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不典型的中枢神经系统特发性炎症性脱髓鞘假瘤(CLIPPERS)表现及MRI检查在正确诊断评估中的作用:一例报告

Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: A case report.

作者信息

Vola Elena, Russo Camilla, Macera Antonio, Del Vecchio Walter, Puoti Gianfranco, Caranci Ferdinando, Elefante Andrea

机构信息

Dipartimento di Scienze Biomediche Avanzate, Università degli Studi di Napoli "Federico II", Napoli, Italy.

Istituto di Biostrutture e Bioimaging, Consiglio Nazionale delle Ricerche, Napoli, Italy.

出版信息

Eur J Radiol Open. 2019 Jun 6;6:212-214. doi: 10.1016/j.ejro.2019.05.005. eCollection 2019.

Abstract

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids syndrome (CLIPPERS) is a newly described, underestimated CNS inflammatory disorder involving predominantly the midbrain and the cerebellum. CLIPPERS pathogenesis is largely unknown, and its clinical manifestations are polymorphic and sometimes confounding. Recently clinical, radiological and pathological diagnostic criteria have been proposed to discriminate CLIPPERS from potential mimickers, but the diagnosis still remains challenging. Here we present the case of a patient with radiological findings consistent with CLIPPERS but with atypical clinical presentation, highlighting the importance of a proper diagnostic assessment.

摘要

类固醇反应性桥脑周围血管强化慢性淋巴细胞性炎症综合征(CLIPPERS)是一种新描述的、未得到充分认识的中枢神经系统炎症性疾病,主要累及中脑和小脑。CLIPPERS的发病机制很大程度上尚不清楚,其临床表现具有多态性,有时令人困惑。最近已提出临床、放射学和病理学诊断标准以将CLIPPERS与可能的模仿者区分开来,但诊断仍然具有挑战性。在此,我们报告一例患者,其放射学表现符合CLIPPERS,但临床表现不典型,强调了进行适当诊断评估的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/913c/6556810/6802953c165a/gr1.jpg

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