Inflammation in dysferlin myopathy: immunohistochemical characterization of 13 patients.
case_series · Level IV
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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.
Medical subject headings
- CD4-Positive T-Lymphocytes
- CD8-Positive T-Lymphocytes
- Chromosomes, Human, Pair 2
- Membrane Proteins
- Muscle Proteins
- Muscular Dystrophies
- Myositis