Northern Clinical School, University of Sydney, St Leonards, New South Wales, Australia.
J Neurol Neurosurg Psychiatry. 2012 Jan;83(1):15-22. doi: 10.1136/jnnp-2011-301054. Epub 2011 Nov 5.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings.
伴有桥脑被膜血管周围强化的慢性淋巴细胞性炎症伴反应性类固醇（CLIPPERS）是一种新近描述的中枢神经系统炎症性疾病，好发于后脑，对免疫治疗有反应。本文描述了另外 5 例病例，包括详细的病理学和长期评估。CLIPPERS 并非良性疾病，如果没有慢性免疫抑制，疾病可能会复发。影像学分布不仅集中在脑桥，还集中在脑桥臂和小脑。即使在及时治疗的情况下，桥脑小脑萎缩也很早就出现了。一些病例出现了明显的认知障碍，并伴有额外的脑萎缩。病理学包括独特的组织细胞和淋巴细胞成分，以及神经轴突损伤的证据。在进一步的检查中还发现了其他亚临床系统性表现。复发与皮质类固醇的停用有关，而在那些及时治疗和缓解的病例中，残疾程度最低。我们建议将该综合征的名称修改为伴有桥脑小脑被膜血管周围强化的慢性淋巴细胞性炎症伴类固醇反应，以更准确地反映影像学发现的分布。
