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- Title
Primary α-sarcoglycan deficiency responsive to immunosuppression over three years.
- Authors
Connolly, Anne M.; Pestronk, Alan; Mehta, Shobhna; Al-Lozi, Muhammed
- Abstract
An 8-year-old girl developed weakness over 2 years and an elevated creatine kinase. The biopsy was most consistent with an active dystrophy with many inflammatory cells present. A trial of immunosuppression was started. In the first 2 months of treatment with prednisone, she had functionally and quantitatively significant improvement in her proximal strength. Over 3 years of treatment she maintained stable strength. Subsequent genetic studies showed that she had primary α-sarcoglycan deficiency. The timing and the degree of benefit in strength were similar to that seen in boys with Duchenne muscular dystrophy who are treated with prednisone. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1549-1553, 1998
- Publication
Muscle & Nerve, 1998, Vol 21, Issue 11, p1549
- ISSN
0148-639X
- Publication type
Article
- DOI
10.1002/(SICI)1097-4598(199811)21:11<1549::AID-MUS30>3.0.CO;2-T