Persistence of the treatment effect of idebenone in Leber’s hereditary optic neuropathy
نویسندگان
چکیده
1 Department of Neurology, Friedrich-Baur-Institute, Ludwig-Maximilians-University, 80336 Munich, Germany 2 Santhera Pharmaceuticals, 4410 Liestal, Switzerland 3 Departments of Neurology and Ophthalmology, Newcastle upon Tyne Foundation Hospital NHS Foundation Trust, NE1 4LP UK 4 Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, NE1 3BZ, UK 5 Department of Ophthalmology, Ludwig-Maximilians-University, Munich 80336, Germany 6 Department of Ophthalmology, Centre Hospitalier de l’Université de Montréal (CHUM), Montreal, Quebec H2L 4M1, Canada
منابع مشابه
Idebenone Protects against Retinal Damage and Loss of Vision in a Mouse Model of Leber’s Hereditary Optic Neuropathy
Leber's hereditary optic neuropathy (LHON) is an inherited disease caused by mutations in complex I of the mitochondrial respiratory chain. The disease is characterized by loss of central vision due to retinal ganglion cell (RGC) dysfunction and optic nerve atrophy. Despite progress towards a better understanding of the disease, no therapeutic treatment is currently approved for this devastatin...
متن کاملA randomized placebo-controlled trial of idebenone in Leber’s hereditary optic neuropathy
Major advances in understanding the pathogenesis of inherited metabolic disease caused by mitochondrial DNA mutations have yet to translate into treatments of proven efficacy. Leber's hereditary optic neuropathy is the most common mitochondrial DNA disorder causing irreversible blindness in young adult life. Anecdotal reports support the use of idebenone in Leber's hereditary optic neuropathy, ...
متن کاملAuthor Response: Increased mtDNA Copy Number Protects Against LHON.
Although we appreciate the major interest of Josef Finsterer and Sinda Zarrouk-Mahjoub in our recent publication in Investigative Ophthalmology and Visual Science entitled ‘‘High Mitochondrial DNA Copy Number Is a Protective Factor from Vision Loss in Heteroplasmic Leber’s Hereditary Optic Neuropathy (LHON),’’ we wish to clarify two issues. First, in our published studies, our findings strongly...
متن کامل[Response to idebenone and multivitamin therapy in Leber's hereditary optic neuropathy].
OBJECTIVE To ascertain the efficacy of idebenone and multivitamin treatment in Leber's hereditary optic neuropathy (LHON). METHOD Two patients diagnosed of unilateral LHON were treated with megadoses of idebenone, vitamin C and riboflavin for one year. They were examined clinically before, during and after treatment. RESULTS No improvement of visual function was observed. Despite the ideben...
متن کاملNovel use of idebenone in Leber's hereditary optic neuropathy in Hong Kong.
We report a case of a young Chinese male presenting with sequential, painless, bilateral visual loss in Hong Kong. He was diagnosed to have Leber's hereditary optic neuropathy with genetic workup showing G11778A mutation with over 80% heteroplasmy. He was started on idebenone treatment 11 months after onset of the binocular disease. To our best knowledge, this is the first case of Leber's hered...
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