Assuring Pediatric Nutrition in the Community

FREQUENTLY ASKED QUESTIONS:
Specific Diagnoses - Cerebral palsy

Is there any evidence that carnitine impacts the linear growth of a child with cerebral palsy?

If a child with cerebral palsy is carnitine deficient, growth would be impacted. Carnitine is involved in CoA and activated carboxylic acid transport across cell membranes; this results in delivery of needed substrated, elimination of accumulated toxins, and transport of high energy from one subcellular or cellular location to another. Carnitine is needed for oxidation of fatty acids. Thus carnitine is very important in facilitating use of energy. Biochemical symptoms of carnitine deficiency include altered intravenous fat tolerance, decreased oxidation of exogenous fatty acids and decreased production of ketone bodies. Clinical symptoms would include failure to thrive and impaired function of organs, such as cardiac muscle and skeletal muscle that are highly dependent on fatty acid oxidation for fuel.

Preterm infants are frequently carnitine deficient, as significant carnitine stores are accumulated during the last trimester of pregnancy. Human milk and cow's milk are good dietary sources of carnitine; if an infant is fed parenterally, s/he will not build carnitine stores, and is vulnerable to carnitine deficiency. It has been shown that high dose IV carnitine supplementation is not beneficial. Small doses seem to be more beneficial.

There are metabolic abnormalities in which carnitine supplementation, over and above that in a typical diet is necessary. Valproic Acid, used for seizure control can cause a deficiency in carnitine, which is treated with carnitine supplementation. In these cases high doses of oral carnitine are required.

If a child with CP has symptoms of carnitine deficiency, especially if s/he is being treated with Valproic Acid, both free and acyl carnitine levels should be evaluated, and supplements given if a deficiency is found. This should be supervised by a physician (e.g., a Neurologist) with experience in dealing with carnitine deficiency.

References
Borum P. Carnitine in neonatal nutrition. J Child Neurol, 1995;10(Suppl):2S25-2S31.

Sulkers EJ, Lafeber HN, Degenhart HJ, et al. Effects of high carnitine supplementation on substrate utilization in low-birth-weight infants receiving total parenteral nutrition. Amer J Clinical Nutrition; 1990; 52(5):889-94.