Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Neuroepidemiology. 2017;48(3-4):87-94. doi: 10.1159/000477300. Epub 2017 Jun 10.
BACKGROUND/AIMS: Neurosarcoidosis is a rare condition with serious health consequences. However, little is known about clinical characteristics and outcome of neurosarcoidosis in the community setting.
Patients with neurosarcoidosis were identified from a previously described cohort of patients with incident sarcoidosis from Olmsted County, Minnesota, United States from 1976 to 2013 using individual medical record review. Data on clinical characteristics, treatment, and outcome were collected.
Neurological involvement by sarcoidosis occurred in 11 patients (3% of all patients with sarcoidosis). Cranial neuropathy was the most common type of neurological disease (5 patients; 45%) followed by peripheral neuropathy (3 patients; 27%), and meningitis (3 patients; 27%). Cerebrospinal fluid (CSF) pleocytosis and elevated CSF protein levels were observed in patients with meningitis, intramedullary spinal cord sarcoidosis, intracranial mass lesion and some patients with cranial neuropathy but were normal in patients with peripheral neuropathy. All patients received high-dose glucocorticoids as initial treatment and almost all responded to this therapy. Relapse after glucocorticoid dose reduction necessitated subsequent treatment with steroid-sparing agents in 4 patients.
Neurosarcoidosis is an uncommon manifestation of sarcoidosis. Neurosarcoidosis manifestations generally responded well to high-dose glucocorticoids in the majority of patients, but relapse was common.
背景/目的：神经结节病是一种罕见的疾病，会对健康造成严重影响。然而，对于社区环境中的神经结节病的临床特征和预后，人们知之甚少。
通过对来自美国明尼苏达州奥姆斯特德县的一个队列中从 1976 年到 2013 年发生的结节病患者进行个体病历回顾，从之前描述的队列中确定神经结节病患者。收集了有关临床特征、治疗和结局的数据。
11 名患者（所有结节病患者的 3%）出现结节病的神经受累。最常见的神经疾病类型是颅神经病变（5 例；45%），其次是周围神经病变（3 例；27%）和脑膜炎（3 例；27%）。脑膜炎、脊髓内结节病、颅内肿块病变和一些颅神经病变患者的脑脊液（CSF）中存在细胞增多和 CSF 蛋白水平升高，但周围神经病变患者的 CSF 中正常。所有患者均接受大剂量糖皮质激素作为初始治疗，几乎所有患者均对此治疗有反应。在 4 名患者中，由于糖皮质激素剂量减少后复发，需要随后使用类固醇保留剂进行治疗。
神经结节病是结节病的一种罕见表现。大多数患者的神经结节病表现通常对大剂量糖皮质激素反应良好，但复发很常见。
