Stimulation frequency-dependent neuromuscular junction transmission defects in patients with prior poliomyelitis.
Generalized fatigue and muscle fatiguability are major symptoms of post-poliomyelitis syndrome (PPS), and may be due to neuromuscular junction transmission defects, as suggested by increased jitter on single fiber electromyography (SFEMG). To determine the etiology of this defect, we studied jitter at low (1, 5 Hz) and high (10, 15, 20 Hz) frequency stimulation with stimulation SFEMG in 17 post-polio patients with muscle fatiguability, and in 9 normal controls. In 5 of 17 PPS patients and in 1 of 9 controls, jitter was significantly higher (unpaired t-test, P < 0.05) at high frequency stimulation (HFS). In the remaining PPS patients and controls there was no significant difference in jitter at high and low stimulation frequencies. PPS patients with increased jitter at HFS had a significantly longer time interval since acute polio (mean 48.5 years) than PPS patients without increased jitter at HFS (mean 40 years; P < 0.05), but were not distinguished by other historical or clinical criteria. We conclude that the neuromuscular junction defect in post-polio patients is similar to that observed in amyotrophic lateral sclerosis, and is probably due to ineffective conduction along immature nerve sprouts and exhaustion of acetylcholine stores. The appearance of an increase in jitter with HFS in post-polio patients may be dependent upon time after acute polio.
Trojan
D A
DA
Department of Neurology, McGill University, Montreal Neurological Institute and Hospital, Quebec, Canada.
Gendron
D
D
Cashman
N R
NR
D000328:Adult; D000368:Aged; D004558:Electric Stimulation; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009412:Nerve Fibers / Q000502:physiology; D009469:Neuromuscular Junction / Q000502:physiology*; D016262:Postpoliomyelitis Syndrome / Q000503:physiopathology*; D009435:Synaptic Transmission / Q000502:physiology*