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巴西专家关于婴儿癫痫痉挛综合征治疗的共识。

Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants.

机构信息

Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, São Paulo SP, Brazil.

Instituto de Medicina Integral Professor Fernando Figueira, Recife PE, Brazil.

出版信息

Arq Neuropsiquiatr. 2023 Sep;81(9):844-856. doi: 10.1055/s-0043-1772835. Epub 2023 Oct 4.


DOI:10.1055/s-0043-1772835
PMID:37793406
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10550353/
Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/b939d51583a1/10-1055-s-0043-1772835-i220327-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/ecef0a130d74/10-1055-s-0043-1772835-i220327-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/b32a2c078891/10-1055-s-0043-1772835-i220327-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/b939d51583a1/10-1055-s-0043-1772835-i220327-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/ecef0a130d74/10-1055-s-0043-1772835-i220327-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/b32a2c078891/10-1055-s-0043-1772835-i220327-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1db9/10550353/b939d51583a1/10-1055-s-0043-1772835-i220327-3.jpg

相似文献

[1]
Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants.

Arq Neuropsiquiatr. 2023-9

[2]
Temporal trends in the cost and use of first-line treatments for infantile epileptic spasms syndrome.

Epilepsia. 2023-3

[3]
Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial.

Lancet Child Adolesc Health. 2018-8-29

[4]
Prednisolone or tetracosactide depot for infantile epileptic spasms syndrome? A prospective analysis of data embedded within two randomised controlled trials.

Eur J Paediatr Neurol. 2023-1

[5]
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Neurol Sci. 2024-2

[6]
Long-term analysis of adrenocorticotropic hormone monotherapy for infantile epileptic spasms syndrome with periventricular leukomalacia.

Seizure. 2023-7

[7]
Epileptic spasms relapse is associated with response latency but not conventional attributes of post-treatment EEG.

Epilepsia Open. 2024-6

[8]
Efficacy of vigabatrin in the treatment of infantile epileptic spasms syndrome: A systematic review and meta-analysis.

Epilepsia Open. 2023-6

[9]
Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial.

Lancet Neurol. 2016-11-10

[10]
Response to sequential treatment with prednisolone and vigabatrin in infantile spasms.

J Paediatr Child Health. 2022-12

引用本文的文献

[1]
Factors Influencing the Response of Patients with Infantile Epileptic Spasms Syndrome to ACTH as Repeated First-Line Therapy.

Neurol Ther. 2025-6

本文引用的文献

[1]
Prednisolone or tetracosactide depot for infantile epileptic spasms syndrome? A prospective analysis of data embedded within two randomised controlled trials.

Eur J Paediatr Neurol. 2023-1

[2]
ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions.

Epilepsia. 2022-6

[3]
Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations.

Pediatr Neurol. 2021-10

[4]
Epilepsy in Mitochondrial Diseases-Current State of Knowledge on Aetiology and Treatment.

Children (Basel). 2021-6-22

[5]
Epilepsy Outcome at Four Years in a Randomized Clinical Trial Comparing Oral Prednisolone and Intramuscular ACTH in West Syndrome.

Pediatr Neurol. 2021-6

[6]
Evaluation of the Modified Atkins Diet for the Treatment of Epileptic Spasms Refractory to Hormonal Therapy: A Randomized Controlled Trial.

J Child Neurol. 2021-7

[7]
Efficacy of the ketogenic diet on ACTH- or corticosteroid-resistant infantile spasm: a multicentre prospective control study.

Epileptic Disord. 2021-4-1

[8]
Vigabatrin-associated Reversible MRI Abnormalities in an Infant with Tuberous Sclerosis.

J Radiol Case Rep. 2021-2

[9]
Intravenous Methylprednisolone Versus Oral Prednisolone for West Syndrome: A Randomized Open-Label Trial.

Indian J Pediatr. 2021-8

[10]
ACTH Treatment of Infantile Spasms: Low-Moderate- Versus High-Dose, Natural Versus Synthetic ACTH-A Retrospective Cohort Study.

Pediatr Neurol. 2020-10

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