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嗜酸性粒细胞增多性肌痛综合征的神经肌肉病理学

The neuromuscular pathology of the Eosinophilia-Myalgia syndrome.

作者信息

Seidman R J, Kaufman L D, Sokoloff L, Miller F, Iliya A, Peress N S

机构信息

Department of Pathology, State University of New York, Stony Brook 11794-8691.

出版信息

J Neuropathol Exp Neurol. 1991 Jan;50(1):49-62. doi: 10.1097/00005072-199101000-00004.

Abstract

The Eosinophilia-Myalgia Syndrome (EMS) is a recently reorganized disorder in patients ingesting pharmacologic doses of L-tryptophan. We studied the lesions of skeletal muscle, peripheral nerve and skin in 12 cases of EMS. Perimyositis was severe in four, moderate in two, mild in three and absent in three cases. The lesions contained many eosinophils, T-helper cells, mast cells and activated macrophages. Type 2 myofiber atrophy was present in five cases and in one, this was the only pathologic finding. Severe epineurial inflammation was seen in the three sural nerve biopsies. Indirect evidence for peripheral neurologic involvement in three other cases consisted of inflammation surrounding intramuscular nerve twigs (two cases) and neurogenic atrophy (one case). Phlebitis accompanied the connective tissue inflammation in five cases and endarteritis in one. Fasciitis was present in three of four skin biopsies and dermal fibrosis in one.

摘要

嗜酸性粒细胞增多性肌痛综合征(EMS)是一种最近重新归类的疾病,见于摄入药理剂量L-色氨酸的患者。我们研究了12例EMS患者的骨骼肌、周围神经和皮肤病变。4例为重度肌束膜炎,2例为中度,3例为轻度,3例无肌束膜炎。病变中有许多嗜酸性粒细胞、辅助性T细胞、肥大细胞和活化巨噬细胞。5例存在2型肌纤维萎缩,其中1例这是唯一的病理表现。在3例腓肠神经活检中可见严重的神经外膜炎症。另外3例周围神经受累的间接证据包括肌内神经小分支周围炎症(2例)和神经源性萎缩(1例)。5例伴有静脉炎的结缔组织炎症,1例伴有动脉内膜炎。4例皮肤活检中有3例存在筋膜炎,1例存在皮肤纤维化。

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