Neuromuscular junction transmission defect in post-poliomyelitis syndrome

Citation
A. Frese et al., Neuromuscular junction transmission defect in post-poliomyelitis syndrome, KLIN NEUROP, 30(3), 1999, pp. 157-159
Citations number
13
Categorie Soggetti
Neurology
Journal title
KLINISCHE NEUROPHYSIOLOGIE
ISSN journal
14340275 → ACNP
Volume
30
Issue
3
Year of publication
1999
Pages
157 - 159
Database
ISI
SICI code
1434-0275(199909)30:3<157:NJTDIP>2.0.ZU;2-C
Abstract
Successful Therapy with an Anticholinesterase Agent: Post-poliomyelitis syn drome (PPS) is characterised by the appearance of new neuromuscular symptom s 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 o f her right leg remained. Before admittance the paresis of the right leg in creased progressively. Additionally, muscle fatigue and pain on exertion in the other extremities and generalised fatigue occurred. Physical examinati on including repetitive muscular contraction revealed a myasthenic syndrome of non-paretic muscles. Single-fiber electromyography of nonparetic muscle s showed increased jitter indicating a neuromuscular junction transmission defect. Both myasthenic syndrome and pathological jitter improved under the rapy with low-dose pyridostigmine. The pathogenesis of PPS is discussed in the light of the clinical findings, successful therapy with pyridostigmine, and current literature.