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Progression despite replacement of a myopathic form of coenzyme Q₁₀ defect

From INSERM U582, Institut de Myologie (Drs. Auré, Romero, and Lombès), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris; and Service de Biochimie A (Drs. Benoist and Rigal) and Service de Neurologie et Maladies Métaboliques (Dr. Ogier de Baulny), Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, France.

Address correspondence and reprint requests to Dr. Anne Lombès, INSERM U582, Groupe hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, 75013 Paris Cedex, France; e-mail: a.lombes{at}myologie.chups.jussieu.fr

The authors report 7 years of follow-up evaluation of a patient with coenzyme Q₁₀ (CoQ₁₀) deficiency. Initial symptoms of exercise intolerance and hyperlactatemia improved markedly with substitutive treatment. However, CoQ₁₀ supplementation did not prevent the onset of a cerebellar syndrome. A switch to idebenone treatment resulted in clinical and metabolic worsening, which disappeared with subsequent CoQ₁₀ treatment. CoQ₁₀ defects may cause progressive neurologic disease despite supplementation.

Received January 8, 2004. Accepted in final form April 14, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the August 24 issue to find the title link for this article.

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