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[神经结节病——诊断、临床表现及治疗]

[Neurosarcoidosis - diagnosis, clinical picture and therapy].

作者信息

Hebel Renata, Dubaniewicz-Wybieralska Mirosława, Dubaniewicz Anna

机构信息

Medical University of Gdansk, Poland: 1Department of Neurology.

Medical University of Gdansk, Poland: Department of Radiology.

出版信息

Pol Merkur Lekarski. 2018 Mar 27;44(261):130-134.

Abstract

Sarcoidosis (SA) is a granulomatous, multisystem disease of unknown etiology. Most often the disease affects lungs and mediastinal lymph nodes, but it may occur in other organs. Neurosarcoidosis (NS) more commonly occurs with other sarcoidosis forms, in 1% of cases it involves only nervous system. Symptomatic NS occurs but on autopsy study up to 25% of cases are confirmed. NS can affect central nervous system: the brain, spinal cord and peripheral nerves, and muscles. The diagnosis of neurosarcoidosis facilitates diagnostic criteria: histopathological, imaging and cerebrospinal fluid examination, and clinical symptoms. At present, there are no set standards for treatment of patients suffering from NS. Early therapy of symptomatic patients is recommended. Corticosteroids still are the first line of treatment for NS patients. In cases of steroids resistance, lack of their effectiveness or existence of contraindication to their use, immunosuppressant treatment is recommended. The latest NS algorithm with immunosuppressive treatment is discussed.

摘要

结节病(SA)是一种病因不明的肉芽肿性多系统疾病。该病最常累及肺部和纵隔淋巴结,但也可能发生于其他器官。神经结节病(NS)更常与其他结节病形式同时出现,1%的病例仅累及神经系统。有症状的NS确实存在,但尸检研究证实高达25%的病例存在该病。NS可影响中枢神经系统:脑、脊髓、周围神经和肌肉。神经结节病的诊断有助于诊断标准的制定:组织病理学、影像学和脑脊液检查以及临床症状。目前,对于患有NS的患者尚无既定的治疗标准。建议对有症状的患者进行早期治疗。皮质类固醇仍然是NS患者的一线治疗药物。在出现类固醇耐药、缺乏疗效或存在使用禁忌的情况下,建议进行免疫抑制治疗。讨论了最新的免疫抑制治疗NS算法。

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