Valeyre D, Chapelon-Abric C, Belin C, Dumas J L
Service de pneumologie, Hôpital Avicenne, Bobigny, France.
Rev Med Interne. 1998 Jun;19(6):409-14. doi: 10.1016/s0248-8663(98)80865-5.
Current knowledge on central nervous system sarcoidosis.
Sarcoidosis is localized in the central nervous system in 5 to 16% of the cases. Various neurological manifestations are observed, including: seizures, cognitive or psychic manifestations, hypothalamic and pituitary involvement, local pseudotumors, and hydrocephalus very frequently associated with asymptomatic lymphocytic meningitis and with cranial nerve palsy, particularly palsy of the seventh nerve, occurring less regularly. CNS localization is most often an early manifestation of the disease, unmasking sarcoidosis. It is often part of primary or secondary systemic polyvisceral sarcoidosis. The diagnosis is mainly based on two arguments: confirmation of the existence of systemic sarcoidosis and clinical and paraclinical compatibility of neurological abnormalities (particularly at magnetic resonance imaging [MRI]). Neurological histopathology is rarely necessary to confirm the diagnosis. Corticotherapy is indicated in all symptomatic cases and most often leads to a more or less complete clinical response evidenced by regression of active lesions identified on MRI. The treatment must often be prolonged for several years, and clinical and MRI evolution help guide therapeutical choices for dosages and threshold doses. CNS involvement is potentially severe with mortality and morbidity rates that are not insignificant. Limiting of iatrogenic risks requires adequate follow-up.
Multicenter studies are necessary to determine factors influencing the incidence and long-term prognosis of CNS sarcoidosis treated with corticotherapy. The efficacy of treatments other than corticotherapy must be evaluated.
关于中枢神经系统结节病的现有知识。
结节病累及中枢神经系统的病例占5%至16%。可观察到多种神经学表现，包括：癫痫发作、认知或精神表现、下丘脑和垂体受累、局部假瘤以及脑积水，后者常与无症状淋巴细胞性脑膜炎及脑神经麻痹相关，尤其是面神经麻痹，发生频率较低。中枢神经系统受累通常是该疾病的早期表现，可揭示结节病。它常是原发性或继发性系统性多脏器结节病的一部分。诊断主要基于两个依据：确认存在系统性结节病以及神经学异常的临床和辅助检查相符性（特别是在磁共振成像[MRI]检查时）。很少需要神经组织病理学检查来确诊。所有有症状的病例均需使用皮质类固醇治疗，且多数情况下会导致或多或少完全的临床反应，MRI显示的活动性病变消退可证明这一点。治疗通常必须持续数年，临床和MRI的演变有助于指导剂量及阈值剂量的治疗选择。中枢神经系统受累可能很严重，死亡率和发病率都不容小觑。限制医源性风险需要进行充分的随访。
有必要开展多中心研究，以确定影响接受皮质类固醇治疗的中枢神经系统结节病发病率和长期预后的因素。必须评估皮质类固醇治疗以外的其他治疗方法的疗效。
