Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up

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.