Department of Neurology, Pomeranian Medical University, 70-110 Szczecin, Poland.
Medicina (Kaunas). 2023 Mar 11;59(3):549. doi: 10.3390/medicina59030549.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a rare inflammatory disease of an undetermined aetiology. The condition is characterised by a range of clinical manifestations generally associated with damage to brainstem structures, the cerebellum, with characteristic magnetic resonance imaging (MRI) findings. The main feature is a good clinical and radiological response to glucocorticosteroid (GCS)-based immunosuppressive treatment. The diagnosis of CLIPPERS is difficult and requires extensive differential diagnosis. A specific biomarker in serum or cerebrospinal fluid (CSF) for this disorder is currently unknown. The pathogenesis of CLIPPERS remains poorly understood and its nosological position has not yet been established. Whether CLIPPERS represents an independent, genuine new disorder or a syndrome in the course of diseases with heterogeneous aetiology and/or their precursor stages remains debatable and incompletely clarified. We present a case report of a patient who was diagnosed with CLIPPERS syndrome on the basis of her clinical and radiological features and by performing an extensive differential diagnosis. The patient has been under neurological follow-up for five years.
伴有桥脑血管周围强化反应的慢性淋巴细胞性炎症（CLIPPERS）综合征是一种病因不明的罕见炎症性疾病。该疾病的特征是一系列临床表现，通常与脑干结构、小脑损伤相关，并具有特征性的磁共振成像（MRI）表现。主要特征是对基于糖皮质激素（GCS）的免疫抑制治疗有良好的临床和影像学反应。CLIPPERS 的诊断较为困难，需要广泛的鉴别诊断。目前尚不清楚该疾病在血清或脑脊液（CSF）中是否存在特定的生物标志物。CLIPPERS 的发病机制仍不清楚，其分类位置尚未确定。CLIPPERS 是否代表一种独立的、真正的新疾病，或者是一种病因和/或其前体阶段具有异质性的疾病过程中的综合征，仍存在争议，且尚未完全阐明。我们报告了一例患者，该患者根据其临床和影像学特征以及广泛的鉴别诊断被诊断为 CLIPPERS 综合征。该患者已接受了五年的神经学随访。
