Spontaneous occlusion of the circle of Willis, i.e., moyamoya disease
(MMD), is a clinical disease entity angiographically characterized by
progressive and bilateral stenosis of the carotid bifurcation, with a
hazy collateral network at the base or convexity of the brain. Althoug
h the importance of computed tomography (CT) and conventional magnetic
resonance (MR) imaging in diagnosing MMD has already been determined,
conventional arteriography is still regarded as necessary for definit
ive diagnosis. Magnetic resonance angiography (MR-A) is a very recent
vascular imaging technique which allows noninvasive and direct imaging
of vessels without the use of contrast medium. We present four pediat
ric cases of MMD, evaluated by conventional angiography, CT, MR imagin
g, and MR-A. Our data demonstrate that MR-A is successful both in allo
wing detection of occlusive disease of the basal portion of the intern
al carotid artery and large branch basal cerebral vessels and demonstr
ating the collateral vessels at the base of the brain. As a noninvasiv
e procedure, MR-A promises to become a valuable alternative to classic
al angiography in the diagnosis of MMD.