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Non-convulsive status epilepticus — SCE Neurology MCQ

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HardEpilepsyNon-convulsive status epilepticusSCE Neurology

A patient develops rapidly progressive confusion, startle-induced multifocal myoclonus and gait ataxia over eight weeks. MRI shows cortical ribboning and caudate diffusion restriction; CSF is acellular. Which test provides the most specific supportive evidence for sporadic Creutzfeldt–Jakob disease?

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Correct answer: ECSF real-time quaking-induced conversion assay for prion seeding

The best answer is “CSF real-time quaking-induced conversion assay for prion seeding”. RT-QuIC detects prion-seeding activity with high specificity and, combined with a compatible rapidly progressive syndrome and diffusion MRI, strongly supports sporadic CJD without requiring brain biopsy. “Serum creatine kinase with skeletal-muscle isoenzyme analysis” is less appropriate because creatine kinase does not identify prion disease “Carotid duplex ultrasonography with flow-velocity measurement” is less appropriate because extracranial arterial imaging cannot explain the diffuse cortical and striatal process “Acetylcholine-receptor antibody testing by validated immunoassay” is less appropriate because junctional antibodies do not assess rapidly progressive encephalopathy “Routine EEG interpreted as an exclusion test after one recording” is less appropriate because periodic EEG changes may be absent, so a normal recording does not exclude the disease

Reference: NICE NG127: Suspected neurological conditions — recommendations. https://www.nice.org.uk/guidance/ng127/chapter/Recommendations