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Hemi-convulsion-hemiplegia syndrome revisited: longitudinal MRI findings in 10 children

Abstract

Hemi-convulsion-hemiplegia syndrome (HHS) is a rare severe epilepsy of infancy consisting of unilateral convulsive status epilepticus immediately followed by transient or lasting ipsilateral hemiplegia. HHS may occur either in patients with previous brain pathology or without any identified cause, so-called 'idiopathic HHS'. We retrospectively analysed clinical and MRI longitudinal findings of a series of 10 patients (six females, four males) presenting with HHS. Age at the study inclusion ranged from 2 years 6 months to 15 years (mean of 5 y 10 mo, median 4 y 2 mo). After defining magnetic resonance imaging (MRI) features as 'typical', i.e. strictly unilateral involvement, and 'atypical', i.e. bilateral, we compared clinical data from both groups. Cognitive level was assessed using Brunet-Lezine or Wechsler scales. HHS occurred at a mean age of 20.5 months (range 8-48 mo). In all cases, status epilepticus lasted for more than 1 hour 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-organ failure 2 weeks after the status epilepticus and the other from a second episode of ipsilateral intractable febrile status epilepticus 3 years after the first episode. Early MRI (days 1-7 from  More>>
Authors:
Barcia,; Giulia,; Chemaly, Nicole; Dulac, Olivier; Nabbout, Rima; [1]  Inserm U663, Paris; [2]  University Paris Descartes, CEA, Gif sur Yvette (France)]; Desguerre, Isabelle; Carmona, Orietta; Barnerias, Christine; Gitiaux, Cyril; [1]  Brunelle, Francis; Boddaert, Nathalie [3] 
  1. Reference Centre for Rare Epilepsies, Department of Paediatric Neurology, Hopital Necker-Enfants malades, APHP, Paris (France)
  2. France
  3. Paediatric Radiology Department, Hopital Necker-Enfants malades, APHP, Paris, (France)
Publication Date:
Jul 01, 2013
Product Type:
Journal Article
Resource Relation:
Journal Name: Developmental Medicine and Child Neurology; Journal Volume: 55; Other Information: 26 refs.; Country of input: France
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; EPILEPSY; GANGLIONS; HIPPOCAMPUS; INFANTS; NMR IMAGING
OSTI ID:
22448836
Country of Origin:
United Kingdom
Language:
English
Other Identifying Numbers:
Journal ID: ISSN 0012-1622; TRN: FR16J0504018378
Availability:
Available from doi: http://dx.doi.org/10.1111/dmcn.12233
Submitting Site:
INIS
Size:
page(s) 1150-1158
Announcement Date:
Mar 22, 2016

Citation Formats

Barcia,, Giulia,, Chemaly, Nicole, Dulac, Olivier, Nabbout, Rima, Inserm U663, Paris, University Paris Descartes, CEA, Gif sur Yvette (France)], Desguerre, Isabelle, Carmona, Orietta, Barnerias, Christine, Gitiaux, Cyril, Brunelle, Francis, and Boddaert, Nathalie. Hemi-convulsion-hemiplegia syndrome revisited: longitudinal MRI findings in 10 children. United Kingdom: N. p., 2013. Web. doi:10.1111/DMCN.12233.
Barcia,, Giulia,, Chemaly, Nicole, Dulac, Olivier, Nabbout, Rima, Inserm U663, Paris, University Paris Descartes, CEA, Gif sur Yvette (France)], Desguerre, Isabelle, Carmona, Orietta, Barnerias, Christine, Gitiaux, Cyril, Brunelle, Francis, & Boddaert, Nathalie. Hemi-convulsion-hemiplegia syndrome revisited: longitudinal MRI findings in 10 children. United Kingdom. doi:10.1111/DMCN.12233.
Barcia,, Giulia,, Chemaly, Nicole, Dulac, Olivier, Nabbout, Rima, Inserm U663, Paris, University Paris Descartes, CEA, Gif sur Yvette (France)], Desguerre, Isabelle, Carmona, Orietta, Barnerias, Christine, Gitiaux, Cyril, Brunelle, Francis, and Boddaert, Nathalie. 2013. "Hemi-convulsion-hemiplegia syndrome revisited: longitudinal MRI findings in 10 children." United Kingdom. doi:10.1111/DMCN.12233. https://www.osti.gov/servlets/purl/10.1111/DMCN.12233.
@misc{etde_22448836,
title = {Hemi-convulsion-hemiplegia syndrome revisited: longitudinal MRI findings in 10 children}
author = {Barcia,, Giulia,, Chemaly, Nicole, Dulac, Olivier, Nabbout, Rima, Inserm U663, Paris, University Paris Descartes, CEA, Gif sur Yvette (France)], Desguerre, Isabelle, Carmona, Orietta, Barnerias, Christine, Gitiaux, Cyril, Brunelle, Francis, and Boddaert, Nathalie}
abstractNote = {Hemi-convulsion-hemiplegia syndrome (HHS) is a rare severe epilepsy of infancy consisting of unilateral convulsive status epilepticus immediately followed by transient or lasting ipsilateral hemiplegia. HHS may occur either in patients with previous brain pathology or without any identified cause, so-called 'idiopathic HHS'. We retrospectively analysed clinical and MRI longitudinal findings of a series of 10 patients (six females, four males) presenting with HHS. Age at the study inclusion ranged from 2 years 6 months to 15 years (mean of 5 y 10 mo, median 4 y 2 mo). After defining magnetic resonance imaging (MRI) features as 'typical', i.e. strictly unilateral involvement, and 'atypical', i.e. bilateral, we compared clinical data from both groups. Cognitive level was assessed using Brunet-Lezine or Wechsler scales. HHS occurred at a mean age of 20.5 months (range 8-48 mo). In all cases, status epilepticus lasted for more than 1 hour 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-organ failure 2 weeks after the status epilepticus and the other from a second episode of ipsilateral intractable febrile status epilepticus 3 years after the first episode. Early MRI (days 1-7 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 month (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 'typical' features, to those with 'atypical' findings, the second group presented psychomotor delay before status epilepticus. This series underlines the major value of early MRI for the prompt diagnosis of HHS, and shows that involvement of subcortical structures has been underestimated. Hippocampal involvement is not constant. (authors)}
doi = {10.1111/DMCN.12233}
journal = {Developmental Medicine and Child Neurology}
volume = {55}
journal type = {AC}
place = {United Kingdom}
year = {2013}
month = {Jul}
}