Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: Insights from a retrospective case series

Abstract Introduction Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years. Objective To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. Method A retrospective des...

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Veröffentlicht in:European journal of paediatric neurology 2012-03, Vol.16 (2), p.142-148
Hauptverfasser: van Toorn, Ronald, Janse van Rensburg, Pieter, Solomons, Regan, Ndondo, Alvin P, Schoeman, Johan F
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container_end_page 148
container_issue 2
container_start_page 142
container_title European journal of paediatric neurology
container_volume 16
creator van Toorn, Ronald
Janse van Rensburg, Pieter
Solomons, Regan
Ndondo, Alvin P
Schoeman, Johan F
description Abstract Introduction Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years. Objective To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. Method A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period. Results The median age of onset of convulsive status was 16 months (range: 9–36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved. Conclusion In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.
doi_str_mv 10.1016/j.ejpn.2011.06.009
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Objective To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. Method A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period. Results The median age of onset of convulsive status was 16 months (range: 9–36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved. Conclusion In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.</description><identifier>ISSN: 1090-3798</identifier><identifier>EISSN: 1532-2130</identifier><identifier>DOI: 10.1016/j.ejpn.2011.06.009</identifier><identifier>PMID: 21788146</identifier><language>eng</language><publisher>England: Elsevier Ltd</publisher><subject>Atrophy ; Brain - pathology ; Cerebral hemiatrophy ; Child Development ; Child, Preschool ; Cognition - physiology ; Developing Countries ; Diagnosis, Differential ; Epilepsy - diagnostic imaging ; Epilepsy - physiopathology ; Epilepsy - therapy ; Female ; Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) ; Hemiplegia - diagnostic imaging ; Hemiplegia - physiopathology ; Hemiplegia - therapy ; Humans ; Infant ; Intellectual Disability - etiology ; Intellectual Disability - psychology ; Intelligence Tests ; Magnetic Resonance Imaging ; Male ; Movement - physiology ; Neurology ; Partial status epilepticus ; Pediatrics ; Retrospective Studies ; Seizures - diagnostic imaging ; Seizures - physiopathology ; Seizures - therapy ; Seizures, Febrile - complications ; South Africa ; Syndrome ; Tomography, X-Ray Computed ; Treatment Outcome</subject><ispartof>European journal of paediatric neurology, 2012-03, Vol.16 (2), p.142-148</ispartof><rights>European Paediatric Neurology Society</rights><rights>2011 European Paediatric Neurology Society</rights><rights>Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c442t-932310c0339be8e5285dce556df2ded07f5d9b005505a5529e2b4ee4d34f17e53</citedby><cites>FETCH-LOGICAL-c442t-932310c0339be8e5285dce556df2ded07f5d9b005505a5529e2b4ee4d34f17e53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S1090379811001322$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,65309</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21788146$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>van Toorn, Ronald</creatorcontrib><creatorcontrib>Janse van Rensburg, Pieter</creatorcontrib><creatorcontrib>Solomons, Regan</creatorcontrib><creatorcontrib>Ndondo, Alvin P</creatorcontrib><creatorcontrib>Schoeman, Johan F</creatorcontrib><title>Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: Insights from a retrospective case series</title><title>European journal of paediatric neurology</title><addtitle>Eur J Paediatr Neurol</addtitle><description>Abstract Introduction Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years. Objective To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. Method A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period. Results The median age of onset of convulsive status was 16 months (range: 9–36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved. Conclusion In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.</description><subject>Atrophy</subject><subject>Brain - pathology</subject><subject>Cerebral hemiatrophy</subject><subject>Child Development</subject><subject>Child, Preschool</subject><subject>Cognition - physiology</subject><subject>Developing Countries</subject><subject>Diagnosis, Differential</subject><subject>Epilepsy - diagnostic imaging</subject><subject>Epilepsy - physiopathology</subject><subject>Epilepsy - therapy</subject><subject>Female</subject><subject>Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE)</subject><subject>Hemiplegia - diagnostic imaging</subject><subject>Hemiplegia - physiopathology</subject><subject>Hemiplegia - therapy</subject><subject>Humans</subject><subject>Infant</subject><subject>Intellectual Disability - etiology</subject><subject>Intellectual Disability - psychology</subject><subject>Intelligence Tests</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Movement - physiology</subject><subject>Neurology</subject><subject>Partial status epilepticus</subject><subject>Pediatrics</subject><subject>Retrospective Studies</subject><subject>Seizures - diagnostic imaging</subject><subject>Seizures - physiopathology</subject><subject>Seizures - therapy</subject><subject>Seizures, Febrile - complications</subject><subject>South Africa</subject><subject>Syndrome</subject><subject>Tomography, X-Ray Computed</subject><subject>Treatment Outcome</subject><issn>1090-3798</issn><issn>1532-2130</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkk9v1DAQxSMEon_gC3BAvnFKOrbjbIwQUlVBW6kSh8LZytqTrkPiBE-y0n57HG3h0EN78lj6vSfNe5NlHzgUHHh10RXYTaEQwHkBVQGgX2WnXEmRCy7hdZpBQy43uj7Jzog6SEQpqrfZieCbuuZldZpNNzh4O4b90pMfQ75L36nHB9_kOPkeJzowOgQXxwGZD-x-XOYdu2yjt01gdud7FzF8ZreB_MNuJtYmkjUs4hxHmtDOfo_MNoSMMHqkd9mbtukJ3z--59mv799-Xt3kdz-ub68u73JblmLOtRSSgwUp9RZrVKJWzqJSlWuFQwebVjm9BVAKVKOU0Ci2JWLpZNnyDSp5nn06-k5x_LMgzWbwZLHvm4DjQkZXNa-0UPxlUggFXIsykeJI2rQbRWzNFP3QxIPhYNZKTGfWSsxaiYHKpMCT6OOj_bId0P2X_OsgAV-OAKY49h6jIesxWHQ-pvyMG_3z_l-fyG3vQ6qn_40HpG5cYkhBG25IGDD361GsN8E5AJdCyL-r2rNV</recordid><startdate>20120301</startdate><enddate>20120301</enddate><creator>van Toorn, Ronald</creator><creator>Janse van Rensburg, Pieter</creator><creator>Solomons, Regan</creator><creator>Ndondo, Alvin P</creator><creator>Schoeman, Johan F</creator><general>Elsevier Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7TK</scope></search><sort><creationdate>20120301</creationdate><title>Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: Insights from a retrospective case series</title><author>van Toorn, Ronald ; 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Objective To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. Method A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period. Results The median age of onset of convulsive status was 16 months (range: 9–36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved. Conclusion In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.</abstract><cop>England</cop><pub>Elsevier Ltd</pub><pmid>21788146</pmid><doi>10.1016/j.ejpn.2011.06.009</doi><tpages>7</tpages></addata></record>
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subjects Atrophy
Brain - pathology
Cerebral hemiatrophy
Child Development
Child, Preschool
Cognition - physiology
Developing Countries
Diagnosis, Differential
Epilepsy - diagnostic imaging
Epilepsy - physiopathology
Epilepsy - therapy
Female
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE)
Hemiplegia - diagnostic imaging
Hemiplegia - physiopathology
Hemiplegia - therapy
Humans
Infant
Intellectual Disability - etiology
Intellectual Disability - psychology
Intelligence Tests
Magnetic Resonance Imaging
Male
Movement - physiology
Neurology
Partial status epilepticus
Pediatrics
Retrospective Studies
Seizures - diagnostic imaging
Seizures - physiopathology
Seizures - therapy
Seizures, Febrile - complications
South Africa
Syndrome
Tomography, X-Ray Computed
Treatment Outcome
title Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: Insights from a retrospective case series
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