Department of Neurology, Amsterdam University Medical Center, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands.
Eur J Neurol. 2022 Jun;29(6):1763-1770. doi: 10.1111/ene.15295. Epub 2022 Mar 3.
Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics, treatment and prognosis of patients with myelitis attributable to neurosarcoidosis.
We performed a retrospective cohort study and a systematic review and meta-analysis of neurosarcoidosis-associated myelitis.
Myelitis was identified in 41 of 153 (27%) neurosarcoidosis patients seen at our clinic from 2015 to 2020. Classification of neurosarcoidosis was definite in three (7%), probable in 29 (71%) and possible in nine patients (22%). The median (interquartile range) age at onset was 49 (41-53) years and 20 of the patients were female (49%). The presenting symptoms included muscle weakness in 31 of 41 patients (78%), sensory loss in 35 (88%) and micturition abnormalities in 30 (75%). Spinal magnetic resonance imaging showed longitudinally extensive myelitis in 27 of 36 patients (75%) and cerebrospinal fluid examination showed an elevated leukocyte count in 21 patients (81%). Initial treatment consisted of glucocorticoids in 38 of 41 patients (93%), with additional methotrexate or azathioprine in 21 of 41 patients (51%) and infliximab in 10 of 41 patients (24%). Treatment led to remission, improvement or stabilization of disease in 37 of 39 patients (95%). Despite treatment, 18 of 30 patients (60%) could not walk independently at the end of follow-up (median 36 months). A review of the literature published between 2000 and 2020 identified 215 patients with comparable clinical characteristics and results of ancillary investigations.
Sarcoidosis-associated myelitis is observed in 27% of neurosarcoidosis patients. Although treatment often led to a decrease in disease activity, residual neurological deficits leading to loss of ambulation occurred frequently.
神经结节病可影响神经系统的各个部位，其中脊髓炎较为常见。本研究旨在描述由神经结节病引起的脊髓炎患者的临床特征、治疗和预后。
我们进行了一项回顾性队列研究和一项对神经结节病相关性脊髓炎的系统回顾和荟萃分析。
2015 年至 2020 年期间，我们在诊所共诊治了 153 例神经结节病患者，其中 41 例（27%）患有脊髓炎。神经结节病的分类为明确诊断 3 例（7%），可能诊断 29 例（71%），可疑诊断 9 例（22%）。发病时的中位（四分位间距）年龄为 49（41-53）岁，20 例患者为女性（49%）。首发症状包括 31 例（78%）肌无力、35 例（88%）感觉缺失和 30 例（75%）排尿异常。36 例脊髓磁共振成像（MRI）中有 27 例（75%）显示纵向广泛脊髓炎，21 例（81%）脑脊液检查显示白细胞计数升高。41 例患者初始治疗均为糖皮质激素，其中 21 例（51%）加用甲氨蝶呤或硫唑嘌呤，10 例（24%）加用英夫利昔单抗。39 例患者中有 37 例（95%）治疗后疾病缓解、改善或稳定。尽管进行了治疗，但在随访结束时（中位 36 个月），30 例患者中有 18 例（60%）仍无法独立行走。对 2000 年至 2020 年期间发表的文献进行综述，发现了 215 例具有类似临床特征和辅助检查结果的患者。
神经结节病患者中，27%患有结节病相关性脊髓炎。尽管治疗通常可降低疾病活动度，但常导致遗留神经功能缺损，导致行走能力丧失。
