Haridas Babitha, Testino Alexander, Kossoff Eric H
Department of Neurology and Pediatrics, Johns Hopkins Hospital, Baltimore, Maryland, USA.
Epileptic Disord. 2025 Apr;27(2):144-155. doi: 10.1002/epd2.20320. Epub 2024 Dec 12.
In 1921, the classic ketogenic diet was created at the Mayo Clinic in Rochester, Minnesota to treat epilepsy in children and adults. Over a century later, it is a widely used, standard-of-care therapy for typically treatment-resistant epilepsy worldwide. There are currently five versions of ketogenic diet therapy that can be started either in or out of the hospital setting. It is overall effective in approximately half of children started, usually within a few months. Established indications for ketogenic diet therapy exist, in which this treatment may potentially even be more advantageous than antiseizure medications. Some of these indications include Glut1 deficiency, pyruvate dehydrogenase deficiency, infantile epileptic spasms syndrome, epilepsy with myoclonic-atonic seizures, and formula-fed children. Although most children are also receiving antiseizure medications with ketogenic diet therapy, its use may lead to medication reduction or withdrawal in some cases, and improvement in cognition and quality of life. Supplements are begun when ketogenic diet therapy is initiated in order to prevent common side effects, including constipation, kidney stones, growth disturbance, and dyslipidemia. Typically, after 2 years in most children, ketogenic diet therapy is discontinued gradually.
1921年,经典的生酮饮食在明尼苏达州罗切斯特市的梅奥诊所被创立,用于治疗儿童和成人的癫痫。一个多世纪后,它成为全球范围内广泛使用的、针对通常难治性癫痫的标准治疗方法。目前有五种生酮饮食疗法版本,可在医院环境内或外开始实施。总体而言,约一半开始接受该疗法的儿童有效,通常在几个月内起效。生酮饮食疗法有既定的适用指征,在这些情况下,这种治疗可能甚至比抗癫痫药物更具优势。其中一些指征包括葡萄糖转运体1缺乏症、丙酮酸脱氢酶缺乏症、婴儿痉挛症综合征、肌阵挛-失张力发作癫痫以及配方奶喂养的儿童。虽然大多数儿童在接受生酮饮食疗法时也同时服用抗癫痫药物,但在某些情况下,其使用可能导致药物减少或停用,并改善认知和生活质量。在开始生酮饮食疗法时就开始补充剂,以预防常见的副作用,包括便秘、肾结石、生长发育障碍和血脂异常。通常,大多数儿童在2年后逐渐停用生酮饮食疗法。