Brain iron accumulation in Wilson's disease: A longitudinal imaging case study during anticopper treatment using 7.0T MRI and transcranial sonography

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10366687" target="_blank" >RIV/00216208:11110/18:10366687 - isvavai.cz</a>

Alternative codes found

RIV/61989592:15120/18:73593720 RIV/00064165:_____/18:10366687

Result on the web

<a href="http://dx.doi.org/10.1002/jmri.25702" target="_blank" >http://dx.doi.org/10.1002/jmri.25702</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1002/jmri.25702" target="_blank" >10.1002/jmri.25702</a>

Result language

angličtina

Original language name

Brain iron accumulation in Wilson's disease: A longitudinal imaging case study during anticopper treatment using 7.0T MRI and transcranial sonography

Original language description

Imaging studies in Wilson&apos;s disease (WD) commonly show hyperechogenicity of the lentiform nucleus (LN) on transcranial sonography (TCS)1 and hypointense lesions in the deep gray matter (DGM) on T2 -weighted magnetic resonance imaging (MRI).2,3 In a postmortem MRI-histopathology correlation study, cerebral T 2 lesions in WD were associated with increased iron content and the presence of iron-containing macrophages, but not with copper concentration.4 WD can be treated by inducing a negative copper balance. However, early worsening after treatment initiation occurs in up to 50% of patients with neurologic symptoms, and results in severe permanent disability in 20% of them.5 Possible connections between iron accumulation, neurodegeneration, and clinical worsening on anticopper treatment in WD are poorly understood. Rapid mobilization of copper from tissues with subsequent elevation of toxic free copper may accelerate neurodegenerative changes that could be accompanied by the influx of iron and activation of macrophages. Chelation therapy can also prevent incorporation of copper into ceruloplasmin and negatively affect its ferroxidase activity, which is necessary for tissue iron efflux. Ceruloplasmin dysfunction inherent to WD pathophysiology and aggravated by chelation therapy may thus contribute to iron accumulation in WD.6

Czech name

—

Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30103 - Neurosciences (including psychophysiology)

Project

<a href="/en/project/NV15-25602A" target="_blank" >NV15-25602A: Biomarkers of progression and treatment response in neurodegenerative disorders</a><br>

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2018

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Journal of Magnetic Resonance Imaging

ISSN

1053-1807

e-ISSN

—

Volume of the periodical

47

Issue of the periodical within the volume

1

Country of publishing house

US - UNITED STATES

Number of pages

4

Pages from-to

282-285

UT code for WoS article

000417880000029

EID of the result in the Scopus database

2-s2.0-85017345607