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iatrogenic Creutzfeldt-Jacob disease
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The use of prion-contaminated medication, grafts and instruements may result in iatrogenic Creutzfeldt-Jacob disease (CJD).
The clinical features of the disease vary according to the route of innoculation.
Central innoculation, such as an infected dural graft, results in a rapidly progressive neurodegenerative disease similar to sporadic CJD, characterised by:
- ataxia
- dementia
- myoclonus
- rididity
- akinetic mutism
- survival is 2-12 months from presentation
Peripheral innoculation, such as the old human growth hormone products, results in a slightly less acute disease disease characterised by:
- progressive cerebellar ataxia
- dementia is not invariably present
- survival is 8-18 months from presentation
Last reviewed 01/2018
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