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

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

Along with antioxidant properties, carnitine is an important regulator of lipid metabolism in humans. While beneficial effects of carnitine have been demonstrated in animal models of Huntington’s disease (HD), metabolism of carnitine has not been studied in humans with this illness. In this retrospe...

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Bibliographic Details
Published in:Neurological sciences Vol. 34; no. 1; pp. 93 - 98
Main Authors: Cuturic, Miroslav, Abramson, Ruth K., Moran, Robert R., Hardin, James W., Frank, Elaine M., Sellers, Andrea A.
Format: Journal Article
Language:English
Published: Milan Springer Milan 2013
Springer Nature B.V
Subjects:
Adult
Anticonvulsants - adverse effects
Anticonvulsants - therapeutic use
Brief Communication
Carnitine - blood
Carnitine - deficiency
Carnitine - therapeutic use
Databases, Factual
Diet
Dietary Supplements
Female
Hospitalization
Humans
Huntington Disease - blood
Huntington Disease - drug therapy
Male
Medicine
Medicine & Public Health
Middle Aged
Neurology
Neuropsychological Tests
Neuroradiology
Neurosciences
Neurosurgery
Nutritional Status
Psychiatry
Retrospective Studies
Valproic Acid - adverse effects
Valproic Acid - therapeutic use
Levocarnitine
Catabolism
Huntington’s disease
Valproic acid
Carnitine
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Summary:Along with antioxidant properties, carnitine is an important regulator of lipid metabolism in humans. While beneficial effects of carnitine have been demonstrated in animal models of Huntington’s disease (HD), metabolism of carnitine has not been studied in humans with this illness. In this retrospective database review from 23 patients admitted to a HD-specialized nursing home unit, we found a relatively high prevalence of hypocarnitinemia (6 cases, 26%). Our review suggests that catabolism and chronic valproate use predisposed our patients to develop hypocarnitinemia. The patients with low serum carnitine levels who received levocarnitine supplementation, during a mean period of 7.3 months, showed improvement in motor, cognitive and behavioral measures. We hypothesize that observed improvement related to the resolution of reversible metabolic encephalopathy and myopathy associated with secondary carnitine deficiency. In conclusion, notwithstanding its limitations, this is the first study to report measurements of carnitine levels in HD patients, revealing relatively high prevalence of hypocarnitinemia in our population. Our findings suggest that HD patients with hypocarnitinemia may benefit from low-dose levocarnitine supplementation. Further studies of carnitine metabolism and supplementation in HD patients are warranted.
ISSN:1590-1874
1590-3478
DOI:10.1007/s10072-012-0952-x