Major or Mild Neurocognitive Disorder due to Prion Disease
Major or Mild Neurocognitive Disorder due to Prion Disease
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Prevalence
Prevalence
Prevalence is unknown but very low given the short survival.
Risk factors
Risk factors
Environmental
Environmental
Cross-species transmission of prion infections, with agents that are closely related to the human form, has been demonstrated (e.g., the outbreak of bovine spongiform encephalopathy inducing variant CJD in the United Kingdom during the mid- 1990s).
Transmission by corneal transplantation and by human growth factor injection has been documented, and anecdotal cases of transmission to health care workers have been reported.
Genetic and physiological
Genetic and physiological
There is a genetic component in up to 15% of cases, associated with an autosomal dominant mutation.
Symptoms
Symptoms
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.
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