(CR34) Chronic Lymphocytic Inflammation of The Pons with Perivascular Enhancement Responsive To Steroid (CLIPPERS) Syndrome.

Introduction:

CLIPPERS syndrome was first described by in the year 2010 by Pittock et al when they studied 8 patients with a clinically distinct pontine-predominant encephalomyelitis which was given the name ‘chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids’ (CLIPPERS). It is defined by the combination of clinical, radiological, pathological criteria and response to steroids.

Case Report/Description:

Chronic lymphocytic inflammation of the pons with perivascular enhancement responsive to steroid (CLIPPERS) is a rare and increasingly identifiable disease. Although the pathogenesis is still unknown, majority of patients were treated based on clinical features, radiological findings and after exclusion of other differential diagnosis. We present an 8 years old patient who presented with diplopia, blurred vision and ataxia for 1 week. He was initially treated as meningitis in which antiviral and antibiotics were endorsed resulting in no response to treatment. MRI showed typical punctuate and linear gadolinium enhancement mainly involving the pons on MRI which resorted to the diagnosis of CLIPPERS syndrome. Steroid treatment was initiated and symptoms rapidly improved with the patient being able to carry out normal daily activity upon day 5 of steroid treatment. 

Discussion/Conclusion:

MRI should be considered when the diagnosis of CLIPPERS syndrome is taken into consideration as imaging findings are typical. Diagnosis is crucial as prompt treatment with steroids has good clinical outcome, whereas delay in treatment may cause further irreversible neurological deterioration. To the best of our knowledge this is the youngest patient yet to be diagnosed with CLIPPERS syndrome.