From the Department of Neurology, Baylor College of Medicine (D.C.), The
Greater Houston Neurosurgery Center (P.S.), and the Department of Neurology,
University of Texas Houston Health Sciences Center (P.B., J.C.G.), Houston,
Tex.
Correspondence to David Chiu, MD, Baylor College of Medicine, Department of Neurology, Suite 1801, 6550 Fannin, Houston, TX 77030. E-mail dchiu{at}bcm.tmc.edu
Background and Purpose—We report the
clinical features and longitudinal outcome of the largest cohort of
patients with moyamoya disease described from a single institution
in the western hemisphere. Moyamoya disease in Asia usually
presents with ischemic stroke in children and intracranial
hemorrhage in adults.
Methods—Our study population included all patients with
moyamoya disease evaluated at a university hospital in Houston,
Texas from 1985 through 1995 (n=35). We used Kaplan-Meier methods to
estimate individual and hemispheric stroke risk by treatment status
(medical versus surgical). Predictors of neurological outcome were
assessed.
Results—The ethnic background of our patients was
representative of the general population in Texas. The
mean age at diagnosis was 32 years (range, 6 to 59 years).
Ischemic stroke or transient ischemic attack was the
predominant initial symptom in both adults and children. Of the 6
patients with intracranial hemorrhage, 5 had an
intraventricular site of hemorrhage. The
crude stroke recurrence rate was 10.3% per year in 116
patient-years of follow-up. Twenty patients underwent surgical
revascularization, the most common procedure being
encephaloduroarteriosynangiosis. The 5-year risk of ipsilateral stroke
after synangiosis was 15%, compared with 20% for medical treatment
and 22% overall for surgery.
Conclusions—Our observations indicate that moyamoya disease
may have a different clinical expression in the United States than in
Asia, and may demonstrate a trend toward a lower stroke
recurrence rate and better functional outcome after
synangiosis.
© 1998 American Heart Association, Inc.
Original Contributions
Clinical Features of Moyamoya Disease in the United States
Key Words: moyamoya disease • stroke, ischemic • intraventricular hemorrhage • epidemiology
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