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Abstract Details

Is there a distinct phenotype for non-Asian moyamoya patients?
Cerebrovascular Disease and Interventional Neurology
P2 - Poster Session 2 (5:30 PM-6:30 PM)
3-068
We aimed to describe idiopathic moyamoya syndrome patients in New York and identify differences between Asians and non-Asians in this population.
Idiopathic moyamoya syndrome (IMMS) is a progressive narrowing of the anterior circulation arteries resulting in the development of a tenuous collateral blood supply which is prone to rupture or thrombosis. IMMS has mostly been studied in East Asian populations where it was first described, with limited data describing this syndrome in non-Asians. 

Using the New York State Department of Health Statewide Planning and Research Cooperative System database from 2000-2014 we identified all newly diagnosed cases of IMMS (ICD-9 437.5) in adults aged 18 and older. We excluded patients with down syndrome, neurofibromatosis type 1 and sickle cell disease. We compared patient demographics, comorbidities and extracranial-intracranial revascularization (ECIC) rates for IMMS patients that self-identified Asian race/ethnicity vs those who did not.

All IMMS diagnoses occurred in urban hospitals. There were 554 IMMS patients in total; 89.4% (n=495) were non-Asian while only 10.6% (n=59) were Asian. A smaller proportion of non-Asian IMMS patients were diagnosed in smaller, non-academic hospitals compared to Asians (3.8% vs 10.2%, p=0.03) (Table). Both groups showed comparable rates of characteristics and comorbidities. Stroke risks following IMMS diagnosis were similar for both Asians and non-Asians (13.6% vs 22.4%, p=0.1); strokes occurred later for non-Asian patients with ECIC than in those without (2.1 + 2.8 vs 1.4 + 2.1 years, p=0.04). No subsequent strokes occurred in Asians after ECIC.

Individuals of non-Asian origin account for almost 90% of IMMS diagnoses in New York State, but they are infrequently diagnosed in small, non-academic hospitals. Further research is needed to identify a distinctive phenotype and genotypic profile of IMMS in non-Asians.

Authors/Disclosures
Hajere Gatollari
PRESENTER
No disclosure on file
Eliza C. Miller, MD (University of Pittsburgh) Dr. Miller has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for medical malpractice cases. The institution of Dr. Miller has received research support from National Institutes of Health. Dr. Miller has a non-compensated relationship as a member of ASA Advisory Council with American Heart Association/American Stroke Association that is relevant to AAN interests or activities.
Amelia K. Boehme, PhD (Columbia University) Dr. Boehme has nothing to disclose.
No disclosure on file
Mitchell S. Elkind, MD, MS, FAAN Dr. Elkind has received personal compensation for serving as an employee of American Heart Association. Dr. Elkind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Atria Academy.
Joshua Z. Willey, MD, FAAN (Columbia University) Dr. Willey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbott. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Edwards Scientific. Dr. Willey has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for RECARDIO. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbott. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BrainQ. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Journal of the American College of Cardiology. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Uptodate. The institution of Dr. Willey has received research support from NIH. Dr. Willey has received personal compensation in the range of $500-$4,999 for serving as a Review chapter author, MKSAP 16-20 with American College of Physicians.