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结节病的神经系统表现:文献综述及23例报告

Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases.

作者信息

Delaney P

出版信息

Ann Intern Med. 1977 Sep;87(3):336-45. doi: 10.7326/0003-4819-87-3-336.

Abstract

Nervous system involvement by sarcoidosis has been considered rare, yet more than 400 cases have been reported. We present 23 additional cases here, including 14 with autopsies, and we review the literature. The overall frequency of neurologic involvement is 5%. Patients usually have other stigmata of sarcoidosis; however, neurologic dysfunction is frequently the presenting finding. The protean manifestations of central nervous system (CNS) involvement usually occur in the early phase of the disease, while those of peripheral nervous system and skeletal muscle involvement are characteristically seen in the chronic stages. Basal granulomatous meningitis causes most of the CNS manifestations either by infiltration or compression of adjacent structures. Steroids are the mainstay of therapy, and the overall response is quite variable. The course is also variable, being transient in some and chronic in others. The prognosis is better with peripheral than with central nervous system involvement.

摘要

结节病累及神经系统曾被认为较为罕见,但已有400多例相关报道。我们在此报告另外23例病例,其中14例进行了尸检,并对相关文献进行综述。神经系统受累的总体发生率为5%。患者通常有结节病的其他体征;然而,神经功能障碍常为首发表现。中枢神经系统(CNS)受累的多样表现通常出现在疾病早期,而周围神经系统和骨骼肌受累的表现则典型地见于慢性期。基底肉芽肿性脑膜炎通过浸润或压迫相邻结构导致大多数CNS表现。类固醇是主要治疗手段,总体反应差异较大。病程也各不相同,有些为短暂性,有些为慢性。周围神经系统受累的预后优于中枢神经系统受累。

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