At the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose. Creutzfeldt-Jakob disease CJD is a transmissible spongiform encephalopathy that results in rapidly progressive dementia and death usually within a year from onset.
The vast majority are sporadic, but familial and acquired forms are occasionally encountered. On imaging, it classically manifests as hyperintense signal on DWI and usually FLAIR in regions of the cerebral grey matter cortex, followed by the striatum, followed by thalamus. Sporadic Creutzfeldt-Jakob disease is characterized by rapidly progressive dementia and other features of neuropsychiatric decline resulting in death within a year of onset.
Other common features include myoclonus, visual hallucinations, cerebellar dysfunction such as ataxia and nystagmus , pyramidal or extrapyramidal signs such as spasticity, rigidity, dystonia, or bradykinesia , and eventually akinetic mutism Brownell-Oppenheimer variant : initial cerebellar ataxia.
Heidenhain variant : initial visual symptoms such as impaired visual acuity, distortions of shapes and colors, and visual hallucinations. Stern-Garcin variant : initial extrapyramidal features. Variant Creutzfeldt-Jakob disease presents mostly with psychiatric symptoms such as depression and sensory symptoms dysesthesias or paresthesias. CSF protein : positive result in a patient suspected clinically of having sporadic Creutzfeldt-Jakob disease. A definitive diagnosis requires a brain biopsy, although in many institutions the difficulty involved in sterilizing equipment renders a biopsy undesirable.
Creutzfeldt-Jakob disease is mediated via prions , a type of protein, which manifests in sheep as the disease scrapie, and in cows as bovine spongiform encephalopathy. Prions are considered infectious in the sense that they can alter the structure of neighboring proteins. Creutzfeldt-Jakob disease leads to spongiform degeneration of the brain, which is thought to be caused by the conversion of normal prion protein to proteinaceous infectious particles that accumulate in and around neurons and lead to cell death.
