目的 探讨类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS)的临床及影像学特征。方法 回顾性分析1例CLIPPERS患儿的临床特征、影像学特点、治疗、预后及随访情况。结果 女性患儿,12岁,表现为四肢无力、步态不稳、右侧视野缺损。神经系统体征主要为双眼球外展受限,双眼球水平位及直立位震颤,左侧面瘫,共济失调。头颅磁共振(MRI)表现为脑桥、延髓、双侧桥臂及左侧中脑呈“胡椒粉样”点状强化病灶。予激素治疗后临床表现及头颅影像明显好转。结论 儿童CLIPPERS的临床表现不典型,目前诊断主要依靠影像学及治疗反应。
Objective To discuss the clinical and imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Methods Clinical characteristics, imaging features, treatment, prognosis, and follow up data from one pediatric patient diagnosed CLIPPERS were analyzed retrospectively. Results Female, 12 years old, presented with limb weakness, gait ataxia, and right visual field defect. Neurological signs mainly presented with oculomotor palsies, nystagmus, the left facial nerve palsy and cerebellar ataxia. Brain magnetic resonance imaging (MRI) showed appearance with punctate and curvilinear gadolinium enhancement 'peppering' the pons, medulla oblongata, bilateral arm, and the left side of the midbrain. After treatment with steroids, the clinical and imaging manifestations were rapidly improved. Conclusions Pediatric patient of CLIPPERS may have more atypical presentations. The diagnosis of CLIPPERS mainly depends on imaging and treatment response.