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Comparison of Treatment Modalities in Infantile Spasm Syndrome: Vigabatrin, Hormonotherapy, and Combined Therapies

Objective: A comparative study designed for the effectiveness of treatment modalities in infantile spasm syndrome (ISS). Methods: From the Division of Pediatric Neurology Archives of Ege University Hospital, 280 patients who had been followed with a diagnosis of ISS were included in the cohort between 2010-2020. For comparison of departmental treatment modalities, three ISS therapy groups were designed: (I) pharmacotherapy with vigabatrin (75-100mg/kg/day)), (II) hormonotherapy (ACTH therapy with a six-week protocol; 50IU/day for infants <10 kg, and 100IU/day for infants >10kg) or oral prednisolone therapy (2-4mg/kg/day)) and (III) combined therapy (vigabatrin plus hormonotherapy). The efficacy of treatment modalities was evaluated with spasm cessation time during follow-up, resolution of hypsarrhythmia on EEG, the ISS relapse rate, and evolution to different epileptic syndromes. Results: The etiology specific diagnosed was performed; structural (n:135, 48.2%), metabolic (n:13, 4.6%), genetic (n:24, 8.6%), immune-infectious (n:10, 3.6%), and unknown (n:98, 35%). Among the 280 patients, 129 (46.1%) of them received pharmacotherapy, 73 (26.1%) hormonotherapy, and 78 (27.9%) combined therapy. The spasm cessation time was significantly delayed in the immune-infectious group (p=0.041). The evolution to a different epileptic syndrome was the most frequent (p=0.017) in the genetic group. However, in terms of electrophysiological and clinical remission, relapse rate, and evolution to different epileptic syndromes, there was no significant difference between ISS therapy groups (Table 1). Conclusions: This retrospective cohort study revealed that there is no significant efficacy difference between the treatment modalities (vigabatrin, hormonotherapy, and combination therapy).
Keywords: infantile spasm syndrome, ACTH, vigabatrin