In vitro microelectrode study of neuromuscular transmission in a case of botulism.
We performed in vitro microelectrode studies in the anconeus muscle biopsy of a 6-week-old infant intoxicated with Clostridium botulinum toxin B. The most striking abnormalities were the severe reduction of the endplate potential (EPP) quantal content and the marked variability of EPP latencies. The increased variability was often limited to a "single quantum" component of the EPP. Neither the amplitudes nor the frequencies of spontaneous miniature endplate potentials (MEEPs) were decreased. However, there was a wide range of amplitudes and frequencies of MEPPs. This unique combination of electrophysiologic findings indicates a severe presynaptic failure of neuromuscular transmission, which appears to result from an impairment of the process of synaptic vesicle release taking place after the stimulus induced influx of calcium into the motor nerve terminals.
Maselli
R A
RA
Department of Neurology, University of Chicago, Illinois.
Burnett
M E
ME
Tonsgard
J H
JH
D001706:Biopsy; D001905:Botulinum Toxins / Q000494:pharmacology; D001906:Botulism / Q000209:etiology / Q000503:physiopathology*; D004576:Electromyography; D005260:Female; D006801:Humans; D007223:Infant; D009045:Motor Endplate / Q000187:drug effects; D009132:Muscles / Q000473:pathology; D009469:Neuromuscular Junction / Q000502:physiology*; D009435:Synaptic Transmission / Q000502:physiology*; D013572:Synaptic Vesicles / Q000187:drug effects
D001905:Botulinum Toxins