Honda H, Mano Y, Takahashi A
Department of Neurology, Nagoya University School of Medicine, Japan.
J Neurol. 1987 Aug;234(6):408-11. doi: 10.1007/BF00314086.
A 34-year-old man developed slowly progressive muscular wasting of facioscapulohumeral (FSH) distribution with marked asymmetry. Clinical features, family history and laboratory findings were consistent with the diagnosis of FSH dystrophy. However, muscle biopsy revealed an inflammatory reaction with perivascular and endomysial infiltrate, myophagocytosis and invasion of the fibres by mononuclear cells. Monoclonal antibody analysis of the phenotypes of cells in the biopsy specimen suggested that cell-mediated immune response may occur during muscle fibre injury in FSH dystrophy.
一名34岁男性出现了缓慢进展的面肩肱型（FSH）分布的肌肉萎缩，且明显不对称。临床特征、家族史和实验室检查结果均符合FSH型肌营养不良的诊断。然而，肌肉活检显示有炎症反应，伴有血管周围和肌内膜浸润、肌细胞吞噬以及单核细胞侵入纤维。对活检标本中细胞表型的单克隆抗体分析表明，在FSH型肌营养不良的肌纤维损伤过程中可能发生细胞介导的免疫反应。
