Page last updated: 2024-10-28

idebenone and Hereditary Optic Neuroretinopathy

idebenone has been researched along with Hereditary Optic Neuroretinopathy in 46 studies

Research Excerpts

ExcerptRelevanceReference
"Idebenone was well tolerated, with most adverse events classified as minor."2.94Real-World Clinical Experience With Idebenone in the Treatment of Leber Hereditary Optic Neuropathy. ( Acaroglu, G; Banik, R; Black, GCM; Castillo, L; Catarino, CB; Doncel, C; Fraser, CL; Friedburg, C; Halfpenny, CA; Jakubaszko, J; Klopstock, T; Landau, K; Langenegger, SJ; Lincoln, JA; Llòria, X; Lob, F; Matloob, S; Metz, G; Mikazans, O; Muñoz-Negrete, FJ; Newman, NJ; Poulton, J; Priglinger, C; Rudolph, G; Scoppettuolo, E; Silva, M; Subramanian, P; Tamhankar, MA; Toosy, AT; Traber, GL; Vidal, M; Vincent, AL; von Livonius, B; Votruba, M; Zarowski, M; Zermansky, A, 2020)
"Treatment with idebenone improved tritan color vision compared with placebo (P = 0."2.78Effects of idebenone on color vision in patients with leber hereditary optic neuropathy. ( Al-Tamami, J; Büchner, B; Dimitriadis, K; Heck, S; Klopstock, T; Leinonen, M; Meier, T; Rudolph, G; Rummey, C; Seidensticker, F, 2013)
"Idebenone was safe and well tolerated."2.76A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. ( Atawan, A; Bailie, M; Chattopadhyay, S; Chinnery, PF; Dimitriadis, K; Garip, A; Griffiths, PG; Heck, S; Kernt, M; Klopstock, T; Leinonen, M; Meier, T; Metz, G; Petraki, D; Rouleau, J; Rummey, C; Schubert, M; Yu-Wai-Man, P, 2011)
"Idebenone is a rapidly absorbed, safe and well-tolerated drug and is currently the only clinically proven treatment option for Leber's hereditary optic neuropathy (LHON) patients."2.53Idebenone for Leber's hereditary optic neuropathy. ( Gueven, N, 2016)
"Idebenone has been proposed as a means of bypassing defective complex I activity and a free radical scavenger to prevent oxidative damage."2.52Is there treatment for Leber hereditary optic neuropathy? ( Newman, NJ; Peragallo, JH, 2015)
"Leber's hereditary optic neuropathy is a rare genetic disorder affecting the retinal ganglion cells leading to a persistent severe bilateral loss of visual acuity within weeks or months."2.50[Leber's hereditary optic neuropathy - phenotype, genetics, therapeutic options]. ( Gallenmüller, C; Klopstock, T, 2014)
"Two months later, the diagnosis of Leber hereditary optic neuropathy (LHON) 11778/ND4 was established and the patient was treated with 900 mg of idebenone daily."2.48Is Leber hereditary optic neuropathy treatable? Encouraging results with idebenone in both prospective and retrospective trials and an illustrative case. ( Khaderi, KR; Sabet-Peyman, EJ; Sadun, AA, 2012)
"Oral treatment with idebenone 300 mg tid was started in seven patients 5 to 51 years after LHON onset."1.51Visual function in chronic Leber's hereditary optic neuropathy during idebenone treatment initiated 5 to 50 years after onset. ( Kircher, K; Pemp, B; Reitner, A, 2019)
"Idebenone is now a treatment option for LHON."1.48A teenager with acute bilateral visual loss. ( Jørstad, ØK; Kerty, E; Ødegaard, EM, 2018)
"Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to <0."1.46Visual prognosis better in eyes with less severe reduction of visual acuity one year after onset of Leber hereditary optic neuropathy caused by the 11,778 mutation. ( Kigasawa, K; Mashima, Y; Oguchi, Y; Shinoda, K; Wakakura, M, 2017)
"He was diagnosed to have Leber's hereditary optic neuropathy with genetic workup showing G11778A mutation with over 80% heteroplasmy."1.40Novel use of idebenone in Leber's hereditary optic neuropathy in Hong Kong. ( Cheng, SW; Ko, CH; Lee, CY; Mak, C; Yau, SK; Yuen, YF, 2014)
" Consistent with the reported dosing and observed effects in LHON patients, we describe that in mice, idebenone penetrated into the eye at concentrations equivalent to those which protected RGC-5 cells from complex I dysfunction in vitro."1.38Idebenone protects against retinal damage and loss of vision in a mouse model of Leber's hereditary optic neuropathy. ( Anklin, C; Erb, M; Gueven, N; Heitz, FD; Pernet, V; Robay, D, 2012)

Research

Studies (46)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (4.35)29.6817
2010's28 (60.87)24.3611
2020's16 (34.78)2.80

Authors

AuthorsStudies
Varricchio, C1
Beirne, K1
Aeschlimann, P1
Heard, C1
Rozanowska, M1
Votruba, M4
Brancale, A1
Varela-Fernández, R2
Lema-Gesto, MI2
González-Barcia, M2
Otero-Espinar, FJ2
Chan, JW1
Sultan, W1
Karanjia, R1
Sadun, AA4
Bendicho-Lavilla, C1
Martin-Pastor, M1
Herrero Vanrell, R1
van Everdingen, JAM1
Pott, JWR2
Bauer, NJC1
Krijnen, AM1
Lushchyk, T1
Wubbels, RJ1
Borrelli, E1
Berni, A1
Cascavilla, ML1
Barresi, C1
Battista, M1
Lari, G1
Reibaldi, M1
Bandello, F1
Barboni, P3
Schworm, B1
Siedlecki, J1
Catarino, C1
von Livonius, B4
Muth, DR1
Rudolph, G4
Havla, J1
Klopstock, T9
Priglinger, C3
Baglivo, M1
Nasca, A1
Lamantea, E1
Vinci, S1
Spagnolo, M1
Marchet, S1
Prokisch, H1
Catania, A1
Lamperti, C1
Ghezzi, D1
Pemp, B1
Kircher, K1
Reitner, A1
Finsterer, J2
Catarino, CB3
Banik, R1
Matloob, S1
Tamhankar, MA1
Castillo, L1
Friedburg, C1
Halfpenny, CA1
Lincoln, JA1
Traber, GL1
Acaroglu, G1
Black, GCM1
Doncel, C1
Fraser, CL1
Jakubaszko, J1
Landau, K2
Langenegger, SJ1
Muñoz-Negrete, FJ1
Newman, NJ6
Poulton, J1
Scoppettuolo, E1
Subramanian, P1
Toosy, AT1
Vidal, M1
Vincent, AL1
Zarowski, M1
Zermansky, A1
Lob, F1
Mikazans, O1
Silva, M1
Llòria, X1
Metz, G3
Ishikawa, H2
Masuda, Y1
Shikisima, K1
Goseki, T1
Kezuka, T1
Terao, M1
Miyazaki, A1
Matsumoto, K1
Nishikawa, H1
Gomi, F1
Mimura, O1
Tonagel, F1
Wilhelm, H1
Richter, P1
Kelbsch, C1
Wang, Y1
Hu, LF1
Cui, PF1
Qi, LY1
Xing, L1
Jiang, HL1
Bianco, A1
Bisceglia, L1
Russo, L1
Palese, LL1
D'Agruma, L1
Emperador, S1
Montoya, J1
Guerriero, S2
Petruzzella, V1
Bulwa, Z1
Nichols, J1
Gupta, N1
Carelli, V3
Carbonelli, M2
de Coo, IF1
Kawasaki, A1
Lagrèze, WA2
La Morgia, C2
Orssaud, C2
van Everdingen, J1
Vignal-Clermont, C1
Yu-Wai-Man, P4
Mashima, Y1
Kigasawa, K1
Shinoda, K1
Wakakura, M1
Oguchi, Y1
Jørstad, ØK2
Ødegaard, EM2
Heimdal, KR1
Kerty, E2
Theodorou-Kanakari, A1
Karampitianis, S1
Karageorgou, V1
Kampourelli, E1
Kapasakis, E1
Theodossiadis, P1
Chatziralli, I1
Chang, M1
Bidot, S1
Lamirel, C1
Brémond Gignac, D1
Touitou, V1
Vignal, C1
Iyer, S1
Gallenmüller, C2
Cheng, SW1
Ko, CH1
Yau, SK1
Mak, C1
Yuen, YF1
Lee, CY1
Peragallo, JH1
Eckenweiler, M1
Gallenmueller, C1
Korinthenberg, R1
Kirschner, J1
Lyseng-Williamson, KA1
Schiff, M1
Rustin, P1
Gueven, N2
Soiferman, D1
Moore, DG1
Burté, F1
Saada, A1
Szewka, AJ1
Toffoli, D1
Biousse, V1
Dimitriadis, K2
Rouleau, J2
Heck, S2
Bailie, M2
Atawan, A1
Chattopadhyay, S1
Schubert, M1
Garip, A1
Kernt, M1
Petraki, D1
Rummey, C2
Leinonen, M2
Griffiths, PG2
Meier, T3
Chinnery, PF2
Valentino, ML2
Rizzo, G1
De Negri, AM1
Sadun, F1
Carta, A1
Simonelli, F1
Aggarwal, D1
Liguori, R2
Avoni, P1
Baruzzi, A2
Zeviani, M1
Montagna, P2
Sabet-Peyman, EJ1
Khaderi, KR1
Heitz, FD1
Erb, M1
Anklin, C1
Robay, D1
Pernet, V1
Büchner, B2
Al-Tamami, J1
Seidensticker, F1
Nwali, N1
Reznicek, L1
Coppard, N1
Barnils, N1
Mesa, E1
Muñoz, S1
Ferrer-Artola, A1
Arruga, J1
Meletti, S1
Vetrugno, R1
Provini, F1
Mancardi, GL1
Bandini, F1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
A Double-Blind, Randomised, Placebo-Controlled Study of the Efficacy, Safety and Tolerability of Idebenone in the Treatment of Patients With Leber's Hereditary Optic Neuropathy[NCT00747487]Phase 285 participants (Actual)Interventional2007-11-30Completed
Safety and Efficacy Study of a Single Intravitreal Injection of rAAV2-ND4 Treatment of Leber Hereditary Optic Neuropathy[NCT01267422]9 participants (Actual)Interventional2011-04-30Completed
A Randomized, Double-blinded, Placebo-controlled Trial of Idebenone in the Prevention of Episodic Migraine[NCT04151472]Phase 3180 participants (Anticipated)Interventional2021-12-08Recruiting
A Single Visit, Observational, Follow-up Study of Patients With Leber's Hereditary Optic Neuropathy Following Participation in SNT-II-003 Trial[NCT01421381]60 participants (Actual)Observational2011-09-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Intraocular Pressure;

(NCT01267422)
Timeframe: Up to 3 years

InterventionmmHg (Mean)
IOP Before Treatment14
IOP After Treatment16

Neutralizing Antibody Assay

The mean of Neutralizing antibody assay of 8 patients before and after treatment (NCT01267422)
Timeframe: up to 3 years

Interventiontiter (Mean)
The Mean of Neutralizing Antibody Assay Before Treatment0.9221
The Mean of Neutralizing Antibody Assay After Treatment0.8723

Average RNFL Thickness Througth Optical Coherence Tomography(OCT) Test

Average RNFL thickness of 8 patients througth Optical coherence tomography(OCT) test before and after treatment (NCT01267422)
Timeframe: Up to 3 years

,
InterventionMicrometer (Mean)
injected eyeuninjected eye
Average RNFL Thickness After Treatment46.781347.9688
Average RNFL Thickness Before Treatment47.312550.1875

Computerized Visual Field(MD: Mean Deviation, the Value Close to 0 Regarded Normal)

MD: mean deviation, the value Close to 0 regarded normal.VFI/MD:The bigger one was more close to the normal value. (NCT01267422)
Timeframe: up to 3 years

,
InterventiondB (Mean)
injected eyeuninjected eye
Mean MD After Treatment-23.813-23.427
Mean MD Before Treatment-29.377-28.701

Computerized Visual Field(VFI: Visual Field Index ,the Value Close to 100% Regarded Normal)

VFI: visual field index ,the value Close to 100% regarded normal. VFI/MD:The bigger one was more close to the normal value. (NCT01267422)
Timeframe: up to 3 years

,
InterventionPercentage of normal (Mean)
injected eyeuninjected eye
Mean VFI After Treatment2825
Mean VFI Before Treatment1110.5

Results of CD3/CD4/CD8 Test

The mean percentage of CD3+/CD4+/CD8+ test before and after treatment (NCT01267422)
Timeframe: up to 6 months

,
InterventionPercentage of total cells (Mean)
Values of CD3+Values of CD4+Values of CD8+
The Mean Percentage of CD3+/CD4+/CD8+ After Treatment532013
The Mean Percentage of CD3+/CD4+/CD8+ Before Treatment512620

The Best Corrected Visual Acuity(BCVA)

(NCT01267422)
Timeframe: Up to 3 years

,
InterventionlogMAR (Mean)
before treament1 months after treament3 months after treament6 months after treament9 months after treament
BCVA of Treated Eyes1.691.581.381.231.27
BCVA of Un-treated Eyes1.401.361.311.201.23

Reviews

7 reviews available for idebenone and Hereditary Optic Neuroretinopathy

ArticleYear
Current and Emerging Treatment Modalities for Leber's Hereditary Optic Neuropathy: A Review of the Literature.
    Advances in therapy, 2018, Volume: 35, Issue:10

    Topics: Antioxidants; Genetic Therapy; Humans; Optic Atrophy, Hereditary, Leber; Stem Cell Transplantation;

2018
Novel therapeutic approaches for Leber's hereditary optic neuropathy.
    Discovery medicine, 2013, Volume: 15, Issue:82

    Topics: Animals; Antioxidants; Dietary Supplements; Disease Models, Animal; DNA-Binding Proteins; DNA, Mitoc

2013
[Leber's hereditary optic neuropathy - phenotype, genetics, therapeutic options].
    Klinische Monatsblatter fur Augenheilkunde, 2014, Volume: 231, Issue:3

    Topics: Antioxidants; DNA, Mitochondrial; Genetic Predisposition to Disease; Humans; Molecular Diagnostic Te

2014
Is there treatment for Leber hereditary optic neuropathy?
    Current opinion in ophthalmology, 2015, Volume: 26, Issue:6

    Topics: Animals; Genetic Counseling; Genetic Therapy; Humans; Mitochondria; Optic Atrophy, Hereditary, Leber

2015
Idebenone in Friedreich ataxia and Leber's hereditary optic neuropathy: close mechanisms, similar therapy?
    Brain : a journal of neurology, 2016, Volume: 139, Issue:Pt 7

    Topics: Antioxidants; Clinical Trials as Topic; Friedreich Ataxia; Humans; Optic Atrophy, Hereditary, Leber;

2016
Idebenone for Leber's hereditary optic neuropathy.
    Drugs of today (Barcelona, Spain : 1998), 2016, Volume: 52, Issue:3

    Topics: Antioxidants; Humans; Optic Atrophy, Hereditary, Leber; Ubiquinone

2016
Is Leber hereditary optic neuropathy treatable? Encouraging results with idebenone in both prospective and retrospective trials and an illustrative case.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2012, Volume: 32, Issue:1

    Topics: Adult; Antioxidants; Clinical Trials as Topic; Female; Humans; Optic Atrophy, Hereditary, Leber; Pro

2012

Trials

5 trials available for idebenone and Hereditary Optic Neuroretinopathy

ArticleYear
Clinical outcomes of treatment with idebenone in Leber's hereditary optic neuropathy in the Netherlands: A national cohort study.
    Acta ophthalmologica, 2022, Volume: 100, Issue:6

    Topics: Antioxidants; Cohort Studies; Humans; Netherlands; Optic Atrophy, Hereditary, Leber; Retrospective S

2022
Real-World Clinical Experience With Idebenone in the Treatment of Leber Hereditary Optic Neuropathy.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2020, Volume: 40, Issue:4

    Topics: Adolescent; Adult; Aged; Antioxidants; Child; Female; Follow-Up Studies; Humans; Male; Middle Aged;

2020
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Adolescent; Adult; Aged; Antioxidants; Contrast Sensitivity; DNA, Mitochondrial; Double-Blind Method

2011
Effects of idebenone on color vision in patients with leber hereditary optic neuropathy.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2013, Volume: 33, Issue:1

    Topics: Adolescent; Adult; Antioxidants; Color Perception; Double-Blind Method; Female; Humans; Male; Middle

2013
Persistence of the treatment effect of idebenone in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2013, Volume: 136, Issue:Pt 2

    Topics: Adolescent; Adult; Aged; Cohort Studies; Double-Blind Method; Female; Follow-Up Studies; Humans; Mal

2013

Other Studies

34 other studies available for idebenone and Hereditary Optic Neuroretinopathy

ArticleYear
Discovery of Novel 2-Aniline-1,4-naphthoquinones as Potential New Drug Treatment for Leber's Hereditary Optic Neuropathy (LHON).
    Journal of medicinal chemistry, 2020, 11-25, Volume: 63, Issue:22

    Topics: Animals; Cell Survival; Dose-Response Relationship, Drug; Female; Hep G2 Cells; Humans; Mice; Mice,

2020
Design, development, and characterization of an idebenone-loaded poly-ε-caprolactone intravitreal implant as a new therapeutic approach for LHON treatment.
    European journal of pharmaceutics and biopharmaceutics : official journal of Arbeitsgemeinschaft fur Pharmazeutische Verfahrenstechnik e.V, 2021, Volume: 168

    Topics: Animals; Chemistry, Pharmaceutical; Chickens; Chorioallantoic Membrane; Delayed-Action Preparations;

2021
Altering neuronal circuitry with 4-aminopyridine for visual improvement in Leber's hereditary optic neuropathy (LHON).
    Mitochondrion, 2022, Volume: 62

    Topics: 4-Aminopyridine; Adult; DNA, Mitochondrial; Drug Therapy, Combination; Humans; Male; Nerve Net; Neur

2022
Design, optimization, and in vitro characterization of idebenone-loaded PLGA microspheres for LHON treatment.
    International journal of pharmaceutics, 2022, Mar-25, Volume: 616

    Topics: Humans; Microspheres; Optic Atrophy, Hereditary, Leber; Particle Size; Polylactic Acid-Polyglycolic

2022
Visual Outcomes and Optical Coherence Tomography Biomarkers of Vision Improvement in Patients With Leber Hereditary Optic Neuropathy Treated With Idebenone.
    American journal of ophthalmology, 2023, Volume: 247

    Topics: Biomarkers; Cohort Studies; Humans; Optic Atrophy, Hereditary, Leber; Retinal Ganglion Cells; Retros

2023
Age-dependent retinal neuroaxonal degeneration in children and adolescents with Leber hereditary optic neuropathy under idebenone therapy.
    European journal of neurology, 2023, Volume: 30, Issue:8

    Topics: Adolescent; Child; Cross-Sectional Studies; Humans; Optic Atrophy, Hereditary, Leber; Retinal Gangli

2023
Evaluation of Mitochondrial Dysfunction and Idebenone Responsiveness in Fibroblasts from Leber's Hereditary Optic Neuropathy (LHON) Subjects.
    International journal of molecular sciences, 2023, Aug-08, Volume: 24, Issue:16

    Topics: DNA, Mitochondrial; Fibroblasts; Humans; Mitochondria; Optic Atrophy, Hereditary, Leber

2023
Visual function in chronic Leber's hereditary optic neuropathy during idebenone treatment initiated 5 to 50 years after onset.
    Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie, 2019, Volume: 257, Issue:12

    Topics: Adult; Aged; Antioxidants; Chronic Disease; Follow-Up Studies; Forecasting; Humans; Male; Middle Age

2019
Assessment of the Idebenone Effect on LHON Eyes Requires High-quality Studies.
    Current eye research, 2020, Volume: 45, Issue:11

    Topics: DNA, Mitochondrial; Humans; Optic Atrophy, Hereditary, Leber; Ubiquinone

2020
Characteristics of Japanese patients with Leber's hereditary optic neuropathy and idebenone trial: a prospective, interventional, non-comparative study.
    Japanese journal of ophthalmology, 2021, Volume: 65, Issue:1

    Topics: Humans; Japan; Male; Optic Atrophy, Hereditary, Leber; Prospective Studies; Ubiquinone; Visual Acuit

2021
Leber's hereditary optic neuropathy: course of disease in consideration of idebenone treatment and type of mutation.
    Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie, 2021, Volume: 259, Issue:4

    Topics: DNA, Mitochondrial; Humans; Mutation; Optic Atrophy, Hereditary, Leber; Retrospective Studies; Ubiqu

2021
Characteristics of Japanese patients with Leber's hereditary optic neuropathy and idebenone trial: a prospective, interventional, non-comparative study.
    Japanese journal of ophthalmology, 2021, Volume: 65, Issue:2

    Topics: Humans; Japan; Optic Atrophy, Hereditary, Leber; Prospective Studies; Ubiquinone

2021
Assessing the Treatment Effect of Idebenone in Leber Hereditary Optic Neuropathy Requires Appropriate Study Designs.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2023, 09-01, Volume: 43, Issue:3

    Topics: Antioxidants; DNA, Mitochondrial; Humans; Optic Atrophy, Hereditary, Leber; Ubiquinone

2023
Real-World Clinical Experience With Idebenone in the Treatment of Leber Hereditary Optic Neuropathy-Response to Dr. Finsterer's Letter.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2023, 09-01, Volume: 43, Issue:3

    Topics: Antioxidants; Humans; Optic Atrophy, Hereditary, Leber; Ubiquinone

2023
Pathologically Responsive Mitochondrial Gene Therapy in an Allotopic Expression-Independent Manner Cures Leber's Hereditary Optic Neuropathy.
    Advanced materials (Deerfield Beach, Fla.), 2021, Volume: 33, Issue:41

    Topics: Animals; Disease Models, Animal; DNA; DNA, Mitochondrial; Electron Transport Complex I; Fluorescent

2021
High Mitochondrial DNA Copy Number Is a Protective Factor From Vision Loss in Heteroplasmic Leber's Hereditary Optic Neuropathy (LHON).
    Investigative ophthalmology & visual science, 2017, Apr-01, Volume: 58, Issue:4

    Topics: Antioxidants; Blindness; DNA Copy Number Variations; DNA, Mitochondrial; Female; Genes, Mitochondria

2017
Clinical Reasoning: A 10-year-old boy with bilateral vision loss.
    Neurology, 2017, 06-06, Volume: 88, Issue:23

    Topics: Antioxidants; Child; Humans; Male; Optic Atrophy, Hereditary, Leber; Ubiquinone; Vision, Low

2017
International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic Neuropathy.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2017, Volume: 37, Issue:4

    Topics: Antioxidants; Congresses as Topic; Consensus; Disease Management; Humans; International Cooperation;

2017
Visual prognosis better in eyes with less severe reduction of visual acuity one year after onset of Leber hereditary optic neuropathy caused by the 11,778 mutation.
    BMC ophthalmology, 2017, Oct-18, Volume: 17, Issue:1

    Topics: Adolescent; Adult; Age of Onset; Aged; Antioxidants; Ascorbic Acid; Child; DNA Mutational Analysis;

2017
Leber Hereditary Optic Neuropathy Caused by a Mitochondrial DNA 10663T>C Point Mutation and Its Response to Idebenone Treatment.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2018, Volume: 38, Issue:1

    Topics: Consensus; DNA, Mitochondrial; Humans; Optic Atrophy, Hereditary, Leber; Point Mutation; Ubiquinone

2018
A teenager with acute bilateral visual loss.
    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke, 2018, 08-21, Volume: 138, Issue:12

    Topics: Adolescent; Antioxidants; Humans; Male; Ophthalmoscopy; Optic Atrophy, Hereditary, Leber; Point Muta

2018
Leber's hereditary optic neuropathy misdiagnosed as optic neuritis and Lyme disease in a patient with multiple sclerosis.
    BMJ case reports, 2018, Dec-07, Volume: 11, Issue:1

    Topics: Adult; Antioxidants; Diagnosis, Differential; Diagnostic Errors; Humans; Lyme Disease; Magnetic Reso

2018
[Raxone in the Leber optical neuropathy: Parisian experience].
    Journal francais d'ophtalmologie, 2019, Volume: 42, Issue:3

    Topics: Adolescent; Adult; Disease Progression; Female; Humans; Male; Middle Aged; Optic Atrophy, Hereditary

2019
Idebenone (Raxone°) and Leber hereditary optic neuropathy.
    Prescrire international, 2017, Volume: 26, Issue:179

    Topics: Antioxidants; Humans; Optic Atrophy, Hereditary, Leber; Treatment Outcome; Ubiquinone

2017
Novel use of idebenone in Leber's hereditary optic neuropathy in Hong Kong.
    Hong Kong medical journal = Xianggang yi xue za zhi, 2014, Volume: 20, Issue:5

    Topics: Adolescent; Antioxidants; Diagnosis, Differential; Hong Kong; Humans; Male; Optic Atrophy, Hereditar

2014
Mitochondrial DNA mutation 14487T>C manifesting as Leber's hereditary optic neuropathy.
    Journal of neurology, 2015, Volume: 262, Issue:12

    Topics: Adolescent; DNA, Mitochondrial; Humans; Male; Mutation; NADH Dehydrogenase; Optic Atrophy, Hereditar

2015
Idebenone: A Review in Leber's Hereditary Optic Neuropathy.
    Drugs, 2016, Volume: 76, Issue:7

    Topics: Antioxidants; Humans; Optic Atrophy, Hereditary, Leber; Randomized Controlled Trials as Topic; Ubiqu

2016
Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy.
    Mitochondrion, 2017, Volume: 36

    Topics: Adenosine Triphosphate; Adolescent; Antioxidants; Cells, Cultured; Energy Metabolism; Fibroblasts; H

2017
Updates in neuro-ophthalmology. Highlights from the 37th annual meeting of the North American Neuro-Ophthalmology Society,February 5-10, 2011, Vancouver, Canada.
    Reviews in neurological diseases, 2011, Volume: 8, Issue:1-2

    Topics: Burkholderia; Canada; Giant Cell Arteritis; Humans; Multiple Sclerosis; Neurology; Ophthalmology; Op

2011
Idebenone treatment in Leber's hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Antioxidants; Female; Humans; Male; Optic Atrophy, Hereditary, Leber; Placebos; Ubiquinone

2011
Treatment of Leber hereditary optic neuropathy.
    Brain : a journal of neurology, 2011, Volume: 134, Issue:Pt 9

    Topics: Antioxidants; Female; Humans; Male; Optic Atrophy, Hereditary, Leber; Placebos; Ubiquinone

2011
Idebenone protects against retinal damage and loss of vision in a mouse model of Leber's hereditary optic neuropathy.
    PloS one, 2012, Volume: 7, Issue:9

    Topics: Administration, Oral; Animals; Antioxidants; Cell Line; Cell Survival; Disease Models, Animal; Drug

2012
[Response to idebenone and multivitamin therapy in Leber's hereditary optic neuropathy].
    Archivos de la Sociedad Espanola de Oftalmologia, 2007, Volume: 82, Issue:6

    Topics: Adult; Antioxidants; Ascorbic Acid; Benzoquinones; Female; Humans; Male; Optic Atrophy, Hereditary,

2007
Leber's hereditary optic neuropathy (LHON/11778) with myoclonus: report of two cases.
    Journal of neurology, neurosurgery, and psychiatry, 2001, Volume: 71, Issue:6

    Topics: Adult; Anti-Inflammatory Agents; Antioxidants; Benzoquinones; Biopsy; Disease Progression; Electroen

2001