Elsevier

Epilepsy Research

Volume 100, Issue 3, July 2012, Pages 267-271
Epilepsy Research

Carnitine, nutritional supplementation and discontinuation of ketogenic diet therapies

https://doi.org/10.1016/j.eplepsyres.2012.04.021Get rights and content

Summary

Nutritional adequacy of a prescribed diet is integral to clinical implementation of the ketogenic diet therapies in intractable epilepsy. This review discusses the evidence for using additional carnitine and the importance of full micronutrient supplementation. The optimal duration of a diet therapy is also discussed, drawing on results of an internationally applied questionnaire.

Section snippets

Carnitine

The ketogenic diet (KD) is a high fat, low carbohydrate dietary regimen used since the 1920s (Wilder, 1921). It is designed to imitate the metabolic changes occurring during fasting and has been shown to be successful in treating intractable epilepsy (Freeman et al., 1998, Lefevre and Aronson, 2000, Henderson et al., 2006, Neal et al., 2008). The classical KD is usually prescribed at a ratio of 3 or 4 g of fat to every 1 g of carbohydrate and protein combined, thus providing 87–90% of total

Nutritional supplementation

General nutritional supplementation for the KD is less controversial and well established within clinical implementation. The KD restricts intake of dairy products, fruit, vegetables, cereals and grain products. These high carbohydrate foods contain many of the 28 known essential micronutrients. The KD is deficient in major nutrients when compared to the Dietary Reference Intakes (DRIs). The DRIs are micronutrient standards established by the United States Food and Nutrition Board of the

Diet discontinuation: an international survey

A frequently asked question is what is the optimal duration of KD therapy? Doctors and dietitians have traditionally recommended that the KD be used for no longer than two years due to potential long term side effects, such as dyslipidemia, kidney stones, and decreased growth velocity (Groesbeck et al., 2006). Over the past 90 years of clinical use, the implementation of the diet has changed with the liberalization of calories and fluid restriction as well as vitamin and mineral supplementation

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      sThe ketogenic diet is a valuable asset in the treatment of medically intractable epilepsy with radical and diffuse effects on the metabolism of every cell in the body due to its classic composition of 80–90 percent fat with approximately 5–7 percent protein and 3 percent carbohydrate (Bergqvist, 2012; Zupec-Kania and Zupanc, 2008). This diet lacks vitamins, trace minerals and electrolytes and needs to be properly supplemented (Neal et al., 2012). Prescribing such a diet for a long period therefore warrants monitoring of laboratory parameters (labs) to evaluate- among other things- degree of ketosis, electrolyte abnormalities and deficits in vitamins, minerals and trace elements.

    • Investigating owner use of dietary supplements in dogs with idiopathic epilepsy

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      KD may have anti-inflammatory properties (Dupuis et al., 2015) and prevent neuronal hyperexcitability via pH, direct inhibition of ion channels, influencing mitochondrial functions and alteration in amino acid metabolism linked to inhibitory neurotransmitter production (Degiorgio et al., 2015; Degiorgio & Taha, 2016). In addition to complete dietary interventions such as the KD, dietary supplements (DS) of different vitamins (Snoeijen-Schouwenaars et al., 2015), minerals (Kumar et al., 2015; Yuen and Sander, 2012), fatty acids (Bromfield et al., 2008; Chang et al., 2013; Degiorgio et al., 2015; Degiorgio & Taha, 2016; Taha et al., 2010; Yuen et al., 2012; Yuen et al., 2005) or other nutrients (Ciarlone et al., 2016) have been extensively explored in rodent models of epilepsy (Betti et al., 2011; Flores-Mancilla et al., 2014; Girotto et al., 2013; Liu et al., 2012; Smeland et al., 2012) and human patients (Jackson et al., 2015; Lee and Chung, 2010; Neal et al., 2012; Zupec-Kania and Zupanc, 2008). While some studies found that dietary supplementation with individual nutrients can improve seizure control (Degiorgio & Taha, 2016; Kumar et al., 2015; Scorza et al., 2009b), reduce seizure frequency (Ciarlone et al., 2016; Yuen et al., 2005) and improve other health aspects of epileptic patients, others have failed to confirm similar findings (Matthews et al., 2012; Sarmento Vasconcelos et al., 2016; Yuen et al., 2012).

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      Repeated measurements showed altered levels of total carnitine and fasting lipids. Although high fat intake may increase utilization of carnitine stores, deficiency during KD is not commonly present and supplementation rarely needed [7,8]. In pregnancy, cholesterol and triglyceride requirements are increased due to enhanced fat tissue lipolytic activity [9].

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