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.
Central nervous system (CNS) malformations are frequently severe and often fatal. Diagnosis during pregnancy is of fundamental importance for a correct clinical evaluation and pharmacological management or, where possible, surgery. Modern imaging technology, pre and post-natal ultrasound scans, but above all magnetic resonance (MR) imaging have revolutionized the diagnostic study of cerebral malformations. In particular, MR provides images of the various brain sections by means of a multidisciplinary non invasive study that today is particularly rapid. MR allows an accurate assessment of cortical development, normal myelinization and the evolution of malformations. We report here our experience with MR carried out after birth on 5000 patients for the study of non vascular cortical anomalies. In only one case was the examination carried out during pregnancy, following ultrasound investigations that showed a developmental anomaly of the corpus callosum. We confirmed 112 malformations with an incidence of 2.24%, greater in males and affecting principally the anatomy of the supratentorial encephalic structures. These cases were selected and MR was carried out based on the clinical picture present at the time of the examination or a suspected diagnosis formulated following ultrasound. The ultrasound investigations were carried out principally in the obstetrics department; only a few were carried out in our unit, together with most of the postnatal ultrasound scans. The percentage of the various malformations was higher than those reported in the literature.
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