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European Journal of Pediatrics

, Volume 156, Issue 7, pp 562–564 | Cite as

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy

  • C. W. Lam
  • C. H. Lau
  • J. C. Williams
  • Y. W. Chan
  • L. J. C. Wong
NEUROPEDIATRICS

Abstract

We report in this study a patient who developed repeated convulsions as a result of valproate therapy. MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) was subsequently diagnosed and a nucleotide 3243 A→G mutation was detected in the mitochondrial DNA. This mutation predisposes the patient to the detrimental effects of valproate on oxidative phosphorylation.

Conclusion We support the suggestion of Ponchaut et al. [14] that valproate should not be given to patients suspected of having mitochondrial diseases. In addition, for patients whose seizures worsen with valproate therapy, an inborn error of mitochondrial metabolism should be suspected. The underlying mitochondrial DNA defects should be sought for family screening and genetic counselling.

Key words Valproate Mitochondrial encephalomyopathy MELAS syndrome Mitochondrial mutation 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1997

Authors and Affiliations

  • C. W. Lam
    • 1
  • C. H. Lau
    • 2
  • J. C. Williams
    • 3
  • Y. W. Chan
    • 1
  • L. J. C. Wong
    • 4
  1. 1.Department of Pathology, Princess Margaret Hospital, Lai Chi Kok, Hong KongHK
  2. 2.Department of Paediatrics, Princess Margaret Hospital, Lai Chi Kok, Hong KongHK
  3. 3.Division of Medical Genetics, Children's Hospital of Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA 90027 USAUS
  4. 4.Molecular Genetics Laboratory, Children's Hospital of Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA 90027, USAUS

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