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目的探讨多发性周围神经黏液变性的临床病理学特征、诊断及预后。方法对3例多发性周围神经黏液变性的尸检病例进行常规组织切片,HE染色,光镜观察;部分组织进行免疫组化及特殊染色。结果心脏自主神经、部分纵隔内、甲状舌骨肌、胸骨舌骨肌及舌根部肌群内周围神经纤维出现多发性节段性黏液变性;AB-PAS染色显示病变神经纤维呈节段性黏液变性,免疫组化显示变性神经纤维内营养血管结构与密度异常改变。结论周围神经黏液变性可呈多发性,主要病理改变为周围神经纤维多发性、节段性黏液变性,严重时可见病变神经纤维束内神经纤维显著减少。该病变除可累及其他部位神经纤维外,心脏自主神经亦可受累。
Objective To investigate the clinicopathological features, diagnosis and prognosis of multiple peripheral mucinous degeneration. Methods Three cases of autopsy with multiple perineural mucinous degeneration were subjected to routine histological sectioning, HE staining and light microscopy. Some tissues were immunohistochemically and specially stained. RESULTS: Multiple segmental mucocutaneous degeneration occurred in the autonomic nerve of the heart, in part of the mediastinum, in the thyrogoid, sternohyoid, and the lingual root muscles. AB-PAS staining showed segmental mucoid degeneration in the diseased nerve fibers Immunohistochemistry showed that the structure and density of nutrient vessels in degenerative nerve fibers changed abnormally. Conclusions Peripheral neuromuscular degeneration may be multiple. The main pathological changes are multiple and segmental mucosal degeneration of peripheral nerve fibers. In severe cases, the number of nerve fibers in the lesioned nerve fiber bundle is significantly reduced. In addition to the lesion can affect other parts of the nerve fibers, the heart can also be involved in autonomic nerve involvement.