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Transcranial sonography changes in patients with Wilson’s Disease during de-coppering therapy
, 1, 1, 1
Affiliations- Institute of Psychiatry and Neurology, Warsaw, Poland
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Abstract
Introduction. Wilson’s Disease (WD) is an inherited disorder of impaired hepatic copper metabolism that leads to copper accumulation in organs such as the liver and brain. Using transcranial sonography (TCS), we investigated brain changes in WD patients during de-coppering treatment.
Methods. Forty-one consecutive treatment-naïve WD patients were classified as having hepatic (WDh; n = 20) or neurological WD (WDn; n = 21) based on symptoms at diagnosis; all patients received either D-penicillamine or zinc sulfate and were observed for 24 months. TCS was performed at regular intervals from study entry (month 0) to month 24.
Results. At study entry, bilateral lenticular nucleus (LN) hyperechogenicity was found in 18 patients with WDn and in nine with WDh (p = 0.006). Substantia nigra (SN) hyperechogenicity was found in nine patients with WDn) and four with WDh (p = ns). After 24 months of treatment, bilateral LN hyperechogenicity was still present in 17 patients with WDn and 14 with WDh (p = ns). SN hyperechogenicity was present in one patient with WDn and two with WDh (p = ns). The decrease in the number of patients with SN hyperechogenicity was significant in the WDn group (p < 0.05).
Conclusions. LN hyperechogenicity is the most common TCS abnormality in WD patients, and was observed despite two years of de-coppering treatment. SN hyperechogenicity was less common, and decreased after treatment introduction.
Abstract
Introduction. Wilson’s Disease (WD) is an inherited disorder of impaired hepatic copper metabolism that leads to copper accumulation in organs such as the liver and brain. Using transcranial sonography (TCS), we investigated brain changes in WD patients during de-coppering treatment.
Methods. Forty-one consecutive treatment-naïve WD patients were classified as having hepatic (WDh; n = 20) or neurological WD (WDn; n = 21) based on symptoms at diagnosis; all patients received either D-penicillamine or zinc sulfate and were observed for 24 months. TCS was performed at regular intervals from study entry (month 0) to month 24.
Results. At study entry, bilateral lenticular nucleus (LN) hyperechogenicity was found in 18 patients with WDn and in nine with WDh (p = 0.006). Substantia nigra (SN) hyperechogenicity was found in nine patients with WDn) and four with WDh (p = ns). After 24 months of treatment, bilateral LN hyperechogenicity was still present in 17 patients with WDn and 14 with WDh (p = ns). SN hyperechogenicity was present in one patient with WDn and two with WDh (p = ns). The decrease in the number of patients with SN hyperechogenicity was significant in the WDn group (p < 0.05).
Conclusions. LN hyperechogenicity is the most common TCS abnormality in WD patients, and was observed despite two years of de-coppering treatment. SN hyperechogenicity was less common, and decreased after treatment introduction.
Keywords
transcranial sonography, Wilson’s Disease, therapy, substantia nigra, lenticular nucleus
About this articleTitle
Transcranial sonography changes in patients with Wilson’s Disease during de-coppering therapy
Journal
Neurologia i Neurochirurgia Polska
Issue
Article type
Research Paper
Pages
185-192
Published online
2020-04-07
Page views
1274
Article views/downloads
375
DOI
Pubmed
Bibliographic record
Neurol Neurochir Pol 2020;54(2):185-192.
Keywords
transcranial sonography
Wilson’s Disease
therapy
substantia nigra
lenticular nucleus
Authors
Marta Skowrońska
Tomasz Litwin
Iwona Kurkowska-Jastrzębska
Anna Członkowska
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