Prion Diseases Flashcards
1
Q
Creutzfeld-Jakob Disease (CJD) and MSA are caused by the accumulation of and of…?
A
PrPSc and α-synuclein respectively
2
Q
What is a fundamental event underlying CJD ?
A
The transition from a structure rich in α-helix to one with a high amount of β-structure
3
Q
Some neuropathologic characteristics of CJD ?
A
A lack of inflammatory response
4
Q
Clinical features of CJD ?
A
Deficits in higher cortical function, psychiatric symptoms such as depression, psychosis amd visual hallucinations. About 90% of patients exhibit myoclonus.Unlike other involuntary movements it persists during sleep.
5
Q
Radiological characteristics of CJD ?
A
Cortical ribboning and basal ganglia hyperintensity on FLAIR and DWI MRI (both highly sensitive and specific).
