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Factors associated with the presentation of moyamoya in childhood
Catherine Amlie-Lefond1, Richard G Ellenbogen2
1Department of Neurology, Seattle Children's Hospital, University of Washington, Seattle, Washington.
Insights
Moyamoya disease in children often presents with underlying conditions and a history of transient ischemic attacks (TIAs), with Native Americans potentially at higher risk. Early diagnosis is crucial for better outcomes.
Area of Science:
- Pediatric Neurology
- Cerebrovascular Diseases
- Medical Genetics
Background:
- Moyamoya disease is a leading cause of stroke in children, frequently diagnosed late.
- Delayed diagnosis occurs even in high-risk groups and despite prior transient ischemic attacks (TIAs).
Purpose of the Study:
- To identify risk factors and presentation patterns in pediatric moyamoya.
- To better define at-risk populations for moyamoya disease.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with moyamoya.
- Analysis of patient demographics, medical history, and presenting symptoms.
Main Results:
- Fifty-four pediatric moyamoya cases identified; mean age 7.5 years, 56% female.
- 59% had associated predisposing conditions; 31% were of Asian descent; 15% were Native American.
- 70% had a history of TIAs; 48% presented with stroke; 52% reported headaches.
Conclusions:
- Most children in this series had predisposing conditions, unlike other US studies.
- Fewer patients presented with stroke, but more had TIAs, headaches, and Native American heritage.
- Native American children may represent a higher-risk group for moyamoya disease.
Background:
Moyamoya accounts for approximately one fifth of cerebral arteriopathies in children presenting with acute stroke; however, often is not diagnosed until stroke occurs, even in higher risk populations and often despite a history of transient ischemic attacks (TIAs). This study was undertaken to better define which groups of children are at risk for moyamoya and how they present.
Methods:
With institutional review board approval, the charts of all patients with a diagnosis of moyamoya were reviewed.
Results:
Fifty-four patients were identified. Mean age at presentation was 7.5 years; 56% were female; 32 (59%) had predisposing medical conditions associated with moyamoya; and 17 (31%) were of Asian descent. Only 11 children (20%) had no underlying predisposing condition identified, of whom, 4 had underlying conditions not previously reported in association with moyamoya. Eight patients (15%) were Native American; 38 (70%) had a history of TIAs; and 26 (48%) presented with acute stroke, of whom, three fourth had left and one fourth had right hemisphere strokes. Fifty-two percent had a history of headache.
Conclusions:
Most children with moyamoya in this series had an underlying predisposing condition, compared with other US series, where almost half had no predisposing condition identified. In this series, fewer children (48% versus 68%) presented with stroke, many (70% versus 43%) had a history of TIAs, and many (52% versus 22%) had a history of headaches than in previous reports. In addition, the Native American population may be at higher risk of moyamoya than previously recognized.
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