Disease Directory Creutzfeldt-Jakob Disease
Neurological

Creutzfeldt-Jakob Disease

Also known as: CJD, sporadic CJD, variant CJD (vCJD), familial CJD, prion disease, transmissible spongiform encephalopathy

Prevalence

1-9 per 1,000,000 (Orphanet)

Onset

Adult

Type

Prion (sporadic, genetic PRNP mutation, or acquired)

Gene

PRNP

About Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease is the most common human prion disease, caused by misfolding and aggregation of the prion protein (PrP) encoded by PRNP. Sporadic CJD (sCJD) accounts for ~85% of cases and arises spontaneously; familial CJD is caused by PRNP mutations; variant CJD (vCJD) is linked to BSE ('mad cow disease') exposure. The disease causes rapidly progressive dementia, myoclonus, cerebellar ataxia, and visual disturbances, with death typically within 1 year of onset in sCJD.

Common Clinical Features

Rapidly progressive dementia Myoclonus Cerebellar ataxia Visual disturbances Periodic sharp-wave complexes on EEG 14-3-3 protein in CSF Cortical ribboning on DWI MRI

Clinical Trial Eligibility Tips

What to know before applying to Creutzfeldt-Jakob Disease trials.

RT-QuIC assay on CSF is now the gold standard diagnostic test — positive result is required for most trial enrollment

CJD subtype (sporadic, familial, variant) determines trial eligibility — PRNP genotyping (codon 129 MM/MV/VV) is required

Rapidly progressive nature requires urgent trial contact — eligibility windows may be only weeks from symptom onset

Brain MRI DWI pattern (cortical ribboning, basal ganglia signal) and EEG periodic sharp waves are required diagnostic documentation

Patient Resources

Patient Organization

CJD Foundation

Visit website ↗

Natural History Registry

National Prion Disease Pathology Surveillance Center

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:204)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Creutzfeldt-Jakob Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Creutzfeldt-Jakob Disease. Updated daily.

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