Dementia 1

Question 1

Amyloid precursor protein is associated with what disease?

How?

What chromosome is APP gene located in?

Answer 1

Alzheimer’s disease

APP is the protein that makes up amyloid plaques which are the hallmark of pathology of AD.

Chromosome 21

Question 2

What is the diagnostic test of choice for the prion disease Creutzfeldt-Jakob disease?

What is the sensitivity and specificity of this test?

Answer 2

CSF assay of 14-3-3 and tau proteins.

Sensitivity and Specificity is >90%.

Question 3

What is the incidence of Creutzfeldt-Jakob disease?

What is the most common cause of CJD?

Answer 3

1 in a million.

So…300 new cases in USA a year.

Sporadic mutation is the most common cause (85%)

Question 4

What is the classic EEG pattern seen on Creutzfeldt-Jakob disease?

Answer 4

1 to 2 cycles-per-second triphasic sharp wave pattern superimposed on a background of electrical depression.

Question 5

What is the clinical course of Creutzfeldt-Jakob disease?

Answer 5

1. Prodrome = vegetative symptoms (asthenia = lack of energy), sleep and appetite disturbance.
2. Then rapidly progressive depression, self neglect, personality changes, myoclonic jerks, dementia and death (usually 2-7 months after symptom onset)

Question 6

What are the 5 Surgical procedures that can help in Parkinsons Disease?

Answer 6

1. Stereotactic Thalatomy
2. Thalamic DBS
3. Sterotactic Post Ventral Pallidotomy
4. Pallidal DBS
5. Subthalamic DBS (most preferred)

Question 7

What is dementia pugilistica?

What chromosomal mutation is associated with increased risk of developing it?

Answer 7

Post traumatic dementia due to repeated head injury, causing axonal shearing…think boxers!

Carriers of ApoE4 (Chromosome 19) are at increased risk of bother dementia pugilistica and Alzheimers.

Question 8

What are the classic deficits seen in dementia pugilistic or post-traumatic dementia?

Answer 8

1. Severe memory and attention difficulty.
2. Executive function difficulty
3. EPS

Question 9

Triphasic waves on EEG suggest what etiology of delirium?

Answer 9

HEPATIC encephalopathy or metabolic encephalopathy

Question 10

What are the histological findings found in Picks disease or Frontotemporal dementia?

Answer 10

1. Picks inclusion bodies in neurons
2. Gliosis (non specific reactive changes in glial cells)
3. Swollen Neurons