Published Online
on April 27, 2006

A more recent version of this article appeared on June 1, 2006

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Submitted on January 16, 2006
Revised on March 15, 2006
Accepted on March 17, 2006

Clinical Features and Outcome in North American Adults With Moyamoya Phenomenon

Christopher L. Hallemeier BA; Keith M. Rich MD; Robert L. Grubb Jr MD; Michael R. Chicoine MD; Christopher J. Moran MD; DeWitte T. Cross III MD; Gregory J. Zipfel MD; Ralph G. Dacey Jr MD; and Colin P. Derdeyn MD^*

From the Interventional Neuroradiology Section (C.L.H., C.P.D., C.J.M., D.T.C.), Mallinckrodt Institute of Radiology, and the Departments of Neurological Surgery (C.P.D., K.M.R., R.L.G., M.R.C., C.J.M., D.T.C., G.J.Z., R.G.D.), and Neurology (C.P.D., G.J.Z.), Washington University School of Medicine, St Louis, Mo.

^* To whom correspondence should be addressed. E-mail: derdeync{at}wustl.edu.

Background and Purpose--To describe baseline clinical features and outcomes of adults with moyamoya phenomenon treated at a single North American institution.

Methods--We identified 34 adults with moyamoya phenomenon by review of angiographic records. Clinical presentation and baseline stroke risk factors were obtained by chart review. Follow-up was obtained prospectively. A 5-year Kaplan-Meier stroke risk was calculated.

Results--The median age was 42 (range 20 to 79) years. Twenty-five were women. The initial symptom was ischemia, hemorrhage, or asymptomatic in 24, 7, and 3 patients, respectively. Twenty-two had bilateral involvement and 12 had unilateral moyamoya vessels. Baseline stroke risk factors were similar between groups. The median follow-up in 31 living patients was 5.1 (range 0.2 to 19.6) years. Fourteen patients were treated with surgical revascularization (20 total hemispheres). In medically treated symptomatic hemispheres, the 5-year risk of recurrent ipsilateral stroke was 65% after the initial symptom and 27% after angiographic diagnosis. Patients with bilateral involvement presenting with ischemic symptoms were at the highest risk of subsequent stroke (n=17, 5-year risk of stroke with medical treatment after first symptom of 82%). In surgically treated hemispheres, the 5-year risk of perioperative or subsequent ipsilateral stroke or death was 17%. This was significantly different compared with medical treatment after first symptom (P=0.02) but not after angiographic diagnosis.

Conclusion--Moyamoya phenomenon in North American adults is associated with a high risk of recurrent stroke, particularly those with bilateral involvement and ischemic symptoms. These data suggest a potential benefit with surgery if diagnosis could be made earlier.

Key words: cerebral hemorrhage • cerebral infarction • cerebral revascularization • Moyamoya disease • retrospective studies

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