Early neurological deterioration in Wilson's disease: a systematic literature review and meta-analysis.
Chelators
Magnetic resonance imaging
Neurological deterioration
UWDRS
Wilson’s disease
Zinc salts
Journal
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
received:
19
02
2023
accepted:
02
06
2023
medline:
12
9
2023
pubmed:
14
6
2023
entrez:
13
6
2023
Statut:
ppublish
Résumé
Neurological deterioration, soon after anti-copper treatment initiation, is problematic in the management of Wilson's disease (WD) and yet reports in the literature are limited. The aim of our study was to systematically assess the data according to early neurological deteriorations in WD, its outcome and risk factors. Using PRISMA guidelines, a systematic review of available data on early neurological deteriorations was performed by searching the PubMed database and reference lists. Random effects meta-analytic models summarized cases of neurological deterioration by disease phenotype. Across the 32 included articles, 217 cases of early neurological deterioration occurred in 1512 WD patients (frequency 14.3%), most commonly in patients with neurological WD (21.8%; 167/763), rarely in hepatic disease (1.3%; 5/377), and with no cases among asymptomatic individuals. Most neurological deterioration occurred in patients treated with d-penicillamine (70.5%; 153/217), trientine (14.2%; 31/217) or zinc salts (6.9%; 15/217); the data did not allow to determine if that reflects how often treatments were chosen as first line therapy or if the risk of deterioration differed with therapy. Symptoms completely resolved in 24.2% of patients (31/128), resolved partially in 27.3% (35/128), did not improve in 39.8% (51/128), with 11 patients lost to follow-up. Given its occurrence in up to 21.8% of patients with neurological WD in this meta-analysis of small studies, there is a need for further investigations to distinguish the natural time course of WD from treatment-related early deterioration and to develop a standard definition for treatment-induced effects.
Identifiants
pubmed: 37311952
doi: 10.1007/s10072-023-06895-6
pii: 10.1007/s10072-023-06895-6
pmc: PMC10495500
doi:
Substances chimiques
Penicillamine
GNN1DV99GX
Trientine
SJ76Y07H5F
Copper
789U1901C5
Types de publication
Systematic Review
Meta-Analysis
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
3443-3455Informations de copyright
© 2023. The Author(s).
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