{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T12:03:45Z","timestamp":1791115425061,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2013,8,8]],"date-time":"2013-08-08T00:00:00Z","timestamp":1375920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Hemiconvulsion\u2013hemiplegia syndrome (<jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content>) is a rare severe epilepsy of infancy consisting of unilateral convulsive status epilepticus immediately followed by transient or lasting ipsilateral hemiplegia. <jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content> may occur either in patients with previous brain pathology or without any identified cause, so\u2010called \u2018idiopathic <jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content>\u2019.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>We retrospectively analysed clinical and <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> longitudinal findings of a series of 10 patients (six females, four males) presenting with <jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content>. Age at the study inclusion ranged from 2\u00a0years 6\u00a0months to 15\u00a0years (mean of 5y 10mo, median 4y 2mo). After defining magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>) features as \u2018typical\u2019, i.e. strictly unilateral involvement, and \u2018atypical\u2019, i.e. bilateral, we compared clinical data from both groups. Cognitive level was assessed using Brunet\u2010L\u00e9zine or Wechsler scales.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content> occurred at a mean age of 20.5\u00a0months (range 8\u201348mo). In all cases, status epilepticus lasted for more than 1\u00a0hour and was characterised by unilateral clonic seizures followed by ipsilateral hemiplegia (persistent in five patients). Two patients in this series died: the first from multi\u2010organ failure 2\u00a0weeks after the status epilepticus and the other from a second episode of ipsilateral intractable febrile status epilepticus 3\u00a0years after the first episode. Early <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> (days 1\u20137 from status epilepticus) showed hemispheric cytotoxic oedema in all, extending to the contralateral side for one. T2 hyperintensity in the basal ganglia was disclosed in 70% of patients and in the hippocampus in 60%. After 1\u00a0month (in intermediate and chronic phases), all surviving patients but one showed hemispheric cortical atrophy corresponding to the regions involved during the early stage. Comparing clinical features of patients presenting with \u2018typical\u2019 features, to those with \u2018atypical\u2019 findings, the second group presented psychomotor delay before status epilepticus.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>This series underlines the major value of early <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> for the prompt diagnosis of <jats:styled-content style=\"fixed-case\">HHS<\/jats:styled-content>, and shows that involvement of subcortical structures has been underestimated. Hippocampal involvement is not constant.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.12233","type":"journal-article","created":{"date-parts":[[2013,8,8]],"date-time":"2013-08-08T08:28:56Z","timestamp":1375950536000},"page":"1150-1158","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Hemiconvulsion\u2013hemiplegia syndrome revisited: longitudinal <scp>MRI<\/scp> findings in 10 children"],"prefix":"10.1111","volume":"55","author":[{"given":"Giulia","family":"Barcia","sequence":"first","affiliation":[{"name":"Reference Centre for Rare Epilepsies Department of Paediatric Neurology H\u00f4pital Necker\u2010Enfants malades APHP Paris France"},{"name":"Inserm U663 Paris France"},{"name":"University Paris Descartes CEA Gif sur Yvette France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Desguerre","sequence":"additional","affiliation":[{"name":"Reference Centre for Rare Epilepsies 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