Department of Neurology, Hospital General Universitario de Alicante, Alicante, Spain.
J Neuroimaging. 2013 Apr;23(2):254-5. doi: 10.1111/j.1552-6569.2011.00631.x. Epub 2011 Aug 17.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently defined inflammatory central nervous system disorder responsive to steroids with characteristic magnetic resonance imaging (MRI) features. We report a 69-year-old man presenting with gait ataxia with the characteristic MRI features of CLIPPERS and describe the clinical, MRI, and magnetic resonance spectroscopy (MRS) follow-up after treatment with glucocorticosteroids. Brain and spine MRI showed punctate enhancement peppering the brainstem, cerebellar peduncles, and upper cervical cord. In MRS, the ratio of N-acetyl aspartate to creatine (NAA/Cr) was significantly decreased in the pons and both thalami. An extensive evaluation found no alternative diagnoses. Treatment with steroids led to rapid clinical improvement. Repeat MRI and MRS showed complete resolution of gadolinium-enhancing lesions and recovery of NAA/Cr levels in the pons and thalami. After 1 month of tapering oral steroids, weekly oral methotrexate was started and the patient has remained stable for the past 6 months.
伴有桥脑血管周围强化的慢性淋巴细胞性炎症反应伴对类固醇有反应（CLIPPERS）是一种新定义的中枢神经系统炎症性疾病，对类固醇有反应，具有特征性的磁共振成像（MRI）特征。我们报告了一例 69 岁男性，表现为步态共济失调，具有 CLIPPERS 的特征性 MRI 特征，并描述了在接受糖皮质激素治疗后的临床、MRI 和磁共振波谱（MRS）随访情况。脑部和脊柱 MRI 显示点状增强，累及脑干、小脑脚和上颈髓。在 MRS 中，桥脑和双侧丘脑的 N-乙酰天冬氨酸与肌酸的比值（NAA/Cr）显著降低。广泛的评估未发现其他诊断。类固醇治疗导致快速的临床改善。重复 MRI 和 MRS 显示钆增强病变完全消退，桥脑和丘脑的 NAA/Cr 水平恢复正常。口服类固醇逐渐减量 1 个月后，开始每周口服甲氨蝶呤，此后患者在过去 6 个月内一直保持稳定。
