Inflammation in dysferlin myopathy: immunohistochemical characterization of 13 patients

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...

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Bibliographic Details
Published inNeurology Vol. 57; no. 11; p. 2136
Main Authors Gallardo, E, Rojas-García, R, de Luna, N, Pou, A, Brown, Jr, R H, Illa, I
Format Journal Article
LanguageEnglish
Published United States 11.12.2001
Subjects
Adolescent
Adult
Biopsy
CD4-Positive T-Lymphocytes - pathology
CD8-Positive T-Lymphocytes - pathology
Chromosomes, Human, Pair 2
Dysferlin
Female
Humans
Male
Membrane Proteins
Muscle Proteins - genetics
Muscle, Skeletal - pathology
Muscular Dystrophies - genetics
Muscular Dystrophies - pathology
Myositis - genetics
Myositis - pathology
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Summary: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.
ISSN:0028-3878
DOI:10.1212/wnl.57.11.2136