ArticleActa neurologica Belgica2017
Optical coherence tomography as a marker of neurodegeneration in patients with Wilson's disease.
Article in Acta neurologica Belgica, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 27 citations in OpenAlex.
- Diagnostic Pitfalls in Wilson Disease with Autoimmune Features: A Case Report.GE Portuguese journal of gastroenterology · 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
- Mapping neurological symptoms and muscle tension representations in impaired gray matter volume of Wilson disease.Frontiers in neurology · 2025Article
- Chinese Multidisciplinary Expert Consensus on Orphan/Anticopper Drugs and Other Non-drug Management of Hepatolenticular Degeneration.Current neuropharmacology · 2025Review
- 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
- 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
- Wilson's Disease: Facing the Challenge of Diagnosing a Rare Disease.Biomedicines · 2021Review
- Exploring the role of macular thickness as a potential early biomarker of neurodegeneration in acute schizophrenia.International ophthalmology · 2021Article
- Management Perspective of Wilson's Disease: Early Diagnosis and Individualized Therapy.Current neuropharmacology · 2021Article
- Brain volume is related to neurological impairment and to copper overload in Wilson's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2019Article
- The Place of Optical Coherence Tomography in Patients with Obsessive Compulsive Disorder.The Eurasian journal of medicine · 2019Article
- Clinical manifestations of Wilson disease in organs other than the liver and brain.Annals of translational medicine · 2019Review
- Wilson disease.Nature reviews. Disease primers · 2018Review
- Reduced retinal nerve fiber layer (RNFL) thickness in ALS patients: a window to disease progression.Journal of neurology · 2018Article
- Characteristics of a newly diagnosed Polish cohort of patients with neurological manifestations of Wilson disease evaluated with the Unified Wilson's Disease Rating Scale.BMC neurology · 2018Article
- Retinal Degeneration in Patients with Wilson's Disease: An OCT Study in Asian Indian Population.Annals of Indian Academy of NeurologyArticle
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Wilson's disease (WD) is an inherited autosomal recessive disorder that leads to pathological copper accumulation in different organs. Optical coherence tomography (OCT) is proposed as a marker of neurodegeneration in many neurological diseases. Thinning of the total retinal nerve fiber layer (RNFL) and macular thickness (Mth) examined by OCT was detected in patients with WD, especially those with brain magnetic resonance imaging changes. The aim of this study was to evaluate the relationship between OCT parameters and the progression of neurological signs measured by the Unified Wilson's Disease Rating Scale (UWDRS) in patients with WD. Consecutive patients with WD admitted to the Department of Neurology underwent OCT to assess the thickness of the macula and total RNFL. Patients also had neurologic assessments according to the UWDRS part III. Patients were divided into two groups based on the presence (UWDRS+) and absence (UWDRS-) of neurological symptoms. Fifty-eight patients (34 females, 24 males) were enrolled. Mean duration of treatment was 9 years (standard deviation [SD], ±10.8). The mean UWDRS score at the time of study was 8.4 (range 1-52; SD ±13.9) points. Total RNFL as well as macula thickness were significantly decreased in the UWDRS+ group versus the UWDRS- group. A significant negative correlation was found between OCT parameters (RNFL and Mth measurements) and neurological impairment according the UWDRS scale. This study confirms that OCT may be a useful tool for measuring the degree of neurodegeneration in patients with WD, and may play role in monitoring disease progression.
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