Sketchy Path: Neuro - "Creutzfeldt-Jakob Disease, Parkinson's Disease & Huntington's Disease"

Question 1

Mad cow disease has what two other names?

Answer 1

Creutzfeldt-Jakob disease or bovine spongiform encephalopathy

Question 2

What is the notation for the normal and abnormal protein in CJD?

Answer 2

PrPc is normal and PrPsc is abnormal (the sc stands for scravy)

Question 3

Describe the protein shape that results in CJD.

Answer 3

Normal alpha helices get converted to beta-pleated sheets (like the normal alpha lanyard next to the bent beta keys)

Question 4

The brain of a person with CJD will be _____________ on pathologic exam.

Answer 4

spongy with vacuoles in the gray matter (like the spongy car seat)

Question 5

CJD presents with the classic duo of _____________.

Answer 5

rapidly progressive dementia with myoclonus

Question 6

What diagnostic can help definitively diagnose CJD?

Answer 6

EEG showing sharp waves (like the sharp waves on the side of his car)

Question 7

Review the genetic inheritance features of Huntington disease.

Answer 7

• Autosomal dominant (like the dominos around the Hunting Town store)
• 100% penetrance (there’s a lot of dominos)
• CAG repeats (like the repeating pockets on his CArGo pants)
• Trinucleotide
• Chromosome 4
• Shows anticipation (like the “ANTICIPATE great savings” sign on the store)

Question 8

Which part of the brain is affected by HD?

Answer 8

Caudate (like the CoD on the Hunting Town store)

Question 9

What cells are affected in HD?

Answer 9

Loss of GABA-A releasing cells in the caudate (like the GABA cab next to the store)

Question 10

Describe the symptoms of HD.

Answer 10

• Chorea (rapid jerking movements like the guy dancing)

* Psychiatric changes (like his mask)

Question 11

The four signs of parkinsonism are ______________.

Answer 11

• Bradykinesia (most common, like the slow sign)
• Resting tremor (guy shaking with his money)
• Postural instability (guy falling)
• Rigidity (bank door)

Question 12

Which receptor is involved in parkinsonism?

Answer 12

D2 (like the Rx sign with two ropes)

Question 13

Go through Sketchy’s symbols for the basal ganglia activation pathway.

Answer 13

1) The substantia nigra disinhibits the striatum (like how the black bag –SN –allowed the guy in the STRIped suit to get into the bank)
2) The striatum activates the subthalamic nucleus (like the STRIped suit guy activating the SUBmachine gun)
3) The STN inhibits the thalamus (like how the SUBmachine gun scares the lady who represents the thalamus)

Question 14

Deep brain stimulation is inserted into which brain structure?

Answer 14

STN (like the cop zapping the SUBmachine gun)