Abstract Details

Title Clinical Characteristics and Predictors of Outcome in Patients with Moyamoya Disease in the United States: Experience from Two Clinical Centers

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P06 - (-)

Poster/Presentation
Number 260

Objective

Background BACKGROUND: Moyamoya is a cerebrovascular disorder characterized by progressive narrowing of the proximal vessels of the circle of Willis, with subsequent development of collaterals. It was originally described in Japan, where adults present more often with hemorrhagic than ischemic strokes. Its incidence in the United States is increasing, accounting for more than 2000 admissions nationwide in the last decade. Differences in presentation among US patients, necessitates further research into clinical characteristics.

Design/Methods DESIGN/METHODS: This is a retrospective chart review. Patients diagnosed with moyamoya disease at two centers in Houston, TX from January 1, 2002 to December 1, 2011 were identified using the ICD-9 code 437.5.

Results RESULTS: 32 adult (age>18) and 13 pediatric (age<18) charts were reviewed, and adult data is presented. Mean age at presentation was 37.3 years, and 81% patients were female. 40% patients were Caucasian, 25% African Americans, 25% Hispanics and 6% Asians. Presenting symptoms included ischemic strokes (37.5%), TIAs (25%), headaches (15%) and ICH (12%). Hypertension (47%) and tobacco use (25%) were the most frequent comorbidities. 12.5% adults had sickle cell disease, compared to 38.5% children. Stroke recurrences were higher in ischemic than hemorrhagic subtypes (53% VS 6.3%). All patients had angiographically proven anterior circulation involvement and 21% had concomitant PCA stenosis. Of patients with ischemic strokes, 45% underwent EC-IC bypass and 15% underwent Encephaloduroarteriosynangiosis. There were no in-hospital deaths, with 85% of hospitalized patients discharged to home. 3 of 4 patients with ICH were discharged to LTAC.

Conclusions CONCLUSIONS: Predominant ischemic strokes, comorbid vascular risk factors, lower incidence of sickle cell disease, and posterior circulation involvement in our patients suggest a different clinical picture and pathophysiology for adult onset moyamoya disease in the US. Prognosis is generally good, though hemorrhagic strokes may carry worse outcomes.

Authors/Disclosures
Sahar Zafar, MD PRESENTER	Dr. Zafar has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer. Dr. Zafar has received research support from NIH. Dr. Zafar has received personal compensation in the range of $5,000-$9,999 for serving as a Speaker for a lecture with Marinus.
Kasey L. Gildersleeve, MD	No disclosure on file
Chethan P. Venkatasubba Rao, MD (Baylor College of Medicine)	Dr. Venkatasubba Rao has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Maestro Inc.
Michael E. Newmark, MD, FAAN (Kelsey-seybold Clinic)	No disclosure on file
	No disclosure on file
Jose I. Suarez, MD, FAAN (Johns Hopkins Hospital)	Dr. Suarez has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Idorsia. Dr. Suarez has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for The Mayo Clinic.
Eric Bershad, MD (Baylor College of Medicine)	Dr. Bershad has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various.
Björn Tackenberg, MD, FAAN (F. Hoffmann-La Roche AG)	Dr. Tackenberg has received personal compensation for serving as an employee of F. Hoffmann-La Roche AG. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck Serono. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for GILEAD. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Celgene. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Tackenberg has received personal compensation in the range of $50,000-$99,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Celgene. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Roche. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Merck Serono. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Novartis. Dr. Tackenberg has received stock or an ownership interest from F. Hoffmann-La Roche AG. The institution of Dr. Tackenberg has received research support from Biogen. The institution of Dr. Tackenberg has received research support from Novartis. The institution of Dr. Tackenberg has received research support from Roche.

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