (Bruno and Frick, 1991). CMP outpatients received three one hour sessions while PPS outpatients received two one hour sessions of physical and occupational therapy per week. CMP or PPS inpatients received 17.5 hours of physical and occupational therapy per week. All clients received one hour per week of individual psychotherapy; CMP clients received four hours per month and PPS clients two hours per month of group psychotherapy. A complete rehabilitation program lasted from eight to twelve weeks.

Definition of Non-Compliance. Two types of non-compliance were noted: clients either failed to increase or decrease their levels of activity. For example, clients who failed to increase their level of activity refused to leave bed in the morning, returned to bed prematurely or remained sedentary during the day. Some also failed to attend therapy sessions or left sessions prematurely, failed to perform the prescribed number or type of therapeutic activities or exercises, failed to correct poor posture or to independently perform self-care activities (e.g., failed to stop asking others to help with ADLs). Non-compliant CMP clients also failed to discard unnecessary assistive devices (e.g., a cane, crutch or wheelchair). These types of behaviors were termed anergic non-compliance.

The second type of non-compliance was characterized by clients failing to decrease their general level of activity and eliminate behaviors that had been identified as maintaining or exacerbating their symptoms. Typically, these clients failed to attend therapy sessions, perform prescribed physical therapy, relaxation and biofeedback exercises or take prescribed breaks during the day because of schedules that burgeoned with numerous activities in service of employers, social organizations, family members and friends. Clients also failed to decrease the number, duration , intensity or extent of their daily activities or to request appropriate assistance in performing them. PPS clients failed to keep daily activity and symptom logs or to use newly prescribed assistive devices (e.g. a cane, crutch, brace or wheelchair). These types of behaviors were termed hyperactive non-compliance.

Discharge Procedure. When non-compliant behaviors were observed by the treatment team, the need for their alteration was discussed with the client. If the behaviors did not change by the next therapy session, a behavioral contract was written by the team that detailed the specific behavior(s) to be altered and stated that the client would be discharged from all therapies if a behavior change was not effected. The contract

was presented to the client by the treatment team and signed by the client.

If the client refused to sign the contract, he was immediately discharged from all therapies until he was willing to participate in the rehabilitation program. If the client signed but failed to comply with the contract by the next therapy session the client again met with the team. The non-compliant behavior(s) were again discussed and the terms of the contract reiterated. The client was immediately discharged from all therapies if the contract was violated a second time and readmitted only when he was willing to participate in the rehabilitation program. Clients were classified as anergic or hyperactive non-compliers depending upon the type of behavior they emitted that caused their discharge from therapy (see above).

Data Analysis. Descriptive statistics were calculated for all variables. Two-tailed independent-groups ttests, with p values corrected for multiple comparisons using the Bonferroni inequality, were used to compare the control subject’s BDI and RMS scores to those of the CMP and PPS client groups (Table 2) and the three outcome subgroups (those who completed rehabilitation or were discharged for anergic or hyperactive non-compliance) (Table 3) and within the three outcome subgroups.

RESULTS

Twenty-five (31%) of the CMP clients and 14 (34%) of the PPS clients were prematurely discharged for non-compliance with their rehabilitation program. Fifteen (18%) CMP clients and 3 (7%) PPS clients were discharged for anergic non-compliance, while ten (13%) CMP clients and 11 (27% ) PPS clients were discharged for hyperactive non-compliance.

CMP clients were significantly younger (40 +/- 10 years) than the controls (50 +/- 13 years)(t=5.91; p < 0.01) and PPS clients (48 +/- 11 years)(t=3.83; p<.01). However, there were no significant differences in age within the CMP or PPS outcome subgroups or in years of education between the CMP and PPS groups or within the outcome subgroups. There were also no significant differences within the PPS subgroups in years since polio onset. However, CMP clients discharged for hyperactive non-compliance reported significantly more years since pain onset (6.3 +/- 7.4) as compared to the CMP clients who completed rehabilitation (2.6 +/- 2.4)(t=3.01; p<0.01).

Beck Depression Inventory Scores. The mean BDI score in the CMP group was statistically but not clinically (not >17) significantly elevated as compared to the control and PPS group means (Table 2) (Beck, et al ., 1961). The BDI scores for the CMP clients who completed rehabilitation and those who were discharged for anergic non-compliance were statistically significantly elevated as compared to the control group mean (Table 3). However, only the mean BDI score of 19 in the CMP clients discharged for anergic non-compliance was clinically elevated indicating that these clients as a group demonstrated symptoms of “mild” depression (Beck, et al., 1961).

There were no significant differences in BDI scores between the PPS clients as a group and the controls, within the PPS subgroups or between the controls and any PPS subgroup.

Type A Behavior Score. The mean Type A Behavior scale (TAB) score was significantly elevated in CMP clients as a group as compared to the controls (Table 2). The mean TAB score was also significantly elevated as compared to the controls in the CMP clients who completed rehabilitation and elevated even higher in those who were discharged for hyperactive non-compliance (Table 3). However, there were no significant differences in TAB score between the CMP clients discharged for anergic non-compliance and the controls or within the CMP subgroups. There were no significant differences in TAB scores between the PPS clients as a group and the controls, within the PPS subgroups or between the controls and any PPS subgroup.

Negative Reinforcement Motivation Score. The mean Negative Reinforcement Motivation scale (NEG) score was significantly elevated in the CMP clients as a group (Table 4) and in each of the CMP subgroups as compared to the controls (Table 5). However, there were no significant differences in NEG scores within the CMP subgroups. There were also no significant differences in NEG scores between the PPS clients as a group and the controls, within the PPS subgroups or between the controls and any PPS subgroup.

Sensitivity to Criticism and Failure. There was no significant difference in the mean Sensitivity to Criticism and Failure scale (SEN) score between the CMP clients as a group and the controls (Table 2) or between any CMP subgroup and the controls (Table 3). However, the SEN score was significantly elevated in the CMP clients discharged for hyperactive as compared to anergic non-compliance.

The mean SEN score was significantly elevated in the PPS clients as a group as compared to the controls (Table 2). The mean SEN score was also significantly elevated as compared to the controls in the PPS clients who completed rehabilitation and elevated even further in those who were discharged for hyperactive non-compliance (Table 3). However, there was no significant difference in SEN score between the PPS clients discharged for anergic non-compliance and the controls or within the PPS subgroups.

DISCUSSION

These findings document that discharge from a chronic pain management program results not only from anergia but also from hyperactivity, since 40% of CMP clients were discharged for hyperactive noncompliance. Further, hyperactive CMP clients did not report significantly more symptoms of depression than did those discharged for anergic non-compliance or those who completed rehabilitation. These findings contradict those of Blumer and Heilbronn (1989) but support the data of King and Snow (1989). Thus, anergia is not always demonstrated by CMP clients, nor are symptoms of depression necessary for the genesis of chronic pain.

The significantly elevated Type A behavior and Negative Reinforcement Motivation scores in CMP clients as a group support the description by Van Houdenhove, et al. (1987) of “pain-prone” individuals demonstrating premorbid, internally-driven hyperactivity that serves in part to protect against feelings of guilt (Van Houdenhove, et al., 1987). The lack of significant differences in Negative Reinforcement Motivation scores between CMP subgroups suggests that negative reinforcement motivation may be a trait in individuals who develop CMP and is not a factor that affects compliance with rehabilitation.

The significantly elevated Type A behavior score in CMP clients who completed rehabilitation suggests that an elevated level of work-oriented behavior may be helpful or even necessary to complete rehabilitation in a pain management program. This conclusion is supported by the below average Type A behavior score of 39 recorded in CMP clients who were documented to be malingering (Bruno, 1991). These clients demonstrated profound anergia and refused to participate in their own rehabilitation.

However, an extreme elevation in Type A score, as seen in CMP clients discharged for hyperactive noncompliance, suggests that excessive Type A behavior may be inimical to the self-care behaviors and moderation of activity that are required to manage chronic pain in clients demonstrating hyperactivity.

The significant elevation of the Sensitivity to Criticism and Failure score in CMP and PPS clients discharged for hyperactive non-compliance, as well as in the PPS clients who completed rehabilitation, may be interpreted in a similar fashion. A certain level of sensitivity to criticism and concern about failure may be required for clients to listen to the treatment team and make the significant life-style changes that

are necessary to treat both CMP and PPS. This conclusion is supported by the below average Sensitivity to Criticism and Failure score in the clients discharged for anergic non-compliance (Table 3) and by the report of an even lower score of 23 in malingering CMP clients, who appeared insensitive to input from the treatment team or the needs of and impact on others of their continuing disability (Bruno, 1991).

On the other hand, extreme sensitivity to criticism and a constant fear of failure would prevent hyperactive individuals from making lifestyle modifications that are readily noticed by others (such as reduced caretaking, eliminating overtime work or using a wheelchair) and that could generate negative comments by family members, friends and colleagues. Thus, the significantly elevated Sensitivity to Criticism and Failure score in the PPS group could explain their 79% rate of hyperactive non-compliance (versus 40% in the CMP group) and the near 4:1 ratio of discharge for hyperactive versus anergic non-compliance in the polio survivors. These clients’ heightened sensitivity may also explain why individuals with supportive and less critical family members were found to be more likely to complete a pain management program (King and Snow, 1989) or rehabilitation for PPS (Creange and Bruno, 1994).

Using the RMS in Developing a Rehabilitation Plan. These findings indicate that the Reinforcement Motivation Survey can be useful in both identifying clients with the potential for non-compliance and indicating the form non-compliance will take even before the client begins therapy. Using the RMS to identify potential non-compliers should be of great help to the vocational rehabilitation counselor and insurance nurse who are responsible for returning clients on long-term disability to work in a pressured managed care environment that demands rapid results but allows a minimum expenditure of insurance company dollars. If behaviors that will likely subvert rehabilitation are identified and managed from before the first day of treatment, the client’s potential for success in therapy and returning to work will be maximized. Clients who are unable or unwilling to modify their non-compliant behaviors – in spite of the best interdisciplinary efforts of the case manager, therapists and psychologist – could be discharged from rehabilitation with cause, compensation stopped and their cases closed.

For example, clients with markedly elevated Type A behavior or Sensitivity to Criticism and Failure scores (i.e., more than one standard deviation above the control group mean) would be identified as potential hyperactive non-compliers. The early identification of hyperactive non-compliers is especially important, since hyperactive behavior is often overlooked or even welcomed, since counselors are pleased when a client appears “extremely well motivated for therapy.” Hyperactive behaviors that were present premorbidly and that are related to the exacerbation or maintenance of symptoms should be discussed with the client before treatment begins. Clients should be cautioned that unless hyperactivity is decreased , their symptoms will continue, rehabilitation will be subverted and the likelihood of returning to work will be significantly decreased.

A rehabilitation plan should then be developed that includes clients keeping daily symptom and activity logs that are reviewed before each therapy session. The presence of hyperactivity should be immediately discussed with the client and its persistence should be addressed by requiring the that client sign a behavioral contract. This contract should make clear that continued treatment and compensation income are contingent on decreasing hyperactive behaviors and full compliance with therapy. A client’s inability to decrease hyperactive behaviors also necessitates referral to a psychologist who can help identify the motivations for Type A behaviors and modify elevated sensitivity to criticism and failure.

Clients having extremely low Type A behavior or Sensitivity to Criticism and Failure scores (i.e., below the control group mean) would be identified as potential anergic non-compliers. Even before therapy begins, these clients should sign a behavioral contract stating that all therapy session will be attended, daily logs completed and home exercises and ADL activities performed as prescribed or rehabilitation and

compensation income will be discontinued. When physical and occupational therapy begin, the contract is expanded to include an individualized home exercise program and the specific activities to be performed outside of the clinic.

We have found the RMS to be very helpful over the past five years in identifying potential non-compliers and guiding the creation of individualized rehabilitation plans. However, we are much more successful in slowing hyperactive clients than in activating those who are anergic. Even the RMS prompting behavioral contracting before treatment begins – contracts promising that therapy and even compensation income will be discontinued with the persistence of non-compliance – cannot motivate the profoundly anergic client to participate in his own rehabilitation. More applied research is needed to identify ways in which the anergic client can be motivated.

REFERENCES

Anastasi, A., Psychological Testing (6th Edition). New York, Macmillan, 1988.

Aronoff, G.M. and Evans, W.O., The prediction of treatment outcome at a multidisciplinary pain center, Pain, 14 (1982) 67-73. [PubMed Abstract]

Beck, A.T., Ward, C.H., Mendelson, M., Mock, J., & Erbaugh, J., An inventory for measuring depression, Arch. Gen. Psych., 4 (1961) 53-61.

Blumer, D. and Heilbronn, M., The pain-prone disorder: A clinical and psychological profile, Psychosomatics, 22 (1981) 395-402. [PubMed Abstract]

Blumer, D. and Heilbronn, M., Chronic pain as a variant of depressive disease, J. Nerv. Ment. Dis, 170 (1982) 381-406. [PubMed Abstract]

Blumer, D. and Heilbronn, M., Dysthymic pain disorder: The treatment of chronic pain as a variant of depression. In: C.D. Tolloson (Ed.) Handbook of Chronic Pain Management, Williams & Wilkins, Baltimore, 1989.

Bruno, R.L., Predicting pain management program outcome, Arch. Phys. Med. Rehab., 10 (1990) 785.

Bruno, R.L. and Frick, N.M., Stress and “Type A” behavior as precipitants of post-polio sequelae. In: L.S. Halstead and D.O. Wiechers (Eds.) Research and Clinical Aspects of the Late-Effects of Poliomyelitis, March of Dimes, White Plains, 1987. [Lincolnshire Library Full Text]

Bruno, R.L. and Frick, N.M., Psychology of polio as prelude to post-polio sequelae: Psychotherapy and behavior modification, Orthopedics, 14 (1991) 1185-1193.[Lincolnshire Library Full Text] Cameron, K. and Gregor, F., Chronic illness and compliance, J. Advan. Nurs., 12 (1987) 671-676. [PubMed Abstract]

Chapman, S.L., Brena, S.F. and Bradford, L.A., Treatment outcome in a chronic pain rehabilitation program, Pain, 11 (1981) 255-268. [PubMed Abstract]

Creange, S.J. and Bruno, R.L., Family support as a predictor of participation in rehabilitation for post-polio sequelae, N.J. Rehab., 8 (1994) 8-11.

Ekerling, L. and Kohrs, M.B., Research on compliance with diabetic regimens: Application to practice, Am. Dietetic Assoc., 84 (1984) 805-809.

Frick, N.M. and Bruno, R.L., Post-Polio Sequelae: Physiological and psychological overview, Rehab. Lit., 47 (1986) 106-111. [Lincolnshire Library Full Text]

Gentry, W.D., Shows, W.D. and Thomas, M., Chronic low back pain: A psychological profile, Psychosomatics, 15 (1974), 174-177.

Gluck, T.P., Meilman, P.W., Skultety, F.M. and Dowd, E.T., Prediction of long-term outcome of

multidisciplinary pain treatment, Arch. Phys. Med . Rehab., 67 (1986) 293-296.

Jette, A.M., Improving patient cooperation with arthritis treatment regimens, Arth. Rheum., 25

(1982) 447-453. [PubMed Abstract]

King, S.A. and Snow, B.R., Factors for predicting premature termination from a multidisciplinary

inpatient chronic pain program, Pain, 39 (1989) 281-287. [PubMed Abstract]

Lonnberg, F., Late onset polio sequelae in Denmark, Scand. J. Rehab. Med, Suppl. 28 (1993) 24-31.

[PubMed Abstract]

Maruta, T, Swanson, D.W. and Swenson, W.M., Chronic pain: Which patient may a pain program help, Pain, 7 (1979) 321-329. [PubMed Abstract]

Painter, J.R., Seres, J.L. and Newman, R.I., Assessing benefits of the pain center: Why some patients regress, Pain, 8 (1980) 101-113. [PubMed Abstract]

Perkins, K.A. Heart rate change in type a and type b males as a function of response to cost and task difficulty, Psychophysiology, 21 (1984) 14-21. [PubMed Abstract]

Powell, L.H., Friedman, M., Thoresen, C.E., et al., Can the Type A behavior pattern be altered after myocardial infarction, Psychosom. Med., 46 (1984) 293-313. [PubMed Abstract]

Rosenman, R.H., Friedman, M., Straus R., et al., A predictive study of coronary heart disease, JAMA, 189 (1964) 113-124.

Sackett, D.L. and Snow, J.C., The magnitude of compliance and non-compliance. In: R.B. Haynes, D.W. Taylor and D.L. Sackett (Eds.) Compliance in Health Care, Johns Hopkins University Press, Baltimore, 1979.

Van Houdenhove, B., Stans, L., and Verstraeten, D., Is there a link between “pain-proneness” and “action-proneness?”, Pain, 29 (1987), 113-117. [PubMed Abstract]

Young, L.D. and Barboriak, J.J., Reliability of a brief scale for assessment of coronary-prone behavior and standard measures of Type A behavior, Percept. Mot. Skills, 55 (1982), 1039-1042.

[PubMed Abstract]

Table 1 [below]

Correlations (r) between individual RMS scale items and total scale scores. (Answers scored with a “1” in parentheses; a,b: Item pairs correlated to assess internal consistency.)

r	Type A Behavior Scale Items.
.59	Do you consider yourself to be ‘hard driving?’ (True)
.54 Do you set at least one deadline per day for yourself? (True)
.53 Is it very important for you personally to get ahead in life? (True)
.47 Do you set at least one deadline per week for yourself? (True)
.45 Do you spend more than 8 hours per week working overtime? (True)
.41 Do you usually wake up in the morning not feeling well rested? (True)
.39 Have you taken less than one vacation per year during the past 5 years? (True)
.34 Do you spend less than 5 days on an average vacation? (True)
.29 Do you enjoy competition? (True)
.18 Do you have a temper that’s hard to control, ‘fiery?’ (True)
r Negative Reinforcement Scale Items.
.49 I don’t need much from people. (True)
.42 I would dislike very much working alone in some isolated place. (False) a
.42 I sometimes need people to prod me to work. (False)
.41 Once I begin a task I try to finish it immediately, even if there’s no deadline. (True) b
.41 When I’m sick, I prefer that my friends leave me alone. ( True)

.35	I feel dissatisfied if I remain unnoticed. (False)
.35	I’d rather not be by myself. (False)
.34 I do my best work when I know it will be appreciated. (False)
.32 I feel very uncomfortable when tasks I’ve started go unfinished. (True) b
.28 When I was growing up, I didn’t care to be a member of a group or crowd. (True)
.27 It bothers me that I never get all the praise I deserve for the work I do. (False)
.27 I prefer to work alone. (True) a
.26 I tend to be interested in several different activities, rather than working at one for a long time.(False)
.25 I find it impossible to relax if there’s work left to be done. (True)
.24 I’m never happier than when people say I’ve done a good job. (False)
.22 I have the habit of starting things and then losing interest in them. (False)
.21 I have very many close friends. (False)
.17 More than once, I’ve very nearly let my car run out of gas. (False)
r Sensitivity to Criticism and Failure Scale Items.
.64 What people think of my work very much affects how I feel about myself. (True) a
.60 Criticism disturbs me very much. (True)
.59 Thoughts of my past mistakes often bother me. (True) b
.58 I give little thought to my failures after they’re past. (False) b
.57 If something goes wrong in my work, it can make me feel depressed. (True)
.53 Peoples’ comments about me don’t bother me. (False)
.50 My image of myself depends greatly on success in the work that I do. (True) a
.39 I am the only person I have to please. (False)

Table 2 [below]

Mean (± standard deviation) BDI and RMS scale scores for the control, chronic musculoskeletal pain (CMP) and post-polio sequelae (PPS) groups.

CONTROLS	CMP	PPS
Sample Size	125	80	41
Beck Depression Inventory 11 (± 8) a 16 (± 9) 14 (± 9)
Type A Score 47 (± 21) 63 (± 20) 55 (± 18)
Negative Reinforcement Score 52 (± 17) 65 (± 16) 51 (± 14)
Sensitivity to Criticism and Failure Score 59 (± 27) 65 (± 28) 77 (± 22)

a: Mean Beck Depression Inventory score of a group (n=115) diagnosed as not being depressed (from Beck, et al, 1961). Mean score significantly elevated (p<0.01) as compared to the control ( ) or postpolio group ( ¥ ) mean. Editorial Note: Unable to determine from source document to which table entries the latter 2 suffixes apply

Tables 3 to 5 missing in source document.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/rms.html> Created: 12th February 1997

Last modification: 29th January 2010.

Predicting Hyperactive Behavior as a Cause of Non-Compliance with Rehabilitation Read More »

Silicone Sex and Polio Survivors

SILICON, SEX and POLIO SURVIVORS

Dr. Richard L. Bruno

Post-Polio Service

Kessler Institute for Rehabilitation 1995

Last January I got a brand new Macintosh computer that came with a modem. Ever since I’ve been “surfing” the Internet, hardly stopping for food or sleep. You’d be amazed at what I’m finding on the disABILITIES bulletin boards: Sex. Yes, unbridled, undiluted and unimpeded discussions about sex among people with all kinds of disabilities, from high-level quads to quadruple amputees. However, there is one place where I’ve heard no discussion of sex: the Post-Polio bulletin board. There are lots of questions about PPS that you had the answers to years ago: Do I have ALS; does exercise make you weak; where can I get a scooter? But, not one question or comment about sex.

I guess I shouldn’t be surprised. Many of our patients at Kessler don’t mention sex, either. Some mention that spouses do not believe that PPS symptoms are “real.” But, most often spouses are very supportive about the need for their post-polio mate to slow down and take care of themselves, even if that means a new brace, crutches or a scooter. So, if spouses are so supportive, why is there no mention of sex?

ALL’S TOO QUITE ON THE SOUTHERN FRONT

There are probably several reasons for the lack of talk about sex:

Many polio survivors grew up in the 50’s when discussing sex was the same as walking down Main Street without your pants. Also, 40 years ago, even more than today, people with disabilities were thought not to be fully human, let alone sexual beings;

Also, it was vital back in the dark ages that anything not “normal” (that is anything not seen on Ozzie and Harriette) be hidden. So, if a polio survivor expected to have a relationship with someone the disability had to be hidden. Since the polio residuals were often hard to hide, the disability was often just ignored or denied. It amazes me still how many survivors tell me that they have never, ever discussed the fact they had polio with their spouse, let alone the experience of having had polio or – God forbid – their feelings about it.

The process of hiding, denying or ignoring the reality of what’s happening in one’s body caused problems. Many people just turned off any awareness of their bodies and any feelings they have below the neck. Others have turned off ALL feelings, both below and above the neck. Since you can’t turn off pain without turning off pleasure as well, an inability to feel will make sensuality or sexuality nearly impossible – or as appealing as eating dry toast.

Also, walling off one’s feelings also cuts off the ability to be intimate with oneself and with others. And,

intimacy is the gateway to sexuality.

Having a visible disability at an early age can result in other barriers to intimacy and sexuality. There is a disturbingly high frequency of physical, emotional and sexual abuse among polio survivors. Nearly one third of the patients we treat have been physically or emotionally abused, and 25% of the women have been sexually abused. Not surprisingly, those who have been abused are much less likely to risk intimacy or be interested in sex.

Also not surprisingly, the more obvious the assistive device people used following polio the more likely it was that [they] had been abused. No wonder polio survivors discarded their braces and crutches and don’t want them back – ever! Now, 40 years later when PPS symptoms start and braces, crutches and wheelchairs are appearing again, the painful memories of the past and the reality of disability can no longer be hidden. Old fears of unacceptability and new fears of rejection surface and cut self-esteem off at the pass. And, the old viscious Rules of Society also come screaming back:

Rule #1: If you’re disabled, you’re not attractive;

Rule #2: If you’re not a attractive you can’t be sexual;

Rule #3: If you can’t be sexual, you shouldn’t have sexual feelings;

Rule #4: Wear flannel pajamas and sleep on the couch.

“IF I THINK I’M SEXY AND I LIKE MY BODY…”

Well, sexy is a sexy thinks. One of the disABILITIES bulletin boards is actually called “Sex is 99% Mental.” Sure you may not look like Cindy Crawford or Mel Gibson. But, how many non-disabled people do? What counts is how you feel about yourself, not how you look to others. If you’re not acceptable to yourself, you wont be sexually available to or even intimate with others.

The first step to intimacy and sexuality is recognizing and dealing with the emotional reality of the original polio and any abuse you have experienced because if it. Next, you need to identify your own negative feelings about yourself and stop projecting them in to the heads of potential friends and lovers. Since a whopping 72% of polio survivors are married, it’s your spouse’s head that you’re filling with your own negative thoughts about yourself and fears of rejection.

Finally, if you turned off your body to stop feeling physical pain, you need the experience of good physical feelings. You need to decrease your PPS fatigue, muscle weakness, and pain by decreasing physical and emotional stress. Then, you need to start sending pleasant physical sensation to your brain. Try long hot baths, a whirlpool or best of all a massage. When your make nice to your body and good feelings start traveling to your head, other good physical sensations (like the erotic ones) will follow that “stairway (or ramp) to heaven.”

For those who are single, get a computer, a modem and start “surfing the net.” It is actually possible to meet hundreds of people without leaving you living room. Whether you decide to let them into your life (or your bedroom), the safety of the computer gives you the freedom to be yourself and even practice being intimate with others, without worrying that everyone is looking ONLY at your brace.

When all is said and done, here’s the Golden Rule for being an intimate and sexy polio survivor (with apologies to Nike):

Turn off your thoughts, turn on your body and JUST DO IT!

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

The Secretary, Lincolnshire Post-Polio Network

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

Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk

Web Site: www.lincolnshirepostpolio.org.uk

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

Created: 14th November 1998

Last modification: 22nd January 2010.

Silicone Sex and Polio Survivors Read More »

The Paradigms of the 50s

POST-POLIO SEQUELAE AND THE PARADIGMS OF THE 50’s:

Newtie, Ozzie and Harriet versus Paradigms of Caring and a Future for Rehabilitation in America.

Richard L. Bruno, Ph.D.

The 45th annual John Stanley Coulter Memorial Lecture presented to the American Congress of Rehabilitation Medicine, Alexandria, Virginia, June 24, 1995

Archives of Physical Medicine and Rehabilitation, 76 (12): 1093-1096.

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

Post-Polio Rehabilitation and Research Service

Kessler Institute for Rehabilitation

Saddle Brook, New Jersey

and

Department of Physical Medicine and Rehabilitation

New Jersey Medical School/UMDNJ

Newark, New Jersey

Please address correspondence to: Dr. Richard L. Bruno, Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, Saddle Brook, New Jersey 07663.

POST-POLIO SEQUELAE AND THE PARADIGMS OF THE 50’s: Newtie, Ozzie and Harriet versus Paradigms of Caring and a Future for Rehabilitation in America.

Richard L. Bruno, Ph.D.

We find ourselves at an extremely interesting and extremely extreme point in our nation’s history. The pendulum of what American’s believe government should do has swung all the way from FDR’s New Deal, gaining momentum as it flew past LBJ’s Great Society to hit Bill Clinton squarely between the eyes. In listening to the political rhetoric since last year’s Republican coup, I believe that not only have the times changed but also that time itself has changed. I have the feeling it is not June 24, 1995. It feels to me as if the clock has been turned back exactly forty years. So, put on your poodle skirts, slick back your D.A. and let’s return to those thrilling days of yesteryear so we can view the childhood and adolescence of rehabilitation through the eyes of those who have grown up with it: the survivors of America’s polio epidemics. Let’s see what lessons have been learned, now that both the polio poster children and rehabilitation have reached mid-life, and ask this most important question: Given the current ideological timewarp, will polio survivors – will rehabilitation itself – have any future at all, let alone enjoy their golden years?

FRIDAY, JUNE 24, 1955.

So it’s Friday, June 24, 1955, and everyone likes Ike in these 48 United States. To date, only a California politician named Douglas and the victims of Joe McCarthy hate our Vice-President. Bob Dole is a practicing lawyer in Russell, Kansas ten years after his spinal cord was damaged during a mortar attack in Italy. Little Newtie Gingrich turned twelve just last Friday. I see him praying fervently in his sixth grade classroom that God make Congress understand that U.S. troops should not be keeping the peace in Korea.

In 1955, polio has been at the forefront of our nation’s consciousness for more than twenty years. Rehabilitation professionals are intimate with the literature on polio, its symptoms, pathophysiology and treatment. Two years ago, they read in J.A.M.A. that at least 39 percent of those having had non-paralytic polio showed measurable weakness in at least one muscle group, and 43 percent reported “increased fatiguability,” as much as six years after their acute polio [1,2]

These findings were not surprising. As early as 1940, David Bodian concluded that at least 60 percent of motor neurons had to be killed by the poliovirus for there to be any symptoms of weakness. But, even more important, Bodian found that poliomyelitis was an afterthought of the poliovirus. He concluded that an encephalitis exists whenever the poliovirus enters the CNS “whether symptoms (of paralysis) are present or not.” [3] This polioencephalitis was found to consistently and often severely damage specific brain regions: the reticular formation, hypothalamus – even the basal ganglia. [4] This damage was invoked by Bodian and others to explain the “fatiguability and fleeting attention” that were seen to persist for months after the acute poliovirus infection. [5]

Unfortunately, these important facts about polio, along with many others, are about to be erased from the consciousness of rehabilitation professionals. Why?

Because it’s 1955 and we believe in The Paradigm of Cure. Americans believe we have ‘cured’ polio by finding an effective preventative. Seven million Americans have thus far been inoculated with the new Salk vaccine. Last week, only 266 cases of polio were reported, the lowest weekly total since 1951. 43,000 cases of polio will be reported this year, down from the record high of 80,000 in 1952. Still, it will take until 1960 for the annual number of polio cases to dip below 10,000.

It’s 1955, and we act according to The Us versus Them Paradigm. Today the House of Representatives unanimously passed a resolution condemning “colonialism and Soviet imperialism.” The man on the street will angrily tell you that Americans are superior to ‘those Godless Commies’ and that it is better to be ‘dead than red.’

And, the ubiquitous symbol of ‘them,’ the adorably pitiful poster children of the National Foundation for Infantile Paralysis, have done their job well. Clad in heavy metal braces, leaning tenuously on their crutches, they called down from their posters to America’s mothers and fathers, saying: “Give money to find the polio vaccine. Don’t let your child become crippled, like me!” But, it’s 1955 and the vaccine has been found. The poster children are needed no more.

In 1955 rehabilitation is provided according to The Institutional Paradigm. To house and treat the thousands who contract polio each year, special hospitals have been built. Children are ripped away from their families and admitted for months, sometimes years. Huge wards provide assembly-line care as scores of scores of polio survivors are fed, bathed and treated. Parental visits, which always disrupt the hospital routine, are allowed rarely, briefly or not at all. Patients are slapped or beaten with rubber truncheons by therapists to ‘motivate’ them to stand up and walk. Patient’s tears or an angry word – triggered by pain, fright, homesickness, or even a nightmare – not infrequently result in the application of a straight jacket or being locked in a dark closet for the night.

It’s 1955, and we also practice The Paradigm of Shame and Shunning. Much is shameful in 1955 and many are shunned. Young, pregnant women are regularly sent away to homes for wayward girls or to back alleys. A ‘Gentleman’s Agreement’ is still in effect, and many Americans remain in the closet while others are allowed to ride only in the back of the bus.

So it is not surprising that merely having had polio, let alone not recovering completely from paralysis, are cause for both shame and shunning. Polio survivors and their entire families are often shunned for having been tainted by the ‘dreaded disease.’ Braces and crutches are certainly cause for shunning, repulsive reminders of our own physical vulnerability and of the medical profession’s shame at its failure to actually cure polio.

Finally, and most distressing, in 1955 we believe in The Paradigm of Normalcy. Tonight, at 8:00 on A.B.C, a cardigan-clad Ozzie Nelson will sit in his (and our) living room while wife Harriet waxes the kitchen floor wearing high-heels and pearls. Sons Rickie and David will come running in from a sock hop. Needless to say, no character on “The Adventures of Ozzie and Harriet” will be seen to have had polio or to have any other disability.

Thus polio survivors are taught that if they are to be accepted back into society, they must become ‘normal’ again. They must work hard and then harder in physical therapy to rid themselves of the wooden and steel stigmata of the terror that is polio. If they are to function in a totally inaccessible world, even paraplegic polio survivors must be able to walk.

So, polio survivors do work hard, harder than anyone ever has. They discard their braces and crutches. Then they go even further. They go to college, when other Americans only finish high school. They marry and have children. They work more hours of overtime and take fewer sick days than non-disabled workers. [6] They are hard-driving, overachieving perfectionists who become the leaders of their communities, their professions, the chief executives of the largest corporations. [7,8] Normal? Polio survivors transcend mere normalcy to become America’s best and brightest as our country’s clock runs forward.

THE CLOCK RUNS FORWARD . . .

During the next decades America – along with its 1.63 million polio survivors – happily forget about polio as we experience wonderful advances in rehabilitation and violent changes in our society:

The domino theory is displaced by detent and followed by dissolution of the Soviet empire; Wooden legs become polypropylene prostheses;

Our leaders and our innocence are assassinated again, and again, and again;

Wheelchairs once wicker and wood become carbon composite;

The War Against Poverty is overshadowed by the Cold War become hot in Vietnam; Paternalistic institutions become independent living centers;

Richard Nixon, Vice-President, becomes President, unindicted co-conspirator, pariah and finally, inexplicably, elder statesman;

Charity appeals for ‘those poor, pitiful cripples’ are finally made unacceptable by the demands of people with disabilities for their civil rights;

Multidisciplinary, becomes interdisciplinary becomes managed care;

The Voting Rights Act is followed by Section 504 and finally by the A.D.A.

. . . AND THEN THE CLOCK RUNS BACKWARD

But, in 1980 America’s clock stopped moving forward and began to actually run backward. An old rhetorical cold warrior became President by asking us to remember how happy we were in 1955 watching – and he believed living – “The Adventures of Ozzie and Harriet.”

But, for America’s polio survivors, the clock had already been running backward. They were being reminded of a 1955 that was not happy. Forty years after polio, their bodies were beginning to break down as a direct result of the paradigms of the 50’s. Society’s Paradigm of Normalcy, applied so successfully by polio survivors, was now extracting its price in disabling fatigue, muscle weakness and pain. [9] Polio survivors were losing the very abilities that allowed them to appear normal, that prevented shame and shunning, that freed them from institutions and allowed them to pass for normal, to become one of ‘us.’

But today, in 1995, the plight of polio survivors is of no interest to America. Why? Because it’s 1955 again. Polio has been conquered and the poster children are gone. “Forget about polio,” says our society. The National Foundation for Infantile Paralysis, now the March of Dimes Birth Defects Foundation, was asked to fund research on Post-Polio Sequelae ( PPS). “No,” said the March of Dimes, “Polio is gone. We’re curing birth defects now. If we help polio survivors, our donors will become confused. Why don’t you ask Congress for help with this orphan disease?”

So, every year since 1985 Congress has been asked to set aside funds for research on PPS, just one dollar for each polio survivor. And every year Congress says, “No. There will be no set aside. The few remaining polio survivors will be dead soon anyway. Why don’t you study an important disease or ask a private foundation for research funding?”

Of course, the ‘few remaining polio survivors’ conservatively number more than one and a half million. Polio is still the second most frequent cause of paralysis, after stroke. [10] And, polio survivors are hardly moribund; our typical patient is a working mother of 48. Polio survivors are still America’s best and brightest, both the foundation and the leaders of our society. But these facts are of no importance. The clock has been turned back and the paradigms of the 50’s have returned:

In 1995, The Paradigm of Normalcy has returned. Conservative Christian, two-parent families are normal. Anything else, to quote House Speaker Gingrich, is “corrupt and bizarre.”

The senior Senator from Kansas is running for President, again. Why? Because of his vision to lead our country forward into the new millennium? No. And not even because he wants to lead America back to the glorious 50’s. According to Senator Dole’s niece, Nancy Poche, “He still has this one

thing he needs to do. He’s still proving that he’s not handicapped.” [11] Bob Dole is clearly following the Paradigm of Normalcy: Crippled is unacceptable, normal is necessary and only achievements above and beyond normal can make ‘the handicapped’ acceptable to society. As we have seen once before in this century, only being elected President of the United States can convince a disabled man that he is normal again. [12]

In 1995, The Paradigm of Shame has also returned. Americans should be shamed into changing their circumstances, or at least their behavior. The homeless are prostitutes, alcoholics and drug addicts who should be ashamed of their lives and ‘pull themselves up by their boot straps.’ Irresponsible single women should be shamed into abstaining from sex so as not to become pregnant out of wedlock and end up on the dole.

But if shame doesn’t change behavior The Institutional Paradigm should again be applied. The Poor House should be rebuilt; children should be removed from their shameful single mothers and raised in orphanages.

The Institutional Paradigm can also be used as an adjunct to the 1950’s tried and true Paradigm of Cure. Those having a disease whose effects leave them unable to contribute meaningfully to the economy should not be allowed to place a drain on the free market. All forms of “compassionate excess,” to use the House Speaker’s phrase, should be eliminated : i.e., Medicaid, Medicare, SSI, SSDI, and especially funds for rehabilitation. Even today, Medicare will only cover outpatient rehabilitation if an individual is ‘falling or unsafe,’ the maxim being don’t let them die, but don’t help them regain function, either. This current policy is tragic for those whose PPS have forced them to go out on disability but who want to regain function and return to work.

But, you know, it would be much less expensive to forgo rehabilitation completely and simply institutionalize all of those too disabled to work. This will be an about-face from current policy, where the only people who shouldn’t be institutionalized are the homeless and the recently reinstitutionalized mentally ill. But, such a policy reversal could be a deficit busting bonanza if we heed the advice of New York’s Governor and require that residents of homeless shelters pay rent. Imagine the tremendous cost savings to society if the government stops paying for rehabilitation and actually makes money by collecting rent from those it institutionalizes.

Finally, in 1995, The Us versus Them Paradigm has returned. In a remarkable echo from the 1950’s, House majority leader Dick Armey published a letter last May black-balling left-leaning charities. Principle among these communist organizations was the American Cancer Society, tarred as an enemy of the free-market because it opposes the sale of cigarettes.

Representative Armey and his troops have also stated that everyone who is not ‘us’ – that is white, working, born-again, non-disabled males – is not a ‘true’ American and should be regulated – that is shamed, cured or institutionalized – into normalcy. However, all regulation of ‘us’ – that is white, business-owning, non-disabled males – should be removed. Clean air and water statutes, safe food regulations, and especially OSHA (including its new repetitive motion disorder guidelines) should be eliminated because they constrain the operation of the free market. Can repeal of workers’ compensation, child labor laws and the spendthrift A.D.A. be far behind?

But think about it. The elimination of regulation should be a boon to the medical industry in general and rehabilitation in particular. Just think of how much carpal tunnel syndrome we will be treating. However, the people most affected by eliminating regulation will be ‘them:’ workers with no clout, no voice, no cash to pay for a $500-a-plate campaign fund raiser for Congressman Armey. Their

medical insurance will have been lost along with their jobs and government funding will no longer be available to pay for rehabilitation. There will be nothing left for ‘them’ to do but beg for spare change to pay rent at the shelter.

SATURDAY JUNE 24, 1995

It is vital that each one of us remembers it is not 1955. No matter how loudly the strident and self-assured voices across the Potomac proclaim that they are restoring the old world order, the clock has not been turned back that far . . . at least not yet. It is still 1995, where we find ourselves at an extremely dangerous point in the life of our nation. The pendulum of what Americans believe government should do and our nation should be is swinging back to a simpler and more simplistic time. Will polio survivors and rehabilitation have any future, let alone their golden years? It is up to us, as individuals, rehabilitation practitioners, and members of this Congress, to insure the future by not allowing the pendulum to swing back and by preventing America from reverting to the paradigms of the 50’s.

We must prevent a return to The Paradigm of Normalcy and instead embrace Paradigms of Quality. Rehabilitation is the one specialty where normalcy – that is the absence of disease or its symptoms – is not the goal. Rehabilitation’s focus on function, on nothing less than quality of life is uniquely ours as a specialty. But, promoting quality is not easy. Recommending that our post-polio patients use new ‘abnormal-looking’ assistive devices is emotionally painful, both for our patients and for us on the treatment team. However, it is only this departure from 40 years of ‘normalcy’ that allows our patients to regain function and maintain their quality of life.

Further, promoting Paradigms of Quality will have effects far beyond our clinics. Our philosophy of quality of life places us in the eye of the vitriolic hurricane of debate about life itself. Should an elevated a – fetoprotein be an automatic criterion for abortion because no child with spina bifida could possibly have quality of life? Should a C2 quad, like Christopher Reeve, or woman in Wisconsin with CP or the paraplegic director of a post-polio service – be allowed or even assisted to die because having any disability is, as one insurance company describes it, “a living death?” [13]

Our unique experience and philosophy can help this society make reasoned decisions about whether life should end when its quality is gone. In the process, we will begin to eliminate society’s notion of disability as “a living death.” [14,15]

Further, if we reject The Paradigm of Normalcy we will also be eliminating The Paradigm of Shame. It must no longer be shameful in America to have a disability. We must end society’s view of people with disabilities as ‘them.’ We must act together to finally end Jerry Lewis’ demeaning Labor Day spectacle. We must ask well-meaning A.C.R.M. members to stop injury prevention programs that send former SCI and TBI patients to high schools and Rotary Clubs as a warning to careless drivers and careless divers. The message of these programs is the same as that of the polio poster children forty years ago: “Don’t become crippled, like me. Don’t become one of the living dead.”

We must also continue to fight The Institutional Paradigm as we have for more than a generation. We must rededicate ourselves to self-advocacy, self-determination and independent living – to Roberts Rules if you will – in memory of polio survivor and vocational rehabilitation pioneer Ed Roberts.

Finally, we must reject The Paradigm of Cure and replace it with Paradigms of Care and, even more, with Paradigms of Caring. We rehabilitation professionals must be the voice of caring in our society, demanding that Congress set priorities for policy beyond merely cutting the budget deficit.

We must state clearly that assuring medical care for all Americans is not the redistribution of wealth, but the redistribution of health; that managed care is not desirable merely because it manages to cut costs. We must demand that the emphasis always be on care that is also carefully managed. We must state unequivocally that medical care is an American’s first right, the right that makes possible not only life, but also liberty and the pursuit of happiness.

In 1995, at this extreme point in the life of our nation, we have three choices: We can react, revert and return to “The Adventures of Ozzie and Harriet;” we can remain in the present, recline and retire with “Rosanne” and “ER;” or, we can can reject ‘us versus them,’ normalcy, shame, inhuman institutions and simplistic cures. We can remember our past, remake our present and restore the possibility of a future – and even golden years – for polio survivors and for rehabilitation in America.

REFERENCES

Mascots E, Kaplan LI. Follow-up studies in seventy-five cases of nonparalytic poliomyelitis. Journal of the American Medical Association 1953; 152:1505-1506.
Shaw EB, Levin M. The infrequent incidence of nonparalytic poliomyelitis. Journal of Pediatrics 1954; 44: 237-243.
Howe, HA, Bodian D. Neural Mechanisms of Poliomyelitis. New York: The Commonwealth Fund, 1942.
Bruno RL, Frick NM, Cohen, J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae. Orthopedics 1991; 14 (11): 1269-1276. [Lincolnshire Library Full Text]
Bruno RL, Sapolsky R, Zimmerman JR, Frick NM. The pathophysiology of a central cause of post polio fatigue. Annals of the New York Academy of Sciences 1995; 753: 257 – 275. [Lincolnshire Library Full Text]
Lonnberg, F. Late onset polio sequelae in Denmark. Scand. J. Rehab. Med. 1993; Suppl. 28: 1-32. [PubMed Abstract]
Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae. 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 Library Full Text]
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 Library Full Text]
Frick NM, Bruno RL. Post-Polio Sequelae: Physiological and psychological overview. Rehabilitation Literature 1986; 47 (5-6): 106-111. [Lincolnshire Library Full Text] 10. Parsons PE. Data on polio survivors from the National Health Interview Survey. Washington, D.C.: National Center for Health Statistics, 1989.
Roberts SV. A warhorse’s toughest fight. U.S. News and World Report 1995; 118: 40-45. 12. Gallagher HG. FDR’s Splendid Deception. Arlington: Vandamere Press, 1994. 13. Gallagher HG. By trust betrayed: Patients, physicians and the license to kill in the Third Reich. New York: Henry Holt and Company, 1990
Singer, P. Rethinking life and death. New York: St. Martin’s Press, 1995.
Hendin, H. Suicide in America. New York: W.W. Norton, 1995.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

The Secretary, Lincolnshire Post-Polio Network

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

Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk

Web Site: www.lincolnshirepostpolio.org.uk

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/coulter.html> Created: 16th January 1997

Last modification: 29th January 2010.

The Paradigms of the 50s Read More »

PPS Questions and Answers

PPS QUESTIONS AND ANSWERS, MARCH, 1995 by Drs Richard Bruno and Nancy Frick

Compiled by Tom Walter

From America Online computer information service PPS Bulletin Board

PLEASE NOTE: READ ME FIRST!

These general answers and our on-line discussion on 3/7/95 represent what is written in the medical literature on PPS, our newest research and how we treat patients at the Kessler Post-Polio Service. They are NOT intended as therapeutic recommendations for you personally nor as a substitute for your being evaluated by your own personal doctor and a doctor who knows about PPS.

Every polio survivor (PS) is different and prescribed treatments must take those differences into account.

Post-Polio Sequelae (PPS) is a diagnosis of exclusion, meaning a physician must evaluate you and determine that no other condition is causing your symptoms. You may have PPS alone, PPS and another problem or a condition not related to polio at all. There are no medical tests that can prove you have PPS.

Read these answers, read the journal articles listed with each, and understand them fully. Take them to your doctor or therapist and ask them to read and understand them fully. Do not apply therapeutic techniques described here (especially exercise) on your own.

We very much hope these answers serve an educational purpose and that you integrate them with your own experience, personal wisdom and doctors’ recommendations so that you can thrive, not just survive, with PPS!

Best wishes, Drs. Richard Bruno and Nancy Frick

ABOUT PPS PREVENTION & PROGRESSION

There are no long-term studies of whether Polio Survivors (PS) who have no symptoms will develop PPS. Every time a study of PS is done, the percentage with new problems increases. The percentage was 22.4% in the first Mayo clinic study in 1982 and increased to 78% in a British polio hospital follow-up in 1987. At the NY Academy of Sciences Symposium on PPS April, 1994, the Mayo group and others thought that more than 90% of PS would develop some problems related to their polio.

Every PS is different. But, the rules that apply to every mortal apply to PS. Anyone who has too few

overworked, damaged nerves compensating for muscle weakness for over 40 years is bound to have some problems. And, when you.add PS super Type A lifestyles and the fact the they work more hours of overtime than non-disabled people, something’s got to give.

But, the good news it that the study cited below and our new follow-up study of all the patients we have treated show that if you listen to your body and stop doing the things that cause weakness, fatigue and pain, PPS often plateau and can even decrease (see below: Exercise & Activity). One example: 18 months after their evaluation with us, PS who completed therapy reported 22% LESS fatigue; however, PS who refused treatment or dropped out of therapy early had 21% MORE fatigue at 18 months.

References

Peach PE, Olejnik S Effect of treatment and non-compliance on Post-Polio Sequelae. ORTHOPEDICS, 1991, 14(11) : 1199. [Lincolnshire Library Full Text]

ABOUT EXERCISE & ACTIVITY

Exercise has been the most hotly debated area in PPS. The studies cited below say three things:

Listen to your body! If you are doing things that cause fatigue, weakness or pain STOP DOING THEM!!! PS need to pace their activities, that is work and then rest about 2 times the amount they worked. Jim Agre showed that PS can do 240% more work if they PACE. You don’t drive your car until it’s out of gas; you shouldn’t drive yourself to exhaustion, weakness or pain;
We only prescribe the non-fatiguing exercises devised by Rubin Feldman that have been shown not to hurt PS. But, these exercises are not given to all patients and are only prescribed after PS learn to pace and conserve energy! Patients are usually given gentle exercise after they get a new brace to keep muscle tone. The key word is NON-FATIGUING;
We have seen a small handful of PS who became deconditioned after surgery or illness. Pacing and resting doesn’t mean sitting or sleeping all day and not moving. You should be doing what you need to do for yourself, and at your job, but in a paced, energy conserving, Type B fashion.

In our follow-up of all the patients we have treated, the three factors that were related to a significant decrease in fatigue were

completing the PPS therapy program;
doing absolutely nothing for 15 minutes twice a day;
using a wheelchair or scooter for distances.

References

Young GR. Energy conservation, occupational therapy and the treatment of Post-Polio Sequelae. ORTHOPEDICS, 1991, 14(11): 1233. [Lincolnshire Library Full Text]

Feldman RM, Soskolne CL. The use of non-fatiguing strengthening exercises in Post-Polio Syndrome. In LS Halstead and DO Wiechers (Eds) Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains: March of Dimes Birth Defects Foundation, 1987. [PubMed Abstract]

Fillyaw MJ, et al. The effects of long-term non-fatiguing resistance exercise in subjects with Post-Polio Syndrome. ORTHOPEDICS,1991,14(11):1253. [PubMed Abstract]

Agre JC and Rodriguez AA. Neuromuscular function in polio survivors. ORTHOPEDICS, 1991, 14(12): 1343. [PubMed Abstract]

ABOUT FEET & LEGS

PS are notorious for having cold and purple “polio feet,” caused in part by the smooth muscle around the veins being partially paralysed by the original polio. Without muscle to control their size, veins fill with blood and cause your feet to appear purple. Your feet become cold because the heat in this pooled blood

escapes into the air. Back in 1983, we found that PS’s nerves and veins act as if it’s 20 degrees colder than the air, making it hard for the nerves, muscles and connective tissues to work. PS lost 75% of their muscle strength when the room temperature dropped from 85 to 65 degrees Fahrenheit.

Cold is the #2 cause of muscle weakness in PS but is the easiest to treat. We suggest that our patients take a bath in the morning, dry off and put on Polypropylene socks or long johns while they are still warm. Polypropylene (trade name: Gortex, Thinsulate) is a silk-like plastic that holds heat in but allows sweating.

Also, engorged veins can cause swelling, especially when feet get hot in Summer or after a long bath. Jobst compression stockings sometimes help, as well as keeping your feet up a lot during the day. But, leg swelling must be evaluated by your doctor!

References

Bruno RL, Johnson JC, Berman WS. Vasomotor abnormalities as Post-Polio Sequelae. ORTHOPEDICS, 1985, 8 (7): 865-869. [PubMed Abstract]

ABOUT GETTING OTHER DISEASES

PS can get all the diseases everyone else gets. That’s why your doctor has to exclude all other causes for your new symptoms before you settle on PPS. In our 1985 National Post-Polio Survey we discovered that PS were on average more Type A – pressured, time-conscious, overachieving, perfectionistic than any other group of Americans, including those who already had had heart attacks. However, PS were no more likely to have heart attacks or high blood pressure than anyone else.

We found that PS also had 3 to 6 times more trouble with gut problems diarrhoea, constipation, ulcer and colitis – as compared to the general population. PS also have more headaches (but not migraines) and muscle pain (often called Fibromyalgia). And, there is also one study that suggests that more PS have hypothyroidism.

Also, 66% of PS report frequent anxiety and 31% of those who see us for evaluation have a Major Depressive Episode – that’s 6 times the rate for the general population. Both old and new types of antidepressants are effective if prescribed for PS. Again, your doctor needs to evaluate any and all new Syrnptoms!

References

Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae: The Felician/Columbia Survey. In LS Halstead and DO Wiechers (Eds.): Research & Clinical Aspects of the Late Effects of Poliomyelitis. White Plains: March of Dimes Research Foundation, 1987. [Lincolnshire

Library Full Text]

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 Library Full Text]

Halstead LS. Assessment and differential diagnosis for Post-Polio Syndrome. ORTHOPEDICS, 1991, 14(1l): 1209. [Lincolnshire Library Full Text]

ABOUT DRUGS FOR PPS

A number of drug studies were presented at the NY Academy of Sciences Symposium on PPS in April, 1994. Prednisone (a steroid) and Amantidine were tried without success to treat PPS weakness and fatigue. Growth hormone was not found to be helpful to treat new muscle weakness in one paper, but a multi-center study is beginning.

Neal Cashman again reported on the use of Mestinon to treat muscle fatigue. He found that a portion of PS whose motor nerves don’t communicate well with muscles report a decrease in muscle fatigue while using Mestinon. However, the effect of Mestinon seems to wear off over time.

We presented our pilot study of a drug to treat PPS brain fatigue. In the 10% of our patients whose fatigue did not improve following the standard treatment for PPS (see above: Exercise and Activity), 60% reported less morning fatigue and less trouble staying awake on drug versus placebo. However, even if drugs are found that help, PS must still listen to their bodies and live their lives in a paced, energy conserving, Type B fashion. There is no magic pill.

References

Bruno RL, Frick NM, Lewis T, Creange SJ. The physiology of post-polio fatigue: A model for post-viral fatigue syndromes and a brain fatigue generator. CFIDS Chronicle, 1994, 7(4): 36-42.

ABOUT MUSCLE TWITCHING

In the 1985 National PPS Survey, we found that 63% of all PS report that their muscles twitch and jump as they fall asleep; 33% reported that their sleep was disturbed by twitching. This sleep disorder, called Generalized Random Myoclonus, is often only noticed by the PS’s bed partner. In doing sleep studies, we found that a small dose of Ativan before bed usually stops the movements and restores sleep.

Other sleep disorders, such as sleep apnea, are not uncommon in PS. If you snore, wake not rested, with a headache or are depressed, you should talk to your doctor about a sleep study.

Many PS report another kind of muscle movement during the day: fasiculations. These are muscle twitches you can see or feel. Fasiculations are found in many non-disabled people. Usually, they are a sign, as is muscle pain, of muscle overuse. Again, talk to your doctor about any twitching.

References

Library Full Text]

Bruno RL, Frick NM, Creange SJ. Nocturnal generalized myoclonus as a post-polio sequelae. Archives of Physical Medicine and Rehabilitation, 1995, (in press).

Bach JR and Alba AA. Pulmonary dysfunction and sleep disordered breathing as Post-Polio Sequelae: Evaluation and management. ORTHOPEDICS,1991,14(12):1329. [Lincolnshire Library Full Text]

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

The Secretary, Lincolnshire Post-Polio Network

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

Telephone: +44 (0)1522 888601

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Email: info@lincolnshirepostpolio.org.uk

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Last modification: 30th January 2010.

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Neuroanatomy of Post-Polio Fatigue

THE NEUROANATOMY OF POST-POLIO FATIGUE

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

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

1. Post-Polio Rehabilitation and Research Service

and

Department of Psychology Kessler Institute for Rehabilitation East Orange, New Jersey

and

Department of Physical Medicine and Rehabilitation UMDNJ/New Jersey Medical School

Newark, New Jersey

2. Medical Imaging Center of the Oranges

West Orange, New Jersey

3. Harvest Center

Hackensack, New Jersey

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

Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation,

240 Central Avenue,

East Orange, New Jersey 07018.

(201) 731-3600 (extension 547).

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This research was supported by grants from the George Ohl, Jr. Foundation and the Joel Leff Charitable Trust.

Running Head: Neuroanatomy of Post-Polio Fatigue. THE NEUROANATOMY OF POST-POLIO FATIGUE

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

ABSTRACT

Fatigue is the most commonly reported, most debilitating and most poorly understood Post-Polio Sequelae (PPS). Postmortem studies of fifty years ago documented frequent and severe poliovirusinduced lesions within the reticular activating system (RAS). Recently, neuropsychological testing has documented marked attention deficits in polio survivors reporting severe fatigue. However, neither of these findings has been related to the pathophysiology of post-polio fatigue. Magnetic resonance imaging of the brain was performed in 22 polio survivors carefully screened to eliminate the effect of comorbidities. Subjects rated the severity of their daily fatigue and subjective problems with attention, cognition and memory. Small discrete or multiple punctate areas of hyperintense signal (HS) in the reticular formation, putamen, medial leminiscus or white matter tracts were imaged in 55% of the subjects reporting high fatigue and in none those reporting low fatigue. The presence of HS significantly correlated with fatigue severity and subjective problems in attention, concentration, staying awake, recent memory and thinking clearly. The lack of significant correlations between HS or fatigue severity and age, severity of the acute polio, depressive symptoms or difficulty sleeping militates against these factors as either causing HS or producing fatigue. These preliminary findings suggest that poliovirus-induced lesions in the RAS may underlie the subjective fatigue and attention deficits associated with PPS fatigue.

Polio; Post-Polio Sequelae; Magnetic Resonance Imaging; Fatigue; Attention

THE NEUROANATOMY OF POST-POLIO FATIGUE

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

Fatigue is the most commonly reported, most debilitating and most poorly understood Post-Polio Sequelae (PPS) affecting the more than 1.63 million American polio survivors (1). In the 1985 National Survey of 676 polio survivors, 91% reported new or increased fatigue, 41% reported fatigue significantly interfering with performing or completing their work and 25% reported fatigue interfering with self-care activities (2).

Importantly, polio survivors differentiate between physical tiredness that they associate with new muscles weakness and decreased physical endurance, and ‘brain fatigue’ that is characterized by

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problems with attention and cognition. In the 1990 National Survey of 373 polio survivors, between 70% and 96% of respondents reported that fatigue was accompanied by problems with concentration, memory, attention, word-finding, maintaining wakefulness and thinking clearly, with 77% reporting moderate to severe difficulty with these functions (3). These problems with attention and cognition suggest that the symptoms of post-polio fatigue cannot be explained merely by poliovirus-induced damaged to anterior horn motor neurons (4). Postmortem histopathology from nearly fifty years ago demonstrated the consistent presence of poliovirus lesions in specific brain areas. Brainstem centers were found to be “involved in even mild cases” of polio (5), with Guizetti (6) stating in 1933 that “only one area is always severely altered (by the poliovirus:) the so-called substantia reticularis,” i.e. the midbrain reticular formation. This conclusion was supported by others who found the reticular formation to be “heavily peppered throughout” (7,8,9,10) with lesions that were “very common and often severe” (7), with Luhan stating that it was the brain area “most uniformly involved” (11) following poliovirus infection.

In addition to the reticular formation, neurosecretory neurons were damaged or destroyed by the poliovirus, including the periaquiductal gray (5,8), locus ceruleus (11), median raphe nuclei (10) and substantia nigra (5,9,10,11). Hypothalamic lesions were also documented in both the paraventricular nucleus and preoptic area, with the posterior region thought to be the most severely damaged (11). Lesions were also noted in lateral, median and midline thalamic nuclei as well as in the putamen, caudate and septal nuclei (12) Cortical lesions, while frequent and severe, were limited only to the motor and pre-motor areas (7,12).

These findings indicate that the poliovirus consistently and often severely damaged brain areas responsible for cortical activation and attention – the reticular formation (13,14), posterior hypothalamus (15), thalamus (16), as well as the putamen, caudate (16,17,18), locus ceruleus and substantia nigra (17,19,20,21) – which comprise the Reticular Activating System (RAS). Clinical reports written during the polio epidemics seem to corroborate the pathological evidence of poliovirus damage to the RAS since “drowsiness” and even coma were described as sequelae of the acute poliovirus infection (7,12,22). Even months after the acute episode, Meyer (23) 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.”

Poliovirus-induced damage to the RAS impairing cortical activation has also been hypothesized as a cause of late-onset fatigue and subjective problems with attention in polio survivors (3). This hypothesis has received some empirical support. Clinically significant deficits on neuropsychological tests measuring attention, concentration and information processing speed were documented only in polio survivors reporting severe fatigue and not in subjects reporting mild fatigue (24). Importantly, no impairment of higher-order cognitive ability or verbal memory was seen in any of the post-polio subjects.

In additional, small discrete and multiple punctate areas of hyperintense signal (HS) were imaged in the thalamus, caudate nucleus, centrum semiovale, deep and periventricular white matter in eleven of twelve polio survivors who underwent magnetic resonance imaging of the brain (3). These areas of HS were interpreted as evidence of damage to the RAS and its associated

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corticofugal white matter tracts caused by the original poliovirus infection. However, no attempt was made to correlate HS with reports of fatigue severity nor were variables controlled for that have been associated with HS, e.g. advanced age, hypertension and depression (25,26).

The purpose of this study was to test the hypothesis that areas of hyperintense signal on MRI of the brain are associated with late-onset fatigue and subjective problems with attention in polio survivors.

METHODS

Subjects. Volunteer subjects were recruited through the media, post-polio support groups and by direct mail over two years. One-hundred forty six (146) polio survivors requested and returned survey forms on which they were asked to rate their usual daily level of fatigue on a six point scale from “none” to “severe” and to list their complete medical history, including a description of the effects of the original poliovirus infection.

One-hundred (108) volunteers were excluded as a result of comorbidities that could have caused HS, fatigue or cognitive problems, e.g. a major depressive episode, hypertension, anemia, lupus, diabetes, traumatic brain injury, or thyroid, respiratory, cerebrovascular or cardiac disease. Volunteers were also excluded if they were above 65 years of age or were taking sedating medications for sleep, including antidepressants or benzodiazepines. Of the 38 remaining volunteers, 22 agreed to have an MRI of the brain. All had unequivocal histories of polio and none had received treatment for post-polio fatigue.

Procedure. Subjects completed the Post-Polio Fatigue Questionnaire (3) that requested subjective ratings of their usual daily level of fatigue and the frequency and severity of problems with attention, concentration, staying awake during the day, thinking clearly, sleeping and muscle weakness on a six-point scale from “none” through “severe.” They also completed the brief Type A questionnaire (27) since Type A behavior has been associated with fatigue and cognitive problems in polio survivors (2,3).

After giving written informed consent, an MRI of the brain was performed on a Diasonics MT/S system operating at 0.35 Tesla. A quadrature head coil was employed with spin echo technique. Imaging consisted of sagital and coronal T1 sections with TR=500-600 msec and TE=25/30 msec. Ten millimeter slices were obtained with a 256 x 256 matrix and 0.95 x 0.95 mm resolution. T2 images were performed in the transaxial and coronal planes with a TR=3,000 msec and TE=30 and 80 msec. Five millimeter T2 sections were obtained with a 256 x 256 matrix and 1.0 mm x 1.0 mm resolution. MRI films were read by one of us (JMC) who was blind to the patients symptoms.

Data Analysis. Descriptive statistics, product-moment correlations, linear regression and independent-groups t-tests were performed using the Macintosh version of Statview (28). Since a daily fatigue severity rating of moderate is considered clinically significant, subjects who reported no or mild fatigue were classified has having low fatigue while those reporting fatigue as moderate or higher were classified has having high fatigue.

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Although none of the subjects met DSM-IIIR criteria for an affective disorder, ten subjects reported “frequent low mood” and seven reported that they had been diagnosed in the past as “depressed.” These subjects were all classified as having “depressive symptoms” to control for the possible effect of “low mood” on subjective symptoms of fatigue and because HS have been imaged in elderly patients with depression (26).

RESULTS

MRI Findings. Areas of hyperintense signal in gray and white matter were imaged in eight of the subjects. This 36% incidence of HS is significantly greater (p<0.05) than the 24% incidence that would be expected to occur in a healthy sample with a mean age of 46.7 years drawn from the general population (Figure 1).

All eight subjects with HS reported high fatigue. No areas of HS were seen in subjects reporting low fatigue. However, HS was not imaged in seven (45%) of the subjects reporting high fatigue. In four subjects multiple punctate areas of HS were imaged in the periventricular and deep white matter. In one of these subjects, a small discrete area of HS was also imaged in the right putamen. A single discrete area of HS was imaged in the rostral reticular formation in one subject and in the right medial leminiscus in another. A seventh subject demonstrated three discrete areas of HS in the centrum semiovale, each approximately 5.0 mm² in area (Figure 2). In an eight subject, one discrete area of HS was imaged in the left putamen and a 4.0 x 6.0 mm² area of HS was seen in the centrum semiovale.

Demographics. Subjects with and without HS were equal in terms of age, years of education, year of polio, age at polio and the severity of acute polio as measured by the number of limbs originally paralyzed and being hospitalized by polio (Table 1). Subjects with HS were six years further from the acute polio as well as having a clinically and statistically significant elevation in mean Type A score as compared to those without HS.

Problem Severity. The presence of HS was significantly correlated with fatigue severity, year of acute polio, years since polio and Type A score (Table 2). The presence of HS and fatigue severity were also significantly correlated with the frequency of problems with thinking clearly, mind wandering, staying awake and recent memory, and the severity of problems with thinking clearly, mind wandering, attention, concentration and recent memory. It is noteworthy that the severity of problems with attention was highly correlated with the severity problems with thinking clearly (product-moment r = 0.79), mind wandering (product-moment r = 0.76) and concentration (product-moment r = 0.70).

Problem severity was compared between subjects with and without HS who reported high fatigue (Figure 3). Subjects with HS had elevated ratings for all problems (except sleeping) although only the between-groups difference for the problems with mind wandering (independent groups t-test = 2.39; p = 0.033), attention (independent groups t-test = 2.30; p = 0 .039) and thinking clearly (independent groups t-test = 2.25; p = 0.044) were statistically significant.

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DISCUSSION

These findings of this preliminary study support the hypothesis that areas of hyperintense signal on MRI of the brain are associated with late-onset fatigue and subjective problems with attention in polio survivors. The correlation of HS imaged in the RAS with fatigue severity and problems in attention, concentration and stying awake, in addition to the significantly elevated severity of attention problems in high fatigue subjects with HS, suggest that HS are related to the pathophysiology of post-polio fatigue (24). The lack of correlation between HS or fatigue severity with age, severity of the acute polio, depressive symptoms or difficulty sleeping militates against these factors as either causing HS or producing symptoms of fatigue.

The significant correlation of fatigue severity and HS with problems in thinking clearly and recent memory indicate that subjects’ quantification of fatigue depends on more than just subjective problems with attention. However, subjective problems with cognition and memory in polio survivors may be more perceived than real. Cognition and memory were found to be intact on neuropsychological testing even in post-polio subjects reporting severe fatigue; however, attention and information processing speed in these subjects were significantly impaired (24). Problems with thinking clearly were highly correlated with the severity of problems in attention. Thus, subjective problems in information processing and short term memory may be the result of impaired attention.

Clinical Correlation of Gray Matter HS. The correlations between HS and the symptoms of postpolio fatigue can only be explained by under standing the pathogenesis of HS. HS imaged in the putamen and reticular formation most likely represent areas where astroglia have replaced neurons destroyed during the acute poliovirus infection, since gliosis has been correlated with HS in postmortem examinations of the brains of healthy elderly adults (35). Since damage to the putamen and caudate nucleus (16,17,18) and especially the reticular formation (36) has been shown to cause deficits in attention, poliovirus damage to these areas could conceivably impair attention and produce the symptoms of post-polio fatigue.

The size and distribution of poliovirus lesions in the reticular formation may also explain the absence of HS in 50% of the high fatigue subjects. Bodian stated, “In most of our material and that described by others the lesions in the reticular formation (even) in fatal bulbar cases were heavily peppered throughout this region rather than being predominately composed of large discrete areas of destruction” (5). Furthermore, he stated that only “In a few instances clearly delimited areas of complete neuronal destruction were seen, as large as 1 or 3 mm. in diameter” (5). Since the limit of MRI resolution is 1 mm, HS resulting from poliovirus lesions will only infrequently be large enough to be imaged by MRI and will be seen only in those polio survivors having both severe and confluent damage.

If HS are indicative of reticular formation damage that dates from the original poliovirus infection, the emergence of symptoms of fatigue more than thirty years later must be explained. The acute poliovirus infection is thought to have destroyed as many as fifty percent of spinal motor neurons in infected individuals (37). Even if a similar percentage of reticular formation neurons was also destroyed, persistent fatigue and attention deficits may have been prevented by inherent neuronal redundancy and the sprouting of axons such as that which was seen in motor neurons following

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poliomyelitis (4). With aging, the cell bodies of reticular formation neurons may become distorted and dendritic shafts may be lost, changes seen in animal reticular formation neurons that resemble degenerative changes in aging human cortex (38). Thus, the age-related degeneration and possibly attrition of reticular formation neurons, combined with an already diminished neuronal pool, may degrade RAS functioning sufficiently to impair attention and produce symptoms of fatigue as polio survivors reach mid-life.

Clinical Correlation of White Matter HS. Bodian has suggested that damage to other than gray neurons may result from a local reaction of tissue to the passage of the poliovirus. For example, lymphocytic infiltrations following the acute poliovirus 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” (39). Bodian suggested that the combination of this “severe reaction to virus and local circulatory embarrassment” may be the cause of local tissue damage. Since HS were imaged along white matter tracts that have been implicated in the centrifugal spread of the poliovirus (40,41), it is conceivable that poliovirus may have damaged the brain parenchma and produced both enlarged, fluid-filled spaces around arterioles and the local atrophy and degeneration of myelinated axons that have been correlated with HS in postmortem examinations of the brains of healthy elderly adults (35,42).

Diffuse atrophy and degeneration of myelinated axons within corticofugal white matter tracts could conceivably impair transmission, decrease cortical activation and thereby cause the attention deficits and symptoms of fatigue. This notion is supported by a number of other studies that have documented a relationship between HS, impaired attention and fatigue. Periventricular and deep white matter HS have been associated with impairments of attention and information processing speed in demented (43,44) and non-demented (34,45,46,47) elderly adults, impairments similar to those documented in polio survivors reporting severe fatigue (24). In addition, eighty-two percent of patients with Chronic Fatigue Syndrome (CFS) reported subjective problems in attention (29) with impaired attention being the “most pronounced and frequent deficit” seen on neuropsychological testing (30). Between 40% and 100% of CFS patients demonstrate periventricular and deep white matter HS on MRI (29,30). Thus, there may be a common pathophysiology for attention deficits and putative ‘post-viral’ fatigue syndromes.

Conclusion. The imaging of HS in brain areas severely and frequently affected by the poliovirus suggests that a poliovirus-damaged RAS is responsible for the documented attention deficits (24) and subjective symptoms of ‘brain fatigue’ reported by polio survivors.

However, there are several caveats. First, only the correlation of fatigue, subjective problems and impaired attention documented by neuropsychological testing with postmortem histopathology will provide an adequate test of this hypothesis. Since postmortem histopathology is difficult to obtain, the correlation of symptoms and neuropsychological test results with the results of imaging techniques that quantify brain functioning (e.g., measurement of local cerebral blood flow or positron emition tomography) might be an interim methodology.

Second, these preliminary findings need to be replicated on a larger sample that represents a crosssection of the post-polio population. Participants in all studies of PPS are likely to have more

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severe symptoms than are non-participants. Thus, polio survivors without symptoms will be under represented. A larger sample would reveal HS in some percentage of polio survivors who do not report fatigue or problems with attention (see Figure 1). This percentage would have to equal that of controls of similar age and be significantly lower than the percentage of HS in polio survivors with fatigue for the hypothesis to be accepted.

Although preliminary, these findings do have clinical implications. First, polio survivors need reassurance that their ‘brain fatigue’ is not hysterical or hypocondriacal, as they have frequently been told (XX), but is real and may have a physiological basis. Second, the current treatments for post-polio fatigue are limiting and only partially effective . Reducing physical and emotional stress (3), energy conservation (48), adequate rest and the pacing of activities (49) do reduce the symptoms of fatigue. Unfortunately, these treatments require polio survivors to significantly reduce their activities. Further, there is a subgroup of polio survivors who report severe and malignant fatigue that is refractory to any of these treatments. Understanding the physiological basis for postpolio fatigue may allow the development of a pharmacological treatment.

REFERENCES

1. Parsons PE. Data on polio survivors from the National Health Interview Survey. Washington,

D.C.: National Center for Health Statistics, 1989

2. 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]

3. Bruno RL, Frick NM, Cohen J. Polioencephalitis, stress and the etiology of post-polio

sequelae. Orthopedics 1991; 14:11 85-93. [Lincolnshire Library Full Text]

4. Trojan D, Gendron D, Cashman NR. Electrophysiology and electrodiagnosis of the postpolio motor unit. Orthopedics 1991; 14 :1353-1361. [Lincolnshire Library Full Text]

5. Bodian D. Poliomyelitis: Neuropathologic observations in relation to motor symptoms. J Am

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6. Guizetti HU. Betrachtungen zur poliomyelitis des hirnstammes. Deutsch Ztschrf Nervenh

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563-578.

8. Barnhart M, Rhines R, McCarter, Magoun, HW. Distribution of lesions of the brain stem in

poliomyelitis. Arch Neurol Psychiatry 1948; 59: 368-377.

9. Matzke HA, Baker AB. Poliomyelitis: A study of the midbrain. Arch Neurol Psychiatry

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10. Peers JH. The pathology of convalescent poliomyelitis in man. Am J Pathol 1942; 19: 673-

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11. Luhan JA. Epidemic poliomyelitis: Some pathological observations on human material.

Arch Pathol 1946; 42: 245-260.

12. Howe HA, Bodian D. Neural Mechanisms of Poliomyelitis. New York: The Commonwealth

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13. Morruzi G, Magoun HW. Brain stem reticular formation and activation of the EEG. EEG

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Clin. Neurophysiol 1949; 1: 455-473.

14. Bentivoglio M, Steriade M. Brainstem-diencephalic circuits as a structural substrate of the ascending reticular activation concept. In Mancia M and Marini G (eds): The Diencephalon and Sleep. New York: Raven, 1990.

15. Sakai K, El Mansari M, Lin JS, Zhang JG, Vanni-Mercier G. The posterior hypothalamus in the regulation of wakefulness and paradoxical sleep. In Mancia M and Marini G (eds): The Diencephalon and Sleep. New York: Raven, 1990.

16. Mesulam M-M. Attention, confusional states, and neglect. In Mesulam M-M (ed): Principles of Behavioral Neurology. Philadelphia: Davis, 1985.

17. Lou HC, Henriksen L, Bruhn P, et al. Striatal dysfunction in attention deficit and hyperkinetic disorder. Arch Neurol 1989; 46: 48-52. [PubMed Abstract]

18. Pribram KH, McGuinness D. Attention and para-attentional processing: Event-related brain potentials as tests of a model. Ann NY Acad Sci; 658: 65-92. [PubMed Abstract]

19. Jones BE. Influence of the brainstem reticular formation, including intrinsic monoaminergic and cholinergic neurons, on forebrain mechanisms of sleep and waking. In Mancia M and Marini G (eds): The Diencephalon and Sleep. New York: Raven, 1990.

20. Robbins TW. Psychopharmacological and neurobiological aspects of the energetics of information processing. In Hockey GRJ, Gaillard AWK, Coles MGH (eds): Energetics and Human Information Processing. Dordrecht: Martinus Nijhoff, 1986.

21. Mandell AJ. Toward a psychobiology of transcendence. In Davidson JM, Davidson RJ (eds): The Psychobiology of Consciousness. New York: Plenum, 1980.

22. Brown JR, Baker AB, McQuarrie, et al. Bulbar form of poliomyelitis. J Am Med Assoc 1947; 135: 425-428.

23. Meyer E. Psychological considerations in a group of children with poliomyelitis. J Pediatrics 1947; 31: 34-48.

24. Bruno RL, Galski T, DeLuca J. Neuropsychology of Post-Polio Fatigue. Arch Phys Med Rehabil 1993 (in press). [Lincolnshire Library Full Text]

25. Schmitt R, Fazekas F, Offenbacher H, et al. Magnetic resonance imaging white matter lesions and cognitive impairments in hypertensive individuals. Arch Neurol 1991; 48: 417- 420. [PubMed Abstract]

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27. Young LD, Barboriak JJ. Reliability of a brief scale for assessment of coronary-prone behavior and standard measures of Type A behavior. Perceptual and Motor Skills 1982;55:1039-42. [PubMed Abstract]

28. Haycock KA, Roth J, Gagnon J, Finzer WF, Soper C. Statview. Berkeley: Abacus Concepts, 1992.

29. Buchwald D, Cheney PR, Peterson DL, et al. A chronic illness characterized by fatigue, neurologic and immunologic disorders and active human herpesvirus type 6 infection. Ann Int Med 1992; 116:103-113. [PubMed Abstract]

30. Daugherty SA, Henry BE, Peterson DL, Swarts Rl, Bastien A, Thomas RS. Chronic fatigue syndrome in northern Nevada. Reviews of Infectious Diseases 1991; 13 (Suppl 1):S39-44.

[PubMed Abstract]

31. Rao SM, Mittenberg W, Bernardin L, Haughion V, Leo GJ. Neuropsychological test findings

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in subjects with leukoaraiosis. Arch Neurol 1989; 46:40-44. [PubMed Abstract]

32. Fazekas F, Offenbacher H, Fuchs S, et al. Criteria for an increased specificity of MRI

interpretation in elderly subjects with suspected multiple sclerosis. Neurology 1988; 38:

1822-1825. [PubMed Abstract]

33. Leys D, Soetaert G, Petit H, Fauquette A, Pruvo J-P, Steinling M. Periventricular and white

matter magnetic resonance imaging hyperintensities do not differ between Alzheimer’s

disease and normal aging. Arch Neurol 1990; 47:524-527. [PubMed Abstract]

34. Austron MG, Thompson RF, Hendrie HC, et al. Foci of increased T2 signal intensity in MR

images of healthy elderly subjects: A follow-up study. J Am Geriatr Soc 1990; 38:1133-1138.

[PubMed Abstract]

35. Awad IA, Johnson PC, Spetzler RF, Hodak JA. Incidental subcortical lesions identified on

magnetic resonance imaging in the elderly. II. Postmortem pathological considerations.

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36. Parasuraman R, Nestor P. Energetics of attention and Alzheimer’s Disease. In Hockey GRJ,

Gaillard AWK, Coles MGH ( eds): Energetics and Human Information Processing.

Dordrecht: Martinus Nijhoff, 1986.

37. Bodian D. Motorneuron disease and recovery in experimental poliomyelitis. In Hastead LS,

Wiechers DO (eds): Late Effects of Poliomyelitis. Miami, Symposia Foundation, 1985.

38. Machado-Salas J, Scheibel ME, Scheibel AB. Neuronal changes in the aging mouse: Spinal

cord and lower brain stem. Experimental Neurol 1977; 54: 504-512. [PubMed Abstract]

39. Bodian D. Poliomyelitis: Pathologic Anatomy in Poliomyelitis. Philadelphia: Lippincott,

1949.

40. Howe HA, Bodian D. Neuropathological evidence on the portal of entry problem in human

poliomyelitis. Bull Johns Hopkins Hosp 1941; 69: 183-214.

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brains of elderly subjects: Possible pathological basis. Radiology 1987; 162:509-511.

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hyperintensity and neuropsychological functions in dementia and healthy aging. Arch Neurol

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imaging in dementia. Arch Neurol 1990; 47:3 87-391. [PubMed Abstract]

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lesions in healthy elderly subjects: A threshold effect. Arch Neurol 1992; 49:549-554.

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speed of mental processing. Arch Neurol 1990; 47:151-156. [PubMed Abstract]

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white matter changes and dementia. Arch Neurol 1988; 45:637-641. [PubMed Abstract]

48. Young G. Energy conservation, occupational therapy and the treatment of Post-Polio

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14:1343-47. [PubMed Abstract]

ACKNOWLEDGEMENTS

The authors gratefully acknowledge the participation of the subjects, the continued support of the George Ohl Foundation, and the efforts and expertise of Doris Sipos and Mary Ann Solimine, R.N., M.L.S., without whom this work would not have been possible.

FIGURE LEGENDS

Figure 1.

Linear regression of the percent incidence of HS in healthy subjects on the mean subject age to calculate the normal incidence of HS in the general population (data from 25,29,30,31,32,33,34). Arrow indicates the 36% incidence of HS in post-polio subjects having a mean age of 46.7 years which lies outside the 95% confidence limits (curved lines).

Figure not available in source document.

Figure 2.

Two of three discrete foci of hyperintense signal (arrows) in the centrum semiovale in a 42

year old male polio survivor who retired on disability because of severe fatigue and severe problems with attention, thinking clearly and staying awake.

Figure not available in source document.

Figure 3.

Subjective rating of problem severity in subjects reporting low fatigue and reporting high fatigue with and without HS.

Figure not available in source document.

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Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/mri.html>

Created: 24th January 1997

Last modification: 29th January 2010.

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Neuroanatomy of Post-Polio Fatigue Read More »

Neuropsychology of Post-Polio

THE NEUROPSYCHOLOGY OF POST-POLIO FATIGUE

Richard L. Bruno, Ph.D., Thomas Galski, Ph.D., John DeLuca, Ph.D.

Bruno RL, Galski T, DeLuca J. The neuropsychology of post-polio fatigue. Archives of Physical Medicine and Rehabilitation, 1993; 74: 1061-1065.

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

Post-Polio Rehabilitation and Research Service Kessler Institute for Rehabilitation

East Orange, New Jersey and

Department of Physical Medicine and Rehabilitation UMDNJ/New Jersey Medical School

Newark, New Jersey

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

Post-Polio Service,

Kessler Institute for Rehabilitation, 240 Central Avenue,

East Orange, New Jersey 07018.

(201) 731-3600 (extension 547)

This research was supported by grants from the George Ohl Foundation

Short Title: Neuropsychology of Post-Polio Fatigue

THE NEUROPSYCHOLOGY OF POST-POLIO FATIGUE Richard L. Bruno, Ph.D., Thomas Galski, Ph.D., John DeLuca, Ph.D.

ABSTRACT

To test the hypothesis that post-polio fatigue and its concomitant cognitive deficits are associated with an impairment of attention and not of higher-level cognitive processes, six carefully screened polio survivors were administered a battery of neuropsychological tests. Only subjects reporting severe fatigue, and not those with mild fatigue, demonstrated clinically significant deficits on all tests of attention, concentration and information processing speed while showing no impairments of cognitive ability or verbal memory. These findings suggest that an impaired ability to maintain attention and rapidly process complex information appears to be a characteristic in polio survivors reporting severe fatigue, since these deficits were documented even when their subjective rating of fatigue was low. This finding supports the hypothesis that a polio-related impairment of selective attention underlies polio survivors’ subjective experience of fatigue and cognitive problems.

Polio; Post-Polio Sequelae; Fatigue; Attention

THE NEUROPSYCHOLOGY OF POST-POLIO FATIGUE

Richard L. Bruno, Ph.D., Thomas Galski, Ph.D., John DeLuca, Ph.D.

Fatigue is the most commonly reported, most debilitating and most poorly understood Post-Polio Sequelae (PPS) affecting the more than 1.63 million polio survivors (1). In national surveys of polio survivors, 91% reported new or increased fatigue, 41% reported fatigue significantly interfering with performing or completing their work and 25% reported fatigue interfering with self-care activities (2,3). Importantly, polio survivors differentiate between physical tiredness, that is associated with muscles being weak or easily fatigued, and “brain fatigue” that is characterized by problems with attention and cognition. Between 70% and 96% of polio survivors with fatigue reported concomitant problems with concentration, memory, attention, word-finding, maintaining wakefulness and thinking clearly, with 77% percent reporting moderate to severe problems with these functions (3).

It has been suggested that post-polio fatigue is caused by poliovirus-induced damage to the neurons of the Reticular Activating System (RAS) (3). Such damage would impair the ability of the RAS to maintain attention, activate the cortex and would generate the subjective problems with attention, concentration and maintaining wakefulness that polio survivors described as “brain fatigue.” For this hypothesis to be supported, post-polio fatigue would have to be associated with impaired functioning on neuropsychological tests measuring attention and not on those assessing memory, abstraction and intellectual ability.

Freidenberg, et al. (4) did administer a battery of neuropsychological tests to polio survivors with and without “progressive weakness, pain and fatigue.” Their findings did not support the hypothesis since both groups were found to have marked impairments of non-verbal memory and performance on tests of attention was significantly lower in the subjects without symptoms. However, this study did not adequately test the hypothesis since the severity of fatigue was not assessed and fatigue was not measured during testing.

This study was an initial attempt to test the hypothesis that post-polio fatigue is associated with impaired functioning on neuropsychological tests measuring attention – the Paced Auditory Serial-Addition Test, the Double Letter Cancellation Test and the Trail Making Test – and not on tests assessing memory and higher-level cognitive processes.

METHODS

Subjects. Volunteer subjects were recruited through the media, post-polio support groups and by direct mail over two years. One-hundred forty six (146) polio survivors requested and returned survey forms on which they were asked to subjectively rate their daily level of fatigue and cognitive problems occurring with fatigue on a six-point scale from “none” through “severe.” They were also asked to give a complete medical history, including descriptions of the effects of the acute poliovirus infection and their PPS.

Exclusion criteria were established to eliminate subjects whose fatigue could have been caused by factors other than PPS. One-hundred (108) volunteers were excluded as a result of co-morbidities that could cause fatigue and cognitive problems, e.g. psychiatric diagnoses including a major depressive episode, thyroid, cerebrovascular or cardiac disease, anemia, lupus or diabetes. Volunteers were also excluded if they were above 65 years of age, were taking sedating medications, including antidepressants and benzodiazepines for sleep, or if they had a been diagnosed with respiratory problems or had a history of breathing problems associated with the acute poliovirus infection (4).

Of the 38 volunteers remaining, only the 5 reporting “severe fatigue” and the 16 reporting “no fatigue” or “mild fatigue” were asked to participate in order to create two groups that were maximally different in terms of fatigue. Six volunteers agreed to be studied; three reporting “no fatigue” or “mild fatigue” were assigned to the mild fatigue group and three reporting severe fatigue were assigned to the severe fatigue group.

Procedure. After subjects gave written informed consent, they recorded their daily subjective levels of fatigue, emotional stress, motivation, sleep quality and cognitive problems for 14 days, rating them on a six-point scale from “none” through “severe” (Figure 1). On the 15th day, fasting blood was drawn at 11:00 AM to measure hypothalamic-pituitary-adrenal functioning (neuroendocrine data will be presented elsewhere). Subjects then completed the Beck Depression Inventory (5) and brief Type A questionnaire (6) to quantify symptoms of depression and Type A behavior that have both been associated with fatigue and cognitive problems in polio survivors (3). Over the next hour subjects ate lunch and rested.

Beginning at 1:00 PM, a three-hour battery of neuropsychological tests was administered to assess higherlevel cognitive processes and to repeatedly assess attention, concentration and fatigue. Four tests were administered to assess attention and a subjective rating of fatigue on a six-point scale from “none” through “severe” was elicited prior to beginning testing and after each of three, 45 minute testing periods. The Paced Auditory Serial-Addition Test (PASAT) (7) assessed complex attention by requiring subjects to sequentially add a series of 60 digits presented by a tape recording at three presentation speeds: one every 1.6, 2.0 and 2.4 seconds. The Double Letter Cancellation Test (8) required subjects to cross-out two specified letters on a sheet filled with similar letters in the shortest possible time to assess selective and sustained attention. The Trail Making Test (9) assessed visual scanning and visual motor speed by requiring subjects to draw a line to sequentially connect circles containing 25 ascending numbers (Parts A) or sequentially but alternately connect circles containing ascending numbers and the letters of the alphabet (Part B) in the shortest possible time.

During the first testing period four tests were administered. The Vocabulary subtest of the Wechsler Adult Intelligence Scales-Revised (WAIS-R) (10) required subjects to give correct definitions of increasingly difficult words to assess verbal intelligence. The Block Design subtest of the WAIS-R assessed visuoconstructive ability and non-verbal reasoning by requiring subjects to correctly reproduce twodimensional colored designs using colored blocks. The Logical Memory I subtest of the Wechsler Memory Scale-Revised (WMS-R) (11) required subjects to recall two paragraph-length passages

immediately after each reading to assess immediate verbal memory. The Visual Memory I subtest of the WMS-R assessed immediate visual memory by requiring subjects to reproduce several drawings from memory after a 10 second exposure.

During the second testing period three tests were administered. The Token Test (12) required subjects to manipulate plastic tokens in response to a series of hierarchally-ordered commands to assess language comprehension. The Ravens Progressive Matrices (13) assessed visual-spatial analysis and non-verbal reasoning by requiring subjects to select a small patterned insert to complete a large pattern that had a portion omitted. The Visual Form Discrimination Test (14) required subjects to select a match for a complex graphic design from a group of designs to assess visual-spatial analysis.

During the third testing period three tests were administered. The Short Form Category Test (15) assessed abstract reasoning and problem solving by requiring subjects to develop a classification scheme for a series of pictures grouped by conceptual similarity. The Logical Memory II and Visual Memory II subtests of the WMS-R required subjects to recall the passages and reproduce the designs to which they had been exposed during the first testing period to measure delayed verbal and visual memory.

Neuropsychological tests were administered and scored according to accepted procedures. Since the small sample size prevented statistical analysis, the means of the test scores were calculated for the two groups and compared to established clinical norms to determine whether group performance was clinically impaired.

RESULTS

Demographics. Subjects in both groups were essentially equal on demographic variables (Table 1). Subjects in both groups had elevated Type A scores (i.e. >50) (6) and were equally affected by polio and PPS as measured by the number of limbs originally paralyzed or affected by new muscle weakness and pain. No subject had a clinically elevated score (i.e ., >17) on the Beck Depression Inventory (5). Those with severe fatigue were on average younger and had polio at a slightly younger age later in the century than those in the mild fatigue group. Two subjects with severe fatigue had retired on Social Security disability income as a result of fatigue preventing continued employment. The remaining subjects in both groups were working full-time outside or inside the home.

Symptom Logs. Mild fatigue subjects’ mean daily fatigue and functional problems were rated between “none” and “mild” (Figure 1). Severe fatigue subjects rated mean daily fatigue above “moderate” while other functional problems were rated as “mild to moderate” or above. Difficulty with sleeping and emotional stress were rated between “mild” and “mild to moderate” by both groups, suggesting that these symptoms, which have been shown to be related to post-polio fatigue, did not differentially affect the severe fatigue subjects (3).

Neuropsychological Testing. Scores on tests of verbal and non-verbal intellectual abilities (WAIS-R Vocabulary Test, Ravens Progressive Matrices and Short Form Category Test) revealed that subjects in both groups had at least average intellectual ability (Table 2). Although subjects with mild fatigue scored slightly higher on all of these measures, the elevations were not clinically significant. The equivalence of intellectual ability was supported by the mean estimated full-scale intelligence quotient of 111 for both groups (Table 1). Language abilities were also intact in both groups and there was no evidence of deficits in the comprehension of simple or complex verbal material as demonstrated by equivalent mean scores on the Token Test in the two groups.

Tests of immediate memory (WMS-R Logical Memory I and Visual Reproduction I) revealed no

difference between severe and mild fatigue groups in their ability to learn and recall verbal and non-verbal (visual) material. Additionally, both groups demonstrated a normal capacity for delayed recall of verbal information as shown by their scores on WMS-R Logical Memory II. However, both the severe and mild fatigue groups were similarly impaired in their ability to recall visual information after a delay as shown by their scores on WMS-R Visual Reproduction II.

Visuospatial perception and organization and visuopraxis were average or higher for both groups as shown by the nearly equivalent Visual Form Discrimination Test scores and their accuracy of construction on the WAIS-R Block Design Test. Notably, severe fatigue subjects’ slower psychomotor speed prevented them from obtaining higher scaled scores on the Block Design Test.

Clinically significant differences were seen between the two groups on measures of attention and information processing speed. Subjects with severe fatigue required 23% and 67% more time, respectively, to complete the initial presentation of Double Letter Cancellation and Trail Making Tests (Part A), tasks requiring sustained attention and vigilance (Figure 2). In the severe fatigue group, the time required for completion of the first three trials of the Double Letter Cancellation Test was clinically abnormally elevated (i.e., >100 seconds) (7), as was the completion time for the first three trials of the Trail Making Test (Part A) (i.e., >30 seconds) (8). Practice and learning effects were demonstrated by decreased completion times in both groups even though the mean fatigue ratings increased to “severe” for the severe fatigue subjects and “mild to moderate” for the mild fatigue group by the last testing period. However, even with practice, the completion times for the last presentation of these tests for the severe fatigue subjects was still longer than those for the initial presentation in the mild fatigue group. Notably, the number of errors on the Double Letter Cancellation Test was essentially equal and decreased with practice in both groups (Table 2).

Subjects with severe fatigue also demonstrated deficits in their ability to rapidly process complex information and sustain divided attention as measured by the Paced Auditory Serial-Addition Test (PASAT)(Figure 3). In the severe fatigue group, the mean percentage of correct responses was clinically abnormally decreased at the 1.6 second (<66% correct), 2.0 second (<73% correct) and 2.4 second presentation rates (<82% correct) during each of the four testing periods. Again, practice and learning effects were demonstrated by the increase in the percentage of correct responses in both groups across testing periods despite an increase in the fatigue rating during testing. However, the percentage of correct responses for the last testing period in the severe fatigue subjects was still lower than that for the first testing period in the mild fatigue group.

DISCUSSION

These findings provide preliminary support for the hypothesis that post-polio fatigue is associated with impaired functioning on neuropsychological tests that assess attention and not on those assessing higherlevel cognitive processes. An impaired ability to maintain attention and rapidly process complex information appears to be a characteristic of these polio survivors reporting severe fatigue, since the deficits were documented during the first testing period when the subjective rating of fatigue was still low.

The impairment of delayed recall of non-verbal information in both groups of polio survivors was also reported by Freidenberg, et al. (4). These data suggest a right hemisphere information processing deficit in polio survivors that is unrelated to fatigue. As yet there is no empirical or theoretical explanation for a polio-related, lateralized impairment of hemispheric functioning.

It is noteworthy that the severe fatigue subjects’ subjective ratings of cognitive problems on their 14-day

logs were not associated with comparable impairments of cognition and verbal memory on neuropsychological testing. It is possible that these Type A individuals, whose cognitive abilities equaled or exceeded the norms, perceived even small decrements in their cognitive functioning as significant problems. It is also possible that subjects’ continuous expenditure of effort, to compensate for fatigue, impaired attention and maintain a generally high level of cognitive functioning, creates the subjective impression that they are having problems with cognition and memory.

A strength of this study, the careful selection of subjects to rule out causes of fatigue other than polio, is also a weakness since the small sample size limits the generalizability of its findings. If these findings do apply in general to polio survivors with fatigue, they have important implications. Despite their subjective reports of cognitive impairment and documented deficits in attention and information processing speed, polio survivors reporting severe fatigue were able to maintain their ability to learn and perform high-level cognitive tasks even as their fatigue during testing increased. Thus, although the work performance of polio survivors may be impaired if they are required to rapidly process multiple sources of complex information, tasks requiring even high-level cognitive abilities (e.g., accounting, copy editing, computer programming) may be performed accurately with reasonable accommodations that allow increased time for task completion and minimize distractions.

Unfortunately, these job modifications alone will not affect problems such as word-finding or maintaining wakefulness, effort and motivation in the face of increased fatigue. Reducing physical and emotional stress, energy conservation, adequate rest and the pacing of activities remain the treatments of choice for post-polio fatigue (16,17,18). We continue to study the neuroanatomy and neuroendocrinology of postpolio fatigue to identify its cause and are developing pharmacological treatments for fatigue and its associated attention deficits.

ACKNOWLEDGEMENTS

REFERENCES

1. Bruno RL. Post-Polio Sequelae: Research and treatment in the second decade. Orthopedics 1991;

14:1169-70. [PubMed Abstract]

2. Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae. In:

Halstead LS, Wiechers DO, eds. Research and Clinical Aspects of the Late Effects of Poliomyelitis.

White Plains: March of Dimes, 1987:145-55. [Lincolnshire Library Full Text]

3. Bruno RL, Frick NM, Cohen J. Polioencephalitis, stress and the etiology of post-polio sequelae.

Orthopedics 1991; 14:11 85-93. [Lincolnshire Library Full Text]

4. Freidenberg DL, Freeman D, Huber SJ, et al. Postpoliomyelitis syndrome: Assessment of behavioral

features. Neuropsychiatry, Neuropsychology and Behavioral Neurology 1989; 2 :272-81.

5. Beck AT, Ward CH, Mendelson M, Mock J, Erbaugh J. An inventory for measuring depression.

Archives of General Psychiatry 1961;4:561-71.

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

standard measures of Type A behavior. Perceptual and Motor Skills 1982;55:1039-42. [PubMed

Abstract]

7. Gronwall DMA. The paced auditory serial-addition task: A measure of recovery from concussion.

Perceptual and Motor Skills 1977;44:367-73. [PubMed Abstract]

8. Lezak MD. Neuropsychological Assessment. New York: Oxford University Press, 1976.

9. Reitan RM. The Halstead-Reitan Neuropsychological Battery: Theory and Clinical Practice.

Tucson: Neuropsychology Press, 1985.

10. Wechsler D. Wechsler Adult Intelligence Scale – Revised. New York: Psychological Corporation,

1981.

11. Wechsler D. Wechsler Memory Scales – Revised. San Antonio: Psychological Corporation, 1987.

12. De Renzi E, Vignolo L. The token test: A sensitive test to detect receptive disturbances in aphasics.

Brain 1962;85: 665-78.

13. Raven JC, Court JH, Raven J. Manual for Raven’s Progressive Matrices and Vocabulary Scales.

London: H.K. Lewis Ltd, 1985.

14. Benton AL. Revised Visual Retention Test (4th edition). New York: Psychological Corporation,

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15. Wetzel L, Boll T. Short Category Test, manual. Los Angeles: Western Psychological Services, 1987.

16. Bruno RL, Frick NM. The psychology of polio as prelude to post-polio sequelae: Behavior

modification and psychotherapy. Orthopedics 1991; 14:1269-76. [Lincolnshire Library Full Text]

17. Agre JC, Rodriguez AA. Neuromuscular function in polio survivors. Orthopedics 1991; 14:1343-

47. [PubMed Abstract]

18. Young GR. Energy conservation, occupational therapy and the treatment of post-polio sequelae.

Orthopedics 1991; 14:1233-39. [Lincolnshire Library Full Text]

19. Barona A, Reynolds CR, Chastain, R. A demographically based index of pre-morbid intelligence for

the WAIS-R. Journal of Consulting and Clinical Psychology 1984; 52:885-887.

FIGURE LEGENDS

Figure 1.

Fourteen-day mean ratings of fatigue and functional problems.

Figure not available in source document.

Figure 2.

Double letter cancellation and trail making test scores.

Figure not available in source document.

Figure 3.

Paced auditory serial addition (PASAT) test scores.

Figure not available in source document.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/neuropsy.html> Created: 27th January 1997

Last modification: 29th January 2010.

Neuropsychology of Post-Polio Read More »