Ruiz Department of Ophthalmology and Visual Science, McGovern Medical School at The University of Texas Health Science Center in Houston (UTHealth) , Houston , Texas , USA.
Robert Cizik Eye Clinic , Houston , Texas , USA.
Ocul Immunol Inflamm. 2019;27(6):912-917. doi: 10.1080/09273948.2018.1449865. Epub 2018 Apr 13.
: The purpose of the article is to describe a novel case of idiopathic central nervous system inflammatory disease with bilateral human leukocyte antigen (HLA)-B27-positive anterior uveitis. : A 15-year-old African American boy with bilateral HLA-B27-positive anterior uveitis controlled with topical and oral steroids for 8 months acutely developed headaches, left eyelid ptosis, and binocular diplopia. Imaging showed lesions in the right midbrain, superior colliculus, cerebellar peduncles, and cerebellar vermis and leptomeningeal enhancement along the vermian foliae. Cerebral spinal fluid tests showed mild lymphohistiocytic pleocytosis with negative cytology; inflammatory and infectious workup were negative. He received intravenous methylprednisolone without initial symptomatic improvement; repeat magnetic resonance imaging (MRI) showed reduced lesion burden. Oral steroids were continued; his symptoms resolved in 1 month. Repeat MRI 2 months after presentation showed almost complete lesion resolution. : Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) was diagnosed. HLA-B27 positivity may represent a novel association with CLIPPERS.
文章旨在描述一例特发性中枢神经系统炎症性疾病的新病例，伴有双侧人类白细胞抗原（HLA）-B27 阳性前葡萄膜炎。
一名 15 岁的非裔美国男孩，因双侧 HLA-B27 阳性前葡萄膜炎，经局部和口服类固醇治疗 8 个月后得到控制，突然出现头痛、左眼眼睑下垂和双眼复视。影像学显示右侧中脑、上丘、小脑脚和小脑蚓部病变，以及蚓状叶脑膜增强。脑脊髓液检查显示轻度淋巴样组织细胞性白细胞增多，细胞学检查阴性；炎症和感染性检查均为阴性。他接受了静脉注射甲基强的松龙，但最初没有症状改善；重复磁共振成像（MRI）显示病变负担减轻。继续口服类固醇治疗；1 个月后症状缓解。发病后 2 个月重复 MRI 显示几乎完全缓解。
诊断为慢性淋巴细胞性炎症伴桥脑血管周围增强对类固醇反应（CLIPPERS）。HLA-B27 阳性可能是 CLIPPERS 的一个新关联。
