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Fatigue and abnormal neuromuscular transmission in Kennedy's disease
Author(s) -
Meriggioli Matthew N.,
Rowin Julie
Publication year - 2003
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.10295
Subject(s) - neuromuscular transmission , fasciculation , repetitive nerve stimulation , medicine , electromyography , neuromuscular junction , muscle fatigue , physical medicine and rehabilitation , weakness , neuromuscular disease , muscle weakness , anatomy , anesthesia , neuroscience , psychology , disease
We describe a patient with Kennedy's disease (X‐linked bulbospinal neuronopathy) who experienced leg muscle fatigue with long‐distance running. The patient also reported muscle twitching involving the face and extremities and long‐standing muscle cramps. Aside from mild facial and tongue weakness (and fasciculations), his examination was normal, including completely preserved muscle strength in the extremities. Electrodiagnostic evaluation revealed evidence for a chronic motor axonopathy/neuronopathy and abnormal sensory nerve action potentials. In addition, repetitive nerve stimulation studies were normal, but neuromuscular jitter tested in the same muscle was markedly abnormal. The normal clinical strength and repetitive nerve stimulation studies in a muscle showing markedly increased neuromuscular jitter suggested a mechanism for this patient's symptoms of muscle fatigue, related to failure of neuromuscular transmission at a critical number of endplates during extremes of physical activity. Muscle Nerve 27: 249–251, 2003