类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥周围血管异常强化症(CLIPPERS)4例影像及临床表现分析

Analysis of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids(CLIPPERS)Imaging and Clinical Manifestations in 4 Cases

目的探讨类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症(CLIPPERS)影像学特点及临床特征,以提高对该病的认识。方法回顾性分析经病理或临床诊断证实的4例CLIPPERS综合征的影像表现及临床诊疗经过。4例患者自出现症状至确诊时间为0.5~6年。结果 MRI上均可见对称性脑干(桥脑为主)、双侧丘脑、小脑及基底节区等多发等或稍长T_1、长T_2病灶,FLAIR为高信号,DWI为高或等信号,ADC值略升高,增强扫描病灶呈典型的"胡椒粉"样点状及曲线状强化,1例病变广泛伴有基底节区及皮层下受累,1例伴有室管膜下斑点及曲线样异常强化,伴有柔脑膜异常强化1例,MRA检查均无异常,MRS病变区域胆碱峰升高,可见乳酸双峰,PWI一例示病变灌注脑血流量增加。T_2*WI上仅1例治疗后复查片上提示小脑多发微出血灶。所有病例对类固醇激素治疗敏感有效,但是停药后均出现复发,且复发后影像表现可较前加重,再次使用激素或免疫抑制治疗后病情好转,1例患者发病5年后发现皮下及肺内多发结节,经皮下结节活检证实为T细胞淋巴瘤,化疗中,病情稳定,其余3例激素维持治疗,症状有改善。结论 CLIPPERS综合征在MRI上有典型部位和影像学表现,且影像表现可完全反映其病情进展情况并以此评价治疗效果,但仅靠影像诊断该病的证据不够充分,确诊要结合临床表现、实验室检查排除其他可能诊断及激素诊疗效果;必要时要进行脑组织活检病理证实。

Objective To analyze chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（ CLIPPERS） imaging characteristics and clinical features so as to improve the recognition of this disease.Methods The imaging manifestations and clinical diagnosis and treatment of 4 patients with CLIPPERS syndrome confirmed by pathology or clinical diagnosis were retrospectively analyzed. We collected 4 patients in the course of 0. 5-6years. Results MRI showed bilateral symmetrical multiple areas in the brainstem（ pons）,thalamus,cerebellum and basal ganglia with slightly long T1 and long T2 signal. The corresponding areas showed a high signal on FLAIR and DWI,as well as a slightly increased ADC value. The lesions enhanced and showed a typical punctate and curvilinear enhancement.1 case had lesions associated with basal ganglia cortical involvement,1 case was complicated with ventricular ependymal spots and spinal abnormal enhancement and 1 case had accompanying abnormal meningeal enhancement. MRA examination showed no abnormalities,while MRS demonstrated increased choline peak and a double lactate peak. PWI revealed increased cerebral perfusion blood flow of the pontine lesion. Only one case was reexamined on T2＊WI after treatment of cerebellar hemorrhage. All cases of steroid hormone treatment showed that the treatment was sensitive and effective,but recurrence occurred after withdrawal. Imaging findings of recurrent lesions can be worse than before treatment and the use of steroids or immunosuppressive treatment improved the condition. 1 case of the patients developed subcutaneous and pulmonary nodules 5 years later,which were confirmed by biopsy of subcutaneous nodules to be T cell lymphoma. Post-chemotherapy,the condition was stable. For the remaining 3 cases,hormonal treatment was maintained and the symptoms improved. Conclusion CLIPPERS has typical locations and imaging manifestations on MRI. MRI can fully reflect the progress of the disease and the effect of treatment. However,imaging findings alone have insufficient evidence of disease.Clinical manifestations and laboratory examinations are useful in excluding diagnosis,and evaluating the effect of steroids therapy. The diagnosis may be confirmed by biopsy when necessary.