Dementia 8

Question 1

In sporadic/classic CJD, what is the classic EEG finding?

Answer 1

Fast polyphasic waves

Question 2

What is the confirmatory test for diagnosis in sporatic/classic CJD?

Answer 2

14-3-3 Protein assay of CSF

Question 3

In the inheritable/familial form of CJD…what gene is mutated and on what chromosome?

How is the clinical presentation of the familial form different from the classic CJD?

Answer 3

Prion gene (PRNP) on chromosome 20

Same symptoms but slower course/progression.

Question 4

How do you differentiate between sporadic/classic CJD vs acquired/variant CJD?

Answer 4

Classic CJD = EEG changes of periodic fast polyphasic waves. Variant doesn’t have this.

Variant CJD = Pulvinar sign on MRI - thalamus lights up. Classic doesn’t have this.

Question 5

What is the inheritance pattern and genetics of Huntington Disease ?

Answer 5

Autosomal dominant trinucleotide repeat. CAG.

“you HUNT animals and put the in a CAGe”

Question 6

1. What are the two drugs that can be helpful in treating Huntington’s Chorea?
2. What is the MOA?
3. What SE do you need to be wary of?

Answer 6

1. Tetrabenazine and Deutetrabenazine
2. Vesicular Monoamine transporter 2 inhibitor –> Causes Monoamine depletion.
3. Severe depression and suicidality (bc monoamine depletion)

Question 7

Which NT is most implicated in delirium?

Answer 7

Acetylcholine