Ketogenic Diet in Alpers-Huttenlocher Syndrome

被引：48

作者：

Joshi, Charuta N. ^{[2
,3
]}

Greenberg, Cheryl R. ^{[1
,2
]}

Mhanni, Aizeddin A. ^{[1
,2
]}

Salman, Michael S. ^{[2
,3
]}

机构：

[1] Univ Manitoba, Program Genet & Metab, Winnipeg, MB, Canada

[2] Univ Manitoba, Dept Pediat & Child Hlth, Winnipeg, MB R3T 2N2, Canada

[3] Univ Manitoba, Sect Pediat Neurosci, Winnipeg, MB, Canada

来源：

PEDIATRIC NEUROLOGY | 2009年 / 40卷 / 04期

关键词：

POLG1; MUTATIONS; EPILEPSY; COMPLICATIONS; DEFECTS;

D O I：

10.1016/j.pediatrneurol.2008.10.023

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We report on a young girl with Alpers-Huttenlocher syndrome, as confirmed by mitochondrial polymerase gamma sequencing, who was treated with the classic (4 parts fat:1 part each of carbohydrate and protein) ketogenic diet after she presented with epilepsia partialis continua. She improved clinically, and her electroencephalogram improved dramatically. This is the first detailed report on the efficacy of the ketogenic diet in treating the epileptic encephalopathy of Alpers-Huttenlocher syndrome. We present a literature review of the utility of a ketogenic diet in mitochondrial disorders, and speculations as to why the diet may be helpful in Alpers-Huttenlocher syndrome. (C) 2009 by Elsevier Inc. All rights reserved.

引用

收藏

页码：314 / 316

页数：3

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