Article Text
Paper
Endplate dysfunction causing respiratory failure in a patient with prior paralytic poliomyelitis
Abstract
A 56 year old man with late amyotrophic sequelae from poliomyelitis experienced progressive dyspnoea requiring intubation and artificial ventilation in the intensive care unit. Repetitive stimulation studies showed a marked decrement of the trapezius muscle response reversible with edrophonium. Ventilatory function considerably and lastingly improved under anticholinesterase treatment. In the absence of biological evidence for autoimmune myasthenia gravis, it is suggested that a mechanism implying endplate dysfunction related to postpolio syndrome. Repetitive stimulation procedure should be considered in postpolio syndrome patients as some of them may benefit from anticholinesterase treatment.
- postpolio syndrome
- neuromuscular transmission
- anticholinesterase agents
- ALS, amyotrophic lateral sclerosis
- EMG, electromyography
- MIP, maximal mouth inspiratory pressure
- MG, myasthenia gravis
- MuSK, muscle specific receptor tyrosine kinase
- PFT, pulmonary function tests
- PPS, postpolio syndrome
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Footnotes
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Competing interests: none declared.