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Ketogenic Diet being used to treat diverse Neuro disorders...

Discussion in 'General Treatment' started by merylg, Nov 20, 2012.

  1. merylg

    merylg Senior Member

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    Sydney, NSW, Australia
  2. Sasha

    Sasha Fine, thank you

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    That's very interesting, thanks for posting.

    The article goes through several disorders looking at evidence and rationale for a ketogenic diet and says this about mito disorders (my bold):

    The KD in Mitochondrial Disorders

    As mentioned above, given the growing evidence that the KD enhances mitochondrial functioning and biogenesis (Bough et al., 2006; Maalouf et al., 2009; Kim do et al., 2010), it is logical to ask whether patients with known mitochondrial cytopathies might derive a benefit from the KD and/or ketone bodies such as BHB. At the same time, it must be considered that inherent mitochondrial dysfunction might predispose individuals to adverse toxicities from high fatty acid loads that could overwhelm β-oxidation within the mitochondrial matrix. Experimental data described above attest to significant improvements in mitochondrial function, and many lines of evidence point to the rationale of therapeutically targeting mitochondrial bioenergetics for other disease states (Wallace et al., 2010), but is there any clinical evidence in patients with intrinsic mitochondrial disorders? Kang et al. (2007) reported that the KD was both safe and effective in 14 pediatric patients with established mitochondrial defects in complexes I, II, and IV, all of whom had medically intractable epilepsy. These authors observed that half of these patients became seizure-free on the KD, and only four patients failed to respond. Hence, these preliminary data suggest that the KD is not necessarily contraindicated in patients with mitochondrial respiratory chain abnormalities. However, KD treatment is not recommended in individuals with primary carnitine deficiencies [including mutations in carnitine palmitoyl transferase (CPT) I or II and mitochondrial translocase] and fatty acid β-oxidation abnormalities (e.g., medium-chain acyl dehydrogenase deficiency; Kossoff et al., 2009). Thus, it is critical to determine the specific mitochondrial defect when considering treatment with the KD, to avert clinical deterioration.


    Any biologically knowledgable people care to comment?
    merylg likes this.
  3. merylg

    merylg Senior Member

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    Hi Sasha,
    Yes, this did concern me. Today I finally had my appointment with a Neurogeneticist. I guess I'm looking at excluding mitochondrial diseases, but also hoping for any help I can get.
    A ketogenic diet could be damaging if one happened to have certain mutations (inherited or spontaneous) affecting mitochondrial function.

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