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 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.
Keywords
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Article info
Publication history
Accepted:
August 2,
2001
Received in revised form:
July 30,
2001
Received:
April 21,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
