Idebenone
OTHER NAME(S): Hydroxydecyl Benzoquinone, Hyrdroxydecyl Ubiquinone, 2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4-benzoquinone., 10-hydroxydecyl, Idébénone, Idebenona
Overview
Idebenone is a man-made product. It is similar to coenzyme Q10.
Idebenone is most commonly used for Alzheimer disease, an inherited disorder that causes vision loss (Leber hereditary optic neuropathy), and a specific type of inherited disorder that causes muscle weakness and muscle loss (Duchenne muscular dystrophy). It is also used for skinwrinkles from sun damage and many other conditions, but there is no good scientific evidence to support these other uses.
Idebenone seems to have antioxidant activity, and appears to protect a wide variety of cells from oxidative damage.
Idebenone seems to have antioxidant activity, and appears to protect a wide variety of cells from oxidative damage.
Uses
Possibly Effective for
- Treating Alzheimer disease. There's some evidence that taking idebenone slows the decline of thinking skills in people with Alzheimer disease. Idebenone appears most effective in patients with moderately severe Alzheimer disease.
- An inherited condition that causes vision loss (Leber hereditary optic neuropathy). Taking idebenone seems to improve vision in people who have had this condition for less than a year. There's not enough information to know if starting idebenone improves vision in people who were diagnosed with this condition more than 1 year ago.
- A group of inherited disorders that cause muscle weakness and muscle loss (muscular dystrophy). There is some evidence that taking idebenone improves airway function, prevents airway infections, and slows long-term loss of airway function in children and teens with a specific type of muscular dystrophy, called Duchenne muscular dystrophy. But it doesn't seem to benefit people with this condition who are already being treated with steroids.
Possibly Ineffective for
- An inherited disease of the nerves and muscles (Friedreich ataxia). Most research shows that taking idebenone does not improve nerve or heart function in people with Friedreich ataxia.
There is interest in using idebenone for a number of other purposes, but there isn't enough reliable information to say whether it might be helpful.
Side Effects
When taken by mouth: Idebenone is POSSIBLY SAFE for most people when taken by mouth. Side effects are uncommon but can include nausea, vomiting, stomach pain, loose stools, a fast heartbeat, or increased risk of infection.
When applied to the skin: Idebenone is POSSIBLY SAFE when applied to the skin for a short period of time. Some people are allergic to idebenone when it is applied to the skin.
Precautions
When taken by mouth: Idebenone is POSSIBLY SAFE for most people when taken by mouth. Side effects are uncommon but can include nausea, vomiting, stomach pain, loose stools, a fast heartbeat, or increased risk of infection.
When applied to the skin: Idebenone is POSSIBLY SAFE when applied to the skin for a short period of time. Some people are allergic to idebenone when it is applied to the skin.
Pregnancy and breast-feeding: There isn't enough reliable information to know if idebenone is safe to use when pregnant or breast-feeding. Stay on the safe side and avoid use.
Children: Idebenone is POSSIBLY SAFE when taken by mouth by children who are at least 7 years old. Doses of 300 mg, taken three times each day, have been used safely for up to 12 months. In children who are at least 10 years old, it has been used safely for up to 6 years.
Interactions
We currently have no information for Idebenone overview.
Dosing
ADULTS
BY MOUTH
- For Alzheimer disease: 90-120 mg of idebenone three times daily.
- An inherited condition that causes vision loss (Leber hereditary optic neuropathy): 300 mg three times per day with a meal has been used.
CHILDREN
BY MOUTH
- A group of inherited disorders that cause muscle weakness and muscle loss (muscular dystrophy). 900 mg daily for six years has been used in patients aged 10 years and older with a specific type of muscular dystrophy, called Duchenne muscular dystrophy.
- An inherited condition that causes vision loss (Leber hereditary optic neuropathy): 300 mg three times per day with a meal has been used.
References
- 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. .
- 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.
- 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.
- Hausse AO, Aggoun Y, Bonnet D, et al. Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart 2002;87:346-9.
- 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.
- Schols L, Vorgerd M, Schillings M, et al. Idebenone in patients with Friedreich ataxia. Neurosci Lett 2001;306:169-72.
- 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.
- Anonymous. Idebenone - monograph. Altern Med Rev 2001;6:83-6.
- 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.
- 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.
- 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.
- 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. .
- Ikejiri Y, Mori E, Ishii K, et al. Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS. Neurology 1996;47:583-5.
- 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.
- 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.
- Mashima Y, Hiida Y, Oguchi Y. Remission of Leber's hereditary optic neuropathy with idebenone. Lancet 1992;340:368-9.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Catarino CB, von Livonius B, Priglinger C, et al. Real-world clinical experience with idebenone in the treatment of leber hereditary optic neuropathy. J Neuroophthalmol. 2020 Dec;40(4):558-565.
- Servais L, Straathof CSM, Schara U, et al. Long-term data with idebenone on respiratory function outcomes in patients with Duchenne muscular dystrophy. Neuromuscul Disord. 2020 Jan;30(1):5-16.
- Kosa P, Wu T, Phillips J, et al. Idebenone does not inhibit disability progression in primary progressive MS. Mult Scler Relat Disord. 2020 Oct;45:102434.
- Zhao X, Zhang Y, Lu L, Yang H. Therapeutic effects of idebenone on leber hereditary optic neuropathy. Curr Eye Res. 2020 Oct;45(10):1315-1323.
- Garegnani L, Hyland M, Roson Rodriguez P, Escobar Liquitay CM, Franco JV. Antioxidants to prevent respiratory decline in people with Duchenne muscular dystrophy and progressive respiratory decline. Cochrane Database Syst Rev 2021;12(12):CD013720.
- van Everdingen JAM, Pott JWR, Bauer NJC, Krijnen AM, Lushchyk T, Wubbels RJ. Clinical outcomes of treatment with idebenone in Leber's hereditary optic neuropathy in the Netherlands: A national cohort study. Acta Ophthalmol 2022;100(6):700-706.
- Yuan LL, Chen TY, Huang ZQ. Effects of paroxetine hydrochloride combined with idebenone on inflammatory factors and antioxidant molecules in treatment of depression after ischemic stroke. Pak J Med Sci 2023;39(1):17-22.