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

Kód výsledku v IS VaVaI

RIV/00216208:11110/18:10366687 - isvavai.cz

Nalezeny alternativní kódy

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

Výsledek na webu

http://dx.doi.org/10.1002/jmri.25702

DOI - Digital Object Identifier

10.1002/jmri.25702

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

Imaging studies in Wilson'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

Název v anglickém jazyce

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

Popis výsledku anglicky

Imaging studies in Wilson'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

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30103 - Neurosciences (including psychophysiology)

Projekt

NV15-25602A: Biomarkery progrese a terapeutické odpovědi u neurodegenerativních onemocnění

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2018

Kód důvěrnosti údajů

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

Název periodika

Journal of Magnetic Resonance Imaging

ISSN

1053-1807

e-ISSN

—

Svazek periodika

47

Číslo periodika v rámci svazku

1

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

4

Strana od-do

282-285

Kód UT WoS článku

000417880000029

EID výsledku v databázi Scopus

2-s2.0-85017345607