A study of brain perfusion in acute infantile hemiplegia by single photon emission computed tomography (SPECT)

ation of developmental delay. The family history was not contributory. The pregnancy was moderately complicated with toxemia. He was born by means of cesarean section at 41 weeks because of cloudy amniotic fluid and fetal arrhythmia without asphyxia. His birth weight was 2650 g. His arrhythmia and feeding difficulty continued for 2 months. His motor progression was slow and his neck was unstable at 5 months, at which time he was noted to have pes valgus and he was thus referred for physiotherapy. At 8 months and at 8 years, he was subjected to CT and MRI, which revealed abnormal density of the white matter in the occipito-parietal region. Many diagnostic methods were tried unsuccessfully. He visited our hospital at the age of 9 years, 5 months. At this age, his development was that of a 5-year-old boy. On examination, he was a well nourished, happy boy with a head circumference of 51.3 cm (1.0 u), external strabismus, pes valgus, hypotonia, normo-active and symmetric DTR exaggeration, and extensor plantar responses. There was no hepato-splenomegaly or cardiovascular abnormality. His facial appearance was peculiar. Ophthalmologic examination was normal. Laboratory studies of blood and urine for amino acids, organic acids, ammonia, lactate, pyruvate and lysosomal enzymes, and immunological and chromosomal analyses were normal. CSF analysis and EEG including the hyperventilation test, EMG, SEP, VEP and ABR were all normal. Cranial CT disclosed characteristic multiple spots at the basal ganglia with indefinite Wilms’ arterial rings. Another demonstrable finding was low periventricular density in the bilateral parieto-occipital regions. MRI demonstrated many small foci of CSF signals in all pulse sequences in the high-convexity gray matter extending into the centrum semiovale. MR angiography revealed a stenotic region in the C3-C4 portion of the bilateral internal carotid arteries and demonstrated moyamoya vascular changes. lz31MP-SPECT revealed a marked reduction in blood flow in the left hemisphere. MR angiography is a beneficial new technique for the diagnosis and evaluation of moyamoya disease, because it is a noninvasive method and does not require contrast materials.