Hyperexcitability of motor and sensory neurons in neuromyotonia.

Abstract:

:Two members of a family with a neuropathy resembling Charcot-Marie-Tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resembled that of tetany, and Trousseau's and Chvostek's signs were present although no abnormality of calcium or magnesium metabolism could be demonstrated. Muscle spasms ceased during medication with carbamazepine, 600 mg daily. Nerve stimulation, electromyography, and regional neuromuscular blockade with curare indicated that the condition originated in peripheral nerve, while microneurographic recordings showed that sensory as well as motor fibers were hyperexcitable. Sural nerve biopsy revealed axonal degeneration involving myelinated and unmyelinated fibers. It was concluded that the neural hyperexcitability is caused by a membrane defect resulting in a low threshold for excitation throughout the length of the axon.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Lance JW,Burke D,Pollard J

doi

10.1002/ana.410050605

subject

Has Abstract

pub_date

1979-06-01 00:00:00

pages

523-32

issue

6

eissn

0364-5134

issn

1531-8249

journal_volume

5

pub_type

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