生酮飲食治療於兒童神經疾患的應用

生酮飲食治療癲癇的概念起源於西元前300年，歷經2000多年的演變，至今已經是有實證驗證療效的科學性治療。本文除了介紹生酮飲食的歷史沿革，同時也介紹生酮飲食的4種分類及其中的營養素比例，讓讀者對醫用生酮飲食有基本了解。同時也總攬生酮飲食治療兒童神經疾患的應用，包含：(1)癲癇；(2)神經罕病-腦血管屏障葡萄糖輸送缺陷症第1型，丙酮酸鹽脫氫酶缺乏症、卓飛症候群、結節硬化症、粒腺體疾患、雷特氏症；(3)自閉症。文末強調生酮飲食為計劃性飲食，需協助醫師及營養師的協助，以避免嚴重副作用。

The precursor of ketogenic diet originated from 300 B.C. It became an evidenced-based food science after more than 2000 years of evolution. We introduce the history of ketogenic diet, the classifications and nutrition distribution in the four different types of the ketogenic diet herein. We also overviewed the clinical application of ketogenic diet in several aspects of pediatric neurological disorders, including: (1) epilepsy; (2) rare neurological disorders, ex. glucose transport 1 deficiency, pyruvate dehydrogenase deficiency, Dravet syndrome, tuberous sclerosis complex, mitochondrial disorders and Rett syndrome; and (3) autism. We emphasized that to avoid severe adverse effects, users should executed ketogenic diet under the surveillance of physician and nutritionist.