ArticleActa neurologica Belgica2023
Serum neurofilament light chain and initial severity of neurological disease predict the early neurological deterioration in Wilson's disease.
Article in Acta neurologica Belgica, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 29 citations in OpenAlex.
- Evaluation of novel assays of non-ceruloplasmin copper to monitor chelation treatment in patients with Wilson disease.JHEP reports : innovation in hepatology · 2026Trial
- Phenotype-specific white matter microstructural alterations and their clinical correlates in Wilson disease.Scientific reports · 2026Article
- The gut-liver-kidney-brain axis in Wilson disease: copper speciation-flux and barrier-mediated organ crosstalk.Frontiers in immunology · 2026Review
- Plasma Glial Fibrillary Acidic Protein Correlates With Brain Metal Burden in Wilson's Disease.Annals of clinical and translational neurology · 2025Article
- Diffusion Restriction in Wilson's Disease: A Radiological Pointer Toward Clinically Severe Disease.Annals of Indian Academy of Neurology · 2025Article
- Clinical Determinants and Prognostic Impact of Delayed Diagnosis in Wilson's Disease.Diagnostics (Basel, Switzerland) · 2025Article
- Exploring the Prognostic Role of Neurofilaments and SEMA3A in Multiple Sclerosis Progression.International journal of molecular sciences · 2025Observational
- N,N'-bis(2-mercaptoethyl)isophthalamide (NBMI) as a novel chelator for Wilson's disease.Free radical biology & medicine · 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
- DMPS-induced neurological deterioration in neurological Wilson's disease patients: a retrospective case-control study on clinical characteristics and risk factors.Frontiers in neurology · 2025Article
- Introducing neurofilament light chain measure in psychiatry: current evidence, opportunities, and pitfalls.Molecular psychiatry · 2024Review
- Brain Magnetic Resonance Imaging in Wilson's Disease-Significance and Practical Aspects-A Narrative Review.Brain sciences · 2024Review
- The Role of Glia in Wilson's Disease: Clinical, Neuroimaging, Neuropathological and Molecular Perspectives.International journal of molecular sciences · 2024Review
- Correlation between neuroimaging, neurological phenotype, and functional outcomes in Wilson's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Article
- Treating the Neurologic Manifestations of Wilson's Disease With Liver Transplantation.The Neurohospitalist · 2024Article
- Gandouling alleviates nerve injury through PI3K/Akt/FoxO1 and Sirt1/FoxO1 signaling pathway to inhibit autophagy in the rats model of Wilson's disease.Brain and behavior · 2023Article
- Wilson's disease-An early diagnosis to improve outcomes.United European gastroenterology journal · 2023Article
- Copper Deficiency as Wilson's Disease Overtreatment: A Systematic Review.Diagnostics (Basel, Switzerland) · 2023Review
- Optical coherence tomography in patients with Wilson's disease.Brain and behavior · 2023Article
- Blood Based Biomarkers of Central Nervous System Involvement in Wilson's Disease.Diagnostics (Basel, Switzerland) · 2023Review
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn Wilson's disease (WD), early neurological deterioration after treatment initiation is associated with poor outcomes; however, data on this phenomenon are limited. Our study analysed the frequency and risk factors of early neurological deterioration in WD.
methodsEarly neurological deterioration, within 6 months from diagnosis, was defined based on the Unified Wilson's Disease Rating Scale (UWDRS): any increase in part II or an increase of ≥ 4 in part III. In total, 61 newly diagnosed WD patients were included. UWDRS scores, brain magnetic resonance imaging (MRI) scores, copper metabolism parameters, treatment type and serum neuro-filament light chain (sNfL) concentrations at diagnosis were analysed as potential risk factors of early deterioration.
resultsEarly neurological deterioration was observed in 16.3% of all WD patients; all cases of worsening occurred in the neurological phenotype (27.7%). Higher scores were seen in those who deteriorated compared with those who did not for UWDRS part II (4.3 ± 5.0 vs 2.0 ± 5.9; p < 0.05), UWDRS part III (21.5 ± 14.1 vs 9.3 ± 16.4; p < 0.01) and MRI-assessed chronic damage (3.2 ± 1.6 vs 1.4 ± 2.2; p = 0.006); all these variables indicated the initial severity of neurological disease. Pre-treatment sNfL concentrations were significantly higher in patients who deteriorated compared with those who did not (33.2 ± 23.5 vs 27.6 ± 62.7 pg/mL; p < 0.01). In univariate logistic regression amongst all patients, chronic damage MRI scores, UWDRS part III scores and sNfL concentrations predicated early deterioration. In the neurological WD, only sNFL were a significant predictor. In bivariate logistic regression amongst all patients, sNfL remained the only significant predictor of deterioration when corrected for MRI scores.
conclusionsNfL concentrations are a promising biomarker of the risk of early neurological deterioration in WD.
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