Leber's Hereditary Optic Neuropathy (LHON)

Active Ingredient: Idebenone

Indication for Idebenone

Population group: only adolescents (12 years - 18 years old) , adults (18 years old or older)

Idebenone is indicated for the treatment of visual impairment in adolescent and adult patients with Leber’s Hereditary Optic Neuropathy (LHON).

For this indication, competent medicine agencies globally authorize below treatments:

900 mg in 3 divided doses daily

Route of admnistration

Oral

Defined daily dose

900 - 900 mg

Dosage regimen

From 300 To 300 mg 3 time(s) per day every day

Detailed description

The recommended dose is 900 mg/day idebenone (300 mg, 3 times a day).

No data regarding a continuous treatment with idebenone beyond 6 months is available from controlled clinical trials.

Dosage considerations

Idebenone should be administered with food because food increases the bioavailability of idebenone.

Active ingredient

Idebenone

Idebenone is an anti-oxidant assumed to be capable of transferring electrons directly to complex III of the mitochondrial electron transport chain, thereby restoring cellular energy (ATP) generation under experimental conditions of complex I deficiency. Similarly, in LHON idebenone can transfer electrons directly to complex III of the electron transport chain, thereby bypassing complex I which is affected by all three primary mtDNA mutations causing LHON, and restoring cellular ATP generation.

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