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皮质类固醇反应性桥脑周围血管强化综合征(CLIPPERS)的临床及影像学特征:9例分析

Clinical and imaging characteristics of corticosteroid-responsive chronic lymphocytic inflammation with Pontine perivascular enhancement syndrome (CLIPPERS): an analysis of 9 cases.

作者信息

Sun Xiaosa, Li Xiaowen, Raza Hafiz Khuram, Yang Jingwen, Ji Ying, Yuan Xinyi, Kong Yu, Tian Jin, Gu Yaqin, Chen Hao, Chen Li

机构信息

Department of Neurology, The Affiliated Hospital of Xuzhou Medical University, Huaihai West Road, Quanshan District, Xuzhou, Jiangsu, 221002, China.

Academician Expert Workstation of Fengxian District, Shanghai Yuansong Biotechnology Limited Company, Shanghai, 201400, China.

出版信息

Neurol Sci. 2025 Aug;46(8):3819-3826. doi: 10.1007/s10072-025-08208-5. Epub 2025 May 8.

Abstract

PURPOSE

This study aimed to elucidate the clinical and radiological profiles of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS), targeting an enhanced comprehension and more efficient diagnosis of this rare disorder among medical professionals.

METHODS

We conducted a retrospective cohort study, encompassing a detailed analysis of clinical and imaging data from nine CLIPPERS patients diagnosed between 2016 and 2023 at Xuzhou Medical University Affiliated Hospital and Beijing Xuanwu Hospital.

RESULTS

Our study included nine patients, comprising seven males and two females, with ages varying from 9 to 79 years.The clinical spectrum was broad, including lower limb weakness (5 cases), unsteady gait (6), lower limb numbness (2), visual impairments (4), dizziness and headaches (5), cognitive impairments (2), facial sensory abnormalities (2), language disturbances (4), nausea and vomiting (1), limb twitching (1), fever (1), and chest and abdominal discomfort (1).Brain MRI scans uniformly disclosed multifocal lesions with slightly prolonged T1 and pronounced T2 signal characteristics in the brainstem, notably in the pons, as well as in bilateral thalami, cerebellum, and basal ganglia. High signal intensity on FLAIR sequences and variable signal intensity on DWI sequences were observed, with post-contrast imaging exhibiting the pathognomonic punctate and curvilinear 'pepper-like' enhancement. Cervical spinal cord MRIs from two patients mirrored this enhancement pattern. Pathological examination via biopsy in two cases-one of brain tissue and one of spinal cord tissue-demonstrated perivascular infiltration of CD3 + T lymphocytes, accompanied by CD20+, CD5+, and CD68 + cell infiltrations. A favorable response to corticosteroid therapy was observed across all patients, with marked amelioration in clinical symptoms and radiological findings. Remarkably, one patient, diagnosed with classical Hodgkin's lymphoma two years post-onset, showed no signs of recurrence on subsequent PET-CT scans following standard chemotherapy. The remaining patients were either on steroid maintenance therapy or received a combination of steroids and immunosuppressants, with symptomatic improvement noted in all.

CONCLUSIONS

CLIPPERS syndrome is distinguished by its characteristic MRI features and demonstrates a robust response to corticosteroid therapy, leading to rapid clinical and radiological improvement. In scenarios with ambiguous diagnosis, pathological tissue biopsy offers essential confirmation.

摘要

目的

本研究旨在阐明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)的临床和影像学特征,旨在提高医学专业人员对这种罕见疾病的理解并实现更有效的诊断。

方法

我们进行了一项回顾性队列研究,详细分析了2016年至2023年期间在徐州医科大学附属医院和北京宣武医院确诊的9例CLIPPERS患者的临床和影像数据。

结果

我们的研究纳入了9例患者,其中男性7例,女性2例,年龄从9岁至79岁不等。临床症状范围广泛,包括下肢无力(5例)、步态不稳(6例)、下肢麻木(2例)、视力障碍(4例)、头晕头痛(5例)、认知障碍(2例)、面部感觉异常(2例)、语言障碍(4例)、恶心呕吐(1例)、肢体抽搐(1例)、发热(1例)以及胸腹部不适(1例)。脑部MRI扫描均显示脑干多灶性病变,T1稍延长,T2信号特征明显,尤其在脑桥,以及双侧丘脑、小脑和基底节。FLAIR序列上呈高信号强度,DWI序列上信号强度可变,增强后成像显示特征性的点状和曲线状“胡椒样”强化。2例患者的颈段脊髓MRI显示了这种强化模式。2例活检病例(1例脑组织活检和1例脊髓组织活检)的病理检查显示CD3 + T淋巴细胞血管周围浸润,伴有CD20 +、CD5 +和CD68 +细胞浸润。所有患者对皮质类固醇治疗均有良好反应,临床症状和影像学表现均有明显改善。值得注意的是,1例患者在发病两年后被诊断为经典型霍奇金淋巴瘤,在接受标准化疗后的后续PET-CT扫描中未显示复发迹象。其余患者要么接受类固醇维持治疗,要么接受类固醇和免疫抑制剂联合治疗,所有患者症状均有改善。

结论

CLIPPERS综合征以其特征性的MRI表现为特点,对皮质类固醇治疗反应良好,可导致临床和影像学快速改善。在诊断不明确的情况下,病理组织活检提供了重要的确诊依据。

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