ArticlePloS one2012
Retinal neurodegeneration in Wilson's disease revealed by spectral domain optical coherence tomography.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 54 citations in OpenAlex.
- The APOSTEL recommendations for reporting quantitative optical coherence tomography studies.Neurology · 2016Guideline
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- Flecainide mediated sodium channel blockade enhances blood brain barrier integrity and promotes neuroprotection in neuroinflammation.Scientific reports · 2025Article
- Quantitative vessel density around foveal avascular zone as a potential imaging biomarker for detecting central nervous system injury in Wilson's disease: an optical coherence tomography angiography study.Quantitative imaging in medicine and surgery · 2025Article
- Peripapillary Retinal Nerve Fiber Layer (pRNFL) Thickness - A Novel Biomarker of Neurodegeneration in Late-Infantile CLN2 Disease.Eye and brain · 2024Article
- Optical coherence tomography in patients with Wilson's disease.Brain and behavior · 2023Article
- Eye Involvement in Wilson's Disease: A Review of the Literature.Journal of clinical medicine · 2022Review
- Optical coherence tomography in neurodegenerative disorders.Arquivos de neuro-psiquiatria · 2022Review
- CNS Involvement in Chronic Inflammatory Demyelinating Polyneuropathy: Subtle Retinal Changes in Optical Coherence Tomography.Neurology(R) neuroimmunology & neuroinflammation · 2022Article
- Acute-Onset Visual Impairment in Wilson's Disease: A Case Report and Literature Review.Frontiers in neurology · 2022Article
- Case Report: The First Reported Concurrence of Wilson Disease and Bilateral Retinitis Pigmentosa.Frontiers in medicine · 2022Article
- Optical Coherence Tomography to Assess Neurodegeneration in Phenylalanine Hydroxylase Deficiency.Frontiers in neurology · 2021Article
- Retinal layers and visual conductivity changes in a case series of microangiopathic ischemic stroke patients.BMC neurology · 2020Article
- Changes in Retinal OCT and Their Correlations with Neurological Disability in Early ALS Patients, a Follow-Up Study.Brain sciences · 2019Article
- Clinical manifestations of Wilson disease in organs other than the liver and brain.Annals of translational medicine · 2019Review
- Early alpha-lipoic acid therapy protects from degeneration of the inner retinal layers and vision loss in an experimental autoimmune encephalomyelitis-optic neuritis model.Journal of neuroinflammation · 2018Article
- Optical coherence tomography as a marker of neurodegeneration in patients with Wilson's disease.Acta neurologica Belgica · 2017Article
- Optical coherence tomography for the diagnosis and monitoring of idiopathic intracranial hypertension.Journal of neurology · 2017Observational
- Whole-body positional manipulators for ocular imaging of anaesthetised mice and rats: a do-it-yourself guide.BMJ open ophthalmology · 2017Article
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND/
objectiveIn addition to cirrhosis of the liver, Wilson's disease leads to copper accumulation and widespread degeneration of the nervous system. Delayed visual evoked potentials (VEPs) suggest changes to the visual system and potential structural changes of the retina.
methodsWe used the latest generation of spectral domain optical coherence tomography to assess the retinal morphology of 42 patients with Wilson's disease and 76 age- and sex-matched controls. We measured peripapillary retinal nerve fiber layer (RNFL) thickness and total macular thickness and manually segmented all retinal layers in foveal scans of 42 patients with Wilson's disease and 76 age- and sex-matched controls. The results were compared with VEPs and clinical parameters.
resultsThe mean thickness of the RNFL, paramacular region, retinal ganglion cell/inner plexiform layer and inner nuclear layer was reduced in Wilson's disease. VEPs were altered with delayed N75 and P100 latencies, but the N140 latency and amplitude was unchanged. An analysis of the laboratory parameters indicated that the serum concentrations of copper and caeruloplasmin positively correlated with the thickness of the outer plexiform layer and with N75 and P100 VEP latencies.
conclusionNeuronal degeneration in Wilson's disease involves the retina and changes can be quantified by optical coherence tomography. While the VEPs and the thickness of the outer plexiform layer appear to reflect the current copper metabolism, the thicknesses of the RNFL, ganglion cell/inner plexiform layer, inner nuclear layer and the total paramacular thickness may be the best indicators of chronic neuronal degeneration.
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