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51 Optical Coherence Tomography Angiography vs Fluorescein Angiography in Leber's Hereditary Optic Neuropathy (LHON)LHON is a mitochondrial disease with sequential, bilateral and dramatic vision loss. The initial clinical presentation of LHON is often subtle with optic disc and peripapillary vascular changes that are frequently missed on funduscopy. In addition, the typical lab work; for optic neuropathies, are u...Genetic Disease, Diagnostic Tests (ERG, VER, OCT, HRT, mfERG, etc) Eyelid & adnexal disease, Optic neuropathy,Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
52 Peripapillary Fluorescein Leakage in 11778 Lebers Optic NeuropathyAdolescent; Adult; Capillary Permeability; DNA, Mitochondrial/genetics; Female; Fluorescein; Fluorescein Angiography; Fluoresceins/metabolism; Fundus Oculi; Humans; Male; Optic Atrophies, Hereditary/diagnosis; Optic Atrophies, Hereditary/genetics; Optic Atrophies, Hereditary/physiopathology; Optic D...
53 MRI of the Optic Nerves and Chiasm in Patients With Leber Hereditary Optic Neuropathy
54 Mitochondrial DNA Hplogroup Distribution in Pedigrees of Southeast Asian G11778A Leber Hereditary Optic NeuropathyChina, ethnology; DNA Mutational Analysis; DNA, Mitochondrial, genetics; Gene Frequency; Genetic Predisposition to Disease, genetics; Genetic Screening; Haplotypes; Humans; India, ethnology; Mutation, genetics; Optic Atrophy, Hereditary, Leber, ethnology; Optic Atrophy, Hereditary, Leber, genetics; ...
55 Differences in pupillary light reflex between hereditary optic neuropathy and ethambutol induced optic neuropathyTo evaluate the differences in pupillary light reflex (PLR) between ethambutol optic neuropathy (EON) and hereditary optic neuropathy (HON).Design: Retrospective, observational, case control study.Pupils Retina, Optic neuropathyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
56 Clinical Trials of Neuro-Ophthalmic Interest (video)2016 was a good year for clinical trials of interest to neuroophthalmologists. In this syllabus, I will discuss final or preliminary results from five treatment trials dealing with four disorders: primary pseudotumor cerebri syndrome (n=1), leber hereditary optic neuropathy (n=2), giant cell (tempor...Pseudotumor Cerebri Syndrome, Leber Hereditary Optic Neuropathy, Optic Pathway Glioma, Giant Cell Arteritis, TocilizumabNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
57 IE02a Acute Leber Optic NeuropathyAcute Leber Optic Neuropathy, Left eye. Same patient as IE_01 and IE_02b. August 7, 1979. Anatomy: Optic disc. Pathology: Optic neuropathy. Disease/ Diagnosis: Leber's optic neuropathy. Clinical: Asymptomatic.Optic Disc Atrophy with Special Features; Atrophy with Peripapillary Microangiopathy; Leber Optic Neuropathy; Acute, Subacute and End StagesNeuro-Ophthalmology Virtual Education Library: William F. Hoyt Neuro-Ophthalmology Collection: https://novel.utah.edu/Hoyt/
58 Expanded Access Program (EAP) in Leber's Hereditary Optic Neuropathy (LHON) Patients Treated for 24 Months (Slides)LHON is a mitochondrial genetic disorder resulting in severe bilateral central VA loss. Three primary mitochondrial DNA mutations cause over 90% of cases. Data from idebenone EAP for patients with LHON receiving treatment for at least 24 months were analyzed to assess rate of clinically-relevant rec...Optic NeuropathyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
59 International Consensus Statement on the Clinical and Therapeutic Management of Leber Hereditary Optic NeuropathyAntioxidants; Congresses as Topic; Consensus; Disease Management; Humans; International Cooperation; Ophthalmology; Optic Atrophy, Hereditary, Leber; Societies, Medical; Ubiquinone
60 Reactive Oxygen Species in Mitochondrial Optic Neuropathies: ResponseHumans; Optic Chiasm; Optic Nerve Diseases; Scotoma; SuperoxidesNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
61 REFLECT Trial: Efficacy and Safety of Bilateral Gene Therapy for Leber Hereditary Optic NeuropathyLeber Hereditary Optic Neuropathy (LHON) is a blinding orphan disease leading to bilateral vision loss. Lenadogene nolparvovec (rAAV2/2-ND4) is a gene therapy developed for the treatment of LHON caused by a mutation in the ND4 gene.Genetic Disease; Optic Neuropathy; RetinaNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
62 Visual Outcomes in Leber Hereditary Optic Neuropathy Patients With the m.11778GA MTND4 Mitochondrial DNA Mutation
63 Cardiac Disorders in Patients With Leber Hereditary Optic Neuropathy
64 A Family with Lebers Heredity Optic Neuropathy with Mitochondrial DNA Heteroplasmy Related to Disease ExpressionAdult; Older people; Older people, 80 and over; DNA/analysis; DNA Mutational Analysis; DNA Primers/chemistry; DNA, Mitochondrial/genetics; Female; Humans; Male; Middle Older people; Optic Atrophies, Hereditary/genetics; Optic Atrophies, Hereditary/physiopathology; Pedigree; Point Mutation; Polymeras...
65 Mitochondrial Optic Neuropathies - Expanding Genotypes and PhenotypesMitochondrial Optic Neuropathies (MON) are a genetically and phenotypically diverse group of diseases. Despite the different genetic causes, there is considerable phenotypic overlap which makes distinguishing one mutation from another solely based on clinical presentation difficult. Moreover, clinic...Leber Hereditary Optic Neuropathy Plus Syndromes; Harding's Disease; Autosomal Hereditary Optic Neuropathies; Leigh Syndrome; Charles Bonnet SyndromeNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
66 Steroids Are Not Always the Solution!!Leber Hereditary Optic Neuropathy (LHON) is the most common inherited mitochondrial disorder and typically affects young males. (https://eyewiki.aao.org/Leber_Hereditary_Optic_Neuropathy)Leber Hereditary Optic Neuropathy (LHON)Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
67 Leber Hereditary Optic Neuropathy in a Mother and Daughter Associated With m.10197G>A Mutation
68 IE01 Acute Leber Optic NeuropathyPseudo edema with peripapillary microangiopathy in a brother of boy with Leber Optic Neuropathy. Not pale nerve yet. Right eye. Same patient as IE_02a and IE_02b. August 7, 1979. Anatomy: Optic disc. Pathology: Optic neuropathy. Disease/ Diagnosis: Optic neuropathy. Clinical: AsymptomaticOptic Disc Atrophy with Special Features; Atrophy with Peripapillary Microangiopathy; Leber Optic Neuropathy; Acute, Subacute and End StagesNeuro-Ophthalmology Virtual Education Library: William F. Hoyt Neuro-Ophthalmology Collection: https://novel.utah.edu/Hoyt/
69 IE02b Acute Leber Optic NeuropathyAcute Leber Optic Neuropathy. Formation of hemorrhage one year after IE_02a. At this time, he was beginning to lose central vision. .Note thinning of the nerve fiber layer temporally, 3:00 - 4:00 Pair with IE_02a. March 26, 1980. Anatomy: Optic disc. Pathology: Optic neuropathy. Disease/ Diagnosis: ...Optic Disc Atrophy with Special Features; Atrophy with Peripapillary Microangiopahty; Leber Optic Neuropathy; Acute, Subacute and End StagesNeuro-Ophthalmology Virtual Education Library: William F. Hoyt Neuro-Ophthalmology Collection: https://novel.utah.edu/Hoyt/
70 Do Idebenone and Vitamin Therapy Shorten the Time to Achieve Visual Recovery in Leber Hereditary Optic NeuropathyAdolescent; Adult; Age of Onset; Antioxidants/therapeutic use; Ascorbic Acid/therapeutic use; Benzoquinones/therapeutic use; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Male; Middle Older people; Mutation; Optic Atrophies, Hereditary/complications; Optic Atrophies, Hereditary/drug ...
71 Cerebral Stroke Causing Monocular Hemianopia on Automated PerimetryA homonymous hemianopia localizes to the retrochiasmal visual pathway. A monocular hemianopic defect typically localizes anterior to the chiasm. We report three patients with a monocular hemianopia on automated perimetry following cerebral stroke.Visual fields, Stroke TraumaNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
72 Its Not the TumorVision Loss; Leber Hereditary Optic Neuropathy; Radiation TherapyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
73 Energy Charge Is Not Decreased in Lymphocytes of Patients with Lebers Hereditary Optic Neuropathy with the 11778 MutationAdenosine Diphosphate/metabolism; Adenosine Monophosphate/metabolism; Adenosine Triphosphate/metabolism; Chromatography, High Pressure Liquid; DNA, Mitochondrial/genetics; DNA, Mitochondrial/metabolism; Humans; Lymphocytes/metabolism; Optic Atrophies, Hereditary/genetics; Optic Atrophies, Hereditary...
74 Are We There Yet? Is Neuro-Ophthalmology at the Cuspof a Paradigm Shift? Lessons From Leber Hereditary Optic NeuropathyHumans; Mitochondria; Neurology; Ophthalmology; Optic Atrophy, Hereditary, Leber; Optic Nerve; Reactive Oxygen Species
75 It's Not the TumorVision Loss; Leber Hereditary Optic Neuropathy; Radiation TherapyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
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