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Neurology 2001;57:2136-2138
© 2001 American Academy of Neurology
Brief Communications

Inflammation in dysferlin myopathy: Immunohistochemical characterization of 13 patients

E. Gallardo, PhD;, R. Rojas–García, MD;, N. de Luna, PhD;, A. Pou, MD;, R.H. Brown, Jr., MD, PhD and I. Illa, MD

From the Department of Neurology (Drs. Gallardo, Rojas-García, Luna, and Illa), Neuromuscular Unit and Laboratory of Experimental Neurology, Hospital de la Sta Creu i St Pau and Institut de Recerca HSCSP, Universitat Autònoma de Barcelona; Department of Neurology (Dr. Pou), Hospital del Mar, Barcelona; and Day Neuromuscular Research Laboratory (Dr. Brown), Massachusetts General Hospital, Charlestown.

Address correspondence and reprint requests to Dr. Isabel Illa, Department of Neurology Hospital de la Santa Creu i Sant Pau, UAB, Sant Antoni Ma Claret, 167, Barcelona 08025, Spain; e-mail: iilla{at}santpau.es

Article abstract—— Inflammation was detected in 9 of 13 patients with different phenotypes of dysferlin myopathy. Endomysial or perivascular infiltrates consisted of 11.1% ± 6.6% CD8+ cells, 40.6% ± 22.8% CD4+ cells, 36.7% ± 23.7% macrophages, and no B cells. Major histocompatibility complex class I was not upregulated in normal muscle fibers. In young patients with sporadic proximal weakness, very high creatine kinase levels, necrotic fibers and inflammation in the muscle biopsy, a diagnosis of dysferlin myopathy should be considered.




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