Electrophysiological findings in X-linked myopathy with excessive autophagy.

Journal Article (Journal Article)

We report electrophysiological features and magnetic resonance imaging muscle findings in 4 patients and 1 female carrier of X-linked myopathy with excessive autophagy. Motor units were polyphasic with high mean amplitude and normal duration. The thigh muscles were most severely involved, but myotonic discharges were abundant in both clinically affected and unaffected muscles. Along with the clinicopathological features, these electrophysiological findings distinguish X-linked myopathy with excessive autophagy from other limb-girdle myopathies.

Full Text

Duke Authors

Cited Authors

  • Jääskeläinen, SK; Juel, VC; Udd, B; Villanova, M; Liguori, R; Minassian, BA; Falck, B; Niemi, P; Kalimo, H

Published Date

  • May 2002

Published In

Volume / Issue

  • 51 / 5

Start / End Page

  • 648 - 652

PubMed ID

  • 12112116

International Standard Serial Number (ISSN)

  • 0364-5134

Digital Object Identifier (DOI)

  • 10.1002/ana.10173


  • eng

Conference Location

  • United States