Figarella-Branger D, Pellissier J F, Pouget J, Calore E E, Azulay J P, Desnuelle C, Serratrice G
Service d'Anatomie Pathologique et de Neuropathologie, Hôpital de la Timone, Marseille.
Rev Neurol (Paris). 1992;148(4):281-90.
A retrospective study of 40 patients with various neuromuscular disorders and more than 3 muscle fibers with rimmed vacuoles has been performed. Two subgroups of patients were distinguished according to the presence or absence of inflammatory exudates. In the first group (14 patients), inflammatory exudates were observed and numerous fibers showed partial invasion. Abnormal filamentous inclusions (16-18 nm in diameter) were found by electron microscopy in muscle fibers cytoplasm and/or nuclei. The diagnosis of inclusion body myositis (IBM) was made in these cases. They presented with insidious proximal muscle weakness and were not improved by immunosuppressive therapy. Immunohistological studies demonstrated T lymphocytes predominance, only few natural killer and B lymphocytes. The number of T8 lymphocytes was high in endomysial sites while T4 were more numerous in perivascular exudates. Abnormal membranous expression of class I MHC antigens was observed on muscle fibers lying near the inflammatory exudates. In the second group of cases (26 patients), no inflammatory exudate was observed. This group of neuromuscular diseases with rimmed vacuoles was heterogeneous. In 10 cases, abnormal filamentous inclusions (16-18 nm in diameter) were observed in rimmed vacuoles. However, this ultrastructural feature did not help in distinguishing subgroups. Various neuromuscular disorders were observed in this group: oculopharyngeal muscular dystrophy (12 cases with IBM like filaments in 4 cases), chronic spinal atrophy (5 cases with IBM like filaments in 3 cases), post poliomyelitis syndrome (2 cases with IBM-like filaments in one), muscle glycogenosis with IBM like filaments (2 cases), hereditary limb girdle myopathy or distal myopathy (3 cases) and 1 patient clinically presenting with polymyositis and another with cramps and myalgias. No abnormal sarcolemmal expression of class I MHC was found in this group. The pathogenesis of IBM is discussed. Besides T cell mediated cytotoxicity, denervation may be involved. The nature of the abnormal 16-18 nm filamentous inclusions remains unknown. These filaments are not IBM specific.
对40例患有各种神经肌肉疾病且有超过3条边缘空泡肌纤维的患者进行了回顾性研究。根据是否存在炎性渗出物将患者分为两个亚组。第一组（14例患者）观察到炎性渗出物，许多肌纤维显示部分浸润。通过电子显微镜在肌纤维细胞质和/或细胞核中发现了异常的丝状包涵体（直径16 - 18nm）。这些病例诊断为包涵体肌炎（IBM）。他们表现为隐匿性近端肌无力，免疫抑制治疗无效。免疫组织学研究显示T淋巴细胞占优势，只有少数自然杀伤细胞和B淋巴细胞。在肌内膜部位T8淋巴细胞数量较多，而在血管周围渗出物中T4淋巴细胞数量更多。在炎性渗出物附近的肌纤维上观察到I类MHC抗原的异常膜表达。第二组病例（26例患者）未观察到炎性渗出物。这组有边缘空泡的神经肌肉疾病是异质性的。在10例病例中，在边缘空泡中观察到异常的丝状包涵体（直径16 - 18nm）。然而，这种超微结构特征无助于区分亚组。在这组中观察到各种神经肌肉疾病：眼咽型肌营养不良（12例，4例有IBM样细丝）、慢性脊髓萎缩（5例，3例有IBM样细丝）、脊髓灰质炎后综合征（2例，1例有IBM样细丝）、伴有IBM样细丝的肌肉糖原贮积症（2例）、遗传性肢带型肌病或远端肌病（3例），1例临床诊断为多发性肌炎，另1例表现为痉挛和肌痛。该组未发现I类MHC的异常肌膜表达。讨论了IBM的发病机制。除了T细胞介导的细胞毒性外，可能还涉及失神经支配。异常的16 - 18nm丝状包涵体的性质仍然未知。这些细丝并非IBM所特有。
