Single photon emission computed tomography (SPECT) of the brain using N-isopropyl-p-[¹²³I] iodoamphetamine (¹²³I-IMP) was performed in 11 children with port wine stain on the face or head, aged 1.0-14.2 years at investigation.
Four cases without neurologic symptoms had no specific abnormality on SPECT and X-ray computed tomography (CT), In 4 cases of so-called Sturge-Weber syndrome with developmental quotients (DQ) or intelligence quotients (IQ) more than 80 and the neurologic symptoms consisting of seizures and hemiplegia, SPECT showed localized reduction of IMP accumulation, and CT exhibited calcification, atrophy and enhancement in 2 cases of 3 with contrast medium infusion in the same areas. In 3 cases with DQ of 50-60 and severer neurologic symptoms, SPECT showed diffuse reduction or defect of IMP accumulation in the ipsilateral hemisphere, and CT exhibited remarkable atrophy, calcifications and enhancement in 2 cases with contrast medium infusion in the same hemisphere. In one case with severe neurologic symptoms, SPECT performed at an early stage showed high IMP accumulation in the ipsilateral hemisphere.