Nordaa Ludmila, Forsaa Elin Bjelland, Odland Audun, Ajmi Soffien
Tidsskr Nor Laegeforen. 2020 Mar 9;140(4). doi: 10.4045/tidsskr.19.0198. Print 2020 Mar 17.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010).
We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general condition. He had experienced dizziness and unstable gait for a year, and had been periodically confused, especially in the previous month. MR imaging showed characteristic punctuate and curvilinear gadolinium enhancements in the pons. Our patient was diagnosed with CLIPPERS and was given corticosteroid treatment, initially methylprednisolone intravenously and then prednisone orally. Other differential diagnoses, such as CNS lymphoma, high-grade glioma, CNS vasculitis, neurosarcoidosis, demyelinating disease, Bickerstaff brainstem encephalitis, and acute disseminated encephalomyelitis were ruled out. The patient's condition improved dramatically after corticosteroid treatment.
In 2017, the diagnostic criteria for CLIPPERS were published. Based on these criteria we were able to diagnose this patient with possible CLIPPERS, consistent with clinical symptoms, MRI findings, absence of better explanations for the condition, and clinical and radiological improvement after treatment with corticosteroids. An unequivocal diagnosis of CLIPPERS can only be established by characteristic pathological findings.
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）综合征是一种免疫介导的、可治疗的炎性中枢神经系统疾病，由皮托克等人于2010年首次报道。
我们描述了一名66岁男性，既往有糖尿病、心房颤动和高血压病史，因全身状况恶化入院。他头晕和步态不稳已有一年，且有周期性意识模糊，尤其是在前一个月。磁共振成像显示桥脑有特征性的点状和曲线状钆增强。我们的患者被诊断为CLIPPERS，并接受了皮质类固醇治疗，最初静脉注射甲泼尼龙，然后口服泼尼松。排除了其他鉴别诊断，如中枢神经系统淋巴瘤、高级别胶质瘤、中枢神经系统血管炎、神经结节病、脱髓鞘疾病、比克斯特法夫脑干脑炎和急性播散性脑脊髓炎。皮质类固醇治疗后患者病情显著改善。
2017年公布了CLIPPERS的诊断标准。根据这些标准，我们能够诊断该患者为可能的CLIPPERS，这与临床症状、磁共振成像结果、对该病情缺乏更好的解释以及皮质类固醇治疗后的临床和影像学改善相一致。CLIPPERS的明确诊断只能通过特征性病理结果来确立。
