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McArdle's disease presenting with asymmetric, late-onset arm weakness.
- Source :
-
Muscle & nerve [Muscle Nerve] 2000 Apr; Vol. 23 (4), pp. 641-5. - Publication Year :
- 2000
-
Abstract
- 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.<br /> (Copyright 2000 John Wiley & Sons, Inc.)
- Subjects :
- Aged
Aged, 80 and over
Arm
Functional Laterality
Glycogen Storage Disease Type V pathology
Humans
Male
Muscle, Skeletal pathology
Sarcolemma pathology
Sarcolemma ultrastructure
Vacuoles pathology
Vacuoles ultrastructure
Glycogen Storage Disease Type V diagnosis
Glycogen Storage Disease Type V physiopathology
Muscle Weakness
Subjects
Details
- Language :
- English
- ISSN :
- 0148-639X
- Volume :
- 23
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Muscle & nerve
- Publication Type :
- Academic Journal
- Accession number :
- 10716777
- Full Text :
- https://doi.org/10.1002/(sici)1097-4598(200004)23:4<641::aid-mus25>3.0.co;2-m