Clinical Features and Outcome in North American Adults With Moyamoya Phenomenon

Abstract

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 (nϭ17, 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 (Pϭ0.02) 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.

Epidemiological Features of Moyamoya Disease in Japan

“…2) The other report from the United States includes only 22 patients with bilateral moyamoya disease. 4) The female-tomale ratio was 6.3, ischemic stroke and TIA were the predominant initial symptoms, and again, no peak incidence in childhood was observed. A report from Europe evaluated 21 patients with moyamoya disease.…”

Optimal surgical treatment for moyamoya disease in adults: direct versus indirect bypass

“…35,39 A number of studies have provided evidence that moyamoya disease outside of Asia can represent a different phenomenon. 4,7,21,36,38 Patient ethnicities are in proportion to the ethnicities of the people in the region of diagnosis, and adult patients present with ischemic symptoms rather than intracranial hemorrhage.…”

“…Although no completed randomized clinical trials have addressed the benefit of surgical revascularization, the dismal prognosis of moyamoya disease [2][3][4]7,8,21,23,31,33,[41][42][43] and a large number of case series demonstrating an improved outcome in surgically treated patients have provided evidence in support of revascularization procedures in a select group of patients. 4,[7][8][9][10][11]16,17,25,29,30,32,34,40,43 Currently, the Ministry of Health and Welfare of Japan reports that bypass surgery is indicated when there are 1) repeated clinical symptoms due to apparent cerebral ischemia and (2) decreased regional cerebral blood flow, vascular response, and perfusion reserve.…”

“…4,[7][8][9][10][11]16,17,25,29,30,32,34,40,43 Currently, the Ministry of Health and Welfare of Japan reports that bypass surgery is indicated when there are 1) repeated clinical symptoms due to apparent cerebral ischemia and (2) decreased regional cerebral blood flow, vascular response, and perfusion reserve. 6 Some authors have reported a benefit from revascularization in patients presenting with intracranial hemorrhage.…”

Moyamoya disease

“…У взрослых могут развиваться внутричереп-ные кровоизлияния, включая субарахноидальные. У евро-пейцев симптомы болезни появляются в более позднем возрасте, чем у азиатов, и реже встречаются геморрагиче-ские варианты [15][16][17].…”