OBJECTIVE The diagnosis of sarcoid optic neuropathy is time-sensitive, as delayed treatment risks irreversible vision loss. We sought to analyze its characteristics and outcomes. METHODS We performed a multi-center retrospective study of sarcoid optic neuropathy among 5 USA medical centers. Inclusion criteria were: 1) clinical optic neuropathy; 2) optic nerve/sheath enhancement on neuroimaging; 3) pathological confirmation of systemic or nervous system sarcoidosis. RESULTS Fifty-one patients were included. The median onset age of sarcoid optic neuropathy was 50 years (range, 17-70 years) and 71% were female. The median visual acuity at nadir in the most affected eye was 20/80 (range, 20/20 to no-light-perception). Thirty-four of 50 (68%) patients had radiologic evidence of other nervous system involvement and 20 (39%) patients had symptoms/signs of other cranial nerve dysfunction. Cerebrospinal fluid analysis revealed an elevated white blood cell count in 22 of 31 (71%) patients (median: 14/μL; range: 1-643/μL). Pathologic confirmation of sarcoidosis was by biopsy of systemic/pulmonary site, 34 (67%); optic nerve/sheath, 9 (18%); or other nervous system region, 8 (16%). Forty patients improved with treatment (78%), 98% receiving corticosteroids and 65% receiving steroid-sparing immunosuppressants, yet 11/46 patients (24%) had a visual acuity of 20/200 or worse at last follow-up. CONCLUSIONS Sarcoid optic neuropathy frequently occurs with other clinical and radiologic abnormalities caused by neurosarcoidosis and diagnostic confirmation occasionally requires optic nerve/sheath biopsy. Improvement with treatment is common but most patients have some residual visual disability. Improved recognition and a more expeditious diagnosis and treatment may spare patients from permanent vision loss.

中文翻译：

---

肉瘤视神经病变多中心病例系列

目的 肉瘤视神经病变的诊断具有时间敏感性，因为延迟治疗会导致不可逆的视力丧失。我们试图分析其特征和结果。方法 我们在 5 个美国医疗中心中进行了一项多中心回顾性研究，观察结节样视神经病变。纳入标准为：1）临床视神经病变；2) 神经影像学上的视神经/鞘增强；3) 全身或神经系统结节病的病理证实。结果 包括 51 名患者。肉瘤视神经病变的中位发病年龄为 50 岁（范围，17-70 岁），71% 为女性。受影响最严重的眼睛在最低点的中位视力为 20/80（范围为 20/20 至无光感）。50 名 (68%) 患者中有 34 名 (68%) 有其他神经系统受累的放射学证据，20 (39%) 名患者有其他颅神经功能障碍的症状/体征。脑脊液分析显示，31 名患者中有 22 名 (71%) 的白细胞计数升高（中位数：14/μL；范围：1-643/μL）。结节病的病理证实是通过全身/肺部活检，34 (67%)；视神经/鞘，9 (18%); 或其他神经系统区域，8 (16%)。40 名患者 (78%) 接受治疗后改善，98% 接受皮质类固醇治疗，65% 接受保留类固醇的免疫抑制剂，但 11/46 患者 (24%) 在最后一次随访时视力为 20/200 或更差。结论结节性视神经病变经常与由神经结节病引起的其他临床和放射学异常一起发生，诊断性确认有时需要视神经/鞘活检。治疗后改善很常见，但大多数患者有一些残留的视力障碍。改进的识别和更迅速的诊断和治疗可以使患者免于永久性视力丧失。