• Neurol Neurochir Pol · Nov 2013

    Case Reports

    Hemiconvulsion-hemiplegia-epilepsy syndrome. Magnetic resonance findings in a 3-year-old boy.

    • Stefania Salafia, Andrea D Praticò, Enza Pizzo, Filippo Greco, and Domenico Di Bella.
    • 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.
    • Neurol Neurochir Pol. 2013 Nov 1;47(6):584-9.

    AbstractThe 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.

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