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• Vol.54 No.12
• CLINICA NEUROL, 54: 1086−1088, 2014
• Vol.54 No.12 contents

Symposium 17

Motor nerve hyperexcitability in ALS: its pathophysiology and treatment

Kazumoto Shibuya, M.D., Ph.D.¹⁾, Sonoko Misawa, M.D., Ph.D.¹⁾ and Satoshi Kuwabara, M.D., Ph.D.¹⁾

¹⁾Department of Neurology Graduate School of Medicine Chiba University

Wide-spread fasciculations are prominent clinical features in patients with amyotrophic lateral sclerosis (ALS), specifically seen in ALS among disorders with neurogenic muscle atrophy. Fasciculations frequently arise from the motor nerve terminals, and the hyperexcitability of motor axons appears to constitute the pathophysiology of the disease. Neurophysiologic investigations of the upper and lower motor neurons/axons have shown increased excitability. The altered excitability is supposed to relate to motor neuron death. Based on these findings, a clinical trial of mexiletine (non-selective sodium channel blocker) is ongoing.
Full Text of this Article in Japanese PDF (240K)

(CLINICA NEUROL, 54: 1086−1088, 2014)
key words: fasciculation, split hand, hyperexcitability, axonal excitability, sodium current

(Received: 23-May-14)