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Article: West syndrome - The University of Hong Kong experience (1970-2000)

TitleWest syndrome - The University of Hong Kong experience (1970-2000)
Authors
KeywordsAnnual incidence
Children
Epilepsy
Infantile spasm
Prognosis
Risk factors
Seizure
Vigabatrin
West syndrome
Issue Date2001
PublisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/braindev
Citation
Brain And Development, 2001, v. 23 n. 7, p. 609-615 How to Cite?
AbstractObjective: To study the clinical pattern of West syndrome (WS) in a university based hospital.Methodology: The database of children seen in the Epilepsy Clinic of Queen Mary Hospital and Duchess of Kent Children's Hospital during a 30-year period (1970-2000) was reviewed.Results: A total of 105 cases had WS (1.9%). The number of new cases of WS admitted per year ranged from one to eight. The range of annual incidence of WS to newly diagnosed epilepsy was 0.8-4.8%. The etiology included idiopathic (N=19, 18%), cryptogenic (N=23; 22%), symptomatic (N=56; 53.3%) and unknown (N=7; 5.7%). Adrenocorticotropic hormone (ACTH) and/or prednisone were given to 42 children (40%). Most were effective in controlling WS on an all-or-none fashion. Seizure outcome included 12 with remission, persistent in the same form in two and persisting but changed to another form in the rest. Neurological outcome at the last follow up in 2000 December included multiple disabilities (N=16), cerebral palsy (N=22), mental retardation (N=94) and Lennox-Gastaut syndrome (N=13). We analysed the following risk factors in relation to poor outcome: age of onset, age of presentation, time lag before treatment, etiology, family history of epilepsy and hormonal treatment. Only etiology of WS has a positive correlation with poor outcome (P<0.0005).Conclusions: WS is an uncommon epileptic syndrome. The majority had poor outcome, especially those with causes identified. Infantile spasm is a specific epileptic phenomenon in a maturational stage of a child when heterogeneous disorders can present with the same clinical epileptic and electroencephalographic phenomenon. Copyright © 2001 Elsevier Science B.V.
Persistent Identifierhttp://hdl.handle.net/10722/143568
ISSN
2015 Impact Factor: 1.785
2015 SCImago Journal Rankings: 0.840
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorWong, Ven_HK
dc.date.accessioned2011-12-12T03:51:59Z-
dc.date.available2011-12-12T03:51:59Z-
dc.date.issued2001en_HK
dc.identifier.citationBrain And Development, 2001, v. 23 n. 7, p. 609-615en_HK
dc.identifier.issn0387-7604en_HK
dc.identifier.urihttp://hdl.handle.net/10722/143568-
dc.description.abstractObjective: To study the clinical pattern of West syndrome (WS) in a university based hospital.Methodology: The database of children seen in the Epilepsy Clinic of Queen Mary Hospital and Duchess of Kent Children's Hospital during a 30-year period (1970-2000) was reviewed.Results: A total of 105 cases had WS (1.9%). The number of new cases of WS admitted per year ranged from one to eight. The range of annual incidence of WS to newly diagnosed epilepsy was 0.8-4.8%. The etiology included idiopathic (N=19, 18%), cryptogenic (N=23; 22%), symptomatic (N=56; 53.3%) and unknown (N=7; 5.7%). Adrenocorticotropic hormone (ACTH) and/or prednisone were given to 42 children (40%). Most were effective in controlling WS on an all-or-none fashion. Seizure outcome included 12 with remission, persistent in the same form in two and persisting but changed to another form in the rest. Neurological outcome at the last follow up in 2000 December included multiple disabilities (N=16), cerebral palsy (N=22), mental retardation (N=94) and Lennox-Gastaut syndrome (N=13). We analysed the following risk factors in relation to poor outcome: age of onset, age of presentation, time lag before treatment, etiology, family history of epilepsy and hormonal treatment. Only etiology of WS has a positive correlation with poor outcome (P<0.0005).Conclusions: WS is an uncommon epileptic syndrome. The majority had poor outcome, especially those with causes identified. Infantile spasm is a specific epileptic phenomenon in a maturational stage of a child when heterogeneous disorders can present with the same clinical epileptic and electroencephalographic phenomenon. Copyright © 2001 Elsevier Science B.V.en_HK
dc.languageengen_US
dc.publisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/braindeven_HK
dc.relation.ispartofBrain and Developmenten_HK
dc.subjectAnnual incidenceen_HK
dc.subjectChildrenen_HK
dc.subjectEpilepsyen_HK
dc.subjectInfantile spasmen_HK
dc.subjectPrognosisen_HK
dc.subjectRisk factorsen_HK
dc.subjectSeizureen_HK
dc.subjectVigabatrinen_HK
dc.subjectWest syndromeen_HK
dc.subject.meshAnticonvulsants/therapeutic useen_US
dc.subject.meshChild, Hospitalized/*statistics & numerical dataen_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshHong Kong/epidemiologyen_US
dc.subject.meshHumansen_US
dc.subject.meshIncidenceen_US
dc.subject.meshInfanten_US
dc.subject.meshInfant, Newbornen_US
dc.subject.meshMaleen_US
dc.subject.meshPrognosisen_US
dc.subject.meshRisk Factorsen_US
dc.subject.meshSpasms, Infantile/diagnosis/drug therapy/*epidemiologyen_US
dc.subject.meshTreatment Outcomeen_US
dc.subject.meshVigabatrin/therapeutic useen_US
dc.titleWest syndrome - The University of Hong Kong experience (1970-2000)en_HK
dc.typeArticleen_HK
dc.identifier.emailWong, V:vcnwong@hku.hken_HK
dc.identifier.authorityWong, V=rp00334en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1016/S0387-7604(01)00296-0en_HK
dc.identifier.pmid11701265en_HK
dc.identifier.scopuseid_2-s2.0-0034778305en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0034778305&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume23en_HK
dc.identifier.issue7en_HK
dc.identifier.spage609en_HK
dc.identifier.epage615en_HK
dc.identifier.isiWOS:000172638800031-
dc.publisher.placeNetherlandsen_HK
dc.identifier.scopusauthoridWong, V=7202525632en_HK

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