Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) without relapse after early steroid treatment

Yuki Asahara, M.D.1), Makiko Yogo, M.D., Ph.D.2), Shinji Miyagawa, M.D.1) and Masahiko Suzuki, M.D., Ph.D.1)

1) Department of Neurology, the Jikei University Katsushika Medical Center
2) Akebono Home Clinic

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T1-weighted MRI revealed multiple punctate and curvilinear enhancements in the pons and right middle cerebellar peduncle. We started treatment with high-dose intravenous methylprednisolone (IVMP) therapy on the 20th day of the illness. His neurological symptoms dramatically improved. Follow-up MRI showed that the enhancing lesions disappeared. We diagnosed him with CLIPPERS based on the clinical course, radiological findings, and steroid response. He did not take any oral immunosuppressant after discharge. However, there was no clinical and radiological relapse for 1 year after the IVMP therapy. Although this case requires careful follow-up because of recurrence risk, early steroid treatment was possibly related to 1-year remission.
Full Text of this Article in Japanese PDF (1811K)

(CLINICA NEUROL, 60: 682|687, 2020)
key words: CLIPPERS, steroid, relapse

(Received: 17-Nov-19)