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Flashcards in Prion Diseases Deck (5)
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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).