Clinical Characteristics and Diagnosis of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome / 实用儿科临床杂志

ABSTRACT

Objective To summarize the clinical characteristics and diagnosis of hemiconvulsion-hemiplegia-epilepsy(HHE) syndrome.Methods The clinical data of 5 children with HHE syndrome were analyzed.Cranial magnetic resonance imaging MRI and /or computerized tomography(CT) and electroencephalogram(EEG) were done in all patients.Results The age of onset was less than 4 years in 4 of 5 patients and initial seizures were associated with fever in 3 cases.The longest duration of convulsive seizures lasted more than 2 hours in all cases,followed by the appearance of hemiplegia involving the side of the body where the convulsions were localized.Subsequent epilepsy developed within 2 years of the hemiplegia in all 5 cases,with 4 cases simple partial seizure and 1 case psychomotor seizures.All cases had mental retardation.Cranial MRI showed left hippocampal sclerosis in 4 patients and CT brain scan showed right cerebral hemiatrophy in 1 case.The EEG showed abnormality in all patients.Epileptiform discharges were found in 4 cases,and the damage hemisphere showed slowing and low voltage of background activity in 1 case.All cases received mainly carbamazepine therapy,and the seizures were satisfactorily controlled or partly controlled.Conclusions HHE syndrome is recognized as hemiplegia-epilepsy syndrome after prolonged hemiconvulsion.The hippocampal sclerosis may be the consequence of repeated seizures,but not the cause.Better understanding of the syndrome,early and adequate treatment of prolonged seizures,especially of febrile convulsion,will reduce the incidence of HHE syndrome.