Adult Moyamoya Disease in an Urban Center in the United States Is Associated With a High Burden of Watershed Ischemia

Abstract

BackgroundAdult moyamoya disease is rare in the United States, and patients mostly present with cerebral ischemia. However, clinical and neurodiagnostic correlates of ischemia are not well known in this population. We sought to characterize the clinical and radiographic features of moyamoya disease in a large urban center in the United States, with a focus on angiographic and neuroimaging patterns of ischemia.Methods and ResultsWe retrospectively reviewed charts of consecutive adult moyamoya disease patients evaluated at 2 centers in Houston, Texas from January 2002 to December 2011. We reviewed all available cerebral angiograms and neuroimaging studies to evaluate the Suzuki grades, presence of intracranial hemorrhage or ischemia, infarct patterns, and vascular territory distribution. Our analysis was mainly descriptive. We identified 31 adults with moyamoya disease who met our inclusion criteria. The female‐to‐male ratio was 2.4:1. The majority of patients were white, followed by Hispanic, black, and Asian. Most presented with ischemia (61%), followed by headaches, and intracranial hemorrhage. Of the 22 patients with available neuroimaging, 72.7% had ischemic findings, with the vast majority having a watershed pattern (81.3%).ConclusionsWe observed a high burden of ischemia, mostly watershed pattern on neuroimaging in our adult moyamoya disease patients. Long‐term monitoring of adult moyamoya disease patients in the United States would be useful to better understand the natural history of this condition.

Comparison of Stroke Risk Factors between Symptomatic and Asymptomatic Patients in a North American Moyamoya Disease Cohort

“…Our study supports the findings of Hirano et al in which the authors report that the frequency of stroke risk factors such as HTN, HLD, diabetes, smoking, and alcohol in ischemic, hemorrhagic, and asymptomatic [3,6,9,10].…”

“…Our study supports the findings of Hirano et al in which the authors report that the frequency of stroke risk factors such as HTN, HLD, diabetes, smoking, and alcohol in ischemic, hemorrhagic, and asymptomatic moyamoya groups was not significantly different. Although in our study the stroke risk in asymptomatic moyamoya patients was zero, earlier studies have reported ≥2.4% annual stroke risk in asymptomatic patients [ 3 , 6 , 9 , 10 ].…”

“…Moyamoya disease (MMD) is a rare cerebrovascular condition characterized by progressive narrowing of distal internal carotid arteries and development of a prominent network of cerebral collateral vessels [1, 2]. The most common presentation of MMD is stroke or TIA [3, 4]. In a Japanese population, Hirano et al [5] recently reported that there was no significant difference in baseline stroke risk factors between patients with MMD with either ischemic, hemorrhagic, or asymptomatic presentations.…”

Moyamoya Disease: A Rare Vascular Disease of the CNS

“…Adult patients in Japan with MMS mostly present with hemorrhagic stroke in some old reports [20] in contrast to patients from the United States in whom the ischemic stroke was found to be more common [21]. In a study done on 88 Korean patients, 45% of them had only a single ischemic or hemorrhagic stroke, 55% recurrent attacks mostly ischemic, and 64% of them presented with hemiplegia [22].…”

Η Μελέτη Του Ρόλου Των Προγεννητικών Παραγόντων Στην Εμφάνιση Καλοήθων Και Κακοήθων Παθήσεων Του Κεντρικού Νευρικού Συστήμματος (ΚΝΣ) Στα Παιδιά

“…Αποτελέσματα δεν ποικίλει με την ηλικία εμφάνισης. [1039][1040][1041][1042][1043] Η συχνότητα εμφάνισης της εξέλιξης της νόσου σε μία μελέτη ήταν 20% σε ενήλικες ασθενείς με MMD. [1044] Οι γυναίκες ασθενείς είχαν υψηλότερο κίνδυνο εξέλιξης της νόσου από τους άντρες.…”