CHAPTER: Neuro Path

Question 1

2 histo findings and where each are found for Alz - which correlates with the degree of dementia?

Answer 1

NF tangles = tau = intra-cell 
# tangles correlates with degree of dementia 
AB amyloid - plaques @ gray matter 
Complication amyloid angiopathy -> intracranial hemm

Question 2

What are Pick bodies? How does their location in the brain contribute to symptoms?

Answer 2

Pick = tau
@ frontal cortex = behavioral changes
@ temporal cortex = language changes (aphasia)
Can progress to dementia

Question 3

Presentation of Lewy body dementia

Answer 3

Dementia :

1. Visual hallucinations
2. Parkinson features (tremor) - once LBs infiltrate SN

Question 4

Presentation osmotic demyelination syndrome before it progresses to locked in syndrome

Answer 4

Acute paralysis
Change speech 
Diplopia
Lose consciousness 
Think PONS

Question 5

What happens if you correct HYPERna too fast?

Answer 5

Cerebral edema + herniation

Question 6

Presentation of MS

Answer 6

Sudden vision loss w/ blown pupils 
INP 
Hemiparesis / sensory loss
Bladder/bowel problems 
SIN:
Scanning speech
Intention tremor 
Nystagmus

Question 7

Dx MS labs + imaging

Answer 7

Labs: oligoclonal Ig in CSF + myelin basic protein (from demyelin)
Perventi spots on MRI separated in space + time

Question 8

You know GB presents with ascending paralysis - what are other presentations

Answer 8

↑ICP b/c ↑protein in CSF -> papilledema
Facial paralysis
Autonomic dysreg: ↑HR, ↓BP

Question 9

What CNS change might occur after chicken pox//measles infection or rabies//small pox vaccine

Answer 9

Peri-vent demyelin

Lesions all at 1 time (vs MS)

Question 10

Inheritance, problem and presentation of Charcot Marie Tooth disease

Answer 10

AD - can’t make proteins for myelin or peripheral nerves

1. High arch foot
2. LE weakness

Question 11

Inheritance, enzyme def, accum proteins of Krabbe

Answer 11

AR - lysosomal storage
X galactocerebrosidase
↑GC + psychosine = causes damage

Question 12

Symptoms + histo findings Krabbe

Answer 12

Peripheral neuropathy
Mental delay
Optic atrophy
Globoid cells on histo

Question 13

Inheritance, enzyme def, accum of proteins of MCLD

Answer 13

AR - lysosomal storage
X Aryl-sulfatase A
↑Sulfatides

Question 14

Symptoms of MCLD

Answer 14

Peripheral AND central demyelinaiton

Ataxia + dementia

Question 15

Inheritance adrenoleukodystrophy

Answer 15

XL - can’t breakdown very long chain FAs

Question 16

Most common origin partial seizures

Answer 16

Medial temporal lobe

Question 17

3 substances released that cause migraines

Answer 17

Sub P
Calcitonin gene related peptide - CGRP
Vasoactive peptides

Question 18

What’s the difference between trigeminal neuralgia vs cluster headaches?

Answer 18

Trigem = lasts seconds 
Cluster = mins/hrs

Question 19

Describe nystamgus of peripheral vs central vertigo

Answer 19

Peripheral = delayed horizontal 
Central = immediate nystagmus in any direction (brain stem or cerebellar lesion)

Question 20

Tumor syndrome that presents with
Hemangioblastomas in the brain + spine
Cavernous hemangiomas in skin/organs
Bilat RCC
Pheo

Answer 20

VHL - chr 3

Question 21

Disease + inheritance of: 
Cafe au lait 
Lisch nodules = iris hamartomas 
Cutaneous neurofibromas
Optic gliomas
Pheo

Answer 21

NF1- chr 17
AD w/ full penetrance but variable expression
Parent and child with same disorder but different symptoms

Question 22

Problem + symptoms of Sturge Weber

Answer 22

Somatic mutation - bad neural crest cells

1. Port wine birth mark on V1/2 dist of face
2. Angiomas in brain -> seizures
3. ↑IOP -> early glaucoma

Question 23

Adult tumor that presents in frontal lobes

Answer 23

Oligodendroglioma

Question 24

Adult tumor with thin walled capillaries and little parenchyma in the middle

Answer 24

Hemangioblastoma - makes sense! Think VHL

Question 25

Which adult tumor is S100 +

Answer 25

Schwannoma

Question 26

Kid tumor that is HW rosettes and can cause drop mts

Answer 26

Medulloblastoma

Question 27

What kid tumor looks like germ cell tumors

Answer 27

Pinealoma