Tateishi J, Nagara H, Ohta M, Matsumoto T, Fukunaga H, Shida K
Acta Neuropathol. 1984;63(1):24-32. doi: 10.1007/BF00688467.
A 30-year-old man had had chronic progressive wasting and weakness of muscles for 17 years. A muscle biopsy 5 years prior to death revealed myopathic changes were rimmed vacuoles and intranuclear inclusions which corresponded to "inclusion body myositis". At autopsy, intranuclear inclusions were observed in neurons, oligodendroglia, and in parenchymal cells of the adrenal medulla. Ultrastructurally, the inclusions in muscles, nervous tissue, and adrenal medulla were identical and consisted of abnormal tubulolinear structures measuring 10-20 nm in diameter. Similar inclusions have been reported in muscles with "inclusion body myositis" and in the nervous system with "neuronal intranuclear hyaline inclusion disease", respectively. Absence of clinical symptoms related to the CNS and adrenal gland, and well-preserved parenchymal cells in these organs of our patient suggest a benign nature of the disease except in the muscular system. Attempts to isolate a virus from the brain were fruitless . This patient may serve to connect both diseases in muscles and the nervous system, and to disclose the etiology of these inclusions.
一名30岁男性有17年慢性进行性消瘦和肌肉无力病史。死亡前5年的肌肉活检显示肌病性改变为边缘空泡和核内包涵体,符合“包涵体肌炎”。尸检时,在神经元、少突胶质细胞和肾上腺髓质实质细胞中观察到核内包涵体。超微结构上,肌肉、神经组织和肾上腺髓质中的包涵体相同,由直径为10 - 20纳米的异常管状线性结构组成。类似的包涵体分别在患有“包涵体肌炎”的肌肉和患有“神经元核内透明包涵体病”的神经系统中被报道过。我们的患者中枢神经系统和肾上腺无相关临床症状,且这些器官实质细胞保存完好,提示除肌肉系统外,该疾病具有良性性质。从大脑中分离病毒的尝试未成功。该患者可能有助于联系肌肉和神经系统的这两种疾病,并揭示这些包涵体的病因。