Postpolio syndrome (PPS) is a condition that strikes survivors of the disease polio. PPS occurs about 20–30 years after the original bout with polio, and causes slow but progressive weakening of muscles.
Description
Polio is a disease caused by the poliovirus. It most commonly infects younger children, although it can also infect older children and adults. About 90% of people infected by poliovirus develop only a mild case or no illness at all. However, infected people can continue to spread the virus to others. In its most severe form polio causes paralysis of the muscles of the legs, arms, and respiratory system.
About 1% of all people infected with poliovirus develop the actual disease known as polio. In these cases, the virus (which enters the person's body through the mouth) multiplies rapidly within the intestine. The viruses then invade the nearby lymphatic system. Eventually, poliovirus enters the bloodstream, which allows it to gain access to the central nervous system or CNS (the brain and spinal cord). The virus may actually infect a nerve elsewhere in the body, and then spread along that nerve to enter the brain.
The major illness associated with poliovirus often follows a mild illness, which has symptoms of fever, nausea, and vomiting. However, after a symptom-free interval of several days, the patient who is on the way to a major illness develops new symptoms such as headache and back and neck pain. These symptoms are due to invasion of the nervous system. The motor nerves (those nerves responsible for movement of the muscles) become inflamed, injured, and destroyed. The muscles, therefore, no longer receive any messages from the brain or spinal cord. The muscles become weak, floppy, and then totally paralyzed (unable to move). All muscle tone is lost in the affected limb, and the muscle begins to decrease in size (atrophy). The affected muscles are often only on one side (asymmetric paralysis) of the body. Sensation (the person's ability to feel) is not affected in these paralyzed limbs.
The maximum state of paralysis is usually reached within just a few days. The remaining, unaffected nerves then begin the process of attempting to grow branches to compensate (make up for) the destroyed nerves. This process continues for about six months. Whatever function has not been regained in this amount of time will usually be permanently lost.
Causes and symptoms
PPS occurs in about 25% of patients, several decades after their original infection with polio. However, long-term follow-up indicates that two thirds of polio survivors may experience new weakness. Several theories exist as to the cause of this syndrome.
One such theory has looked at the way function is regained by polio survivors. Three mechanisms seem to be at work:
injured nerves recuperate and begin functioning again
muscles which still have working nerve connections grow in size and strength, in order to take over for other paralyzed muscles
working nerves begin to send small branches out to muscles whose original nerves were destroyed by polio
As a person ages, injured nerves that were able to regain function may fail again, as may muscles that have been over-worked for years in order to compensate for other paralyzed muscles. Even the uninjured nerves that provided new nerve twigs to the muscles may begin to falter after years of relative over-activity. This theory, then, suggests that the body's ability to compensate for destroyed nerves may eventually begin to fail. The compensating nerves and muscles grow older, and because they've been working so much harder over the years, they wear out relatively sooner than would be expected of normal nerves and muscles. Some researchers look at this situation as a form of premature aging, brought on by overuse.
Other researchers note that normal aging includes the loss of a fair number of motor nerves. When a patient has already lost motor nerves through polio, normal loss of motor nerves through aging may cause the number of remaining working nerves to drop low enough to cause symptoms of weakness.
Other theories of PPS include the possibility that particles of the original polioviruses remain in the body. These particles may exert a negative effect, decades later, or they may cause the body's immune system to produce substances originally intended to fight the invading virus, but which may accidentally set off a variety of reactions within the body that actually serve to interfere with the normal functioning of the nerves and muscles.
Still other researchers are looking at the possibility that polio patients have important spinal cord changes which, over time, affect the nerves responsible for movement.
The symptoms of PPS include generalized fatigue, low energy, progressively increasing muscle weakness, shrinking muscle size (atrophy), involuntary twitching of the muscle fibers (fasciculations), painful muscles and joints, difficulties with breathing and swallowing, and sleep problems.
Survivors of polio may also develop arthritis of the spine, shoulders, or arms, related to the long-term use of crutches or overcompensation for weak leg muscles.
Diagnosis
Diagnosis is primarily through history. When a patient who has recovered from polio some decades previously begins to experience muscle weakness, PPS must be strongly suspected.
Treatment
Just as there are no treatments available to reverse the original damage of polio, there are also no treatments available to reverse the damaging effects of postpolio syndrome. Attempts can be made to relieve some of the symptoms, however.
Pain and inflammation of the muscles and joints can be treated with anti-inflammatory medications, application of hot packs, stretching exercises, and physical therapy. Exercises to maintain/increase flexibility are particularly important. However, an exercise regimen must be carefully designed, so as not to strain already fatigued muscles and nerves.
Some patients will require new types of braces to provide support for weakening muscles. Others will need to use wheelchairs or motorized scooters to maintain mobility.
Sleep problems and respiratory difficulties may be related to each other. If breathing is labored during sleep, the blood's oxygen content may drop low enough to interfere with the quality of sleep. This may require oxygen supplementation, or even the use of a machine to aid in breathing.
Prognosis
Prognosis for patients with postpolio syndrome is relatively good. It is a very slow, gradually progressing syndrome. Only about 20% of all patients with PPS will need to rely on new aids for mobility or breathing. It appears that the PPS symptoms reach their most severe about 30–34 years after original diagnosis of polio.
Prevention
There is no way to prevent PPS. However, paying attention to what types of exertion worsen symptoms may slow the progression of the syndrome.
BOOKS
Cohen, Jeffrey. "Enteroviruses and Reoviruses." In Harrison's Principles of Internal Medicine, ed. Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.
Price, Richard. "Poliomyelitis." In Cecil Textbook of Medicine, ed. J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders Co., 1996.
Ray, C. George. "Enteroviruses." In Sherris Medical Microbiology: An Introduction to Infectious Diseases. 3rd ed. Ed. Kenneth J. Ryan. Norwalk, CT: Appleton & Lange, 1994.
PERIODICALS
Bartfeld, Harry, and Dong Ma. "Recognizing Post-Polio Syndrome." Hospital Practice 31, no. 5 (15 May 1996): 95+.
Brown, Marybeth. "Post-Polio Syndrome." The New England Journal of Medicine 333, no. 8 (24 Aug. 1995): 532+.
Richardson, Sarah. "New Pain, Old Virus." Discover 16, no. 1 (Jan. 1995): 72+.
ORGANIZATIONS
International Polio Network, 4207 Lindell Blvd., Suite 110, St. Louis, MO 63108-2915. (314) 534-0475.
March of Dimes Birth Defects Foundation. 1275 Mamaroneck Ave., White Plains, NY 10605. (914) 428-7100. <http://www.modimes.org>.
