Major or Mild Neurocognitive Disorder Due to Prion Disease

Major or Mild Neurocognitive Disorder Due to Prion Disease includes creutzfeld-jakob disease, fatal insomnia and kuru, however the most common type is creutzfeld-jakob disease.  Major or mild neurocognitive disorder due to prion disease is a neurodegenerative disease which rapidly progressess over several months. The symptoms include fatigue, anxiety, problems with appetite, sleeping or concentration, which may be followed after several weeks by incoorrdination, altered vision, or abnormal gait or other movements that may be myoclonic, choreoatheoid or ballistic followed by the onset of rapidly progressive dementia. 

Firstly, the criteria needs to be met for either mild or major neurocognitive disorder, there is a gradual and insidious progression of impairment and the disorder is not better explained by another mental disorder or attributable to another medical condition.  Major neurocognitive disorder includes a significant decline from a previous level of performance in either complex attention, executive function, learning and memory, language, perceptual-motor, or social cognitive based on concern of a person who knows the informant or the clinical; and the impairment is documented by a standardised neuropsychological test or another quantified clinical assessment.   Lastly the cognitive deficit is not better explained by another medical condition.  On the other hand, mild neurocognitive disorder is when the person has a modest cognitive decline from a previous level of performance and the deficits does not interfere with the ability to be independent but needs greater effort or compensatory strategies are needed to cope. ​ The  impairment is rapid and insidious, motor features of prion disease (myoclonus or ataxia), or a biomarker are present and there is no attributable  to another medical condition and not better explained by another mental disorder.  

The disorder is transmutable (e.g., mad cow disease) with agents such as abnormal proteins called prions or otherwise known as slow viruses.   The prions build up in the cells of the brain causing shrinkage.  

People of any age may develop major or mild neurocognitive disorder due to prion disease but mostly people between the age of 40-60 are more susceptible to it. 

​Source:
American Psychiatric Association. (2013). Neurocognitive disorders. In Diagnostic and Statisical Manual of Mental Disoders DSM-5 (pp. 591-643). Arlington, VA: American Psychiatric Association.