Abstract Details

Title CLIPPERS: Rare Disease or Tip of the Iceberg? A Case Report and Literature Review

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P03 - (-)

Poster/Presentation
Number 190

Objective

Background BACKGROUND: There is limited literature on the neurological disorder known as CLIPPERS(Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids), first reported in 2010. CLIPPERS is most noted for a signature MRI appearance demonstrating perivascular enhancement involving the cerebral vasculature predominantly in the pons, cerebellum, brachium pontis, with or without associated symptoms ranging from ataxia to pseudobulbar affect.

Design/Methods DESIGN/METHODS: A 70 year old female presented with a 4 week history of frequent falls, progressive right lower-extremity weakness and ataxia. Extensive work up revealed oligoclonal bands and elevated myelin basic protein in the CSF, positive CANCA in blood, and brain MRI abnormalities. Her MRI revealed peppered perivascular enhancement in the brainstem and peri-ventricular areas. This pattern was initially believed to be consistent with AVM, but cerebralangiography was negative. Further review of the MRI indicated features reminiscent of CLIPPERS syndrome. No alternative diagnosis was established despite thorough investigation. Based on a presumptive diagnosis of CLIPPERS syndrome, she was treated with 5 days IV methylprednisone resulting in clinical and radiological improvement. No further steroids were given.

Results RESULTS: More than 8 weeks following her initial presentation, the patient progressively stabilized on MRI and continued to do well clinically. A PubMED search was carried out to review the current literature. Seminal clinical features, presentation, diagnosis, treatment, recurrence and outcome will be discussed and reviewed in detail.

Conclusions CONCLUSIONS: We raise the possibility that CLIPPERS syndrome may be more common than realized. Awareness of the unique radiological findings may suggest this disease. We surmise that careful review of perplexing MRI imaging on difficult cases could reveal CLIPPERS as the diagnosis for many more potentially treatable cases.

Authors/Disclosures
Fawaz Al-Mufti, MD (Westchester Medical Center at New York Medical College) PRESENTER	Dr. Al-Mufti has received personal compensation in the range of $0-$499 for serving as a Consultant for Stryker. Dr. Al-Mufti has received personal compensation in the range of $0-$499 for serving as a Consultant for Cerenovus. Dr. Al-Mufti has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Revalesio .
	No disclosure on file
	No disclosure on file
Patricia K. Coyle, MD, FAAN (SUNY At Stony Brook)	Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Accordant. Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Coyle has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Sanofi Genzyme. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for GlaxoSmithKline. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Horizon Therapeutics. The institution of Dr. Coyle has received research support from CorEvitas LLC. The institution of Dr. Coyle has received research support from Genentech/Roche. The institution of Dr. Coyle has received research support from NINDS. The institution of Dr. Coyle has received research support from Sanofi Genzyme. The institution of Dr. Coyle has received research support from Cleveland Clinic.
	No disclosure on file

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