Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, São Paulo SP, Brazil.
Instituto de Medicina Integral Professor Fernando Figueira, Recife PE, Brazil.
Arq Neuropsiquiatr. 2023 Sep;81(9):844-856. doi: 10.1055/s-0043-1772835. Epub 2023 Oct 4.
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.
背景:婴儿痉挛症(IESS)是一种罕见但严重的疾病,通常发生在儿童早期,且与可识别的脑部疾病有关。它与儿童期的精神运动恶化和成年期的癫痫有关。由于婴儿痉挛可能对大多数抗癫痫药物没有反应,且容易复发,因此治疗具有挑战性。
目的:评估关于 IESS 治疗的文献,并为资源有限的医疗保健系统提供实用指导。
方法:巴西儿童神经病学学会的一个专家委员会审查并讨论了巴西现有药物治疗 IESS 的相关科学证据。
结果:口服泼尼松龙和氨己烯酸是最常用的一线治疗药物;它们是有效且负担得起的治疗方法,因为两者均在巴西统一卫生系统(SUS)中可用。肌肉注射促肾上腺皮质激素(ACTH)与口服泼尼松龙具有相似的疗效,但成本更高,且在巴西不可用。其他抗癫痫药物,如托吡酯、左乙拉西坦或苯二氮䓬类药物,反应有限,作为辅助治疗药物开具。如果卫生服务机构有营养师,对于那些对激素和氨己烯酸治疗无反应的患者,应实施生酮饮食。癫痫手术主要适用于对药物治疗无反应的局灶性病变患者。
结论:在我国,用有效药物早期治疗 IESS 是可行的。使用标准方案可增加在更短时间内实现完全缓解的机会,并降低复发率。
Arq Neuropsiquiatr. 2023-9
J Paediatr Child Health. 2022-12
Children (Basel). 2021-6-22
J Radiol Case Rep. 2021-2