HEMICONVULSION-HEMIPLEGIA-EPILEPSY SYNDROME - A CLINICAL, ELECTROENCEPHALOGRAPHIC AND NEURORADIOLOGICAL STUDY

Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epile psy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemico nvulsion in three of the children and generalized tonic-clonic seizure s in the others, being preceded by hemifacial twitching or head and ey e deviation in two. They were followed by hemiplegia, which cleared wi th time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture de formities and severe mental retardation following repeated status epil epticus. Subsequent epilepsy developed in five patients and was satisf actorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomogr aphy and/or magnetic resonance imaging. Single photon emission compute d tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral sl owing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.