West Syndrome among Down Syndrome Patients - Abstract
Down syndrome is the most common chromosome disorder. One constant concern is the care to improve development and quality of life that may be affected by the onset of West syndrome. The aim of this retrospective study is analyze the prevalence and evolution of West syndrome in patients seen at a Down syndrome outpatient clinic. Data was gathered from the medical files of 1,212 patients with Down syndrome, among whom 22 (1.9%) had been diagnosed with West syndrome. The data was analyzed using StatisticaR software. Among the 22 patients with West syndrome, 11 (50%) were male. The onset of spasms occurred at a median of 7 months (3-57). Patients received vigabatrin as first treatment with total control of spasms in 81.8%. Five patients (22.7%) developed epilepsy. A higher frequency of autistic spectrum disorder (29.4%
versus 5.9%, p < 0.001) and seizures (17.6% versus 2.8%, p < 0.001) was observed. Pediatricians should promptly request an electroencephalogram when faced with a child with spasms. If hypsyarrhythmia is detected, it is West syndrome, improving the prognosis when early treated. Development is recovered after seizure control with the specific treatment (vigabatrin or ACTH) instituted early