ubiquinone and Optic-Nerve-Diseases

The concept that optic nerve fiber loss might be reduced by neuroprotection arose in the mid 1990s. The subsequent research effort, focused mainly on rodent models, has not yet transformed into a successful clinical trial, but provides mechanistic understanding of retinal ganglion cell death and points to potential therapeutic strategies. This review highlights advances made over the last year.. In excitotoxicity and axotomy models retinal ganglion cell death has been shown to result from a complex interaction between retinal neurons and Müller glia, which release toxic molecules including tumor necrosis factor alpha. This counteracts neuroprotection by neurotrophins such as nerve growth factor, which bind to p75NTR receptors on Müller glia stimulating the toxic release. Another negative effect against neurotrophin-mediated protection involves the action of LINGO-1 at trkB brain-derived neurotrophic factor (BDNF) receptors, and BDNF neuroprotection is enhanced by an antagonist to LINGO-1. As an alternative to pharmacotherapy, retinal defences can be stimulated by exposure to infrared radiation.. The mechanisms involved in glaucoma and other optic nerve disorders are being clarified in rodent models, focusing on retrograde degeneration following axonal damage, excitotoxicity and inflammatory/autoimmune mechanisms. Neuroprotective strategies are being refined in the light of the mechanistic understanding.

Topics: Antioxidants; Glaucoma; Humans; Infrared Rays; Intraocular Pressure; Nitric Oxide Synthase; Optic Nerve Diseases; Retinal Ganglion Cells; Ubiquinone

We aimed to evaluate the current practice patterns of neuro-ophthalmologists in diagnosis and management of three optic neuropathies using a national survey in South Korea and to further compare the practices of neuro-ophthalmologists divided into junior and senior groups based on their clinical practice experience.. An anonymous, 15-question survey on the diagnosis and treatment of traumatic optic neuropathy (TON), nonarteritic anterior ischemic optic neuropathy (NAION), and Leber's hereditary optic neuropathy (LHON) was sent to all neuro-ophthalmologists registered with the Korean Neuro-ophthalmology Society. The questions addressed physician's practice duration as neuro-ophthalmologist, choices of MRI scans and laboratory tests for the diagnosis in suspected optic neuropathy, clinical experiences with steroids (e.g., side effects), and choices of treatment modalities and reason in in each optic neuropathy. All participants were classified into senior (≥ 10 years) and junior (< 10 years) groups.. A total of 63 responders (response rate 78.8%) answered the questionnaire. All responders performed the basic blood tests and brain imaging for evaluating optic neuropathy. Observation was the most preferred option for TON (47.6%) and NAION (63.5%). Steroid use was the second most preferred, and the most selected indication of steroid was "when the patient wants" (58.7%) for TON and "severe visual loss or last eye" (66%) for NAION. The most preferred treatment for LHON was "prescribing idebenone" (69.7%) with a dose of 900 mg/day (63.8%). Forty-nine respondents (77.8%) experienced side effects of steroids. There was no significant difference between the senior and junior groups in all questionnaire answers (all p > 0.05).. Optic neuropathies are being managed similarly by the two groups in South Korea, and many of them still use steroids. We provided reliable reasons for our results compared with other countries.

Topics: Adult; Antioxidants; Dose-Response Relationship, Drug; Glucocorticoids; Humans; Incidence; Magnetic Resonance Imaging; Male; Optic Nerve; Optic Nerve Diseases; Republic of Korea; Surveys and Questionnaires; Treatment Outcome; Ubiquinone; Visual Acuity

We report the case of a patient with an optic neuropathy induced by neurotoxicity in the setting of methylmalonic acidemia. The patient responded with a significant and long-term improvement in visual acuity, perimetry, and chromatic function after a neuroprotective treatment with vitamin E and coenzyme Q10 was started. Coenzyme Q10 levels had been proven to be normal before starting treatment. This case report is particularly important because it describes a possible treatment for optic neuropathy in methylmalonic patients. Although the response might be, in part, specific to the individual, it suggests the existence of a cause-effect relationship between the treatment undergone by our patient and the improvement in her visual acuity. To date, no other treatments with beneficial effects have been reported for the few optic neuropathies caused by methylmalonic acidemia. Further studies should determine the applicability of coenzyme Q10 and vitamin E for the treatment of optic neuropathies in methylmalonic acidemia.

Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Antioxidants; Cells, Cultured; Diet, Protein-Restricted; Dietary Supplements; DNA Mutational Analysis; Drug Therapy, Combination; Female; Humans; Neuroprotective Agents; Optic Nerve Diseases; Recovery of Function; Time Factors; Treatment Outcome; Ubiquinone; Visual Acuity; Vitamin E