Background. West syndrome is characterized by a hipsarrhytmia pattern on electroencephalography, spasm type seizures, and psychomotor regression triad. In this study, we aimed to document demographic characteristics, clinical and laboratory findings, treatment responses, neurodevelopmental outcomes, and risk factors developed during long-term follow-up with the diagnosis of West syndrome. Materials and methods. The study included patients who were diagnosed with West syndrome between July 2011 and December 2012 in the Department of Pediatric Neurology of Atatürk University. The following data were collected from each patient’s history. Systemic and neurological examinations, cerebral imaging, and electroencephalography were reviewed. Biochemical tests were performed from laboratory tests. The development of each child was assessed using the Denver 2 developmental screening test, the Ankara Developmental Screening Inventory test. Results. The ratio of male/female of our patients was found 2,28/1 and the mean age of the referral was 8.62 ± 7.20 months (median: 8.0). 59 (85.51 %) of the patients were in the symptomatic group and 10 (14.49 %) were in the idiopathic group. The most common factors in the symptomatic group were anomalies of the congenital central nervous system (45.7 %) and hypoxic-ischemic encephalopathy (28.8 %). There was a significant difference between the idiopathic and symptomatic groups in terms of relapse rates, radiological findings, and prognosis rates (p: 0.035/p < 0.001/p < 0.001). Relapse was detected in 43,5 % of the patients. While 83 % of patients treated with adrenocorticotropic hormone, treatment was responded to, 17 % had resistant seizures. There was no statistically significant difference between the treatment responses with adrenocorticotropic hormone and other treatments (p = 0.093). 46 of 55 children (83.6 %) were found to be a developmental delay. Conclusions. Early diagnosis and treatment, the provision of appropriate and convenient treatment in the West syndrome can make a positive contribution to prognosis according to etiology.
Epilepsy and Neurodevelopmental Outcomes in a Cohort of West Syndrome Beyond Two Years of Age
