Abstracts

Rationale: Purpose of this study was to determine predictive value of etiology in symptomatic West syndrome. Methods: Study comprised 69 patients with symptomatic West syndrome (WS) with average age 6 3/12 ± 5 2/12 years. All patients underwent the same diagnostic protocol at the onset of spasms, after 1, 3 and 12 months. Results: In great majority of patients (63.8%) WS was a result of pre, peri or postnatal asphyxia. Prematurity was the cause of the WS in 21.7%, tuberous sclerosis (TS) in 14.5%, cortical development malformations (CDM) and hypoxic-ischemic encephalopathy in 11.6%, while other causes (postvaccinal, chromosomopathy, intracerebral hemorrhage, postinfectional and as a result of infection during pregnancy) were quite rare. One risk factor for development of WS was present in 66.7%, two risk factors in 23.2% and three and more risk factors in 10.1%. One month after beginning of treatment similar number of patients was with and without seizures, regardless the etiology. At that time half of the patients had partial response to therapy, which was the best in the group of patients with TS, while it was the worst in patients with CDM. Three months after beginning of therapy similar number of patients was with and without seizures, but in different etiologies there were some differences: most of the patients with asphyxia (53.8%) was seizure free, while most of the patients with CDM (87.5%) was not. At that time neurological examination, psychomotor development, EEG findings and response to therapy were unchanged or improved in the majority of patients, mainly in the group of patients with asphyxia and TS, while they were worse or unchanged in most of the patients with CDM. Most of the patients with asphyxia and TS (79.2% and 66.7% respectively) were seizure free after 12 months of therapy, while that was the case with only 37.5% of patients with CDM. Conclusions: In most patients with symptomatic WS adequate therapy results in improvement of neurological finding and psychomotor development. The best outcome can be expected in patients with asphyxia and TS, while CDM usually have the worst outcome.