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Titolo:
West syndrome - The University of Hong Kong experience (1970-2000)
Autore:
Wong, V;
Indirizzi:
Univ Hong Kong, Queen Mary Hosp, Dept Paediat, Pokfulam, Hong Kong, Peoples R China Univ Hong Kong Pokfulam Hong Kong Peoples R China Kong, Peoples R China
Titolo Testata:
BRAIN & DEVELOPMENT
fascicolo: 7, volume: 23, anno: 2001,
pagine: 609 - 615
SICI:
0387-7604(200111)23:7<609:WS-TUO>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
TERM FOLLOW-UP; INFANTILE SPASMS; TUBEROUS SCLEROSIS; PROGNOSTIC FACTORS; NATURAL-HISTORY; ACTH THERAPY; DOSE ACTH; VIGABATRIN; ETIOLOGY;
Keywords:
infantile spasm; West syndrome; children; seizure; epilepsy; vigabatrin; risk factors; prognosis; annual incidence;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
37
Recensione:
Indirizzi per estratti:
Indirizzo: Wong, V Univ Hong Kong, Queen Mary Hosp, Dept Paediat, Pokfulam, Hong Kong, Peoples R China Univ Hong Kong Pokfulam Hong Kong Peoples R China eoples R China
Citazione:
V. Wong, "West syndrome - The University of Hong Kong experience (1970-2000)", BRAIN DEVEL, 23(7), 2001, pp. 609-615

Abstract

Objective: 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 QueenMary 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 WSadmitted 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 formin 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. (C) 2001 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/09/20 alle ore 23:30:47