A Pediatric Case of CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive To Steroids) (P6.265)

Objective: Report of a pediatric case of CLIPPERS and review the pediatric literature. Background: CLIPPERS represents a poorly understood inflammatory disorder of the central nervous system with predilection for rhombencephalic structures, particularly the pons, responsive to immunotherapy. CLIPPERS has distinct radiological features of punctate and/or curvilinear gadolinium enhancement, ‘peppering’ the pons and adjacent hindbrain structures on MRI. CLIPPERS has been well reported in adults, with a paucity of cases described in the pediatric population. Design/Methods: A 14 year old boy presented with two-month history of binocular diplopia which progressed to gait ataxia, incoordination, and dysarthria. Neurological exam demonstrated horizontal and vertical nystagmus, bilateral 6th nerve palsies, left facial palsy, dysarthria, ataxia, and rombergism. MRI brain showed patchy, peppered T2 hyperintensities with perivascular distribution in brainstem, mostly pons, and bilateral cerebellum. MRI spine was unremarkable. Extensive investigations of blood, CSF, and imaging were performed without evidence of underlying infectious, inflammatory, demyelinating, neoplastic, paraneoplastic, or vasculitic processes. Biopsy was not performed. Treatment with 1,000 mg intravenous methylprednisolone for 5 days resulted in robust improvement of symptoms and resolution of enhancement on repeat MRI. He was discharged on a slow prednisone taper. Results: Symptoms remain resolved five weeks after initiation of glucocorticoids. Conclusions: CLIPPERS has been reported mostly in adults and was first described in 2010 by Pittock and colleagues. It remains a diagnosis of exclusion based upon radiographic data and treatment response. There are two previously reported pediatric cases consisting of a 13 year-old boy and a 16 year-old girl. The first child lacked radiographic abnormalities until his twelfth relapse and had fluctuating presence of oligoclonal bands during relapses. The second case had normal CSF and suffered recurrence despite glucocorticoids and azathioprine. One could be concerned that children may have a worse prognosis or atypical presentation as compared to adults. Disclosure: Dr. Mathias has nothing to disclose. Dr. Hickman has nothing to disclose. Dr. Lightner has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Baumann has nothing to disclose.