Optical Coherence Tomography Angiography Findings in Long-Term Follow-up of Leber’s Hereditary Optic Neuropathy: Report of Two Cases

Kızıltunç, Pınar Bingöl; Bilen, Feyza Tüntaş; Atilla, Hüban

doi:10.4274/tjo.galenos.2020.86300

Cited by 5 publications

(4 citation statements)

References 8 publications

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“…Later, in its chronic atrophic phase, optic disc pallor is seen as a consequence of RGC loss [ 68 ]. OCT-A measurements are available, mostly from case reports, of the chronic phase and fewer from the sub-acute phase of the disease [ 68 , 69 , 70 ]. The greatest patient group was analyzed by Yu et al [ 67 ].…”

Section: Oct-a Findings In Neurological Diseasesmentioning

confidence: 99%

The Value of Optical Coherence Tomography Angiography (OCT-A) in Neurological Diseases

Augustin

Atorf

2022

Diagnostics

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Optical coherence tomography angiography (OCT-A) was commercially introduced in 2014. OCT-A allows a fast, non-invasive, three-dimensional analysis of the retinal vasculature from the vitreoretinal interface to the choriocapillaris. The results can be evaluated separately in automated or custom-defined retinal layers. Since its introduction, OCT-A has also been used in patients with neurological diseases in order to find and characterize retinal biomarkers. Many neurological diseases have retinal manifestations, often preceding the key symptoms of the neurological disease. Anatomically and developmentally, the retina is a part of the brain. In contrast to the brain, the retina is easily accessible for imaging methods; moreover, retinal imaging is more cost-effective than brain imaging. In this review, the current knowledge about OCT-A findings and possible OCT-A biomarkers in neurological diseases is summarized and discussed regarding the value of OCT-A as a diagnostic tool in neurological diseases.

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Section: Oct-a Findings In Neurological Diseasesmentioning

confidence: 99%

The Value of Optical Coherence Tomography Angiography (OCT-A) in Neurological Diseases

Augustin

Atorf

2022

Diagnostics

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“…Similarly, OCT-A is significant for involvement of microvasculature of the small axons of the papillomacular bundle, radial peripallary capillary defects, and a decrease in the retinal nerve fiber layer with progress of the optic atrophy. [88] . Patients may experience loss of color vision.…”

Section: Wolfram Syndromementioning

confidence: 99%

“…• Diabetes Inspidus [64,70] • Diabetes Mellitus [64,70] • Peripheral Neuropathy [69] • Cerebellar Ataxia [69] • Myoclonus [69] • Urinary Incontinence [64] • Optic Atrophy [69] • Progressive bilateral vision loss [69,71] • Loss of color vision (blue-yellow spectrum) [71,72] • Cataracts [73] • Pigmentary retinopathy [73] • Nystagmus [73] • SNHL [51,[53][54][55] Leber Hereditary Optic Atrophy Adulthood Males • Decreased muscle control [90,91] • Tremors [90,91] • Cardiac arrhythmia [90,91] • Optic Atrophy [88] • Progressive bilateral vision loss [86] • Loss of color vision (red-green spectrum) [84,89] • SNHL [71,73,82] Marfan Syndrome Infancy, Neonatal No preference • Aortic root dilation [96,97,104] • Mitral valve prolapse [96,97,104] • Aortic aneurysm [96,97,99,100,202] • Aortic Dissection [96,...…”

Section: Adolescence Young Adulthoodmentioning

confidence: 99%

Ocular Manifestations in Patients with Sensorineural Hearing Loss

Parameswarappa

Zaheer

Chhablani

et al. 2022

JOVR

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(SNHL) can have a large impact on the outcome and treatment of pediatric patients. Due to the common co-incidence of ocular manifestations and SNHL in children, both ophthalmologic and hearing loss screening and routine examinations must be conducted to minimize adverse outcomes and worsening of pathology. Early evaluation and diagnosis is imperative for intervention and further development of the patient. Coincidence requires a thorough evaluation that includes a comprehensive history, examination, and diagnostic testing. In this article, a literature review was conducted to analyze the presentations of various diseases and syndromes, such as Alport Syndrome, Waardenburg Syndrome, Norrie Disease, Usher Disease, Stickler Syndrome, Marfan Syndrome, Congenital Rubella, and Hereditary Optic Neuropathies. We divided the various ocular pathologies into anterior and posterior segment presentations and associated systemic findings for better understanding. Additionally, this review aims to include an update on the management of patients with both ocular and hearing loss manifestations.

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“…Interestingly, Rovcanin et al found that both symptomatic and asymptomatic LHON patients showed similar oxidative stress alterations, suggesting that mtDNA point mutations per se induce a pro-oxidative phenotype [136]. Also, some studies indicate that LHON might be a neurovascular disease since decreased vascular density in the retina is observed in both unaffected and affected LHON carriers and precedes RNFL thinning [197][198][199][200][201]. Furthermore, smoking has been shown to negatively influence TAS and OSI in LHON patients [136].…”

Section: Oxidative Stress In Lhonmentioning

confidence: 99%

Oxidative Stress in Optic Neuropathies

et al. 2021

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Increasing evidence indicates that changes in the redox system may contribute to the pathogenesis of multiple optic neuropathies. Optic neuropathies are characterized by the neurodegeneration of the inner-most retinal neurons, the retinal ganglion cells (RGCs), and their axons, which form the optic nerve. Often, optic neuropathies are asymptomatic until advanced stages, when visual impairment or blindness is unavoidable despite existing treatments. In this review, we describe systemic and, whenever possible, ocular redox dysregulations observed in patients with glaucoma, ischemic optic neuropathy, optic neuritis, hereditary optic neuropathies (i.e., Leber’s hereditary optic neuropathy and autosomal dominant optic atrophy), nutritional and toxic optic neuropathies, and optic disc drusen. We discuss aspects related to anti/oxidative stress biomarkers that need further investigation and features related to study design that should be optimized to generate more valuable and comparable results. Understanding the role of oxidative stress in optic neuropathies can serve to develop therapeutic strategies directed at the redox system to arrest the neurodegenerative processes in the retina and RGCs and ultimately prevent vision loss.

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Optical Coherence Tomography Angiography Findings in Long-Term Follow-up of Leber’s Hereditary Optic Neuropathy: Report of Two Cases

Cited by 5 publications

References 8 publications

The Value of Optical Coherence Tomography Angiography (OCT-A) in Neurological Diseases

The Value of Optical Coherence Tomography Angiography (OCT-A) in Neurological Diseases

Ocular Manifestations in Patients with Sensorineural Hearing Loss

Oxidative Stress in Optic Neuropathies

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