Original ArticlePatterns of Cerebral Ischemia in Children With Moyamoya
Introduction
Moyamoya disease, common in Asian countries, is a rare progressive steno-occlusive cerebrovascular disorder of unknown etiology. It is characterized by stenosis and occlusion of the arteries of circle of Willis, with abnormal telangiectatic collateral circulation at the base of the brain. Moyamoya is categorized as moyamoya disease when there is no underlying etiology or association and as moyamoya syndrome if an underlying etiology or association is recognized.1, 2 Childhood moyamoya typically presents with recurrent bilateral ischemic events, often transient ischemic attacks (TIAs), in association with hyperventilation suggesting brain hypoperfusion as the likely mechanism in the already compromised stenotic and occluded vessels and newly formed telangiectatic collateral circulation. The clinical presentation is variable, with either focal neurological deficits (often transient) or nonspecific symptoms such as lethargy, headache, and vomiting, depending on the areas of ischemic involvement and severity of the steno-occlusive disease.1, 2, 3, 4, 5, 6, 7 The need for revascularization surgery in children with moyamoya is dependent on the extent and type of ischemic infarction (bilateral and multiple strokes), the presence of advanced vascular stage and poor cerebrovascular reserve (as indicated by the ivy sign on magnetic resonance imaging [MRI] brain fluid attenuated inversion recovery and contrast-enhanced T1-weighted sequences),8, 9 and cerebrovascular reserve mapping studies including single-photon emission computerized tomography and perfusion scans10, 11 and progression of the disease.12, 13, 14 In the Japanese population, where the moyamoya disease is prevalent, data regarding the severity of the disease have been well studied. However, the literature is limited regarding the patterns of cerebral ischemia in childhood moyamoya, particularly in the non-Japanese population. One recent study in a Korean cohort of patients with moyamoya studied the pattern of ischemic infarction based on diffusion-weighted neuroimaging. They compared the acute diffusion-weighted neuroimaging of 34 adult patients with moyamoya to 32 childhood moyamoya patients when they presented with acute stroke signs. They found that among all patterns of infarction, a gyral pattern was the most common (44%) and cortical border zone and deep lacunar patterns were infrequent, and among children with moyamoya, cortical and border zone patterns were more frequently observed, whereas a honeycomb pattern was common in adult patients and with advanced vascular stages.15 In their study, the border zone pattern only included the typical cortical watershed border zone that is present between two major cerebral arteries, and the honeycomb pattern was the patchy cortical and subcortical infarction. In addition, the study did not assess the patterns of infarction over a longitudinal period or the patterns of chronic or remote infarction using other neuroimaging sequences.15 We therefore sought to determine patterns of cerebral ischemic infarction in a non-Japanese population of children with moyamoya.
Section snippets
Study design
We retrospectively reviewed the records of a prospectively ascertained consecutive cohort of children, 1 month to 18 years, with both moyamoya disease and moyamoya syndrome, observed at the two Canadian Pediatric Ischemic Stroke Registry affiliated centers, The Hospital for Sick Children and the McMaster University Medical Centre. The study period was from January 1992 to June 2006. The study was approved by the institution's research ethics board.
Aims of the study
The primary aim of the study was to review the
Demographics
We identified 23 children, without sickle cell hemoglobinopathy, with a diagnosis of either moyamoya disease or syndrome. Three patients with unavailable radiology films were excluded from the analysis. Of the remaining 20 children, there were five boys and 15 girls, with median age 6.4 years (range, 2 months to 15.5 years).
Clinical presentation
The initial clinical presentation included persistent focal neurological deficits in 17, history consistent with preceding TIAs in seven, headache in eight, focal seizures
Discussion
The etiologic mechanisms and associations of moyamoya disease is extensively described and studied in the medical literature.1, 21, 22 There are scarce data with regards to patterns of ischemic injury in patients with this unique disorder. In addition, both embolic and hemodynamic factors have been implicated as the underlying mechanism of ischemia. Our study is the first to assess the patterns of both acute and chronic ischemic infarction in a North American cohort of children with moyamoya.
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