Single photon emission tomography (SPECT), MRI and EEG were studied in two infants with lissencephaly. One infant had generalized tonic convulsions at 7 month of age and continuous high voltage fast activities on EEG. The other infant developed infantile spasms at the age of 2.5 months, and showed low voltage disorganized background with pseudorhythmic high voltage sharp waves on EEG. Cranial CT and MRI in both infants revealed abnormally thick cortex, dilatation of the lateral ventricles, and smooth or relatively smooth cerebral surface. In SPECT studies, a diffuse increase of tracer uptake was shown at the cerebral cortex compared with the basal ganglia or cerebellum in both infants. The SPECT finding might reflect the abnormal cortical architectures in this cerebral malformation.
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