McArdle's disease presenting with asymmetric, late-onset arm weakness

Gil I. Wolfe, Noel S. Baker, Ronald G. Haller, Dennis K. Burns, Richard J. Barohn

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


McArdle's disease or myophosphorylase deficiency is one of the most common muscle glycogenoses and typically presents in childhood or adolescence with exercise intolerance, myalgia, myoglobinuria, and cramps in exercising muscle. We describe an elderly man who developed asymmetric proximal arm weakness at age 73. He had no history of exercise-induced cramps, myalgias, or myoglobinuria. Creatine kinase levels were elevated, serum lactate did not rise on ischemic exercise testing, and muscle biopsy showed a vacuolar myopathy with absent myophosphorylase activity. This unusual case demonstrates that McArdle's disease may present with fixed, asymmetric proximal weakness at an advanced age and should be considered in this clinical setting, especially when a history of poor exercise tolerance can be elicited. (C) 2000 John Wiley and Sons, Inc.

Original languageEnglish (US)
Pages (from-to)641-645
Number of pages5
JournalMuscle and Nerve
Issue number4
StatePublished - Apr 2000


  • Late-onset
  • McArdle's disease
  • Myophosphorylase
  • Weakness

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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