Abstract
West syndrome (WS) is commonly associated with a poor long-term outcome including
a small but significant mortality, infantile spasms that are resistant to treatment,
the development of other seizure types and impaired cognitive and psychosocial functioning.
It is important to understand that the families of these children also experience
significant psychosocial morbidity, which is usually, but not invariably, correlated
with persisting seizures beyond the first or second year of life. One of the fundamental
points about the prognosis of this epilepsy syndrome is that the natural history (i.e.
the outcome of spasms without any medical or surgical intervention) is not known.
Numerous factors have been implicated as being important in influencing the long-term
prognosis of children with WS. However, the majority of these factors have been identified
from retrospective and markedly heterogeneous studies, including different populations
and different treatment regimes. The most important prognostic factors are generally
recognised to be the underlying aetiology of the syndrome and the presence or absence
of pre-existing seizures and/or developmental abnormalities. The rapidity with which
the diagnosis is made and treatment started from the onset of spasms (often termed
the ‘treatment lag’) is a possible, though controversial and as yet unproven, factor
in the prognosis of WS.
Keywords
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Article info
Publication history
Accepted:
June 16,
2001
Received in revised form:
June 13,
2001
Received:
May 17,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
