The purpose of this survey is to collect visual acuity data from patients with LHON in order to establish the clinical course (natural history) and visual acuity outcomes in patients with a genetically confirmed diagnosis of LHON. In addition, this survey will generate data that will serve as comparator for the open-label study SNT-IV-006.

NCT02796274

Conditions

1. Leber's Hereditary Optic Neuropathy (LHON)

MeSH:Optic Nerve Diseases Optic Atrophy, Hereditary, Leber

HPO:Leber optic atrophy

Clinically Relevant Recovery (CRR) is defined as a VA improvement from off-chart (ETDRS) to 5 letters on-chart, or an on-chart improvement of 10 letters.. Inclusion Criteria: 1. age ≥ 12 years 2. the onset of symptoms is dated after 1999 and is well documented (at least month of onset of symptoms is known for each eye) 3. at least two VA assessments are available within 5 years of onset of symptoms and prior to idebenone use 4. have a genetic diagnosis for LHON for one of the following mitochondrial DNA (mtDNA) mutations: G11778A, G3460A, T14484C Exclusion Criteria: 1. any participation in an interventional clinical trial after the onset of symptoms 2. any other cause of visual impairment (e.g. --- G11778A --- --- G3460A ---

glaucoma, diabetic retinopathy, AIDS related visual impairment, cataract, macular degeneration, etc.) or any active ocular disorder (uveitis, infections, inflammatory retinal disease, thyroid eye disease, etc.) during the data collection period Inclusion Criteria: 1. age ≥ 12 years 2. the onset of symptoms is dated after 1999 and is well documented (at least month of onset of symptoms is known for each eye) 3. at least two VA assessments are available within 5 years of onset of symptoms and prior to idebenone use 4. have a genetic diagnosis for LHON for one of the following mitochondrial DNA (mtDNA) mutations: G11778A, G3460A, T14484C Exclusion Criteria: 1. any participation in an interventional clinical trial after the onset of symptoms 2. any other cause of visual impairment (e.g. --- G11778A --- --- G3460A ---

This study is meant to assess the effectiveness of idebenone on visual function measures in patients with Leber's Hereditary Optic Neuropathy over a 6 months period.

NCT00747487

Conditions

1. Leber's Hereditary Optic Neuropathy

Interventions

1. Drug: Idebenone
2. Drug: Placebo

MeSH:Peripheral Nervous System Diseases Optic Nerve Diseases Optic Atrophy, Hereditary, Leber

HPO:Abnormal peripheral nervous system morphology Leber optic atrophy Peripheral neuropathy Polyneuropathy

Inclusion Criteria: - Age > or = 14 years and < 65 years - Impaired visual acuity in at least one eye due to LHON - Onset of visual loss due to LHON lies five years or less prior to Baseline - Confirmation of either G11778A, T14484C or G3460A LHON mtDNA mutations at >60% in blood - No explanation for the visual failure besides LHON - Body weight ≥ 45 kg - Negative urine pregnancy test at Screening and at Baseline (women of childbearing potential). --- G11778A --- --- T14484C --- --- G3460A ---

Exclusion Criteria: - Treatment with Coenzyme Q10 or idebenone within 1 month prior to Baseline - Pregnancy and/or breast-feeding - Weekly alcohol intake 35 units (men) or 24 units (women) - Current drug abuse - Clinically significant abnormalities of clinical haematology or biochemistry including, but not limited to, elevations greater than 2 times the upper limit of normal of AST, ALT or creatinine - Participation in another clinical trial of any investigational drug within 3 months prior to Baseline - Other factor that, in the investigator's opinion, excludes the patient from entering the study Inclusion Criteria: - Age > or = 14 years and < 65 years - Impaired visual acuity in at least one eye due to LHON - Onset of visual loss due to LHON lies five years or less prior to Baseline - Confirmation of either G11778A, T14484C or G3460A LHON mtDNA mutations at >60% in blood - No explanation for the visual failure besides LHON - Body weight ≥ 45 kg - Negative urine pregnancy test at Screening and at Baseline (women of childbearing potential). --- G11778A --- --- T14484C --- --- G3460A ---

LEROS is an open-label interventional Phase IV study, designed to further assess the efficacy and safety of Raxone® in the long-term treatment of LHON patients.

NCT02774005

Conditions

1. Leber's Hereditary Optic Neuropathy (LHON)

Interventions

1. Drug: Idebenone

MeSH:Optic Nerve Diseases Optic Atrophy, Hereditary, Leber

HPO:Leber optic atrophy

Inclusion Criteria: 1. Impaired visual acuity in affected eyes due to LHON 2. No explanation for visual loss besides LHON 3. Age more or equal 12 years 4. Onset of symptoms ≤5 years of Baseline 5. Confirmation of either G11778A, G3460A or T14484C LHON mtDNA (for the ITT population, not required for enrolment) 6. Written informed consent obtained from the patient 7. Ability and willingness to comply with study procedures and visits 8. Women of Childbearing Potential (WCBP) who have a negative urine or serum pregnancy test at Baseline visit and who are willing to use a highly effective contraceptive measure and maintain it until treatment discontinuation. --- G11778A --- --- G3460A ---

8. Women who are pregnant or have a positive pregnancy test at Baseline visit 9. Women who are breastfeeding Inclusion Criteria: 1. Impaired visual acuity in affected eyes due to LHON 2. No explanation for visual loss besides LHON 3. Age more or equal 12 years 4. Onset of symptoms ≤5 years of Baseline 5. Confirmation of either G11778A, G3460A or T14484C LHON mtDNA (for the ITT population, not required for enrolment) 6. Written informed consent obtained from the patient 7. Ability and willingness to comply with study procedures and visits 8. Women of Childbearing Potential (WCBP) who have a negative urine or serum pregnancy test at Baseline visit and who are willing to use a highly effective contraceptive measure and maintain it until treatment discontinuation. --- G11778A --- --- G3460A ---