Tackling the neurological manifestations in Wilson's disease - currently available treatment options.
Wilson’s disease
dysphagia, dysarthria
dystonia, chorea, parkinsonism
neurological symptoms
symptomatic treatment
Journal
Expert review of neurotherapeutics
ISSN: 1744-8360
Titre abrégé: Expert Rev Neurother
Pays: England
ID NLM: 101129944
Informations de publication
Date de publication:
16 Oct 2023
16 Oct 2023
Historique:
medline:
16
10
2023
pubmed:
16
10
2023
entrez:
16
10
2023
Statut:
aheadofprint
Résumé
Wilson'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's quality of life. 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. 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.
Identifiants
pubmed: 37842984
doi: 10.1080/14737175.2023.2268841
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM