Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study.

Abstract

Respiratory failure can be the direct cause of death in patients with Leigh syndrome. Unfortunately, no effective treatment strategy is available. Here, we report successful treatment of a patient with Leigh syndrome using idebenone, a derivative of coenzyme Q-10. The patient's brainstem function, especially respiratory function, improved after idebenone treatment. Idebenone may be worth trying in patients with Leigh syndrome.

• Authors: Kazuhiro Haginoya, Shigeaki Miyabayashi, Masahiro Kikuchi, Akira Kojima, Katsuya Yamamoto, Kiyoshi Omura, Mitsugu Uematsu, Naomi Hino-Fukuyo, Soichiro Tanaka, Shigeru Tsuchiya
• Journal: Journal of the neurological sciences (J Neurol Sci) Vol. 278 Issue 1-2 Pg. 112-4 (Mar 15 2009) ISSN: 0022-510X [Print] Netherlands
• PMID: 19101701 (Publication Type: Case Reports, Journal Article)

Chemical References

• Ubiquinone
• Lactic Acid
• idebenone

Topics

• Adolescent
• Brain Stem (drug effects, physiopathology)
• Evoked Potentials, Auditory, Brain Stem (drug effects)
• Follow-Up Studies
• Humans
• Lactic Acid (cerebrospinal fluid)
• Leigh Disease (complications)
• Male
• Polysomnography
• Respiration (drug effects)
• Respiratory Insufficiency (drug therapy, etiology)
• Ubiquinone (analogs & derivatives, therapeutic use)