Creutzfeldt-Jakob Disease (CJD) is a rare, fatal, rapidly progressive dementia. It affects about 1 in every 1 million people per year worldwide. CJD has a rapid course and usually appears in late life. There is a very short survival time, death usually occurs within one year of symptom onset.
Major or mild neurocognitive disorder (NCD) due to prion disease includes the group of subacute spongiform encephalopathies including Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia.
The most common subtypes of Creutzfeldt-Jakob disease are:
The criteria are met for major or mild neurocognitive disorder.
There is insidious onset, and rapid progression of impairment is common.
There are motor features of prion disease, such as myoclonus or ataxia, or biomarker evidence.
The neurocognitive disorder is not attributable to another medical condition and is not better expiated by another mental disorder.
CJD should always be considered in a patient with the combination of the 2
cardinal clinical features: