Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Clinical Manifestations in Children Versus Adults.

This article explores the manifestations, radiological findings, associated diseases, treatment, and outcomes in pediatric chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (C.L.I.P.P.E.R.S.). It was inspired by an 11-year-old male who presented with headache, ataxia, nystagmus, persistent vomiting, nausea, and constipation. He was diagnosed with C.L.I.P.P.E.R.S. based on clinical and radiologic findings, and positive response to treatment. The ensuing literature review led us to conclude that pediatric C.L.I.P.P.E.R.S. has a clinical presentation, etiology, evolution, course and prognosis different from adult cases. Future pediatric workup should include genetic studies because of commonly associated mutations such as PRF1. Treatment in pediatric cases should account for a delayed response to steroids, frequent recurrences, and especially in genetic cases, definitive treatment may require hematopoietic stem cell transplant. It seems that C.L.I.P.P.E.R.S. is not an isolated disease, rather a syndrome with unique radiologic findings, a wide range of etiologies, and clinical and neurologic manifestations that distinguish pediatric from adult cases. In order to optimize treatment and follow-up, future diagnostic approaches of C.L.I.P.P.E.R.S. in children should include workup for a primary disease such as EBV B cell lymphoma or hemophagocytic lymphohistiocytosis.