Catalog

Classical sporadic Creutzfeldt-Jakob Disease

No MMS code

Link to WHO Definition

Classical sporadic Creutzfeldt-Jakob Disease – http://id.who.int/icd/entity/1855860109

The most common human prion disease, typically characterised by rapidly progressive dementia, ataxic gait, myoclonus, akinetic mutism, and visual disturbance. Magnetic resonance imaging of the brain, on diffusion-weighted imaging (DWI) sequences, reveals patchy hyperintensity of the cerebral cortices and basal ganglia. Cerebrospinal fluid shows increased 14-3-3 and tau proteins. There are periodic synchronous discharges (PSD) on electroencephalogram (EEG). This is the classical or typical sCJD and its neuropathology is characterised by spongiform encephalopathy consisting of neuronal loss, neuropil vacuolation, and astrogliosis.