Inflammation in dysferlin myopathy: Immunohistochemical characterization of 13 patients

E. Gallardo, R. Rojas-García, N. De Luna, A. Pou, R. H. Brown, Isabel Illa

Research output: Contribution to journalArticleResearchpeer-review

162 Citations (Scopus)

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.
Original languageEnglish
Pages (from-to)2136-2138
JournalNeurology
Volume57
Issue number11
DOIs
Publication statusPublished - 11 Dec 2001

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