Infantile spasms with predominantly unilateral cerebral abnormalities

To investigate the pathogenesis of infantile spasms, 12 children underwent a combination of neurophysiologic and neuroimaging studies including brainstem evoked potentials and single photon emission computed tomography with 99mTcHMPAO. Three of the children had localized cerebral abnormalities on neuroimaging, i.e. right frontotemporal cortical microdysgenesis, left frontotemporal polymicrogyria, and right parietooccipitotemporal porencephalic cyst. Neurophysiologic studies also indicated that a single cerebral hemisphere was predominantly involved, while the other hemisphere and the brainstem were relatively spared, a result compatible with the clinical findings of hemiparesis and hemiconvulsion. In these three patients, administration of carbamazepine was followed by marked improvement of the clinical and electroencephalographic findings. Our results suggest that a combination of noninvasive examinations can distinguish a particular subtype of infantile spasms associated with a localized cerebral abnormality and responsive to carbamazepine.