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POLG Mutation – Valproate Contraindicated — SCE Neurology MCQ

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HardEpilepsy & Seizure DisordersPOLG Mutation – Valproate ContraindicatedSCE Neurology

A 19-year-old man presents with occipital seizures, migrainous visual aura, sensory ataxia and external ophthalmoplegia. His sister died from unexplained hepatic failure after starting an antiseizure medicine. Which proposed treatment should be withheld while urgent genetic assessment proceeds?

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Correct answer: E — Sodium valproate because a POLG-related disorder is suspected

Explanation lettering: C = shown as A · E = shown as B · B = shown as C · A = shown as D · D = shown as E

A has no general mitochondrial renal contraindication. B gives a false syndrome rule. C is mechanistically incorrect. D is correct: occipital epilepsy, ataxia, ophthalmoplegia and familial valproate-associated hepatic failure raise concern for POLG disease, in which valproate is contraindicated because it can precipitate fatal hepatotoxicity. E is not the defining hazard and does not address the family history.

Reference: Sodium valproate: Summary of Product Characteristics: https://www.medicines.org.uk/emc/product/1500/smpc