Dorman James, Warrior Lakshmi, Pandya Vishal, Sun Ying, Ninan Jacob, Trick William, Zhang Helen, Ouyang Bichun
Neurology.
Neurological Sciences, Rush University.
Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(1):25-32. doi: 10.36141/svdld.v36i1.7106. Epub 2019 May 1.
To characterize clinical presentation, laboratory and imaging data, and treatment outcomes for neurosarcoidosis in an urban safety net hospital.
The research database of Cook County Health and Hospitals system was queried for all cases of sarcoidosis from 2006 to 2013. These cases plus those identified through a survey of neurology faculty were reviewed and flagged if suspected to be neurosarcoidosis. Data were extracted in a standardized fashion, upon review by two experienced neurologists; patients were classified as definite, probable or possible neurosarcoidosis. Disagreements on classification were resolved by consensus conference.
1706 cases of sarcoidosis were identified, with 82 (4.8%) classified as neurosarcoidosis. The cohort was predominantly African American (89%). Six were classified as definite, 34 as probable, and 42 as possible neurosarcoidosis. Neurosarcoidosis was the presenting symptom of sarcoidosis in 74% of cases. The most common presenting phenotype was myelopathy (21.7%), followed by optic nerve/chiasm involvement (16.0%) and epilepsy (11.3%). The facial nerve was involved in only 2% of cases. Chest x-ray showed abnormalities of sarcoidosis in 43.3% of cases, while chest CT did so in 78.6%. Corticosteroids were the initial treatment in 91% of cases, and outcomes were good in 53% of cases.
Neurosarcoidosis remains a challenging diagnosis with the majority of patients without a previous diagnosis of systemic sarcoidosis. Chest imaging was supportive of the diagnosis in a majority of patients. Our cohort differs from others in the literature due to a low prevalence of facial nerve involvement. Prospective registry studies are needed.
描述一家城市安全网医院中神经结节病的临床表现、实验室及影像学数据,以及治疗结果。
查询库克县卫生与医院系统的研究数据库,获取2006年至2013年所有结节病病例。这些病例以及通过对神经科教员进行调查确定的病例,经审查若怀疑为神经结节病则进行标记。由两名经验丰富的神经科医生以标准化方式提取数据;患者被分类为确诊、很可能或可能的神经结节病。分类上的分歧通过共识会议解决。
共识别出1706例结节病病例,其中82例(4.8%)被分类为神经结节病。该队列主要为非裔美国人(89%)。6例为确诊,34例为很可能,42例为可能的神经结节病。在74%的病例中,神经结节病是结节病的首发症状。最常见的首发表型是脊髓病(21.7%),其次是视神经/视交叉受累(16.0%)和癫痫(11.3%)。仅2%的病例涉及面神经。胸部X线显示43.3%的病例有结节病异常,而胸部CT显示78.6%的病例有异常。91%的病例初始治疗为使用皮质类固醇,53%的病例治疗结果良好。
神经结节病仍然是一个具有挑战性的诊断,大多数患者之前没有系统性结节病的诊断。大多数患者的胸部影像学有助于诊断。我们的队列与文献中的其他队列不同,因为面神经受累的患病率较低。需要进行前瞻性登记研究。