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Acute disseminated encephalomyelitis — SCE Neurology MCQ

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HardMSAcute disseminated encephalomyelitisSCE Neurology

A 24-year-old develops dysarthria, dystonia and psychiatric change. Slit-lamp examination shows Kayser–Fleischer rings; ceruloplasmin is low and 24-hour urinary copper is high. What is the long-term treatment principle?

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Correct answer: ABegin specialist-supervised lifelong copper-lowering therapy and monitor clinical and biochemical response

The best answer is “Begin specialist-supervised lifelong copper-lowering therapy and monitor clinical and biochemical response”. Neurological Wilson disease requires lifelong anti-copper treatment selected and monitored by an experienced specialist; stopping therapy risks severe relapse. “Use short-course corticosteroids until the rings disappear” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “Give levodopa alone because the disorder is purely dopaminergic” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “Observe after dietary copper restriction because neurological disease is self-limiting” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “Arrange therapeutic phlebotomy as the principal metal-removal treatment” is a credible alternative elsewhere, but it fails the decisive discriminator in this case.

Reference: EASL–ERN clinical practice guideline on Wilson disease: https://pubmed.ncbi.nlm.nih.gov/40089450/