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Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
Letícia Pereira de Brito Sampaio1, Adélia Maria de Miranda Henriques-Souza2,3, Mariana Ribeiro Marcondes da Silveira1
1Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, São Paulo SP, Brazil.
Insights
Early treatment of Infantile Epileptic Spasms Syndrome (IESS) with accessible medications like oral prednisolone and vigabatrin is feasible and effective. Standardized protocols improve outcomes and reduce relapses in children with this severe neurological disorder.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Therapeutics
Background:
- Infantile Epileptic Spasms Syndrome (IESS) is a severe early-onset condition often linked to brain disorders, leading to psychomotor decline and adult epilepsy.
- Treatment for IESS is challenging due to medication resistance and frequent relapses.
Purpose of the Study:
- To review the literature on IESS treatment strategies.
- To offer practical guidance for healthcare systems with limited resources, focusing on available therapies.
Main Methods:
- An expert committee from the Brazilian Society of Child Neurology conducted a review of scientific evidence.
- Focus was placed on treatments for IESS that are accessible within Brazil.
Main Results:
- Oral prednisolone and vigabatrin are recommended first-line therapies due to efficacy and availability in Brazil's public health system.
- Intramuscular ACTH shows similar efficacy but is not available; other antiseizure drugs have limited response.
- Ketogenic diet and epilepsy surgery are options for refractory cases.
Conclusions:
- Early IESS treatment using effective and available drugs is achievable in resource-limited settings.
- Implementing standard treatment protocols can lead to faster cessation of spasms and fewer relapses.
Background:
Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent.
Objective:
To evaluate the literature regarding treatment of IESS and provide a practical guidance to a healthcare system with limited resources.
Methods:
An expert committee from the Brazilian Society of Child Neurology reviewed and discussed relevant scientific evidence in the treatment of IESS regarding the drugs available in Brazil.
Results:
Oral prednisolone and vigabatrin are the most common drugs used as first-line therapy; they are efficient and affordable therapy as both are available in the Brazilian unified health system (SUS, in the Portuguese acronym). Intramuscular adrenocorticotropic hormone (ACTH) presents similar efficacy as oral prednisolone but has a higher cost and is not available in Brazil. Other antiseizure medications such as topiramate, levetiracetam, or benzodiazepines have limited response and are prescribed as adjuvant therapy. If the health service has nutritionists, a ketogenic diet should be implemented for those not responding to hormonal and vigabatrin treatment. Epilepsy surgery is mainly indicated for patients with focal lesions that do not respond to pharmacological therapy.
Conclusion:
Early treatment of IESS with efficient drugs is feasible in our country. Using standard protocols increases the odds of achieving complete cessation in a shorter time and decreases relapse.
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