ArticleJournal of neural transmission (Vienna, Austria : 1996)2014
Lenticular nucleus hyperechogenicity in Wilson's disease reflects local copper, but not iron accumulation.
Article in Journal of neural transmission (Vienna, Austria : 1996), 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 2 of them syntheses that pooled it.
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15 citing papers in PubMed, 2 syntheses or guidelines pooled it, 30 citations in OpenAlex.
- Systematic review-based guideline "Parkinson's disease" of the German Society of Neurology: diagnostic use of transcranial sonography.Journal of neurology · 2024Guideline
- Pooled it
- Use of Estonian Biobank data and participant recall to improve Wilson's disease management.European journal of human genetics : EJHG · 2025Article
- High correlation of quantitative susceptibility mapping and echo intensity measurements of nigral iron overload in Parkinson's disease.Journal of neural transmission (Vienna, Austria : 1996) · 2025Article
- The characteristic and biomarker value of transcranial sonography in cerebellar ataxia.Annals of clinical and translational neurology · 2024Article
- Echogenicity of Brain Structures in Huntington's Disease Patients Evaluated by Transcranial Sonography - Magnetic Resonance Fusion Imaging using Virtual Navigator and Digital Image Analysis.Ultraschall in der Medizin (Stuttgart, Germany : 1980) · 2023Article
- Diabetic Striatopathy: Parenchymal Transcranial Sonography as a Supplement to Diagnosis at the Emergency Department.Diagnostics (Basel, Switzerland) · 2022Article
- Value of ultrasound fusion imaging in detecting vascular cerebral white matter pathology.The ultrasound journal · 2022Article
- Copper Dyshomeostasis in Neurodegenerative Diseases-Therapeutic Implications.International journal of molecular sciences · 2020Review
- Transcranial sonography changes in heterozygotic carriers of the ATP7B gene.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Article
- Cardiac assessment in Wilson's disease patients based on electrocardiography and echocardiography examination.Archives of medical science : AMS · 2019Article
- Wilson disease.Nature reviews. Disease primers · 2018Review
- Transcranial Sonography in Mitochondrial Membrane Protein-Associated Neurodegeneration.Clinical neuroradiology · 2018Article
- [Diagnostics of Wilson's disease].Der Nervenarzt · 2018Review
- Developments in the role of transcranial sonography for the differential diagnosis of parkinsonism.Current neurology and neuroscience reports · 2015Review
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In patients with Wilson's disease (WD) transcranial brain sonography typically reveals areas of increased echogenicity (hyperechogenicity) of the lenticular nucleus (LN). Correlation with T2-hypointensity on magnetic resonance images suggested that LN hyperechogenicity in WD is caused by trace metal accumulation. Accumulation of both, copper and iron, in the brain of WD patients has been reported. The present study was designed to elucidate whether LN hyperechogenicity in WD reflects accumulation of copper or iron. Post-mortem brains of 15 WD patients and one non-WD subject were studied with ultrasonography in an investigator-blinded fashion. LN hyperechogenicity was measured planimetrically by manual tracing as well as using digitized image analysis. The putaminal copper content was determined in samples of 11 WD brains and the non-WD brains using inductively coupled plasma mass spectrometry, and iron content was assessed using flame atomic absorption spectroscopy. LN was normal on ultrasonography only in the non-WD brain, but abnormal (hyperechogenic) in all WD brains. Digitized image analysis measures of LN hyperechogenicity and, by trend, manual measures correlated with putaminal copper content (Pearson test; digitized: r = 0.77, p = 0.04; manual: r = 0.57, p = 0.051) but not with iron content (each, p > 0.18). LN hyperechogenicity measures were unrelated to age at death of patients, age at onset of WD, WD duration, age of brain specimen, serum copper or serum ceruloplasmin (each, p > 0.1). We conclude that LN hyperechogenicity in WD reflects copper, but not iron accumulation. Further studies are warranted to elucidate the use of transcranial brain sonography for monitoring therapeutic effects of chelating agents in WD patients.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.