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| Valproic acid drugs not recommended in CPT II Deficiency | |
| --Sharon Hesterlee, Ph.D. |
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| A recent case report in
the journal Neuromuscular Disorders highlights a potentially
life-threatening response in a person with CPT II deficiency to the
drug valproic acid. A common anti-epileptic drug, valproic acid
(brand name Depakene) is also used in the treatment of bipolar
disorders and migraine. The case report describes a 47-year-old man who had a history of dark urine and exercise intolerance when he began treatment with valproic acid for bipolar disorder. Within four days of starting the new medication, the man was admitted to the hospital with acute rhabdomyolysis (muscle breakdown), a CK level of 49,000 U/L and kidney failure. He was treated with hemodialysis and recovered fully after two weeks. Although it had been suggested in the past that valproic acid might be better avoided in people with mitochondrial diseases, this is the first example of a documented reaction to the drug by someone with a defect in energy metabolism. The authors of the case study concluded that valproic acid likely interferes with the fatty acid breakdown cycle within mitochondria called beta-oxidation. Those with primary defects in fatty acid metabolism, like CPT II deficiency, may be at high risk for adverse reactions with this drug. Reference: Kottlors M, et al. Valproic acid triggers acute rhabdomyolysis in a patient with carnitine palmitoyltransferase type II deficiency. Neuromuscular Disorders 2001 11(8): 757-759. |
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Dr. Hesterlee is Director of Research for the Muscular Dystrophy Association. |
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Related links:
Drug-induced rhabdomyolysis  Extensive article by E. B. Larbi, Ph.D. Drug-induced myopathy Detailed information from the US Pharmacist site. Causes of rhabdomyolysis Table that includes a partial listing of drugs. Rhabdomyolysis Emedicine article with list of drugs and other causes. Final
diagnosis: Acute necrotizing myopathy (rhabdomyolysis) secondary to
lovastatin and niacinIsotretinoin induced rhabdomyolysis? Dermatology case history concerning an acne drug. Rhabdomyolysis
associated with simvastatin-gemfibrozil therapyCase history that requires Adobe Acrobat to view. Cholesterol drug problems are not imaginary People's Pharmacy question and answer. Toxic myopathies Washington University information including drug lists. 
For case histories of rhabdomyolysis in CPT II deficiency,
visit Seeing red, Anatomy
of an attack, Running
in the family, The
great Alaska kick-start.
For an overview, visit Rhabdomyolysis:
Taking it one day at a time.
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Although
drug-induced rhabdomyolysis was uncommon in the past, it is no
longer rare due to the introduction of more and increasingly
potent drugs into clinical practice.
