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Evidence for motor axon depolarization in Fabry disease
Author(s) -
Tan Stella V.,
Lee Philip J.,
Walters Richard J. L.,
Mehta Atul,
Bostock Hugh
Publication year - 2005
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.20390
Subject(s) - depolarization , pathophysiology , fabry disease , axon , medicine , ischemia , cardiology , median nerve , motor nerve , disease , hyperkalemia , neuroscience , anatomy , psychology
To investigate the pathophysiology of neuropathy in Fabry disease, multiple nerve excitability properties of median motor axons in 20 patients with this disorder but without hyperkalemia were compared with 35 age‐matched normal controls. In the patients, depolarizing threshold electrotonus was reduced ( P < 0.0001) and superexcitability was reduced ( P < 0.001), but late subexcitability was normal. These findings indicate that the axons were mildly depolarized, probably due to ischemia, and are consistent with the hypothesis that poor nerve perfusion in Fabry disease contributes to axonal damage. Muscle Nerve, 2005