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Open Access 01-10-2019 | Wilson's Disease | Original Article

Brain volume is related to neurological impairment and to copper overload in Wilson’s disease

Authors: Lukasz Smolinski, Tomasz Litwin, Barbara Redzia-Ogrodnik, Karolina Dziezyc, Iwona Kurkowska-Jastrzebska, Anna Czlonkowska

Published in: Neurological Sciences | Issue 10/2019

Abstract

Introduction

To determine whether brain volume was associated with functional and neurological impairments and with copper overload markers in patients with Wilson’s disease.

Methods

In 48 treatment-naïve patients, we assessed functional and neurological impairments with the Unified Wilson’s Disease Rating Scale, measured normalized brain volumes based on magnetic resonance images, and assessed concentration of non-ceruloplasmin-bound copper. We correlated brain volume measures with functional and neurological impairment scores and copper overload indices.

Results

Functional and neurological impairments correlated with all brain volume measures, including the total brain volume and the volumes of white matter and gray matter (both peripheral gray matter and deep brain nuclei). Higher non-ceruloplasmin-bound copper concentrations were associated with greater functional and neurological impairments and lower brain volumes.

Conclusions

Our findings provided the first in vivo evidence that the severity of brain atrophy is a correlate of functional and neurological impairments in patients with Wilson’s disease and that brain volume could serve as a marker of neurodegeneration induced by copper.

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Title: Brain volume is related to neurological impairment and to copper overload in Wilson’s disease
Authors: Lukasz Smolinski
Tomasz Litwin
Barbara Redzia-Ogrodnik
Karolina Dziezyc
Iwona Kurkowska-Jastrzebska
Anna Czlonkowska
Publication date: 01-10-2019
Publisher: Springer International Publishing
Keywords: Wilson's Disease
Wilson's Disease
Published in: Neurological Sciences / Issue 10/2019
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI: https://doi.org/10.1007/s10072-019-03942-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Brain volume is related to neurological impairment and to copper overload in Wilson’s disease

Abstract

Introduction

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusions

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