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Post Polio Syndrome

Synonyms of Post Polio Syndrome

  • Polio, Late Effects
  • Post-Polio Muscular Atrophy
  • Postpoliomyelitis syndrome
  • Post-Polio Sequelae

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Post-polio syndrome (PPS) is a syndrome that affects some people who have had polio (poliomyelitis) and occurs many years (typically from 10 to 40 years) after recovery from the initial infection. It is characterized by the development of progressive weakness in muscles that were affected by the original polio infection. In addition, those affected may experience extreme fatigue and joint pain. Skeletal deformities, such as scoliosis, may occur as a result of this syndrome. There is variation in the severity of symptoms. In severe cases, symptoms may mimic those of the rare disorder known as Lou Gehrig's disease (amyotrophic lateral sclerosis). The degree of muscle atrophy during the post-polio period appears to reflect the severity of the impact of the initial polio infection. People who were significantly affected by polio are more likely to experience severe symptoms from post-polio syndrome. The cause of this syndrome is not known. Although exact numbers are not available, it has been estimated that there are 300,000 polio survivors in the United States and that from one-fourth to one-half of them may ultimately develop some degree of post-polio syndrome.

Symptoms

Post-polio syndrome occurs at least 10 years after a person has been stricken by polio. It is characterized by gradual deterioration of muscle function and increased weakness that usually occurs in the limbs that were most severely affected by polio. Sometimes, the disorder involves muscles that appeared to be fully recovered or that were not involved in the original polio attack, including muscles necessary for respiration. Other symptoms may include fatigue, muscle pain and twitching (fasciculations).

The fatigue associated with PPS comes on unexpectedly, sometimes as a feeling of total exhaustion throughout the body. Pain in the muscles and joints is not uncommon. Also, the affected person may experience problems related to swallowing, breathing, and sleeping. People with PPS are less able to tolerate the cold, and they are less able to carry on with day-to-day activities such bathing, dressing, and walking.

Causes

The cause of post-polio syndrome is not known at this time. One theory is that it may be related to the recovery from the original polio. During recovery from polio, nerve cells in affected muscles may regrow many smaller branches (dendrites) from the message-transmitting large branches (axons) of nerve cells. These sprouts take over the function of neurons killed by the polio virus. It is thought that, after years of functioning beyond capacity, the nerve cells weaken and lose their ability to maintain these tiny dendrites, which then begin to shrink, and the whole muscle becomes weaker. Additional research is needed to determine whether this may, in fact, be the cause of post-polio syndrome.

Although this syndrome sometimes resembles amyotrophic lateral sclerosis (ALS), it is not considered to be a form of that disease.

Affected Populations

Post-polio syndrome affects people who have had acute episodes of poliomyelitis. It occurs 10 years or more after the original illness, and can occur as long as 40 years afterward. According to one estimate, 25% to 50% of the 300,000 polio survivors in the United States may develop the syndrome. At the present time, there is no known way to prevent the syndrome.

Related Disorders

Symptoms of the following disorders can be similar to those of post-polio syndrome. Comparisons may be useful for differential diagnosis:

Amyotrophic lateral sclerosis (ALS) is one of a group of disorders known as motor neuron diseases. It is characterized by the progressive degeneration and eventual death of nerve cells (motor neurons) in the brain, brainstem and spinal cord that facilitate communication between the nervous system and voluntary muscles of the body. Ordinarily, motor neurons in the brain (upper motor neurons) sent messages to motor neurons in the spinal cord (lower motor neurons), and then to various muscles. ALS affects both the upper and lower motor neurons, so that the transmission of messages is interrupted, and muscles gradually weaken and waste away. As a result, the ability to initiate and control voluntary movement is lost. Ultimately, ALS leads to respiratory failure because affected individuals lose the ability to control muscles in the chest and diaphragm. ALS is often called Lou Gehrig's disease. (For more information on this disorder, choose "amyotrophic lateral sclerosis" as your search term in the Rare Disease Database.)

Multiple sclerosis is a neuroimmunologic (both the nervous system and immunological system are involved) disorder involving the brain, spinal chord and optic nerves. By means of a mechanism that is not clearly understood, the protective insulating sheath (myelin sheath) that covers the nerve is destroyed. The inflammatory attacks that produce the characteristic scarring (plaques or patches) of the myelin sheath occur randomly, in varying intensity, and at multiple sites. The course of the disease may advance, relapse, remit, or stabilize. The randomness of the location of plaques or patches affects the nerve's ability to transmit information (neurotransmission) and causes a wide range of neurological symptoms, which may vary from person to person. (For more information on this disorder, choose "multiple sclerosis" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
Diagnosis of post-polio syndrome is made on the basis of a thorough history, a neurological examination, and the process of excluding other possible diseases through various tests. In making the diagnosis, physicians will be aware of three factors: a prior diagnosis of polio, an interval of one or more decades since the original acute episode, and slow, steady, progressive deterioration.

Treatment
There are no specific treatments for PPS at this time. The goal of management of this disorder is to make the patient as comfortable as possible.

Affected individuals are taught to conserve energy by pacing their activities and combining these with periods of rest. Mechanical assists such as canes, walkers, and scooters may be helpful.

Moderate exercise is seen by many physicians as beneficial for those affected. Swimming is one type of exercise that is sometimes recommended. Speech therapy may be helpful for individuals whose swallowing has been affected.

Also, occupational therapy can lead to adjustments in the home environment that may allow those affected to carry out common activities in ways that are less energy-consuming.

Investigational Therapies

Two clinical trials sponsored by the National Institute of Neurological Disorders and Stroke (NINDS) are being carried out (2005) at the Clinical Center on the NIH campus in Bethesda. For additional information, contact the NIH Clinical Center at the phone numbers or email address listed above.

One is designed to determine whether electromyography (a test of the communication between nerves and muscle cells) is a reliable diagnostic tool for post-polio syndrome. The other is designed to determine whether the drug Modafinil can decrease fatigue in patients with post-polio syndrome.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Post Polio Syndrome

References

TEXTBOOKS
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1486, 2342.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:576, 1578.

Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases. 4th ed. Churchill Livingstone Inc. New York, NY; 1995:1617-18.

Rowland LP. Ed. Merritt's Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:137, 712.

REVIEW ARTICLES
Trojan DA, Cashman NR. Post-poliomyelitis syndrome. Muscle Nerve. 2005;31:6-19.

Bartels MN, Omura A. Aging in polio. Phys Med Rehabil Clin N Am. 2005;16:197-218.

Khan F. Rehabilitation for postpolio sequelae. Aust Fam Physician. 2004;33:621-24.

Jubelt B, Agre JC. Characteristics and management of postpolio syndrome. JAMA. 2000;284:412-14.

JOURNAL ARTICLES
Sorenson EJ, Daube JR, Windebank AJ. A 15-year follow-up of neuromuscular function in patients with prior poliomyelitis. Neurology. 2005;22:1070-72.

Sliwa J. Postpolio syndrome and rehabilitation. Am J Phys Med Rehabil. 2004;83:909.

Sandberg A, Stalberg E. How to interpret normal electromyographic findings in patients with an alleged history of polio. J Rehabil Med. 2004;36:169-76.

Finch LE, Venturini A, Mayo NE, et al. Effort-limited treadmill walk test: reliability and validity in subjects with postpolio syndrome. Am J Phys Med Rehabil. 2004;613-23.

Sanberg A, Stalberg E. changes in macro electromyography over time in patients with a history of polio: A comparison of 2 muscles. Arch Phys Med Rehabil. 2004;85:1174-82.

Gordon T, Hegedus J, Tam SL. Adaptive and maladaptive motor axonal sprouting in aging and motoneuron disease. Neurol. Res. 2004;26:174-85.

Jubelt B. Post-Polio Syndrome. Curr Treat Options Neurol. 2004;6:87-93.

Markstrom A, Sundell K, Lysdahl M, et al. Quality-of-life evaluation of patients with neuromuscular and skeletal diseases treated with noninvasive and invasive home mechanical ventilation. Chest. 2002;122:1695-700.

FROM THE INTERNET
NINDS Post-Polio Syndrome Information Page. NINDS. Last updated March 29, 2005. 2pp.
www.ninds.nih.gov/disorders/post_polio/post_polio_pr.htm

Post-polio syndrome. MayoClinic.com. March 04, 2005. 6pp.
www.mayoclinic.com/invoke.cfm?id=DS00494

Post-Polio Syndrome. Quick Reference and Fact Sheets. March of Dimes. 2005. 2pp.
www.marchofdimes.com/printableArticles/681_1284.asp?printable=true

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Report last updated: 2009/04/08 00:00:00 GMT+0

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