Treatment and long term outcome in West syndrome: The clinical reality. A multicentre follow up study

Abstract

We systematically reviewed the files of 51 infants presenting with infantile spasms and hypsarrhythmia in order to study the initial treatment strategies and the long term outcome. 80% of the infants were classified as symptomatic. In the nine participating centres, different treatment protocols were used, but the large majority of the children received vigabatrin as first line treatment. Second line options included hormonal treatment, topiramate and valproate. The time to reach cessation of infantile spasms was significantly shorter in the cryptogenic group than in the symptomatic group (50% at 13 days versus 66 days respectively) and was irrespective of the treatment used. The late follow up data (>2 years) showed that 60% of the children had epilepsy and that 75% of the children had a delay in their psychomotor development. Again, outcome in the cryptogenic group was better than in the symptomatic group, but also in the cryptogenic group, 50% of the children had a clear developmental delay, even if spasms were controlled early in the course of the disease. Our retrospective study illustrates that not only the underlying brain dysfunction is the major determinant for later outcome in infantile spasms (symptomatic group) but also even a short period of infantile spasms can be responsible for later developmental delay (cryptogenic group).

Long-term outcomes in patients with West syndrome: An outpatient clinical study

“…Riikonen [4,5] reported that patients of WS with a favorable outcome had normal neurodevelopment before the onset of the spasms. Nearly all previous studies on this variable reported similar results [3,7,10,[21][22][23][24][25][26]. The presence of parental consanguinity and epilepsy in near relatives showed no significant effect on prognosis in this study.…”

The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

“…This was greater than that in developed countries, which showed a lag time of 25 -45 days, but less than in other developing countries, at 7.9 months. [11,15] There was no correlation between the number of spasms per cluster or clusters per day and final neurological outcome in our study. The number of spasms at presentation was similar to that recorded in other studies.…”

West syndrome: Clinical characteristics and short-term outcome

“…On sekiz aydan sonra nadir görülmekle birlikte, başlangıcı 4 yaş kadar geç olan olgular bildirilmiş-tir [5]. Önceki çalışmalarla benzer şekilde [10][11][12][13], çalışmamızda başlangıç yaşı ortalama 6.23 aydı.…”

“…Belirtilerin başlangıcından tanıya kadar geçen süre gelişmiş ülkelerde 1-1.5 ay arasında bildirilirken [10,12,13], gelişmekte olan ülkelerde 7.9 ay kadar uzun olabilmektedir [11]. Çalışmamızda ise bu süre 1.36 aydı.…”