ArticleBMC neurology2018
Characteristics of a newly diagnosed Polish cohort of patients with neurological manifestations of Wilson disease evaluated with the Unified Wilson's Disease Rating Scale.
Article in BMC neurology, 2018. 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, 57 citations in OpenAlex.
- Early neurological deterioration in Wilson's disease: a systematic literature review and meta-analysis.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Pooled it
- APASL clinical practice guidance: the diagnosis and management of patients with Wilson's disease.Hepatology international · 2026Article
- Improvement in sleep following treatment initiation in newly diagnosed, treatment-naïve patients with Wilson's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Assessment of neurological symptoms and associated factors in patients with Wilson's disease in Southwest China.Orphanet journal of rare diseases · 2025Article
- Minimal Criteria to Screen for Wilson Disease: A Delphi Consensus in the United States.International journal of hepatology · 2025Article
- Supranuclear Vertical Gaze Palsy in Movement Disorders.Neuro-ophthalmology (Aeolus Press) · 2025Review
- Intrathecal Baclofen Therapy Improves Refractory Status Dystonicus in Neuro-hepatic Wilson's Disease: A Case Report.NMC case report journal · 2025Article
- Long-term outcome of patients with neurological form of Wilson's disease compliant to the de-coppering treatment.Journal of neurology · 2023Article
- Serum neurofilament light chain and initial severity of neurological disease predict the early neurological deterioration in Wilson's disease.Acta neurologica Belgica · 2023Article
- Blood Based Biomarkers of Central Nervous System Involvement in Wilson's Disease.Diagnostics (Basel, Switzerland) · 2023Review
- Dysfunction of the Lenticular Nucleus Is Associated with Dystonia in Wilson's Disease.Brain sciences · 2022Article
- Autoantibodies in Wilson disease: Impact on clinical course.JIMD reports · 2022Article
- Neuroimaging Correlates of Cognitive Deficits in Wilson's Disease.Movement disorders : official journal of the Movement Disorder Society · 2022Article
- Neuroimaging correlates of brain injury in Wilson's disease: a multimodal, whole-brain MRI study.Brain : a journal of neurology · 2022Article
- Blink reflex in newly diagnosed and treated patients with Wilson's disease.Journal of neural transmission (Vienna, Austria : 1996) · 2021Observational
- Article
- Co-occurring Wilson's disease and non-penicillamine-induced systematic lupus erythematosus: a case report and literature review.Clinical rheumatology · 2021Review
- Review
- Plasma Neurofilament Light as a Biomarker of Neurological Involvement in Wilson's Disease.Movement disorders : official journal of the Movement Disorder Society · 2021Article
- Effects of High-Frequency Repetitive Transcranial Magnetic Stimulation on Upper Limb Dystonia in Patients With Wilson's Disease: A Randomized Controlled Trial.Frontiers in neurology · 2021Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundWilson disease is a rare genetic disorder in which impaired copper excretion results in toxic copper levels and tissue damage. Manifestations are primarily hepatic and/or neuropsychiatric, with a variety of neurological phenotypes. The aim of this study was to characterize neurological signs of Wilson disease in newly diagnosed patients and to determine whether they correlated with disability, liver function, and copper metabolism.
methodsFifty-three treatment-naïve patients recently diagnosed with Wilson disease who exhibited neurological symptoms were included. Neurological manifestations were characterized by examination in terms of symptom type and degree of neurological impairment (Unified Wilson's Disease Rating Scale [UWDRS] Part III) and correlated with degree of disability (UWDRS Part II), abnormalities in copper parameters and hepatic status.
resultsMost patients (62.3%) exhibited tremor and ataxia, whereas 15.1% were dystonic, and 11.3% had parkinsonism. Discrete or unclassified signs only were observed in 11.3% of patients. A good correlation between disability (UWDRS Part II) and neurological impairment (UWDRS Part III) was observed (Pearson r = 0.84). However, there was a lack of correlation when either disability or neurological impairment were analyzed with copper parameters or liver impairment.
conclusionsThe predominant neurological manifestations in this cohort of newly diagnosed Wilson disease patients were ataxia and tremor. Neurological impairment measured was highly correlated with the level of disability. However, hepatic manifestations of Wilson disease and copper levels did not appear to be correlated with neurological status and disability. These results highlight the challenges faced when assessing Wilson disease with its highly variable symptomatology.
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