Veerapandiyan Aravindhan, Chaudhari Amit, Deo Prabhav, Ming Xue
Division of Pediatric Neurology, Department of Neurology, Rutgers New Jersey Medical School, Newark, NJ, USA.
Division of Pediatric Neurology, Department of Neurology, Rutgers New Jersey Medical School, Newark, NJ, USA.
Mult Scler Relat Disord. 2017 Oct;17:95-98. doi: 10.1016/j.msard.2017.07.012. Epub 2017 Jul 8.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder involving brainstem, pons in particular, characterized by a predominant T-cell pathology and responsiveness to glucocorticosteroids. We describe a difficult 6 year course of this rare syndrome in a 10-year-old girl.
Our patient presented with diplopia, spastic paraparesis, and unsteady gait. MRI showed characteristic punctate hyper-intensities with enhancement in the brain stem, cerebellar peduncles, and optic chiasm and diffuse nodular enhancement throughout the cervical and thoracic spinal cord. Brain biopsy revealed perivascular inflammatory lesions compatible with CLIPPERS. Pulse intravenous methylprednisolone followed by oral prednisolone resulted in significant clinical improvement. She had multiple clinical relapses associated with new brain and/or spinal cord lesions despite sequential maintenance methotrexate and rituximab with low dose steroids. Each relapse responded to pulse steroids.
Her overall clinical course was progressive but responsive to pulse steroids. Long-term treatment remains challenging.
类固醇反应性桥脑周围血管增强的慢性淋巴细胞性炎症（CLIPPERS）是一种累及脑干，尤其是脑桥的炎症性疾病，其特征为主要的T细胞病理改变以及对糖皮质激素有反应。我们描述了一名10岁女孩患这种罕见综合征的6年艰难病程。
我们的患者出现复视、痉挛性截瘫和步态不稳。MRI显示脑干、小脑脚和视交叉有特征性点状高信号并强化，颈髓和胸髓有弥漫性结节状强化。脑活检显示与CLIPPERS相符的血管周围炎性病变。静脉注射甲泼尼龙冲击治疗后口服泼尼松龙使临床症状显著改善。尽管先后使用甲氨蝶呤、利妥昔单抗及低剂量类固醇进行维持治疗，她仍多次出现与新的脑和/或脊髓病变相关的临床复发。每次复发对类固醇冲击治疗均有反应。
她的总体临床病程呈进行性，但对类固醇冲击治疗有反应。长期治疗仍然具有挑战性。
