**8. IVIg in therapy of post-polio syndrome (PPS)**

Post-polio syndrome (PPS) is characterized by new muscle weakness, muscle atrophy, fatigue and pain developing several years after acute polio. The prevalence of PPS in patients with previous polio is 20–60%.

Post-polio syndrome is caused by an increased degeneration of enlarged motor units, and some motor neurones cannot maintain all their nerve terminals. Muscle overuse may contribute. Immunological and inflammatory signs have been reported in the cerebrospinal fluid and central nervous tissue (EFNS task force, 2008).

There are two RCTs of treatment with IVIg in PPS (class I evidence) including 155 patients. In the study with highest power, a significant increase of mean muscle strength of 8.3% was reported after two IVIg treatment cycles during 3 months. Physical activity and subjective vitality also differed significantly in favour of the IVIG group (Farbu et al., 2007).

Post-polio syndrome is a chronic condition. Although a modest IVIG effect has been described short term, nothing is known about long-term effects. Responders and nonresponders have not been defined.

Any relationship between the clinical response to IVIG treatment and PPS severity, cerebrospinal fluid inflammatory changes and cerebrospinal fluid changes after IVIg is unknown. Optimal dose and IVIG cycle frequency has not been examined. Cost-benefit evaluation has not been performed.

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