Neuro- Pathology

Question 1

What genes are associated with early onset Alz?

Answer 1

APP, PS-1, PS-2

Question 2

What gene is associated with late onset Alz and which gene is protective?

Answer 2

ApoE4- late onset

ApoE2- protective

Question 3

Compare senile plaques and neurofibrillary tangles?

Answer 3

Senile plaque- extraceulluar b amyloid core; amyloid b from cleaving APP
Neurofibrillary tanges- intracellular hyperphosphorylated tau protein

Question 4

Presents as dementia, aphasia, parkinsonian aspects, change in personality

Answer 4

Pick disease (frontotemporal dementia)

Question 5

Describe the microscopic and gross findings of Pick disease?

Answer 5

Pick bodies- spherical tau protein aggregates
frontotemporal atrophy (spares parietal lobe and post 2/3 of superior temporal gyrus)

Question 6

Presents as rapid progressive dementia with myoclonus

Answer 6

CJD

Question 7

What are 2 important diagnostic findings in MS?

Answer 7

oligoclonal IgG bands in CSF

periventricular plaques

Question 8

Presents as scanning speech, intention tremor, nystagums

Answer 8

Charcot triad of MS (SIN)

Question 9

what are the treatments of MS?

Answer 9

b-interferon, immunosuppression, natalizumab

Question 10

What 2 infections are commonly associated with Acute inflammatory demylinating polyradiculopathy?

Answer 10

Campy, CMV

Question 11

Describe adrenuoleukodystrophy

Answer 11

X-linked

impaired metabolism of VLCFA- build up in nervous system, adrenal, testes

Question 12

What viral infection is at increased risk with natalizumab?

Answer 12

PML (JC virus)

Question 13

What cell type is affected in PML?

Answer 13

oligodendrocytes

Question 14

What gene is defective in metachromatic leukodystrophy and what builds up?

Answer 14

arylsulfatase A, AR

buildup of sulfatides (impaired production of myelin sheath)

Question 15

Genetic disorder presents as central and peripheral demyelination with ataxia, dementia

Answer 15

Metachromatic leukodystrophy

Question 16

What genetic defect presents with scoliosis and foot deformities?

Answer 16

Charcot-Marie-Tooth disease

Question 17

What is the inheritance of Charcot-Marie Tooth? What genes are affected?

Answer 17

Aut Dom

proteins involved in structure and function of peripheral nerves or myelin sheath

Question 18

What is status epilepticus?

Answer 18

continuous seizure for >30 min or no gain of consciousness between seizures for >30 min

Question 19

What area of the brain do partial/focal seizures most commonly originate?

Answer 19

Medial temporal lobe

Question 20

Compare partial vs complex partial seizure

Answer 20

simple- motor, sensory, autonomic, psychic; no loss of consciousness
complex- loss of consciousness

Question 21

What EEG waves are seen in abscence seizures?

Answer 21

3hz

Question 22

Describe myoclonic, tonic-clonic, tonic, and atonic seizures

Answer 22

myoclonic- quick, repetitive jerks
tonic-clonic- alternating stiffening and movement
tonic- stiffening
atonic- drop seizure (often mistaken for fainting)

Question 23

How does positional testing distinguish peripheral vs central vertigo?

Answer 23

Peripheral- delayed horizontal nystagmus

central- immediated nystagmus in any direction

Question 24

What brain tumor is found in cerebral hemispheres and can cross corpus callosum

Answer 24

glioblastoma

Question 25

What brain tumor presents with spindle cells concentrically arranged in a whorled pattern and psammoma bodies?

Answer 25

Meningioma

Question 26

Brain tumor associated with chicken-wire capillary pattern and fried egg cells, often calcified

Answer 26

oligodendroglioma

Question 27

Brain tumor often found at cerebellopontine angle, S-100+

Answer 27

shchawnnoma

Question 28

What brain tumor can produce EPO?

Answer 28

hemangioblastoma

Question 29

Brain tumor associated with von hippel lindau and often cerebellar

Answer 29

hemangioblastoma

Question 30

Brain tmor assocated with closely arranged, thin-walled capillaries with minimal interleaving parenchyma

Answer 30

hemangioblastoma

Question 31

Compare the common locations of children vs adult brain tumors?

Answer 31

Children- mostly infratentorial, adult mostly supratentorial

Question 32

Brain tumor presents with Homer-Wright rosettes, small blue cells

Answer 32

medulloblastoma

Question 33

Brain tumor shows perivascular rosettes, rod-shaped blpharoplasts

Answer 33

ependymoma

Question 34

Brain tumor shows rosenthal fibers

Answer 34

pilocytic astrocytoma

Question 35

Where are pilocytic astrocytomas typicall found

Answer 35

posterior fossa (eg cerebellum)

Question 36

Where are ependymomas typically found?

Answer 36

4th ventricle

Question 37

What is the most common childhood supratentorial tumor

Answer 37

craniopharyngioma

Question 38

What type of herniation can compress the ACA?

Answer 38

cingulate herniation under falx cerebri

Question 39

What type of herniation can compress the brain stem and result in coma and death

Answer 39

cerebellar tonsillar herniation

Question 40

What can present as CNII lesion, contralateral homonymous hemianopsia, ipsilateral paralysis?

Answer 40

Uncal herniation