Retrospective Evaluation of Patients with Infantile Spasm Diagnosis

Abstract

Introduction: Infantile spasm (West Syndrome) (IS) is an age-dependent epileptic encephalopathy clinic specific to the first two years of life. Infantile spasm is the most known epileptic syndrome of infancy with typical seizures in the form of spasm, psychomotor retardation and hypsarrhythmia on electroencephalography. Typical spasms are in the form of flexor/extensor contractions that last for about 2-5 seconds, which usually symmetrically involves all muscle groups in the body. Material and Method: In this study, the data of patients, who were followed up with the diagnosis of "infantile spasm" in the Pediatric Neurology Clinic of Mersin City Training and Research Hospital between 2020 and 2022, were retrospectively analyzed. Demographic characteristics, clinical, EEG and MRI findings of the patients were analyzed. Results: Eighteen patients were included in the study. Ten (55.5%) of our patients were male and 8 (44.5%) were female. The mean age of patients was ……. years. 11 (61.1%) patients were in the symptomatic group, 5 (27.7%) patients were in the cryptogenic group, and 2 (11.1%) patients were in the idiopathic group. At the time of diagnosis, there was flexor spasm, extensor spasm, mixt spasm, tonic seizure, myoclonic seizure in 5 (27,7%), 4 (22,2%), 4 (22,2%), 3 (16,6%), and 2 (11,1%) patients, respectively. Hypsarrhythmia, suppression-burst pattern, focal epileptic abnormality and generalized epileptic abnormality was observed in 8 (44,4%), 4 (22,2%), 3 (16,6%), and 3 (16,6%) patients, respectively. Of the 14 patients who were first diagnosed, 8 (57.1%) were treated with ACTH, 4 (28.6%) with phenobarbital, 2 (14.3%) with vigabatrin. Conclusion: Infantile spasm is an important age-related encephalopathy with severe neurological sequelae, resistant seizures, and high morbidity and mortality all over the world and in our country. Spasms cause further damage to the central nervous system, which continues to develop. For this reason, a positive contribution can be made in terms of prognosis with early and appropriate treatment.