Neurological Symptoms of Wilson's Disease: What to Do When Treatment Switch Has Not Achieved Improvement

This protocol addresses patients with Wilson's disease who present with a neurological clinical picture and whose symptoms have continued to worsen despite having already undergone a structured switch of copper-lowering therapy.

Clinical scenario: Wilson's disease with a neurological presentation. Either zinc or chelators should be used in this population, with treatment selection guided by individual patient factors.

Previous treatment line — failure condition

The prior step involved switching the copper-lowering regimen — moving between chelators and zinc, or to trientine as a second-line option.

Escalation to the current protocol is triggered when that switch fails to produce progressive improvement of neurological symptoms — specifically, failure to achieve a decrease or normalisation of the UWDRS score.

Next-line approach: For patients with continuous neurological deterioration despite optimised medical therapy, a transplantation-based approach may be considered in appropriate cases. Full eligibility criteria and the clinical decision pathway are set out in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2024.11.007

Either zinc or chelators should be used in patients with a neurological presentation (LoE 2, strong recommendation, consensus).

LT should be considered on a case-to-case basis in patients with a continuous worsening of neurological symptoms despite at least 6 months of optimised medical treatment (LoE 2, strong recommendation, consensus).

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