Idebenone

Idebenone is a prescription medication used primarily to treat Leber's Hereditary Optic Neuropathy (LHON), a rare genetic disorder that causes sudden vision loss. Idebenone is used for Friedreich's ataxia and Leber's hereditary optic neuropathy (LHON).

Also called: Kirkman (United States)

Pronunciation: (eye-DEB-eh-known).

Generic name: idebenone
Brand names: Ideben, Neurox, Raxone, Idenone, Catena
Drug class: General Medical Info

Availability and supply rules vary by country

Trade names by country: United States · India

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What idebenone is and how it works

Idebenone is a prescription medication used primarily to treat Leber's Hereditary Optic Neuropathy (LHON), a rare genetic disorder that causes sudden vision loss. It is also being studied for other mitochondrial and neurodegenerative diseases. Idebenone works by supporting the function of mitochondria, the energy-producing structures in your cells. It acts as an antioxidant, helping to protect nerve cells from damage caused by oxidative stress. Regulatory note: under the brand name Raxone, idebenone has been approved for LHON in the European Union (since 2015), the UK, Switzerland, and several other countries. As of August 2026, it is NOT approved by the FDA in the United States — after a Priority Review, the FDA issued a Complete Response Letter rather than an approval. Availability therefore depends on the country. Patients should know that idebenone is not a cure for LHON, but it may help slow or partially reverse vision loss if started early. It is important to take the medication exactly as prescribed and to attend regular follow-up appointments to monitor progress and side effects. (2)

Uses, formulations, monitoring, and supply rules can differ by patient, product, and country. Follow the exact local product information and professional advice.

Mechanism of action: how idebenone works

Idebenone is a synthetic short-chain benzoquinone structurally related to coenzyme Q10. It acts as an electron carrier in the mitochondrial respiratory chain, bypassing complex I defects and restoring cellular ATP production. Its antioxidant properties help reduce oxidative stress and protect neuronal cells from free radical-induced damage, particularly in mitochondrial disorders such as LHON.

What is Idebenone used for?

Friedreich's ataxia

Investigational/off-label use only — idebenone is not an approved treatment for Friedreich's ataxia in the countries where it is approved (its approved indication is LHON); any use here would be specialist-directed and outside standard labeling. (2)

Leber's hereditary optic neuropathy (LHON)

The approved indication for idebenone (brand name Raxone) in the European Union, UK, Switzerland, and several other countries, for adolescent and adult patients with visual impairment from LHON. Not FDA-approved in the United States as of August 2026. (2)

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome

Investigational/off-label use only — idebenone is not an approved treatment for MELAS; any use here would be specialist-directed and outside standard labeling. (2)

Before taking idebenone

Do not take / not appropriate for

Do not take idebenone (Raxone) if you are hypersensitive to idebenone or to any of the other ingredients of the tablet. This is the only absolute contraindication in the EU Summary of Product Characteristics. (European Union / European Economic Area)
Raxone tablets contain lactose. Patients with rare hereditary problems of galactose intolerance, total lactase deficiency, or glucose-galactose malabsorption should not take Raxone. (European Union / European Economic Area)
Idebenone is not approved by the US Food and Drug Administration for any medical use, so there is no US prescribing information and no US-authorised medicine to take. Products sold in the United States containing idebenone are cosmetics or supplements, not the licensed medicine described here. (United States)

Tell your clinician about

Any allergy to idebenone or to tablet ingredients, and any intolerance of lactose or of the colouring sunset yellow (E110), which may cause allergic reactions.
Any liver or kidney problems — caution is advised, and liver problems have led to treatment being interrupted or stopped.
If you are pregnant, could become pregnant, or are planning a pregnancy — safety in pregnancy has not been established, and idebenone should only be used if the benefit is judged to outweigh the potential risk.
If you are breastfeeding — idebenone passes into milk in animals and a risk to the infant cannot be excluded, so you and your doctor must decide whether to stop breastfeeding or stop treatment.
Every other medicine you take, especially digoxin, dabigatran, aliskiren, ciclosporin, tacrolimus, sirolimus, fentanyl, quinidine, pimozide or an ergot medicine.
If your urine turns reddish-brown — this chromaturia is a harmless effect of idebenone, but tell your doctor so that other causes are not missed.

Idebenone dosage and how to take it

General Instructions: Take idebenone exactly as prescribed by your doctor. Take it WITH FOOD — food increases how much idebenone your body absorbs by roughly 5 to 7 times, so taking it on an empty stomach can make it much less effective.. Adult Dosing: The approved dose for LHON is 900 mg per day, divided into three doses of 300 mg each.. Pediatric Dosing: For adolescents aged 12 years and older, the dosing is generally the same as adults. Safety and efficacy in children under 12 have not been established.. Administration: Swallow tablets whole with a glass of water, with food. Do not crush or chew the tablets.. When To Stop: Do not stop taking idebenone without consulting your doctor, even if you feel better. Stopping suddenly may reduce its effectiveness.. Storage: Store at room temperature (below 25°C/77°F), away from moisture and heat; the manufacturer states no special storage conditions are required. Keep out of reach of children.
This page does not provide a dose calculator. Dosing must follow the prescriber, pharmacist, or product label.

(2)

Procedures and treatment changes

Tell the treating team before surgery, procedures, or any medicine change. Do not alter treatment without the responsible clinician. (2)

Missed dose

If you miss a dose, take it as soon as you remember. If it is almost time for your next dose, skip the missed dose and resume your regular schedule. Do not double the dose to catch up.

Overdose

If you or someone else takes too much idebenone, call your local poison control center or seek emergency medical attention immediately. Symptoms of overdose may include severe gastrointestinal upset or liver dysfunction. (2)

Idebenone side effects

Side effects and their urgency vary; report severe, persistent, or concerning symptoms to a healthcare professional.