Riboflavin-responsive lipid myopathy and carnitine deficiency

James Edwin Carroll, Jack B. Shumate, Michael H. Brooke, James M. Hagberg

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

We used riboflavin to treat a patient with lipid myopathy, reduced exercise capacity, intolerance to fasting, and reduced concentrations of carnitine in muscle and serum. Although carnitine concentrations did not change, exercise capacity doubled, and response to fasting improved. Muscle enzyme assay showed that palmityl CoA dehydrogenase activity with and without added flavin adenine dinucleotide (a riboflavin product) was normal. Another riboflavin derivative, electron transfer flavoprotein, could be the site of the defect.

Original languageEnglish (US)
Pages (from-to)1557-1559
Number of pages3
JournalNeurology
Volume31
Issue number12
StatePublished - Jan 1 1981
Externally publishedYes

Fingerprint

Riboflavin
Carnitine
Muscular Diseases
Lipids
Fasting
Electron-Transferring Flavoproteins
Exercise
Palmitoyl Coenzyme A
Muscles
Flavin-Adenine Dinucleotide
Enzyme Assays
Oxidoreductases
Serum

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Carroll, J. E., Shumate, J. B., Brooke, M. H., & Hagberg, J. M. (1981). Riboflavin-responsive lipid myopathy and carnitine deficiency. Neurology, 31(12), 1557-1559.

Riboflavin-responsive lipid myopathy and carnitine deficiency. / Carroll, James Edwin; Shumate, Jack B.; Brooke, Michael H.; Hagberg, James M.

In: Neurology, Vol. 31, No. 12, 01.01.1981, p. 1557-1559.

Research output: Contribution to journalArticle

Carroll, JE, Shumate, JB, Brooke, MH & Hagberg, JM 1981, 'Riboflavin-responsive lipid myopathy and carnitine deficiency', Neurology, vol. 31, no. 12, pp. 1557-1559.
Carroll JE, Shumate JB, Brooke MH, Hagberg JM. Riboflavin-responsive lipid myopathy and carnitine deficiency. Neurology. 1981 Jan 1;31(12):1557-1559.
Carroll, James Edwin ; Shumate, Jack B. ; Brooke, Michael H. ; Hagberg, James M. / Riboflavin-responsive lipid myopathy and carnitine deficiency. In: Neurology. 1981 ; Vol. 31, No. 12. pp. 1557-1559.
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