Datema Mirjam, Tannemaat Martijn R, Drent Marjolein, Hoitsma Elske
Leids Universitair Medisch Centrum, afd. Neurologie, Leiden.
Ned Tijdschr Geneeskd. 2015;159:A8383.
The clinical spectrum of neurosarcoidosis is highly diverse; virtually any neurological syndrome can present, depending upon granuloma localization. There are indications that neurosarcoidosis is insufficiently recognized as a separate clinical entity. In the absence of major prospective clinical trials, we launched a large national online registry of neurosarcoidosis patients in the Netherlands in June 2014 at www.neurosarcoidose.nl. When a patient with sarcoidosis presents with neurological symptoms that may compatible with sarcoidosis, it is important to locate sites of disease activity accessible for biopsy outside the nervous system. Small nerve fibre neuropathy is a common neurological complication of sarcoidosis. It is considered an epiphenomenon, and therefore classified as 'para-neurosarcoidosis'. Neurological symptoms causing disability are an indication for immunosuppressive therapy. There is increasing evidence for the effective treatment of refractory neurosarcoidosis using biologicals. Whether the intravenous administration of immunoglobulins or ARA290, an erythropoietin derivative with TNF-α-antagonistic and tissue-protective characteristics, is effective for small nerve fibre neuropathy in sarcoidosis is subject for study.
神经结节病的临床谱高度多样；几乎任何神经综合征都可能出现，这取决于肉芽肿的定位。有迹象表明，神经结节病作为一种独立的临床实体未得到充分认识。由于缺乏大型前瞻性临床试验，我们于2014年6月在荷兰启动了一个大型全国性神经结节病患者在线登记处，网址为www.neurosarcoidose.nl。当结节病患者出现可能与结节病相符的神经症状时，重要的是在神经系统之外找到可进行活检的疾病活动部位。小神经纤维神经病变是结节病常见的神经并发症。它被认为是一种附带现象，因此被归类为“副神经结节病”。导致残疾的神经症状是免疫抑制治疗的指征。越来越多的证据表明，使用生物制剂可有效治疗难治性神经结节病。静脉注射免疫球蛋白或ARA290（一种具有TNF-α拮抗和组织保护特性的促红细胞生成素衍生物）对结节病中的小神经纤维神经病变是否有效有待研究。
