Carnitine “deficiency”: Lack of response to carnitine therapy

James Edwin Carroll, Michael H. Brooke, Darryl C. DeVivo, Jack B. Shumate, Rüdiger Kratz, Steven P. Ringel, James M. Hagberg

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

A 29-year-old woman had muscle weakness, low concentrations of carnitine in muscle and serum, and abnormally low urinary excretion of carnitine. During a fast, exercise capacity declined, ketone body concen-trations rose, metabolic acidosis worsened, and she became hypoglycemic. After treatment with oral carnitine, serum carnitine content returned to normal and blood glucose was maintained during fasting, but ketone body concen-trations in blood were even higher. Muscle carnitine content and exercise capacity did not improve. This patient demonstrated features of both systemic and muscle carnitine deficiency, suggesting that the current classification of carnitine-deficiency syndromes is inadequate.

Original languageEnglish (US)
Pages (from-to)618-626
Number of pages9
JournalNeurology
Volume30
Issue number6
StatePublished - Jan 1 1980
Externally publishedYes

Fingerprint

Carnitine
Ketone Bodies
Muscles
Therapeutics
Exercise
Muscle Weakness
Acidosis
Serum
Hypoglycemic Agents
Blood Glucose
Fasting

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Carroll, J. E., Brooke, M. H., DeVivo, D. C., Shumate, J. B., Kratz, R., Ringel, S. P., & Hagberg, J. M. (1980). Carnitine “deficiency”: Lack of response to carnitine therapy. Neurology, 30(6), 618-626.

Carnitine “deficiency” : Lack of response to carnitine therapy. / Carroll, James Edwin; Brooke, Michael H.; DeVivo, Darryl C.; Shumate, Jack B.; Kratz, Rüdiger; Ringel, Steven P.; Hagberg, James M.

In: Neurology, Vol. 30, No. 6, 01.01.1980, p. 618-626.

Research output: Contribution to journalArticle

Carroll, JE, Brooke, MH, DeVivo, DC, Shumate, JB, Kratz, R, Ringel, SP & Hagberg, JM 1980, 'Carnitine “deficiency”: Lack of response to carnitine therapy', Neurology, vol. 30, no. 6, pp. 618-626.
Carroll JE, Brooke MH, DeVivo DC, Shumate JB, Kratz R, Ringel SP et al. Carnitine “deficiency”: Lack of response to carnitine therapy. Neurology. 1980 Jan 1;30(6):618-626.
Carroll, James Edwin ; Brooke, Michael H. ; DeVivo, Darryl C. ; Shumate, Jack B. ; Kratz, Rüdiger ; Ringel, Steven P. ; Hagberg, James M. / Carnitine “deficiency” : Lack of response to carnitine therapy. In: Neurology. 1980 ; Vol. 30, No. 6. pp. 618-626.
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