Neuromuskuläre Übertragungsstörung beim Post-Polio-Syndrom: Erfolgreiche Therapie mit einem Cholinesterasehemmer

Abstract

Successful Therapy with an Anticholinesterase Agent: Post-poliomyelitis syndrome (PPS) is characterised by the appearance of new neuromuscular symptoms decades after recovery from paralytic poliomyelitis. We describe a thirty-seven-year old Korean lady who suffered from poliomyelitis with transitory tetraparesis in the first year of her life. A permanent atrophic paresis of her right leg remained. Before admittance the paresis of the right leg increased progressively. Additionally, muscle fatigue and pain on exertion in the other extremities and generalised fatigue occurred. Physical examination including repetitive muscular contraction revealed a myasthenic syndrome of non-paretic muscles. Single-fiber electromyography of nonparetic muscles showed increased jitter indicating a neuromuscular junction transmission defect. Both myasthenic syndrome and pathological jitter improved under therapy with low-dose pyridostigmine. The pathogenesis of PPS is discussed in the light of the clinical findings, successful therapy with pyridostigmine, and current literature.