Ketogenic Diet in Alpers-Huttenlocher Syndrome

Charuta N. Joshi, Cheryl R. Greenberg, Aizeddin A. Mhanni, Michael S. Salman

Research output: Contribution to journalArticlepeer-review

49 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)314-316
Number of pages3
JournalPediatric Neurology
Issue number4
StatePublished - Apr 2009
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology


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