IDEBENONE

OTHER NAME(S):

2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4-benzoquinone, Hydroxydecyl Benzoquinone, Hydroxydecyl Ubiquinone, Idebenona, Idébénone.<br/><br/>

Overview

Overview Information

Idebenone is a man-made product. It is similar to coenzyme Q-10.

Idebenone is used for Alzheimer's disease, Huntington’s disease, liver disease, and heart disease. It is also used for a number of inherited disorders including a condition that causes vision loss (Leber’s hereditary optic neuropathy), mitochondrial encephalomyopathies (nerve and muscle disorders), a type of muscular dystrophy called Duchenne muscular dystrophy, and Friedreich's ataxia (a nervous system disorder that worsens over time, producing gait and speech problems, and eventually leading toward heart disease and diabetes).

How does it work?

Idebenone seems to have antioxidant activity, and appears to protect a wide variety of cells from oxidative damage.

Uses

Uses & Effectiveness?

Possibly Effective for

  • Treating Alzheimer's disease. There's some evidence that taking idebenone slows the decline of thinking skills in people with Alzheimer's disease. Idebenone appears most effective in patients with moderately severe Alzheimer's disease.
  • A specific type of muscular dystrophy called Duchenne muscular dystrophy. There is some evidence that taking idebenone improves airway function and prevents airway infections in children and teens with Duchenne muscular dystrophy who are not already being treated with steroids. It doesn’t seems to benefit people with this condition who are already being treated with steroids.
  • An inherited condition that causes vision loss (Leber’s hereditary optic neuropathy). Taking idebenone seems to improve vision in people with early stage Leber’s disease. There’s not enough information to know if idebenone improves vision in people diagnosed with this condition more than 1 year ago.

Possibly Ineffective for

  • An inherited progressive condition affecting the nerves that can lead to heart disease and diabetes (Friedreich's ataxia). Most research shows that taking idebenone does not improve nerve or heart function in people with Friedreich’s ataxia.

Insufficient Evidence for

  • Mitochondrial encephalomyopathies (a group of disorders that lead to muscle and nervous system problems). There is some research that suggests that taking idebenone might reduce symptoms in patients with some of these conditions.
  • Wrinkled skin. Early research shows that applying a lotion containing idebenone might reduce wrinkles and improve skin quality in middle-aged women.
  • Other conditions.
More evidence is needed to rate the effectiveness of idebenone for these uses.

Side Effects

Side Effects & Safety

Idebenone is POSSIBLY SAFE for most people when taken by mouth and when used on the skin. Side effects are uncommon but can include nausea, vomiting, stomach pain, loose stools, a fast heartbeat, or increased risk of infection. Some people are allergic to idebenone when it is applied to the skin.

Special Precautions & Warnings:

Pregnancy and breast-feeding: Not enough is known about the use of idebenone during pregnancy and breast-feeding. Stay on the safe side and avoid use.

Interactions

Interactions?

We currently have no information for IDEBENONE Interactions.

Dosing

Dosing

The following doses have been studied in scientific research:

ADULTS

BY MOUTH:

  • For Alzheimer's disease: 90-120 mg of idebenone three times daily.
  • An inherited condition that causes vision loss (Leber's hereditary optic neuropathy): 300 mg three times per day with a meal has been used.
CHILDREN

BY MOUTH:
  • For Duchenne muscular dystrophy. 900 mg daily for one year has been used in patients aged 10 years and up.
  • An inherited condition that causes vision loss (Leber’s hereditary optic neuropathy): 300 mg three times per day with a meal has been used.

View References

REFERENCES:

  • Anonymous. Idebenone - monograph. Altern Med Rev 2001;6:83-6.
  • Artuch R, Colome C, Vilaseca MA, et al. Monitoring of idebenone treatment in patients with Friedreich's ataxia by high-pressure liquid chromatography with electrochemical detection. J Neurosci Methods 2002;115:63-6. View abstract.
  • Buyse G, Mertens L, Di Salvo G, et al. Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring. Neurology 2003;60:1679-81. . View abstract.
  • Buyse GM, Goemans N, van den Hauwe M, Meier T. Effects of glucocorticoids and idebenone on respiratory function in patients with duchenne muscular dystrophy. Pediatr Pulmonol. 2013;48(9):912-20.View abstract.
  • Buyse GM, Voit T, Schara U, DELOS Study Group. Efficacy of idebenone on respiratory function in patients with Duchenne muscular dystrophy not using glucocorticoids (DELOS): a double-blind randomised placebo-controlled phase 3 trial. Lancet. 2015;385(9979):1748-1757. View abstract.
  • Carelli V, Carbonelli M, de Coo IF, et al. International consensus statement on the clinical and therapeutic management of Leber hereditary optic neuropathy. J Neuroophthalmol. 2017;37(4):371-381. View abstract.
  • Corben LA, Lynch D, Pandolfo M, Schulz JB, Delatycki MB; Clinical Management Guidelines Writing Group. Consensus clinical management guidelines for Friedreich ataxia. Orphanet J Rare Dis. 2014;9:184. View abstract.
  • Di Prospero NA, Baker A, Jeffries N, Fischbeck KH. Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial. Lancet Neurol. 2007;6(10):878-86. View abstract.
  • Di Prospero NA, Sumner CJ, Penzak SR, et al. Safety, tolerability, and pharmacokinetics of high-dose idebenone in patients with Friedreich ataxia. Arch Neurol 2007;64:803-8. View abstract.
  • Esposti MD, Ngo A, Ghelli A, et al. The interaction of Q analogs, particularly hydroxydecyl benzoquinone (idebenone), with the respiratory complexes of heart mitochondria. Arch Biochem Biophys 1996;330:395-400. View abstract.
  • Filla A, Moss AJ. Idebenone for treatment of Friedreich's ataxia? Neurology 2003;60:1569-70.
  • Geromel V, Darin N, Chretien D, et al. Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits. Mol Genet Metab 2002;77:21-30. View abstract.
  • Gutzmann H, Hadler D. Sustained efficacy and safety of idebenone in the treatment of Alzheimer's disease: update on a 2-year double-blind multicentre study. J Neural Transm 1998;54:301-10. View abstract.
  • Hausse AO, Aggoun Y, Bonnet D, et al. Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart 2002;87:346-9. View abstract.
  • Ihara Y, Namba R, Kuroda S, et al. Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone. J Neurol Sci 1989;90:263-71. View abstract.
  • Ikejiri Y, Mori E, Ishii K, et al. Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS. Neurology 1996;47:583-5. View abstract.
  • Klopstock T, Metz G, Yu-Wai-Man P, et al. Persistence of the treatment effect of idebenone in Leber's hereditary optic neuropathy. Brain. 2013;136(Pt 2):e230. View abstract.
  • Klopstock T, Yu-Wai-Man P, Dimitriadis K, et al. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain. 2011;134(Pt 9):2677-86. View abstract.
  • Lagedrost SJ, Sutton MS, Cohen MS, et al. Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA). Am Heart J. 2011;161(3):639-645.e1. View abstract.
  • Lerman-Sagie T, Rustin P, Lev D, et al. Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone. J Inherit Metab Dis 2001;24:28-34. View abstract.
  • Lynch DR, Perlman SL, Meier T. A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia. Arch Neurol. 2010;67(8):941-7. View abstract.
  • Mariotti C, Solari A, Torta D, et al. Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial. Neurology 2003;60:1676-9. View abstract.
  • Mashima Y, Hiida Y, Oguchi Y. Remission of Leber's hereditary optic neuropathy with idebenone. Lancet 1992;340:368-9. View abstract.
  • Mc Aleer MA, Collins P. Allergic contact dermatitis to hydroxydecyl ubiquinone (idebenone) following application of anti-ageing cosmetic cream. Contact Dermatitis. 2008;59(3):178-9. View abstract.
  • McDaniel DH, Neudecker BA, DiNardo JC, Lewis JA 2nd, Maibach HI. Clinical efficacy assessment in photodamaged skin of 0.5% and 1.0% idebenone. J Cosmet Dermatol. 2005;4(3):167-73. View abstract.
  • McDonald CM, Meier T, Voit T; DELOS Study Group. Idebenone reduces respiratory complications in patients with Duchenne muscular dystrophy. Neuromuscul Disord. 2016;26(8):473-80. View abstract.
  • Meier T, Perlman SL, Rummey C, Coppard NJ, Lynch DR. Assessment of neurological efficacy of idebenone in pediatric patients with Friedreich's ataxia: data from a 6-month controlled study followed by a 12-month open-label extension study. J Neurol. 2012;259(2):284-91. View abstract.
  • Pineda M, Arpa J, Montero R, et al. Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up. Eur J Paediatr Neurol. 2008;12(6):470-5. View abstract.
  • Pisano P, Durand A, Autret E, et al. Plasma concentrations and pharmacokinetics of idebenone and its metabolites following single and repeated doses in young patients with mitochondrial encephalomyopathy. Eur J Clin Pharmacol 1996;51:167-9. View abstract.
  • Rego AC, Santos MS, Oliveira CR. Influence of the antioxidants vitamin E and idebenone on retinal cell injury mediated by chemical ischemia, hypoglycemia, or oxidative stress. Free Radic Biol Med 1999;26:1405-17. View abstract.
  • Rustin P, von Kleist-Retzow JC, Chantrel-Groussard K, et al. Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study. Lancet 1999;354:477-9. View abstract.
  • Schols L, Vorgerd M, Schillings M, et al. Idebenone in patients with Friedreich ataxia. Neurosci Lett 2001;306:169-72. View abstract.
  • Shivaram KN, Winklhofer-Roob BM, Straka MS, et al. The effect of idebenone, a coenzyme Q analogue, on hydrophobic bile acid toxicity to isolated rat hepatocytes and hepatic mitochondria. Free Radic Biol Med 1998;25:480-92. View abstract.
  • Weyer G, Babej-Dolle RM, Hadler D, et al. A controlled study of 2 doses of idebenone in the treatment of Alzheimer's disease. Neuropsychobiology 1997;36:73-82. . View abstract.

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CONDITIONS OF USE AND IMPORTANT INFORMATION: This information is meant to supplement, not replace advice from your doctor or healthcare provider and is not meant to cover all possible uses, precautions, interactions or adverse effects. This information may not fit your specific health circumstances. Never delay or disregard seeking professional medical advice from your doctor or other qualified health care provider because of something you have read on WebMD. You should always speak with your doctor or health care professional before you start, stop, or change any prescribed part of your health care plan or treatment and to determine what course of therapy is right for you.

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© Therapeutic Research Faculty 2018.