Cerebral vascular disease is a common and serious complication of sickle ce
ll disease that mainly involves the large blood vessels of the skull base.
Because recurrences are common and residual deficits severe, attention has
turned to detection of preclinical cerebral involvement with the goal of pr
eventing clinical damage, Magnetic resonance imaging (MRI), an extremely se
nsitive tool for detecting cerebral infarction/ischemia, has shown that 10%
of asymptomatic patients exhibit white matter lesions that seem to be asso
ciated with impaired cognitive function and may be predictive of stroke; ma
gnetic resonance angiography demonstrates occlusions of skull base arteries
but is not reliable for the diagnosis of stenosis because of artifacts gen
erated by rapid turbulent flow. Transcranial Doppler is sensitive and speci
fic for the detection of arterial stenosis and occlusion, even in asymptoma
tic patients. Digitized cerebral angiography remains the gold standard inve
stigation for pretreatment confirmation of lesions detected by Doppler and/
or MRI.