Clinical evaluation of patients with infantile spasms

Clinical Evaluation of Patients with Infantile Spasms Aim: Infantile spasms is an age-dependent epileptic encephalopathy specific to the first two years of life. Pathophysiology is still not fully understood. There is no standard treatment scheme accepted by everyone. In this study, we aimed to evaluate the demographic, clinical features, EEG and neuroimaging findings, etiology, treatments, treatment response, follow-up neuromotor development and risk factors of patients with infantile spasms. Material-Method: The study group included 94 cases of infantile spasms who are diagnosed and treated at Inonu University Medical Faculty, Department of Pediatrics, Division of Child Neurology between 2009-2017. Patients' data were analyzed by retrospective file scanning. Results: 94 cases (53 boys and 41 girls) were included in this study. In 91.4% of patients, spasms were found to be under one year of age. The average diagnosis age is 7.01±4.8 months. In the first visit, abnormal neurological examination findings were found in 80.9% of the patients and microcephaly was found in 37.2% of the patients. The most frequent type of seizure was spasms (86.2%). The most frequent type of spasm was flexor spasm. The most frequent EEG findings were hypsarrhythmia (40.4%). Abnormal cranial imaging findings were noted in 69.1% of the cases. 58.5% of the patients were classified as symptomatic, 24.5% as cryptogenic, 17% as idiopathic etiology. The most frequent causes of symptomatic etiology were hypoxic ischemic encephalopathy (25.5%) and cerebral developmental disorders (17%). The success rate of treatment with vigabatrin and ACTH in the initial treatment (83.3%) was significantly higher than the other drugs. There was no correlation between drug used in initial therapy and relapse and seizure prognosis. The response rate (84.6%) was higher in patients with ACTH in follow-up treatment than in the other groups. After the average 31,4±25,6 months follow-up period, 6.4% of the patients did not take any medication and were seizure-free, 21.3% were seizure-free with monotherapy, 50% had drug-resistant epilepsy, 94.7% had mental/developmental retardation. Symptomatic and cryptogenic etiology was found to be associated with poor prognosis in terms of seizure prognosis and neuromotor development. Conclusion: Infantile spasms is an epileptic encephalopathy with serious neurological sequelae, resistant seizures, high morbidity and mortality rate. Etiology is the most important determinant of prognosis. Hypoxic episode is the most important preventable perinatal factor associated with infantile spasms. Early diagnosis, appropriate and adequate therapy might provide a contribution for favourable prognosis in infantile spasms. Key words: Infantile spasms, risk factors, etiology, ACTH, vigabatrin, prognosis