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Hashemi Eye Care - Neuroscience Institute and Research Center

Idebenone (Raxone)

A medication used for Leber hereditary optic neuropathy that may help preserve or restore vision when started early.

Drug Class: mitochondrial

2 min read

Idebenone (brand name Raxone) is a synthetic analogue of coenzyme Q10 used to treat Leber hereditary optic neuropathy (LHON). It may help preserve vision or promote recovery when started early in the disease course.

Key Takeaways

  • Used for LHON—the most common inherited mitochondrial optic neuropathy
  • Works best when started early
  • Supports mitochondrial function
  • Most effective in patients with some remaining vision
  • Generally well-tolerated

How It Works

LHON is caused by mitochondrial dysfunction in the optic nerve. Idebenone can transfer electrons in the mitochondrial respiratory chain, potentially bypassing the defective complex caused by LHON mutations. This may help maintain or restore energy production in surviving nerve cells.

Common Uses

  • Leber hereditary optic neuropathy
  • May help carriers before vision loss occurs (research ongoing)

Dosing

  • Usually 300mg three times daily (900mg total)
  • Take with fatty food (improves absorption)
  • May need long-term treatment

Expected Benefits

  • May preserve remaining vision
  • Some patients experience visual recovery
  • More effective when started early
  • Best results in patients with the 11778 mutation who still have some vision

Side Effects

Common

  • Mild GI symptoms (nausea, diarrhea)
  • Urine color change (orange/red—harmless)
  • Headache

Less Common

  • Elevated liver enzymes
  • Fatigue

Monitoring

Tips for Taking Idebenone

  • Take with meals containing fat (improves absorption by 5-7 fold)
  • Orange/red urine is normal and harmless
  • Continue even if no immediate improvement—benefits may take time

Frequently Asked Questions

How effective is idebenone?

Results vary. Some patients see significant visual improvement; others stabilize without further loss. Early treatment (before significant vision loss) gives better results.

Why take it with fatty food?

Idebenone is fat-soluble and absorbs much better (5-7 times more) when taken with food containing fat.

How long do I need to take it?

Duration is not well-established. Many patients continue long-term, especially those who have responded. Discuss with your doctor.

Is this the same as CoQ10?

Idebenone is a synthetic analogue of CoQ10 with better penetration into cells and brain tissue. Regular CoQ10 supplements are not a substitute.

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