A Rare Presentation of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) as Acute Ischemic Stroke

OBJECTIVE: A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS) presenting with acute brainstem stroke after six months of progressive diplopia and gait difficulty.BACKGROUND: CLIPPERS is a recently described disorder with clinical brainstem symptoms and characteristic radiographic findings of numerous punctate or nodular enhancing lesions throughout the pons, middle cerebral peduncal, and cerebellum due to a lymphocytic infiltrate that is highly responsive to steroids.DESIGN/METHODS: Case report.RESULTS: A 75 year-old man presented for imaging after six months of progressive diplopia, difficulty walking, and fatigue, and negative extensive neuromuscular workup. He experienced acute exacerbation of his symptoms with new-onset dysphagia, dysarthria, and severe ataxia. Inpatient workup revealed a left cerebellar lesion concerning for acute ischemia. On outpatient follow-up four weeks later, his symptoms remained progressive, and repeat imaging with contrast showed progression of the ischemic lesion with periventricular enhancement and numerous lesions involving the pons, cerebellum, and deep ganglionic structures. He underwent extensive neoplastic, paraneoplastic, inflammatory, and infectious workup and was ultimately diagnosed with CLIPPERS given the characteristic imaging findings. Symptoms began to improve after a five-day course of high-dose intravenous methylprednisolone. He remained on steroid maintenance therapy after discharge. The dysarthria resolved completely, diplopia became minimal, and he ambulates without assistive device.CONCLUSIONS: This case illustrates not only a classic clinical and radiographic case of CLIPPERS, but also that this recently recognized disorder can be easily misdiagnosed as a neuromuscular disease in the early stages, and stroke in the more severe stages. Rarely, the inflammatory infiltrate can result in stroke in these patients, and is likely the cause of the acute exacerbation of our patient’s symptoms. This case also demonstrates the need for contrast imaging whenever possible, as the non-contrast imaging failed to identify the characteristic diffuse enhancing punctate lesions. Disclosure: Dr. Gadhia has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Lai has received personal compensation for activities with UCB Pharma and Lilly as a speaker and/or consultant. Dr. Lai has received research support from Medtronic. Dr. Lee has nothing to disclose.