Vol 54, No 2 (2020)
Research paper
Published online: 2020-04-07
Submitted: 2019-08-10
Accepted: 2020-01-09
Get Citation

Transcranial sonography changes in patients with Wilson’s Disease during de-coppering therapy

Marta Skowrońska, Tomasz Litwin, Iwona Kurkowska-Jastrzębska, Anna Członkowska
DOI: 10.5603/PJNNS.a2020.0028
·
Pubmed: 32253748
·
Neurol Neurochir Pol 2020;54(2):185-192.

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Vol 54, No 2 (2020)
Research paper
Published online: 2020-04-07
Submitted: 2019-08-10
Accepted: 2020-01-09

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.

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Keywords

transcranial sonography, Wilson’s Disease, therapy, substantia nigra, lenticular nucleus

About this article
Title

Transcranial sonography changes in patients with Wilson’s Disease during de-coppering therapy

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 54, No 2 (2020)

Pages

185-192

Published online

2020-04-07

DOI

10.5603/PJNNS.a2020.0028

Pubmed

32253748

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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