Factors associated with the presentation of moyamoya in childhood

J Stroke Cerebrovasc Dis. 2015 Jun;24(6):1204-10. doi: 10.1016/j.jstrokecerebrovasdis.2015.01.018. Epub 2015 Apr 10.


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.

Keywords: Moyamoya; childhood; stroke; transient ischemic attack.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease Susceptibility
  • Female
  • Humans
  • Infant
  • Male
  • Moyamoya Disease / complications
  • Moyamoya Disease / diagnosis*
  • Retrospective Studies
  • Risk Factors
  • Stroke / complications
  • Stroke / diagnosis*