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J. Clin. Med. 2017, 6(6), 56; doi:10.3390/jcm6060056

Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders

Chelsea and Westmister Hospital, 369 Fulham road, Chelsea, London SW10 9NH, UK
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Academic Editor: Iain P. Hargreaves
Received: 31 March 2017 / Revised: 16 May 2017 / Accepted: 22 May 2017 / Published: 26 May 2017
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Abstract

Mitochondrial disorders are a clinically heterogeneous group of disorders that are caused by defects in the respiratory chain, the metabolic pathway of the adenosine tri-phosphate (ATP) production system. Epilepsy is a common and important feature of these disorders and its management can be challenging. Epileptic seizures in the context of mitochondrial disease are usually treated with conventional anti-epileptic medication, apart from valproic acid. However, in accordance with the treatment of intractable epilepsy where there are limited treatment options, the ketogenic diet (KD) has been considered as an alternative therapy. The use of the KD and its more palatable formulations has shown promising results. It is especially indicated and effective in the treatment of mitochondrial disorders due to complex I deficiency. Further research into the mechanism of action and the neuroprotective properties of the KD will allow more targeted therapeutic strategies and thus optimize the treatment of both epilepsy in the context of mitochondrial disorders but also in other neurodegenerative disorders. View Full-Text
Keywords: ketogenic diet; mitochondrial disorders; intractable epilepsy; treatment ketogenic diet; mitochondrial disorders; intractable epilepsy; treatment
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Paleologou, E.; Ismayilova, N.; Kinali, M. Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders. J. Clin. Med. 2017, 6, 56.

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