Creutzfeldt-Jakob Disease Flashcards
1
Q
What is Creutzfeldt-Jakob disease?
A
Rapidly progressive neurological condition caused by prion proteins
2
Q
What are the features of Creutzfeldt-Jakob disease?
A
Dementia (rapid onset)
Myoclonus
3
Q
What are the investigations for Creutzfeldt-Jakob disease?
A
CSF normal
EEG: Biphasic, high amplitude sharp waves
MRI: Hyperintense signals in the basal ganglia and thalamus
4
Q
What are the types of Creutzfeldt-Jakob disease?
A
Sporadic (85%) - 10-15% familial, onset age 65yrs
New variant - onset 25yrs, genetic factors, survival 13m
5
Q
What are other prion diseases?
A
Kuru
Fatal familial insomnia
