Inflammation in dysferlin myopathy: immunohistochemical characterization of 13 patients.

E Gallardo, R Rojas-García, N de Luna, A Pou, R H Brown, I Illa

Journal: Neurology 2002;57(11):2136-8

PMID: 11739845

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.

Address: Department of Neurology, Neuromuscular Unit and Laboratory of Experimental Neurology, Hospital de la Sta Creu i St Pau and Institut de Recerca HSCSP, Universitat Autònoma de Barcelona, Spain.

DOI: 10.1212/wnl.57.11.2136

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MeSH Terms: Adolescent, Adult, Biopsy, CD4-Positive T-Lymphocytes, CD8-Positive T-Lymphocytes, Chromosomes, Human, Pair 2, Dysferlin, Female, Humans, Male, Membrane Proteins, Muscle Proteins, Muscle, Skeletal, Muscular Dystrophies, Myositis