Imai Katsumi, Ishihara Eiko, Ikeda Hiroko
Nihon Rinsho. 2014 May;72(5):875-80.
Reappraisal of ketogenic diets (KD) were delayed in Japan compared to USA and Korea. The reasons are unknown, but possible explanations are (1) Japanese food culture prefers rice and less fat and (2) ACTH therapy is preferred for West syndrome in Japan. Since Japanese child neurologists were surprised at dramatic effects on glucose transporter 1 deficiency syndrome (Glut-1DS) in 2003, KD have been slowly accepted for treatment of epilepsy in Japan. New generation KD including modified Atkins diet (mAD) are preferred to classical KD. KD can be causal therapy in Glut-1DS and some of mitochondrial disorders, though anti-epileptic drugs are symptomatic therapy. KD can alleviate intractable seizures in epilepsies with brain malformation in addition to West syndrome and Dravet syndrome, etc. KD may work for brain tumor, cancer, neurodegenerative disorders including Alzheimer's disease. C7-8 triglycerides or fatty acid esters are under development as medicines replacing KD.
与美国和韩国相比，日本对生酮饮食（KD）的重新评估有所延迟。原因尚不清楚，但可能的解释是：（1）日本饮食文化偏爱大米且脂肪摄入较少；（2）在日本，韦斯特综合征首选促肾上腺皮质激素（ACTH）治疗。自2003年日本儿童神经科医生对生酮饮食对葡萄糖转运蛋白1缺乏综合征（Glut - 1DS）的显著疗效感到惊讶以来，生酮饮食在日本已逐渐被接受用于癫痫治疗。包括改良阿特金斯饮食（mAD）在内的新一代生酮饮食比传统生酮饮食更受青睐。生酮饮食在Glut - 1DS和一些线粒体疾病中可作为病因治疗，而抗癫痫药物则是对症治疗。除韦斯特综合征和德雷维特综合征等外，生酮饮食还可缓解伴有脑畸形的癫痫患者的难治性癫痫发作。生酮饮食可能对脑肿瘤、癌症、包括阿尔茨海默病在内的神经退行性疾病有效。C7 - 8甘油三酯或脂肪酸酯正在研发中，有望成为替代生酮饮食的药物。
