West syndrome is a specific, age dependent electroclinical syndrome and one of the most common and well described form of epileptic encephalopathy. In this study, we describe outcome regarding epilepsy and adaptive behavior in patients with WS treated in our clinic.
Patients with WS treated in our clinic from 2000 -2018 were followed up regularly for at least 2 years. For this study, their adaptive behavior was measured using Vineland-II Adaptive Behavior Scales. Adaptive behavior (AB) refers to the skills needed by individuals to function and be self-sufficient within their everyday environments. Vineland-II asses adaptive behavior in four domains: communication, daily living skills, socialization and motor skills, each divided in more subdomains. It provides standardized scores in every domain and is applicable from birth throughout life.
A total of 64 patients were enrolled in this study, 38 males (59%) and 26 females (41%). Symptomatic WS had 46 patient (72%) and 18 patients (28%) had cryptogenic WS. One patient with symptomatic WS died during follow up.
Regardless of response to initial treatment and resolution of infantile spasm, 20 patients (31%) developed different kind of seizures during follow up and are still taking antiepileptic therapy, 17 of symptomatic (37%) and 3 of cryptogenic WS (16%).
Among all patients, 4 (6%) have no deficit in adaptive behavior in all measured domains and subdomains while 40% of patients have severe or profound deficit. Significant differences were found between symptomatic and cryptogenic form of WS. In cryptogenic WS, most of the patients have moderately low AB, 66% of them, 17% have AB and 17% have low AB. In symptomatic WS moderately low AB were measured in 37% of patients, low AB in 62% and only 4% had adequate AB. Low AB was also in negative correlation with seizure freedom.
Analyzing each domain and subdomain of adaptive behavior, the lowest scores in almost every patient (patients with adequate AB were excluded) were measured in communication domain, more specific, in expressive speech and in subdomain of community daily living skills.
In this study we confirmed that the most important prognostic factor for outcome of epilepsy and psychomotor development in WS is underlying etiology of the syndrome. Despite the etiology, the great majority (94%) of the patients with WS need some kind of help in everyday functioning throughout lifetime.
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