Document Detail

McArdle's disease presenting with asymmetric, late-onset arm weakness.
MedLine Citation:
PMID:  10716777     Owner:  NLM     Status:  MEDLINE    
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.
G I Wolfe; N S Baker; R G Haller; D K Burns; R J Barohn
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Muscle & nerve     Volume:  23     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  2000 Apr 
Date Detail:
Created Date:  2000-04-19     Completed Date:  2000-04-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  641-5     Citation Subset:  IM    
Copyright Information:
Copyright 2000 John Wiley & Sons, Inc.
Department of Neurology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, Texas 75235-8897, USA.
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MeSH Terms
Aged, 80 and over
Functional Laterality
Glycogen Storage Disease Type V / diagnosis*,  pathology,  physiopathology*
Muscle Weakness*
Muscle, Skeletal / pathology
Sarcolemma / pathology,  ultrastructure
Vacuoles / pathology,  ultrastructure

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