Tackling the neurological manifestations in Wilson's disease - currently available treatment options

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064165%3A_____%2F23%3A10470919" target="_blank" >RIV/00064165:_____/23:10470919 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11110/23:10470919

Result on the web

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=IydJnrWL2a" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=IydJnrWL2a</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1080/14737175.2023.2268841" target="_blank" >10.1080/14737175.2023.2268841</a>

Result language

angličtina

Original language name

Tackling the neurological manifestations in Wilson's disease - currently available treatment options

Original language description

Introduction: Wilson&apos;s disease (WD) is a potentially treatable, inherited disorder resulting from impaired copper metabolism. Pathological copper accumulation causes a range of symptoms, most commonly hepatic and a wide spectrum of neurological symptoms including tremor, dystonia, chorea, parkinsonism, dysphagia, dysarthria, gait and posture disturbances. To reduce copper overload, anti-copper drugs are used that improve liver function and neurological symptoms in up to 85% of patients. However, in some WD patients, treatment introduction leads to neurological deterioration, and in others, neurological symptoms persist with no improvement or improvement only after several years of treatment, severely affecting the patient&apos;s quality of life. Areas covered: This review appraises the evidence on various pharmacological and non-pharmacological therapies, neurosurgical procedures and liver transplantation for the management of neurological WD symptoms. The authors also discuss the neurological symptoms of WD, causes of deterioration and present symptomatic treatment options. Expert opinion: Based on case and series reports, current recommendations and expert opinion, WD treatment is focused mainly on drugs leading to negative copper body metabolism (chelators or zinc salts) and copper-restricted diet. Treatment of WD neurological symptoms should follow general recommendations of symptomatic treatment. Patients should be always considered individually, especially in the case of severe, disabling neurological symptoms.

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

—

Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2023

Confidentiality

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

Name of the periodical

Expert Review of Neurotherapeutics

ISSN

1473-7175

e-ISSN

1744-8360

Volume of the periodical

23

Issue of the periodical within the volume

12

Country of publishing house

GB - UNITED KINGDOM

Number of pages

11

Pages from-to

1249-1259

UT code for WoS article

001084194000001

EID of the result in the Scopus database

2-s2.0-85174266601