What are the WHO probable sCJD criteria?

Probable sCJD: rapidly progressive dementia + ≥2 of (myoclonus, visual/cerebellar, pyramidal/extrapyramidal, akinetic mutism) + positive biomarker (EEG PSWC, 14-3-3 CSF, positive RT-QuIC in CSF, or typical MRI DWI/FLAIR basal ganglia/cortical ribboning).

What MRI findings suggest CJD?

DWI cortical ribboning (high signal following cortical gyri) and/or basal ganglia hyperintensity are characteristic; sensitivity 90%, specificity 93% for sCJD; "hockey-stick sign" in posterior thalamus suggests variant CJD (vCJD) from BSE exposure.

What is RT-QuIC and how sensitive is it for CJD?

Real-time quaking-induced conversion (RT-QuIC) amplifies misfolded prion protein in CSF; sensitivity ~90%, specificity ~99%; superior to 14-3-3 CSF (sensitivity 80-90%, low specificity); nasal brushing RT-QuIC is an alternative non-invasive approach.

What EEG pattern is characteristic of sCJD?

Periodic sharp wave complexes (PSWC) at 0.5-2 Hz on a suppressed background; seen in ~60-70% of sCJD; sensitivity increases with serial EEG recordings; absent in vCJD; not specific (also in metabolic encephalopathies, NCSE).

What infection control measures are required for CJD?

Standard precautions for routine patient care; high-risk procedures (brain surgery, neurosurgery, spinal cord, eye): single-use instruments or prion sterilization (134°C gravity steam or NaOH/hypochlorite); endoscopy instruments: enhanced decontamination if CJD suspected.

Prion Disease (CJD) Diagnostic Criteria and Surveillance Score

Applies updated WHO/EUROCJD diagnostic criteria for sporadic CJD (sCJD), classifies as possible/probable/definite, scores CSF 14-3-3 protein, MRI DWI/FLAIR, EEG PSWC, and RT-QuIC biomarker support.

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Frequently Asked Questions

What are the WHO probable sCJD criteria?

What MRI findings suggest CJD?

What is RT-QuIC and how sensitive is it for CJD?

What EEG pattern is characteristic of sCJD?

What infection control measures are required for CJD?