Focal time-to-peak changes on perfusion MRI in children with Moyamoya disease: correlation with conventional angiography.
ABSTRACT Moyamoya disease is a chronic progressive steno-occlusion of the distal internal carotid arteries with unknown etiology. As the classical presentation of childhood Moyamoya disease is ischemic stroke, cerebral hemodynamic evaluation is important for patient selection for surgery to prevent recurrent ischemic attacks. Perfusion MR imaging has been applied to evaluate cerebral hemodynamics.
To correlate the 'basal time-to-peak preservation sign', 'auto-synangiosis sign', and 'posterior involvement sign' on time-to-peak map of perfusion MRI with catheter angiography.
Thirty-four children (6.91 ± 3.08 years) with Moyamoya disease who underwent both perfusion-weighted MRI and catheter angiography were enrolled in this study. Given catheter angiography as a reference standard, basal time-to-peak preservation sign, auto-synangiosis sign, and posterior involvement sign were evaluated on time-to-peak maps.
The basal time-to-peak preservation sign was accurate for the diagnosis of childhood Moyamoya disease; both sensitivity and specificity were 100%. The auto-synangiosis sign showed lower sensitivity (65%), however, with an acceptable specificity (98%). The posterior involvement sign showed lower sensitivity (61%) but had an acceptable specificity (96%).
The basal time-to-peak preservation sign may be a universal finding in childhood Moyamoya disease. The auto-synangiosis and posterior involvement sign may be useful in determining transdural collateral status and posterior circulation involvement in childhood Moyamoya disease.
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ABSTRACT: Intracerebral hemorrhage in patients with moyamoya disease is rare in children. We report three unique cases of pediatric moyamoya disease with hemorrhagic onset. Two 7-year-old girls and a 9-year-old girl were admitted to our hospital because of intracerebral hemorrhage associated with angiographically verified moyamoya disease. Two of them did not demonstrate either an ischemic episode or cerebral infarct on the magnetic resonance images. A decreased regional cerebral blood flow was revealed on single photon emission computed tomography in two patients, who developed cerebral infarction in the acute stage following hemorrhage. They underwent superficial temporal artery-middle cerebral artery anastomoses combined with encephalo-myo-synangiosis, and have not experienced any further ischemic episodes thereafter. Hemodynamic insufficiency associated with moyamoya disease could cause intracerebral hemorrhage even in children. Adequate management in the acute stage of hemorrhage and revascularization surgery are recommended to prevent cerebral infarction, which may easily occur in pediatric patients with moyamoya disease.Clinical Neurology and Neurosurgery 04/2008; 110(3):270-5. · 1.23 Impact Factor