Nevo Y, Pestronk A
Department of Neurology, Barnes and St. Louis Children's Hospitals, Washington University School of Medicine, MO 63110, USA.
Pediatr Neurol. 1996 Sep;15(2):150-2. doi: 10.1016/0887-8994(96)00127-0.
Perifascicular atrophy of muscle fibers is generally considered to be a specific feature of autoimmune myopathies, dermatomyositis in particular. We describe a neonate presenting with hypotonia and weakness. A biopsy revealed atrophic and regenerating muscle fibers in a perifascicular distribution, and abnormal alkaline phosphatase activity in neighboring perimysial connective tissue. The weakness was nonprogressive and improved on follow-up even though no long-term treatment was administered. We conclude that the presence of perifascicular myopathic changes and muscle fiber atrophy in infants presenting with hypotonia and weakness is neither diagnostic of progressive dermatomyositis, nor a necessary indication for immunosuppressive therapy.
肌纤维束周萎缩通常被认为是自身免疫性肌病的一个特征性表现，尤其是皮肌炎。我们报告了一名表现为肌张力减退和肌无力的新生儿。活检显示肌纤维呈束周分布的萎缩和再生，且相邻肌束膜结缔组织碱性磷酸酶活性异常。尽管未进行长期治疗，但肌无力未进展且随访时有所改善。我们得出结论，对于表现为肌张力减退和肌无力的婴儿，出现束周肌病性改变和肌纤维萎缩既不能诊断为进行性皮肌炎，也不是免疫抑制治疗的必要指征。
