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West syndrome: long-term prognosis and social aspects

      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.

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