Fatty Acid Oxidation Defects in Muscle

Overview

Journal Curr Opin Neurol

Specialty Neurology

Date 1998 Dec 16

PMID 9847998

Citations 9

Authors

A A MORRIS

D M Turnbull

Affiliations

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Abstract

Fatty acid oxidation defects can cause recurrent rhabdomyolysis or chronic progressive muscle weakness. Diagnosis is often possible on blood using tandem mass spectrometry or molecular genetic techniques. Riboflavin and carnitine are effective in some cases of multiple acyl-CoA dehydrogenase deficiency and primary carnitine deficiency, respectively. Controlled trials are needed to evaluate other proposed forms of treatment.

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Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency and Perioperative Management in Adult Patients.

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Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.

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Carnitine supplementation for inborn errors of metabolism.

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