Psychiatry Flashcards
What type for disease is Creutzfeldt-Jakob disease (CJD)?
A prion disease - thought to arise from the transformation of normal host-encoded prion proteins to aberrantly folded protease resistant isoforms.
How is nvCJD spread?
The new variant prion CJD - nvCJD, is spread to humans by eating meat that has been infected with bovine spongiform encephalopathy in cattle (mad cow diseasE).
What are the four variants of CJD?
- Sporadic - 85% cases
- Hereditary just under 15%
- Iatrogenic CJD
- nvCJD
How does sporadic CJD occur and what is the prevalence of it?
It occurs in approximately 1 in a million, and is due to a spontaneous mutation of the prion protein, it is not transmitted.
What is Gerstmann-Straussler-Scheinker syndrome (GSS)
A rare inherited prion disease characterised by adult onset of memory loss, dementia, ataxia and pathological deposition of amyloid-like plaques in the brain. It is classified as a transmissible spongiform encephalopathy.
How can CJD present?
- Myoclonus
- Visual disturbances
- Cerebella/pyramidal/extrapyramidal signs
- Rapidly progressive cognitive and functional impairment
(often non-specific symptoms which are difficult to diagnose before death)
What is the prognosis of someone with CJD?
Up to 2 years normally - illnesses that are gradual in progression with a duration of more than 3 years are most likely non-prion neurodegenerative brain diseases
Biopsy from where can help with diagnosis of nvCJD?
Tonsil biopsy
What investigations can be useful in trying to distinguish between forms of CJD and perhaps with diagnosis too?
- MRI
- EEG
- Brain biopsy (in life or post-mortem)
PIck’s disease is a form of what type of dementia?
Frontotemporal dementia
What is the pathophysiology of frontotemporal dementia?
There is atrophy of the frontal and temporal lobes. Distribution is lobar, rather than diffuse atrophy of Alzheimer’s disease. There may be loss of neurones or gloss, but no increase in plaque formation.
