Hou Xiaohe, Wang Xiaoke, Xie Bo, Lin Weihong, Liu Jun, Ma Dihui, Zhang Hong-Liang
Neuroscience Center, Department of Neurology, the First Hospital of Jilin University Department of Neurosurgery, the Second Hospital of Jilin University, Jilin University, Changchun, China.
Medicine (Baltimore). 2016 Aug;95(34):e4640. doi: 10.1097/MD.0000000000004640.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic inflammatory disorder in the central nervous system (CNS), which is characterized by magnetic resonance imaging (MRI) appearance with punctate and curvilinear gadolinium enhancement "peppering" the pons. Lesions of CLIPPERS mainly involve the pons and the cerebellum. Adjacent structures such as the medulla and the midbrain may also be involved. It is proposed that CLIPPERS is an immune-mediated inflammatory condition characteristic of T-cell-predominant infiltrates and good responsiveness to corticosteroids.
We report a 46-year-old woman who presented with horizontal eyeball akinesia and gait ataxia with characteristic MRI features of CLIPPERS. The possible pathogenesis, clinical manifestations, imaging features, treatment, and prognosis of this peculiar disorder are summarized.
This report contributes to the clinical understanding of CLIPPERS which may present with horizontal eyeball akinesia as an initial manifestation. The characteristic presentation of a subacute cerebellar and brainstem syndrome and pepper-like gadolinium enhancement was confirmed in this report. Long-term immunosuppressive treatment seems to be mandatory to sustain improvement. Azathioprine alone may be capable of maintaining remission.
类固醇反应性桥脑周围血管强化的慢性淋巴细胞性炎症（CLIPPERS）是中枢神经系统（CNS）一种罕见的慢性炎症性疾病，其特征为磁共振成像（MRI）表现为桥脑出现点状和曲线状钆强化“胡椒样”改变。CLIPPERS病变主要累及桥脑和小脑。延髓和中脑等相邻结构也可能受累。有人提出，CLIPPERS是一种免疫介导的炎症性疾病，其特征是以T细胞为主的浸润以及对皮质类固醇有良好反应。
我们报告了一名46岁女性，她表现为水平眼球运动不能和步态共济失调，具有CLIPPERS的特征性MRI表现。总结了这种特殊疾病的可能发病机制、临床表现、影像学特征、治疗及预后。
本报告有助于临床了解可能以水平眼球运动不能为首发表现的CLIPPERS。本报告证实了亚急性小脑和脑干综合征以及胡椒样钆强化的特征性表现。长期免疫抑制治疗似乎对维持病情改善必不可少。单独使用硫唑嘌呤可能能够维持缓解。
