Lower E E, Broderick J P, Brott T G, Baughman R P
Department of Internal Medicine, University of Cincinnati Medical Center, Ohio, USA.
Arch Intern Med. 1997 Sep 8;157(16):1864-8.
Neurological involvement is a significant cause of morbidity and mortality in patients with sarcoidosis. Detection and management of neurosarcoidosis remains problematic. Our interest in immunosuppressive agents for chronic sarcoidosis has given us experience with various agents for the treatment of sarcoidosis, including cyclophosphamide and methotrexate.
We analyzed all patients with sarcoidosis seen in our clinic during a 10-year period. Evaluation for neurological disease included routine physical examination. Magnetic resonance imaging, cerebral spinal fluid analysis, and neural tissue biopsy were performed where clinically indicated. Patients were treated with corticosteroids, methotrexate, or cyclophosphamide.
Neurological disease was identified in 71 of 554 patients with sarcoidosis. Seventh (facial) cranial nerve paralysis was the most common manifestation identified in 39 patients. This included 24 patients with facial nerve palsy as the only manifestation of neurological sarcoidosis in whom complete recovery was seen in all but 1 patient. Forty-eight patients with disease other than facial nerve palsy received corticosteroids or other therapies. Corticosteroids benefited only 14 patients (29%). Methotrexate successfully treated 17 (61%) of 28 patients and cyclophosphamide controlled disease in 9 (90%) of 10 assessable patients. Methotrexate and cyclophosphamide were each associated with a higher response rate than corticosteroids alone (chi 2, 14.6; P < .001).
Neurological symptoms can be significant manifestations of sarcoidosis. Facial nerve paralysis is a common, but usually self-limited form of disease. Other manifestations are usually chronic and agents other than corticosteroids appear to have increased efficacy with lower morbidity.
神经系统受累是结节病患者发病和死亡的重要原因。神经结节病的检测和管理仍然存在问题。我们对慢性结节病免疫抑制剂的关注使我们积累了使用多种药物治疗结节病的经验，包括环磷酰胺和甲氨蝶呤。
我们分析了10年间在我们诊所就诊的所有结节病患者。对神经疾病的评估包括常规体格检查。在临床指征明确时进行磁共振成像、脑脊液分析和神经组织活检。患者接受皮质类固醇、甲氨蝶呤或环磷酰胺治疗。
554例结节病患者中有71例被发现有神经疾病。第七（面）颅神经麻痹是最常见的表现，在39例患者中出现。这包括24例以面神经麻痹为神经结节病唯一表现的患者，除1例患者外，其余患者均完全康复。48例除面神经麻痹外有其他疾病的患者接受了皮质类固醇或其他治疗。皮质类固醇仅使14例患者（29%）受益。甲氨蝶呤成功治疗了28例患者中的17例（61%），环磷酰胺使10例可评估患者中的9例（90%）病情得到控制。甲氨蝶呤和环磷酰胺的有效率均高于单独使用皮质类固醇（χ²，14.6；P <.001）。
神经症状可能是结节病的重要表现。面神经麻痹是一种常见但通常为自限性的疾病形式。其他表现通常为慢性，除皮质类固醇外的其他药物似乎疗效更高且发病率更低。
