Journal
NEUROMUSCULAR DISORDERS
Volume 19, Issue 4, Pages 261-263Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2009.01.010
Keywords
Calpainopathy; LGMD 2A; Eosinophilic myositis; Muscular dystrophy
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An 11-year-old girl with a calpain-3 gene (CAPN-3) mutation and eosinophilic myositis on muscle biopsy had high serum CK levels and eosinophil counts which showed spontaneous fluctuations. After commencement of immunosuppressive therapy reciprocal changes occured in response to alterations in closes of the medications. Subacutely evolving and spreading muscle weakness developed during tapering of the immunosuppressive medications. These observations suggest that either the occurrence of eosinophilic myositis or the withdrawal of the immunosuppressive treatment may have accelerated the clinical course of the calpainopathy in this case. The positive effect of immunosuppressive therapy might have implications for the management of calpainopathy with all inflammatory component. (C) 2009 Elsevier B.V. All rights reserved.
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