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Serum carnitine levels and levocarnitine supplementation in institutionalized Huntington's disease patients

Miroslav Cuturic1, Ruth K Abramson, Robert R Moran

  • 1Department of Mental Health, University of South Carolina School of Medicine, Columbia, SC, USA. mbc20@scdmh.org

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
|February 2, 2012
PubMed

Insights

Huntington's disease patients often have low carnitine levels, potentially causing neurological issues. Supplementing with levocarnitine improved motor, cognitive, and behavioral symptoms in these patients.

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Biochemistry

Background:

  • Carnitine regulates lipid metabolism and possesses antioxidant properties.
  • Beneficial effects of carnitine are known in animal models of Huntington's disease (HD).
  • Carnitine metabolism in human HD patients remains understudied.

Purpose of the Study:

  • To investigate carnitine levels in human patients with Huntington's disease.
  • To explore the potential benefits of levocarnitine supplementation in HD patients with hypocarnitinemia.

Main Methods:

  • Retrospective database review of 23 patients in an HD-specialized nursing home.
  • Analysis of serum carnitine levels and correlation with clinical factors.
  • Evaluation of outcomes following levocarnitine supplementation in patients with low carnitine.

Main Results:

  • A high prevalence of hypocarnitinemia (26%) was observed in the studied HD patient population.
  • Catabolism and chronic valproate use were identified as potential predisposing factors.
  • Levocarnitine supplementation led to improvements in motor, cognitive, and behavioral measures over a mean of 7.3 months.

Conclusions:

  • This is the first study to report carnitine levels in human HD patients, indicating a significant prevalence of hypocarnitinemia.
  • Hypocarnitinemia in HD may be linked to reversible metabolic encephalopathy and myopathy.
  • Low-dose levocarnitine supplementation shows promise for improving outcomes in HD patients with carnitine deficiency.

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