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高级磁共振成像在诊断和治疗慢性淋巴细胞性炎症伴脑桥血管周围强化且对类固醇有反应(CLIPPERS)病例中的作用及治疗随访

Role of advanced MRI in diagnosing and evolutive follow-up to treatment in a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).

作者信息

Grasso Daniela, Borreggine Carmela, Prencipe Gianluca

机构信息

Department of Diagnostic Imaging, Neuroradiology Unit, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Foggia, Italy.

Diagnostic Imaging, Postgraduate Radiology Medical School, University of Foggia, Foggia, Italy.

出版信息

Radiol Case Rep. 2025 Aug 8;20(11):5458-5465. doi: 10.1016/j.radcr.2025.07.017. eCollection 2025 Nov.

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder that affects the brainstem and cerebellum. Patients often show symptoms like ataxia, diplopia, and dysarthria. Diagnosis can be complex due to the need to exclude other conditions. We present a case of a 35-year-old previously healthy female admitted to our hospital with dizziness, progressively worsening gait, and mild dysarthria. Advanced MRI techniques revealed characteristic lesions in the pons and cerebellum with characteristic "peppering" appearance, supporting a diagnosis of CLIPPERS. The patient improved significantly with corticosteroid treatment, emphasizing the importance of early recognition and treatment. Follow-up MRIs demonstrated a reduction in lesions, reinforcing the effectiveness of corticosteroids. The etiology of CLIPPERS remains unclear, but it is believed to involve immune-mediated mechanisms. Regular monitoring and long-term treatment strategies are essential to manage this condition effectively. This case underscores the critical need for increased awareness of CLIPPERS among clinicians and radiologists. It also highlights the importance of integrating clinical, radiological, and neuropathological findings for accurate diagnosis. Given its rarity and diagnostic complexity, this case report provides valuable pathogenetic insights into a poorly understood condition.

摘要

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)是一种罕见的炎症性疾病,累及脑干和小脑。患者常表现出共济失调、复视和构音障碍等症状。由于需要排除其他疾病,诊断可能较为复杂。我们报告一例35岁既往健康女性,因头晕、步态进行性恶化和轻度构音障碍入院。先进的MRI技术显示桥脑和小脑有特征性病变,呈特征性的“点状”外观,支持CLIPPERS的诊断。患者经皮质类固醇治疗后明显改善,强调了早期识别和治疗的重要性。随访MRI显示病变减少,强化了皮质类固醇的有效性。CLIPPERS的病因尚不清楚,但据信涉及免疫介导机制。定期监测和长期治疗策略对于有效管理这种疾病至关重要。该病例强调了临床医生和放射科医生提高对CLIPPERS认识的迫切需求。它还强调了整合临床、放射学和神经病理学发现以进行准确诊断的重要性。鉴于其罕见性和诊断复杂性,本病例报告为一种了解甚少的疾病提供了有价值的发病机制见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e454/12356297/f90df238d0d3/gr1.jpg

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