Etiology and Prognosis in West Syndrome.

概要

論文の詳細を見る
The clinical and EEG features of 187 cases with West syndrome (WS) were investigated after classifying the cases according to the etiology. The impact of its etiology and underlying diseases on the mental and seizure prognosis was also studied on 128 cases of the total, with follow up of more than three years. In the idiopathic group, the seizure and mental prognosis were significantly more favorable, whereas significantly more pyridoxal phosphate effective cases were seen than in the symptomatic group. The cases with disorders of organogenesis type of the brain malformations subgroup in the prenatal group showed significantly more cases having early seizure onset and/or preceding seizures than those in the other types within brain malformations subgroup. Cases with evolution from Ohtahara syndrome or to Lennox-Gastaut syndrome were also often seen in this type, and the seizure and mental prognosis were extremely poor. The postnatal group had significantly more cases with late seizure onset and/or asymmetric hypsarrhythmia, and the mental prognosis tended to be favorable than the other symptomatic cases. The perinatal group showed no significant characteristics. These findings suggest that the strict classification of WS according to its etiology is beneficial in elucidating its pathophysiology and prognostic evaluation.
一般社団法人日本てんかん学会の論文