Objective To investigate the chronic lymphocytic inflammation with pontine perivascularenhancemen responsive to steroids (CLIPPERS) with manifestations, imaging and pathological changes, and to explore its possible pathogenesis.Method A case report of CLIPPERS retrospectively analyzed with showed multiple small patchy abnormal enhancing signals mainly symmetrical distributed in brainstem, spinal cord, with obscure border as "pepper sign". More lesions concentrated at medulla oblongata and spinal cord. Additionally, bilateral thalami, basal ganglia, and cerebellar hemisphere had similar abnormal enhancing lesions. Multiple lining mild enhancement of the pia mater was also observed. The clinical symptoms relieved with glucocorticoid treatment for 2 months, and most of the abnormal enhancement lesions disappeared on following cranial enhancement MRI,the residual lesions shrinked with fuzzy boundaries and less intensity.Results Some symptoms of case 1 conformed to MELAS, while the others were accorded with MERRF. The MRI features of case 1 disclosed symmetric abnormal signals of midbrain and quadrigemina conforming to Leigh's syndrome except that the common cortical lesions of MELAS were disclosed at the same time. 10158T>C point mutation of mitochondrial gene ND3 was found through next generation sequencing. Although the clinical presentations of case 2 conformed to MELAS, MRI found symmetric lesions of midbrain which indicated Leigh's syndrome. 10191T>C point mutation was found in case2.Conclusion In some CLIPPERS cases,the enhancing lesions on MRI may mainly involved the brainstem and the whole spinal cord,pia matter can also be affected. The lesions could be not always centered on pons. It response well to glucocorticoid. The diagnosis of CLIPPERS should be combined with clinical, imaging and laboratory examination, brain tissue biopsy may be necessary when needed.%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）的临床表现、影像学及病理改变，探讨其可能的发病机制。方法对1例主要累及全脊髓及脑干的CLIPPERS患者临床资料及影像学进行分析，并结合文献复习此病的特征表现及鉴别诊断。结果本例男性29岁，进行性双下肢无力、共济失调2年、及伴腹部麻木1年余。头颅磁共振（MRI）增强扫描示脑干、全脊髓多发小斑片状异常强化信号，边界较清楚，呈“胡椒粉征”。病变分布较均匀，延髓及脊髓分布更密集。另外双侧丘脑、基底核、小脑半球见散在类似异常强化灶。软脑膜及软脊膜也可见多处轻度线样强化。经糖皮质激素治疗2个月后，患者临床症状明显减轻。复查MRI示上述异常强化灶大部分消失，残存病灶缩小且强化程度减轻，边界变模糊。影像学提示病变明显好转。结论在有些CLIPPERS患者，MRI的强化灶主要累及脑干、脊髓全长及软脑（脊）膜，不一定以脑桥为中心。CLIPPERS糖皮质激素治疗效果好。CLIPPERS的诊断应结合临床、影像学及实验室检查，必要时行脑组织活检。
展开▼
