CLIPPERS syndrome responsive to Leflunomide: A case report.

INTRODUCTION

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) was recently described in 2010, therefore the long-term prognosis is uncertain and there is no consensus on which steroid-sparing treatment is the most indicated. We present a case of CLIPPERS successfully treated with leflunomide.

CASE REPORT: A 43-year-old man with progressive symptoms of pontocerebellar dysfunction and radiological findings consistent with CLIPPERS was admitted to our clinic and treated with steroids in the acute phase, then he continue leflunomide for one year, showing sustained clinical and radiological remission. The treatment was changed to azathioprine and continued two more years in remission.

CONCLUSION

As far as we have documented, this is the first case of CLIPPERS kept under control with leflunomide in the medium-term with a good relative prognosis.