Dados do Trabalho

Título

INFANTILE EPILEPTIC SPASMS SYNDROME (IESS): TREATMENT EFFICACY IN PATIENTS TREATED AT FMABC UNIVERSITY CENTER

Introdução

The term Infantile Epileptic Spasms Syndrome (IESS) is a more recent terminology that encompasses the condition previously known as West Syndrome (WS), reflecting a broader understanding of the clinical manifestations associated with epileptic spasms in childhood. The treatment of IESS is challenging, as infantile spasms often do not respond to standard anticonvulsant medications, and relapses are common.
In this regard, this study aims to compare the response rate to different treatments available for patients with IESS treated at FMABC University Center.

Objetivo

To compare the response rate of various pharmacological treatments in patients with IESS and to identify related variables.

Método

The records of 40 patients treated at the pediatric epilepsy clinic at the ABC School of Medicine were analyzed.

Resultados

A total of 40 patients were included, ranging in age from 5 months to 8 years, with 52% being boys (21 patients). The majority (65%) began experiencing epileptic spasms between 4 and 7 months of age (26 patients), 35% (14 patients) had a defined genetic etiology, 32% (13 patients) had an unknown etiology, and the remainder had a structural etiology.
Regarding treatment, 32% of patients underwent combined therapy with vigabatrin and prednisone, 58% started with vigabatrin alone (26 patients), and 10% with ACTH. As a second medication, 4 of the patients who used vigabatrin alone added ACTH for complete resolution of the condition.
Of the patients evaluated, 47% (19 patients) developed other forms of epilepsy, requiring additional anticonvulsant medications and adjunctive therapy, such as cannabidiol (15 patients), ketogenic diet (4 patients), and/or vagus nerve stimulation (VNS) (3 patients).

Conclusão

The results of this study indicate that the therapeutic approach to IESS requires careful personalization, considering the variability in response to available treatments. Initial combined therapy with vigabatrin and prednisone showed greater efficacy in ceasing spasms. However, the persistence of subsequent forms of epilepsy in nearly half of the cases underscores the need for continuous follow-up and the possible adoption of adjunctive therapies.
The diversity of etiologies and varying responses to treatment highlight the importance of standardized but flexible protocols to optimize therapeutic outcomes and minimize relapse.

Referências

TORRES, Célia Maria; GUERREIRO, Carlos Alberto Masseto; CYSNE, Tatiana de Lima; et al. Brazilian experts’ consensus on the treatment of infantile epileptic spasm syndrome in infants. Arquivos de Neuro-Psiquiatria, São Paulo, v. 79, n. 10, p. 864-875, 2021.

Palavras Chave

Infantile Epileptic Spasms Syndrome; Treatment; epilepsy