Congenital cerebral venous dysgenesis. Decreased cerebral blood flow in deep cerebral regions revealed by SPECT.

This report describes a rare case of primary cerebral venous dysgenesis in a 3-year-old child with development retardation. Angiography resulted in nonvisualization not only of deep cerebral veins but also of superficial cerebral veins. In computed tomography and in magnetic resonance imaging the collateral venous circulation appeared as a strange configuration in the pineal region. Single photon emission computed tomography using N-isopropyl-p-[I-123]-iodoamphetamine revealed decreased regional cerebral blood flow in the basal ganglia and thalamus, but cerebral infarction was not detected in the area. These features indicate that in this case, dysgenesis of deep cerebral veins, which probably occurred during prenatal life, had caused hypoperfusion in the deep cerebral regions.