17º CONGRESSO BRASILEIRO DE NEUROLOGIA INFANTIL

Dados do Trabalho


Título

ACTH VERSUS CORTICOSTEROID IN INFANTILE SPASMS, A LITERATURE REVIEW

Introdução

Infantile spasms (IS) represent an age-specific epileptic disorder of infancy and early childhood. Children with infantile spasms typically exhibit epileptic spasms along with the electroencephalographic (EEG) pattern known as hypsarhythmia. Although rare, 1.6 to 4.5 per 10,000 live births, IS is a significant disorder because of the association with developmental delay or regression, high mortality rate, refractoriness to conventional antiseizure medications, and responsiveness to hormonal therapy. Genetic variants and acquired factors such as hypoxic-ischemic injury, infections, and structural abnormalities of the brain, are some of the insults that are associated with IS.

Objetivo

To summarize the knowledge about corticotropin (ACTH) and corticosteroid in IS available in the literature.

Métodos

We performed a literature review using PUBMED and SCIELO search engines up to August 2022 with the terms (infantile spasms) AND (corticosteroid) OR (ACTH).

Resultados

The ACTH formulation is the classical treatment for IS given intramuscularly or subcutaneously. Several meta-analyses of randomized trials comparing the effectiveness of ACTH with oral glucocorticoids have found no difference between the two forms of hormonal treatment for outcomes including cessation of IS, hypsarhythmia resolution, adverse effects, relapse rate, or subsequent development of epilepsy. Data from the National Infantile Spasms Consortium prospective multicenter cohort study also support corticotropin and oral glucocorticoids as effective first-line treatments. Of note, conclusions have been limited by the overall poor methodology and small size of most of the available clinical trials and studies. Lack of adherence to standardized case definitions and outcome measures is one problem with many studies. Another is that inclusion of a control group is critical, as the natural history of the disease is that clinical spasms subside and EEG patterns evolve without therapy, yet many clinicians would be reluctant not to treat, particularly since observational data sugest that delayed therapy may worsen prognosis. As a result, questions remain regarding the optimal drug, dose, and duration of therapy.

Conclusões

Given the advent of data that have suggested, but not proven, that high-dose prednisolone regimens are as effective as ACTH and given considerable reduction in the cost of treatment and ease of administration with oral glucocorticoids, some centers are now using oral glucocorticoids as initial therapy for IS.

Palavras chave

Seizures; Intellectual Disability; Epilepsy

Referências (se houver)

Danielle S Takacs, MDAkshat Katyayan. Infantile spasms: Etiology and pathogenesis. Available on: https://www.uptodate.com/contents/infantile-spasms-etiology-and-pathogenesis?source=history_widget
Danielle S Takacs, Akshat Katyayan. Infantile spasms: Management and prognosis. Available on: https://www.uptodate.com/contents/infantile-spasms-management-and-prognosis?source=history_widget#H36

Fonte de Fomento (se houver)

Declaração de conflito de interesses de TODOS os autores

There is no conflict of interests.

Área

Epilepsias

Instituições

UPF - Rio Grande do Sul - Brasil

Autores

Saulo Bueno de Azeredo, Eduarda Vogel Wollmeister, Lucas Lizot Pozzobon, Maria Fernanda Guadagnin, Martina Estacia Da Cas, Gabriel Soccol Fassina, Valéria Tessaro Grandi, Nicolle Surkamp, Marcos Vinicius Dalla Lana