Highlights
- •Early neurological worsening is observed in almost 11% of WD patients.
- •It is limited to cases with neurological signs at the beginning of WD treatment.
- •It is usually reversible in most of the cases (66%).
- •It may be associated with several clinical, imaging and pharmacological factors.
- •The type of anti-copper therapy seems to have no clear impact.
Abstract
Background
Early neurological worsening during treatment initiation for Wilson's disease (WD)
is an unresolved problem. Our aim was to establish the frequency and outcome of early
neurological worsening in patients with WD.
Methods
We analyzed 143 symptomatic patients diagnosed with WD between 2005 and 2009. Early
neurological deterioration was based on worsening on the Unified Wilson's Disease
Score Scale, scored at baseline through 6 months or occurrence of new neurological symptoms. Reversibility of worsening was
followed up to 24 months.
Results
Early neurological worsening was observed in 11.1% (16/143) and involved only patients
with neurological signs at diagnosis. Mean time to worsening from treatment initiation
was 2.3 ± 1.9 months. Neurological deterioration was completely reversible in 53% (8/15) and partially
in 13% (2/15) of patients over 9.2 ± 5.2 months. Patients who experienced early deterioration had significantly more severe
baseline neurological deficit, higher prevalence of thalamic (66% vs 29%) and brain
stem (73% vs 33%) lesions seen on baseline magnetic resonance imaging, and more often
used concomitant dopamine receptor antagonists (46% vs 5%). Disease duration, treatment
type (d-penicillamine or zinc sulfate), type of neurological manifestations, initial copper
metabolism results, and liver function parameters did not differ between evaluated
groups.
Conclusions
Neurological worsening at the beginning of anti-copper therapy may occur in over 10%
of WD patients. Special attention should be paid to those with severe initial neurological
manifestations, advanced brain injury and using dopamine receptor antagonists. Type
of anti-copper therapy did not show clear association with early neurological worsening.
Keywords
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Article info
Publication history
Published online: June 07, 2015
Accepted:
June 5,
2015
Received in revised form:
May 27,
2015
Received:
December 31,
2014
Identification
Copyright
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
