Axonal excitability during ischemia in MELAS.

Abstract:

INTRODUCTION:In mitochondrial disease, it is likely that energy substrate depletion leads to paralysis of ATPase-dependent pumps, resulting in membrane depolarization. Axonal depolarization has been demonstrated in a crisis, but not in the resting state. We, therefore, stressed axons using ischemia to see if this would reveal abnormal responses, as occurs in diabetes mellitus. METHODS:Excitability of median nerve axons at the wrist was studied in 13 patients with MELAS (6 with glucose intolerance) and 17 control subjects in response to ischemia due to inflation of a cuff around the arm for 10 min. RESULTS:There were no significant differences in preischemic measures of axonal excitability or in the intra- and postischemic responses. CONCLUSIONS:Although depolarization has been noted to occur spontaneously during a crisis, we could not demonstrate a defect of axonal ATP-dependent mechanisms. The mechanisms underlying axonal excitability and neuropathy in diabetes may not apply to MELAS.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Ng K,Kumar KR,Sue C,Burke D

doi

10.1002/mus.23733

subject

Has Abstract

pub_date

2013-05-01 00:00:00

pages

762-5

issue

5

eissn

0148-639X

issn

1097-4598

journal_volume

47

pub_type

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