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Sat, 31 May 2008 17:55:07
By Patricia Khashayar, MD.,

Post Polio Syndrome


How is PPS diagnosed?

Criteria for diagnosis of post-polio syndrome*

Prior paralytic poliomyelitis with evidence of motor neuron loss, as confirmed by history of the acute paralytic illness, signs of residual weakness and atrophy of muscles on neuromuscular examination, and signs of nerve damage on electromyography (EMG). Rarely, persons have subclinical paralytic polio, described as a loss of motor neurons during acute polio but with no obvious deficit. That prior polio now needs to be confirmed with an EMG. Also, a reported history of nonparalytic polio may be inaccurate.

A period of partial or complete functional recovery after acute paralytic poliomyelitis, followed by an interval (usually 15 years or more) of stable neuromuscular function.

Gradual onset of progressive and persistent new muscle weakness or abnormal muscle fatigability (decreased endurance), with or without generalized fatigue, muscle atrophy, or muscle and joint pain. Onset may at times follow trauma, surgery, or a period of inactivity, and can appear to be sudden. Less commonly, symptoms attributed to PPS include new problems with breathing or swallowing.

Symptoms that persist for at least a year.

Exclusion of other neuromuscular, medical, and orthopedic problems as causes of symptoms.

*Modified from: Post-Polio Syndrome: Identifying Best Practices in Diagnosis & Care. March of Dimes, 2001.

PPS may be difficult to diagnose in some people because other medical conditions can complicate the evaluation. Depression, for example, also is associated with fatigue and can be misinterpreted as PPS or vice versa. For this reason, some clinicians use less restrictive diagnostic criteria, while others prefer to categorize new problems as the late effects of polio—for example, shoulder osteoarthritis from walking with crutches, a chronic rotator cuff tear leading to pain and disuse weakness, or breathing insufficiency due to progressive scoliosis.

Polio survivors with PPS symptoms need to visit a physician trained in neuromuscular disorders to clearly establish potential causes for declining strength and to assess progression of weakness not explained by other health problems.

Physicians may use magnetic resonance imaging (MRI), computed tomography (CT), neuroimaging, and electrophysiological studies as tools to investigate the course of decline in muscle strength. Less commonly, they will conduct a muscle biopsy or a spinal fluid analysis. These tests are also important to exclude other, possibly treatable, conditions that mimic PPS, but the tests do not identify survivors at greatest risk for new progression of muscle weakness.

How is PPS treated?There are currently IVIg  treatments for the syndrome itself. However, a number of controlled studies have demonstrated that nonfatiguing exercises can improve muscle strength.

Researchers have found that CIDP is the underlaying issue in post polio syndrome and IVIg helps it,

In an effort to reduce fatigue, increase strength, and improve quality of life in PPS patients, scientists conducted two controlled studies using low doses of the drug pyridostigmine (Mestinon). These studies showed that pyridostigmine is not helpful for PPS patients.

In another controlled study scientists concluded that the drug amantadine is not helpful in reducing fatigue. And other researchers recently evaluated the effectiveness of modifinil (Provigil) on reducing fatigue and found no benefit.

The future of PPS treatment may center on IVIg.




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