Search results for "Outer nuclear layer"

Atrophic Forms (Dry AMD)

2009

Atrophic form of “Dry AMD” is defined by areas of RPE atrophy, often resulting from regression of confluent soft drusen.

Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis

2004

Retinal degeneration is an early and progressive event in many forms of neuronal ceroid lipofuscinoses (NCLs), a heterogeneous group of neurodegenerative disorders with unknown pathogenesis. We here used the mutant motor neuron degeneration (mnd) mouse, a late-infantile NCL variant, to investigate the retinal oxidative state and apoptotic cell death as a function of age and sex. Total superoxide dismutase (SOD) activities and thiobarbituric acid-reactive substance (TBARS) levels revealed progressive increases in retinal oxyradicals and lipid peroxides of mnd mice of both sexes. Female mnd retinas showed a higher oxidation rate and consistently exhibited the 4-hydroxy-2-nonenal (4-HNE)-adduc…

OPTICAL COHERENCE TOMOGRAPHIC FINDINGS IN BIETTI’S CRYSTALLINE TAPETORETINAL DYSTROPHY

2014

Purpose: To report the optical coherence tomography (OCT) findings of Bietti crystalline tapetoretinal dystrophy. Design: Observational case report. Methods: A subject with Bietti crystalline tapetoretinal dystrophy was evaluated with ophthalmoscopy, fluorescein angiography, and OCT (Stratus). Results: OCT showed thinning and hyporeflectivity of the outer nuclear layer in the macula due to photoreceptor degeneration. The retinal areas showing atrophy of the retinal pigment epithelium displayed greater penetration of the optical beam into the choroid and visualization of residual choroidal vessels. The hyperreflective band normally seen under the neurosensory retina was extremely wide when a…

AN ULTRASTRUCTURAL STUDY OF THE RETINA IN HUMAN LATE INFANTILE NEUROAXONAL DYSTROPHY

1993

A case involving a girl who died at 11 years of age and who had developed normally until the age of 18 months, at which time further psychomotor maturation stopped and then regressed, is reported. The patient appeared hypotonic and showed loss of deep tendon reflexes, as well as bulbar signs and increasing immobility. Visual impairment resulted in blindness at the age of 7 years. Her disease was diagnosed as late infantile neuroaxonal dystrophy (LINAD) after examination of sural nerve biopsy samples and after autopsy. Under electron microscopy, retinal axons were filled with tubulocisternal profiles and occasional large lamellar clefts close to or distant from synaptic complexes. These lesi…

Clinical Features and Natural History of AMD on OCT

2009

Despite recent progress, age-related macular degeneration (AMD) remains the leading cause of vision loss in high-income countries, and its incidence appears to be increasing, probably due to longer life span and improved methods of detection.

Polypoidal Choroidal Vasculopathy

2009

Polypoidal choroidal vasculopathy does not constutute part of the classical definition of AMD, but these two en tities have a number of features in common.

Clinical Features and Natural History of AMD

2009

Over recent years, OCT has become an essential part of the examination in routine follow up for AMD to guide clinical practice and analyze the response to treatment. OCT examinations can clearly demonstrate the efficacy of treatment by showing regression or persistence of leakage. Moreover, alterations of the outer retinal layers can also be analyzed.

Magnetic resonance imaging and optical coherence tomography correlations in multiple sclerosis beyond anatomical landmarks.

2020

Abstract Objective To investigate multiple sclerosis (MS) optical coherence tomography (OCT) cross-sectional correlations with central nervous system (CNS) magnetic resonance imaging (MRI). Material and methods Peripapillary retinal nerve fiber layer (pRNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner (INL) and outer nuclear layer (ONL) of 54 relapsing remitting (RRMS) and 38 progressive (PMS, 9 primary and 29 secondary) patients were measured. With less than 3 months brain parenchymal fraction (BPF), spinal cord (SC), total gray matter (GM) and white matter volumes were calculated. Demographical and clinical data was compared according to the history of optic neuritis (H…

2021

Introduction Niemann-Pick type C (NPC) is a lysosomal storage disease that is progressive and life-limiting, with an estimated incidence of 1:120,000 live births. In addition to systemic manifestation with (hepato-)splenomegaly, there are a number of neurological manifestations (ataxia, dysarthria, dementia, cataplexy, epileptic seizures, and psychiatric disorders). Characteristic is vertical supranuclear gaze palsy, which is often overlooked. Early diagnosis and start of therapy improve quality of life. This study aimed to characterize oculomotor dysfunction of NPC patients, and to provide ophthalmologic data including retinal imaging. Methods Eighteen patients with biochemically or genet…

Classic Choroidal Neovascularization

2009

Since Donald Gass’s pioneering work in 1967, which demonstrated the role of choroidal neovascularization (CNV) in wet (exudative) AMD, visible (classic) CNV, well demarcated and well-defined on fluorescein angiography, has become the most characteristic form of AMD.