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[多发性单神经病与血管炎]

[Multiple mononeuropathy and vasculitis].

作者信息

Cartier L, García M, Peñaherrera P, Vergara C

机构信息

Departamento de Ciencias Neurológicas, Facultad de Medicina, Universidad de Chile, Santiago-Chile.

出版信息

Rev Med Chil. 1999 Feb;127(2):189-96.

Abstract

BACKGROUND

Seventy percent of vasculitis are neurologically expressed as multiple mononeuropathy (MM) or asymmetrical neuropathy (AN). Concurrent nerve and muscle biopsy increases the diagnostic accuracy of the disease.

AIM

To define the pathological features of vascular damage in nerve and muscle in patients with MM or AN.

PATIENTS AND METHODS

Between 1980 and 1997, 50 patients with a MM or AN diagnosis, based on neurological and neurophysiological findings, were studied at the Neurology Department of Hospital del Salvador. All underwent nerve and muscle biopsy (of the superficial peroneal nerve and the short peroneal muscle). Slices were stained with hematoxylin eosin, luxol fast blue and Gomori staining.

RESULTS

Forty two patients, aged 52 +/- 15 years old (29 female) had a vasculitis. These subjects with MM or AN associated to vasculitis, corresponded to 22% of neuropathies subjected to nerve biopsy at the Department in the study period. Thirty two cases (76%) had necrotizing arteritis, characterized by wall fibrinoid necrosis and lumen occlusion in large vessels (> 100 microns), with lymphoplasmocytic and macrophage infiltration. Ten cases showed an inflammatory reaction and endothelial proliferation without wall necrosis, specially in small epineural arteries. Vascular recanalization was found in 33% of cases. Diagnostic vascular changes were found in 87% of nerve biopsies and 53% of muscle biopsies. No definitive relationship between the intensity of vascular and nerve lesions was found. All muscle biopsies showed some degree of neurogenic atrophy and 5 had micro infarcts.

CONCLUSIONS

Superficial peroneal nerve biopsy is diagnostic in most patients with MM or AN associated with vasculitis. Nerve and muscle biopsies are complementary in the diagnostic work up.

摘要

背景

70%的血管炎在神经方面表现为多发性单神经病(MM)或不对称性神经病(AN)。同时进行神经和肌肉活检可提高该病的诊断准确性。

目的

明确MM或AN患者神经和肌肉中血管损伤的病理特征。

患者与方法

1980年至1997年间,在萨尔瓦多医院神经科对50例根据神经学和神经生理学检查结果诊断为MM或AN的患者进行了研究。所有患者均接受了神经和肌肉活检(腓浅神经和腓短肌)。切片用苏木精伊红染色法、髓磷脂碱性蛋白染色法和Gomori染色法染色。

结果

42例患者(年龄52±15岁,女性29例)患有血管炎。这些伴有血管炎的MM或AN患者,占研究期间该科室接受神经活检的神经病患者的22%。32例(76%)有坏死性动脉炎,其特征为大血管(>100微米)壁的纤维蛋白样坏死和管腔闭塞,伴有淋巴细胞、浆细胞和巨噬细胞浸润。10例表现为炎症反应和内皮细胞增生,但无管壁坏死,尤其在神经外膜小动脉。33%的病例发现血管再通。87%的神经活检和53%的肌肉活检发现有诊断性血管改变。未发现血管和神经病变强度之间有明确关系。所有肌肉活检均显示一定程度的神经源性萎缩,5例有微梗死。

结论

对于大多数伴有血管炎的MM或AN患者,腓浅神经活检具有诊断价值。神经和肌肉活检在诊断检查中具有互补性。

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