A Brazilian ACTH therapy protocol for west syndrome in environmental treatment

Background: West syndrome (WS) is an epileptic encephalopathy characterized by epileptic spasms, neurodevelopment delay, and hypsarrhythmia on electroencephalography. Infantile spasms have an incidence of about 0.43 per 1000 livebirths and occur between 3 and 12 months of age. The drug's choice is the adrenocorticotrophic hormone (ACTH), with varied response rates and limited efficacy data, relapses, and evolution to other kinds of seizures in the medium-long term in our country.

Objective: This study aims to evaluate the effectiveness of ACTH therapy after failure of vigabatrin in patients with West syndrome.

Methods: This retrospective cohort study included WS children from two Neuropediatric ambulatories, aged 2-144 months, treated with synthetic ACTH from 2001 to 2021, that failed with therapeutic doses of vigabatrin. The primary outcome was efficacy on the 7^th and 30^th days; secondarily, we registered the relapse rates of hormonal therapy during one year of follow-up.

Results: Of 41 patients selected to study, 2 had severe ACTH side effects, and 39 made up the sample. Sixty-eight percent were male, with 87% symptomatic WS presentation. The median of spams onset, ACTH lag to treat, and VGB dose was 6, 5, and 12 months, respectively. On the 7^th and 30^th days, 46,1% and 94,8% had a favorable clinical-electroencephalographic resolution with the drug. Although without statistical significance, the study showed that the favorable clinical response was not associated with the form of WS presentation, etiology, history of other types of seizures, coexistence of two or more diseases, previous diagnosis of epilepsy or gender. Female gender, symptomatic etiology and diagnosis of epilepsy before WS increased the probability of an unfavorable outcome one month after the end of ACTH. The diagnosis of prenatal or perinatal injury also increased the chances of an unfavorable outcome, although these results were not statistically significant.

Conclusions: Our data corroborate the higher rates of ACTH therapy efficacy after vigabatrin failure in West syndrome. Also, we demonstrated minor relapse rates compared to ACTH results, attributed to their higher doses.

 

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Artikel online veröffentlicht:
18. September 2023