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神经系统结节病

Sarcoidosis of the nervous system.

作者信息

Joseph F G, Scolding N J

机构信息

Department of Neurology, Institute of Clinical Neurosciences, University of Bristol, Bristol, UK.

出版信息

Pract Neurol. 2007 Aug;7(4):234-44. doi: 10.1136/jnnp.2007.124263.

Abstract

Although sarcoidosis is rarely confined to the nervous system, any neurological features that do occur frequently happen early in the course of the disease. The most common neurological presentation is with cranial neuropathies, but seizures, chronic meningitis and the effects of mass lesions are also frequent. The diagnostic process should first confirm nervous system involvement and then provide supportive evidence for the underlying disease; in the absence of any positive tissue biopsy, the most useful diagnostic tests are gadolinium enhanced MRI of the brain and CSF analysis, although both are non-specific. The mainstay of treatment is corticosteroids, but these often have to be combined with other immunosuppressants such as methotrexate, hydroxychloroquine or cyclophosphamide. There is increasing evidence that infliximab is a safe treatment with good steroid sparing capacity.

摘要

尽管结节病很少局限于神经系统,但确实出现的任何神经学特征通常在疾病进程早期就会出现。最常见的神经学表现是颅神经病变,但癫痫发作、慢性脑膜炎和占位性病变的影响也很常见。诊断过程应首先确认神经系统受累情况,然后为潜在疾病提供支持性证据;在没有任何阳性组织活检的情况下,最有用的诊断测试是钆增强脑部磁共振成像(MRI)和脑脊液分析,尽管两者都不具有特异性。治疗的主要手段是使用皮质类固醇,但通常必须将其与其他免疫抑制剂联合使用,如甲氨蝶呤、羟氯喹或环磷酰胺。越来越多的证据表明,英夫利昔单抗是一种安全的治疗方法,具有良好的减少类固醇用量的能力。

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