Post-polio syndrome
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Post-polio syndrome (PPS) is a condition that frequently affects survivors of poliomyelitis, a viral infection of the nervous system, after recovery from an initial paralytic attack of the virus. Typically the symptoms appear 20-40 years after the original infection, at an age of 35 to 60. Symptoms include new or increased muscular weakness, pain in the muscles, and fatigue.
Some post-polio patients report having memory problems, or various other cognitive difficulties that are difficult to distinguish from normal aging. Some physicians have suspected that post-polio patients have an increased sensitivity to anesthetics, but rigorous work on the subject remains to be done. Weight gain is also a frequently noted symptom, though it is hard to tell if this is due to the disorder directly or due to the decreased level of physical activity that usually accompanies the disorder.
Fatigue is often the most disabling symptom, as often even slight exertion can produce disabling fatigue and also increase other symptoms.
Diagnosis of post-polio syndrome can be difficult, since the symptoms are hard to separate from the original symptoms of polio and from the normal infirmities of aging. There is no laboratory test for post-polio syndrome, nor is there any other specific diagnostic, so diagnosis is usually a "diagnosis of exclusion" where other possible causes of the symptoms are eliminated.
The precise mechanism that causes post-polio syndrome is unknown. It shares many features in common with myalgic encephalomyelitis, a form of chronic fatigue syndrome that is apparently caused by viral infections, but unlike those disorders it tends to be progressive, and can cause tangible loss of muscle strength.
One theory of the mechanism behind the disorder is that it is due to "neural fatigue" from overworked neurons. The original polio infection generally results in the death of a substantial fraction of the motor neurons controlling skeletal muscles, and the theory is that the remaining neurons are thus overworked and eventually wear out.
Another theory holds that the original viral infection damages portions of the lower brain, possibly including the thalamus and hypothalamus. This somehow upsets the hormones that control muscle metabolism, and the result is a metabolic disorder similar to mitochondrial disorder that causes muscle pain and injury (via rhabdomyolysis) and also causes the fatigue.
Some also believe that the original polio caused the atrophying of some muscles and as the person ages the weakness caused by loss of muscle mass due to aging is accelerated due the the person starting off with less muscle.
Treatment generally is limited to supportive measures, primarily leg braces and energy-saving devices such as powered wheelchairs, plus pain relievers, sleep aids, etc. However, some post-polio patients claim to have found significant relief using large amounts of various food supplements, primarily L-carnitine, CoQ10, and d-ribose.
Anecdotal evidence suggests that exercise can improve some cases.
[edit] Diagnosis
Doctors arrive at a diagnosis of PPS by observing the patient, asking about symptoms, and by excluding other disorders. PPS may be difficult to diagnose in some because it is hard to determine what component of a neuromuscular deficit is old and what is new. Health professionals say that the only way to be sure a person has PPS is through a neurological examination aided by other laboratory studies that exclude all other possible diagnoses. Patients must visit the doctor periodically to establish that their muscle weakness is progressive.
Objective assessment of muscle strength in PPS patients may not be easy. A change in muscle strength is determined in specific muscle groups, or limbs, using various muscle scales, such as the Medical Research Council (MRC) scale or scales that quantify muscle force. Doctors use magnetic resonance imaging (MRI), neuroimaging, and electrophysiological studies, muscle biopsies, or spinal fluid analysis as tools to investigate the course of decline in muscle strength and exclude other conditions.
Once PPS is diagnosed, some patients worry that they have polio again, or even ALS. In fact, they have neither of these disorders. In general, PPS is not life-threatening. The only exception is in patients left with severe residual respiratory difficulties, who may experience new severe respiratory impairment. Studies have proven that, compared to control populations, PPS patients lack any elevation of antibodies against the polio virus, and since PPS affects only certain muscle groups, it is not considered a recurrence of the original polio. Further, there is no evidence that the polio virus can cause a persistent infection in humans. Other studies have demonstrated that ALS, which progressively weakens muscles, does not occur more frequently in PPS patients, and PPS is not a form of ALS.
[edit] Causes
Several theories have been proposed to explain post-polio symptoms:
- Neural fatigue: The most widely accepted theory is the "neural fatigue" one. Motor neuron fibers were originally damaged by the polio virus and were subsequently over-stressed because too few surviving neurons activated too many muscles. Eventually these neurons become fatigued and die, leading to the slowly advancing loss of muscle function that is typical of post-polio. This scenario may be accelerated by the fall-off in production of nerve growth factor (NGF) that occurs with menopause/andropause.
- Mitochondrial disruption: This theory assumes that the major symptoms of PPS are a result of some interference with the action of mitochondria in the muscles and possibly the nerves. Failure of the mitochondria to produce sufficient energy would result in the muscle pain typical of PPS, and would, over time, cause muscle death (rhabdomyolysis) due to exerting the muscle beyond its ability to recover. The cause of this interference with mitochondrial action is presumably a change in the body's hormone balance, as mediated by the hypothalamus and other lower brain areas that control hormones (and which were, presumably, damaged by the original polio virus infection). As with the neural fatigue theory, menopause/andropause accelerates the process, though this time by most likely disrupting the NOTCH pathway that controls cell differentiation and damage repair.
One significant argument in favor of the mitochondrial disruption theory is that it explains the fatigue and cognitive difficulties ("brain fog") symptoms that usually accompany post-polio better than the neural fatigue theory does.
- Reticular activating system damage: Damage to the reticular activating system and related areas such as the thalamus can also produce most of the fatigue, "brain fog", and dysautonomia symptoms of post-polio, and may be able to cause hormonal changes that result in progressive muscle weakness. Post-mortem examinations of polio patients have shown damage to these areas, and some PPS patients show lesions in these areas when examined by MRI. Many authorities believe that these areas are damaged by the initial polio infection, either as a direct result of the polio virus, or due to an autoimmune reaction following the polio infection.
One problem with this theory, though, is that it doesn't easily explain the delayed onset of PPS. It may be that this theory needs to be combined with one of the others to explain delayed onset.
- Mechanical overwork: The stresses placed on nerves, muscles, and joints in a polio survivor are in many cases several times those experienced by other people. Problems with gait, in particular, can greatly overstress joints and the surviving muscles, and the polio survivor is also likely to compensate for weakened arms by jerking more when lifting/pulling something. Over time (and again with menopause/andropause), this results in fatigue and damage.
- Reactivated polio: Though this theory has been largely discredited by laboratory studies that show no active polio virus in the body, there are still some people who believe that post-polio is caused by reactivation of latent polio virus in the body, similar to the way that shingles is a reactivation of the chicken pox virus.
In final analysis, it will likely turn out that post-polio symptoms are due to some combination of mechanisms.
[edit] Treatment
Treatment for post-polio is primarily palliative, as no reliable therapy to reverse symptoms is known. Palliative treatment includes:
- Rest: Very often fatigue is the most disabling symptom of PPS, and many of those with the disease have discovered that by carefully managing energy expenditure they can prevent or reduce the worst fatigue episodes. Further, for many this "energy management" approach appears to reduce pain.
Though most authorities agree that rest is an important component of post-polio treatment, there is significant disagreement as to how much rest is necessary. Some hold that the best approach is to expend the absolute minimum amount of energy necessary to enjoy a reasonable lifestyle, while others feel that there is some threshold below which energy conservation is not helpful and may in fact be harmful (due to the general effects caused by lack of exercise).
- Leg braces: Joint braces and other orthotics can reduce the stress on joints and, in some cases, muscles, and so may slow the progression of joint and muscle damage related to PPS. However, some authorities feel that many PPS patients rely on such items too much and for too long when they should be graduating to a wheelchair.
- Wheelchairs: Wheelchairs (particularly powered wheelchairs) and "scooters" (small battery-powered vehicles) are useful both to conserve energy and to reduce the stress on weakened joints and muscles. Non-powered wheelchairs, however, are not generally recommended since they place too much stress on arm muscles and joints and may take too much energy to operate. In some cases even the scooters are not recommended since operating the "tiller" of the typical scooter can be tiring to arm muscles. A standing frame can be used in conjunction with the wheelchair to provide alternative positioning and prevent secondary complications.
- Surgery: In some cases surgery can be used to repair joint deformities, or to fuse joints (as in the back or ankle) that have become too weak.
- Medications: Post-polio syndrome often causes significant levels of pain, sometimes in specific muscles or joints, and sometimes body-wide. Various forms of narcotic and non-narcotic pain-relievers, muscle relaxants, tranquilizers, and sleep medications may help to deal with the pain and related sleep problems.
Only a few non-palliative treatments for post-polio syndrome have shown any promise, and none have been subjected to any sort of rigorous clinical testing. There are, however, a few treatments that have developed some "following" in the PPS community:
- L-carnitine: This amino acid has several functions in the body, one of the most important being the transport of fatty acids into the mitochondria. Researchers in Australia have had some success using doses of several grams per day.
- Coenzyme Q10: CoQ10 is a general antioxidant, but it also plays a critical role in the function of the mitochondria, transporting electrons between the "complexes" that participate in the energy conversion cycle. A shortage of CoQ10 can cause the fatigue and muscle pain much like that experienced with PPS. Some PPS patients have reported significant improvements in their symptoms when taking several hundred milligrams of CoQ10 per day.
- D-ribose: This pentose sugar is the "R" in RNA and a critical component of DNA, RNA, and enzymes. It is also a component of ATP, the energy-transporting molecule produced by the mitochondria. A shortage of D-ribose can produce fatigue and muscle pain. Some PPS patients have reported significant improvement in pain and fatigue symptoms when taking on the order of 20 grams/day of D-ribose.
[edit] References
- Bruno, Richard L. (2002). The Polio Paradox: Uncovering the Hidden History of Polio to Understand and Treat "Post-polio Syndrome" and Chronic Fatigue. New York: Warner Books. ISBN 0-446-52907-9. (Dr. R. L. Bruno is the Director of The Post-Polio Institute in Englewood, New Jersey)
- Jubelt, B., & Agre, J.C. (2000). "Characteristics and management of postpolio syndrome." Journal of the American Medical Association, 284, 412-414.
- Maynard, F.M., & Headley, J.H. (Eds.) (1999). Handbook on the Late Effects of Poliomyelitis for Physicians and Survivors. Saint Louis, MO: GINI (now Post-Polio Health International). Information on 90 post-polio topics; a compilation of the research and experience of over 40 experts.
- March of Dimes Birth Defects Foundation. (1999). Identifying Best Practices in Diagnosis & Care. Warm Springs, GA: March of Dimes International Conference on Post-Polio Syndrome
- Nollet F. "Perceived health and physical functioning in postpoliomyelitis syndrome". Vrije Universiteit Amsterdam, 2002.
- Nollet, F. "Post-polio syndrome". Orphanet Ecyclopaedia, 2003
- Silver, Julie K. (2001). Post-Polio Syndrome: A Guide for Polio Survivors and Their Families. New Haven: Yale University Press. (Dr. Silver is Medical Director, Spaulding-Framingham Outpatient Center; Assistant Professor, Department of Physical Medicine and Rehabilitation, Harvard Medical School.)
