A motor unit is a nerve cell or neuron and the muscle fibers it activates. Poliovirus attacks specific neurons in the brainstem and the anterior horn cells of the spinal cord, generally resulting in the death of a substantial fraction of the motor neurons controlling skeletal muscles.
Post Polio Syndrome - NORD (National Organization for Rare Disorders)
In an effort to compensate for the loss of these neurons, surviving motor neurons sprout new nerve terminals to the orphaned muscle fibers. The result is some recovery of movement and the development of enlarged motor units. The neural fatigue theory proposes that the enlargement of the motor neuron fibers places added metabolic stress on the nerve cell body to nourish the additional fibers.
After years of use, this stress may be more than the neuron can handle, leading to the gradual deterioration of the sprouted fibers and, eventually, the neuron itself.
This causes muscle weakness and paralysis. Restoration of nerve function may occur in some fibers a second time, but eventually nerve terminals malfunction and permanent weakness occurs. There is an ongoing denervation and reinnervation, but the reinnervation process has an upper limit where the reinnervation cannot compensate for the ongoing denervation, and loss of motor units takes place. With age, most people experience a decrease in the number of spinal motor neurons.
Because polio survivors have already lost a considerable number of motor neurons, further age-related loss of neurons may contribute substantially to new muscle weakness. The overuse and underuse of muscles also may contribute to muscle weakness. Another theory is that people who have recovered from polio lose remaining healthy neurons at a faster rate than normal.
However, little evidence exists to support this idea. Again, compared to neural fatigue, the evidence supporting this theory is quite limited. Diagnosis of post-polio syndrome can be difficult, since the symptoms are hard to separate from complications due to the original poliomyelitis infection, and from the normal infirmities of aging.
There is no laboratory test for post-polio syndrome, nor are there any other specific diagnostic criteria. Three important criteria are recognized, including: previous diagnosis of polio, long interval after recovery and the gradual onset of weakness.
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In general, PPS is a diagnosis of exclusion whereby other possible causes of the symptoms are eliminated. Objective assessment of muscle strength in PPS patients may not be easy. Changes in muscle strength are determined in specific muscle groups using various muscle scales which quantify strength, such as the Medical Research Council MRC scale.
Magnetic resonance imaging MRI , neuroimaging , and electrophysiological studies, muscle biopsies , or spinal fluid analysis may also be useful in establishing a PPS diagnosis. PPS treatment — concerns comfort relieving pain via analgesics and rest via utilisation of mechanisms to make life easier, such as a powered wheelchair — is generally of palliative care. There are no reversive therapies. Fatigue is usually the more disabling symptom. Energy conservation can significantly reduce fatigue episodes. Such can be achieved by lifestyle changes: additional daytime sleep, reducing workload, weight loss for obesity.
Some require lower limb orthotics to reduce energy usage. Medications for fatigue, such as amantadine and pyridostigmine , are ineffective in the management of PPS. Management should focus on treatments such as hydrotherapy and developing other routines that encourage strength but do not affect fatigue levels. PPS increasingly stresses the musculoskeletal system from progressive muscular atrophy. A recent study showed that in a review of PPS patients, 80 percent reported pain in muscles and joints and 87 percent had fatigue.
Points of view or opinions do not, therefore, necessarily represent official Administration for Community Living policy. Reeve Foundation. Roosevelt, polio survivor Resources. History of polio Poliomyelitis is a disease caused by a virus that attacks nerves that control motor function.
The World Health Organization WHO estimates that 12 million people worldwide live with some degree of disability caused by poliomyelitis. What is post-polio syndrome? Medical professionals called this post-polio syndrome PPS. Managing PPS Polio survivors are urged to take care of their health in all the usual ways — by seeking periodic medical attention, being nutrition-wise, avoiding excessive weight gain, and by stopping smoking or overindulging in alcohol.
Franklin D. Roosevelt, polio survivor Franklin D. Roosevelt, seldom seen as a polio survivor, with Ruthie Bye and Fala, Resources If you are looking for more information on post-polio syndrome or have a specific question, our information specialists are available business weekdays, Monday through Friday, toll-free at from 9am to 5pm ET. We encourage you to also reach out to post-polio syndrome support groups and organizations, including: Post-Polio Health International offers information for polio survivors and promotes networking among the post-polio community. Louis 50 years ago. Connect with us t f y l g.
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. 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. 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.
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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. All studies receiving U. Beers MH, Berkow R.
The Merck Manual, 17th ed. Berkow R.
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The Merck Manual-Home Edition. Churchill Livingstone Inc. New York, NY; Rowland LP. Philadelphia, PA. A year follow-up of neuromuscular function in patients with prior poliomyelitis. Sandberg A, Stalberg E. How to interpret normal electromyographic findings in patients with an alleged history of polio.
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J Rehabil Med. Effort-limited treadmill walk test: reliability and validity in subjects with postpolio syndrome. Am J Phys Med Rehabil. Sanberg A, Stalberg E.