title: Hemiconvulsion-hemiplegia-epilepsy syndrome. Magnetic resonance findings in a 3-year-old boy.
abstract: The term 'hemiconvulsion-hemiplegia-epilepsy syndrome' (HHE) was first used by Gastaut et al. to describe the se-quential combination of unilateral or predominantly unilateral clonic seizures (hemiconvulsion), occurring during the first 2 years of life, immediately followed by an ipsilateral flaccid hemiplegia lasting 7 or more days. In the following phase partial epileptic seizures occur. We report a case of HHE syndrome in a 3-year-old boy with partial seizures (hemiconvulsion lasting 15-30 minutes) followed by left hemiplegia and hyporeflexia. Magnetic resonance imaging showed diffuse and high signal hyperintensity of the whole right cerebral hemisphere. Diffusion-weighted images showed a reduction of the apparent diffusion coefficient in the subcortical region. Magnetic resonance arterio-graphy showed a narrow flow signal in the distal territory of the right middle cerebral artery. The authors emphasize the importance of neuroradiological findings in early diagnosis and in the follow-up of HHE syndrome.
authors.affiliation: Dr. Andrea Domenico Praticò, Department of Pediatrics, University of Catania, Via S. Sofia 78, Catania, Italy, phone: 00390953781193, fax: 0039095222532, e-mail: terrenere178@tin.it.
authors.forename: Stefania
authors.initials: S
authors.lastname: Salafia
authors.forename: Andrea D
authors.initials: AD
authors.lastname: Praticò
authors.forename: Enza
authors.initials: E
authors.lastname: Pizzo
authors.forename: Filippo
authors.initials: F
authors.lastname: Greco
authors.forename: Domenico
authors.initials: D
authors.lastname: Di Bella
mesh_terms: D001921:Brain / Q000473:pathology*; D002675:Child, Preschool; D038524:Diffusion Magnetic Resonance Imaging; D004827:Epilepsy / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006429:Hemiplegia / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D012640:Seizures / Q000209:etiology; D013577:Syndrome