Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neuro II > Path II > Flashcards

Path II Flashcards

1
Q

What are the features of Tay Sachs disease?

(use the pneumonic)

A
  • T - testign recommended
  • A - autosomal recessive
  • Y - young death (<4yr)
  • S - spot in maula (cherry red spots)
  • A - ashkeniazi jew
  • C - CNS degeneration
  • H - hex A deficiency
  • S - storage disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the other names for Tay Sachs disease?

A

Neuronal lipidosis

GM2 gangliosidosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

The provided microscopic images are characteristic of what condition?

A

Tay Sachs Disease

Left - ganglion cells w/ lipid vacuolization

Right - prominent lysosome with whorled configuration on EM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the most common lysosomal storage disease?

A

Gaucher Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the cause of Gaucher disease?

A

glucocerebrosidase deficiency

-> accumulation of glucocerebroside (main in macrophages)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What unique cells are shown in the provided bone marrow aspirates & which disease are the characteirstic of?

A

Gaucher cells: histiocytes w/ “crinkled paper” appearance

Gaucher disease

“terribly gauche to arrive at a fancy party in a wrinkled dress”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the cause of Niemann-Pick Disease, types A/B?

A

acid sphingomyelinase deficiency results in storage of sphingomyelin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the clinical picture of a patient with Niemann-Pick Disease, types A/B?

A

early symptoms

failure to thrive -> hepatosplenomegaly & neural regression

brain become atrophic

macular cherry-red spot

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Which disease do you see a macular cherry-red spot?

A

Niemann-Pick Disease, types A/B

Tay-Sachs Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

The provided microscopic images are characteristic of what condition?

What is the name of each of the cell types?

A

Niemann-Pick Disease, Type A/B

Left - Foamy histiocyte

Right - Lamellar bodies on EM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Fill out the provided summary table:

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

The provided images show pathology in what structures?

This is due to what deficiency?

A

Mamillary body

Thiamine (B1) deficiency

  • left - necrosis & petechial hemorrhage
  • right - yellow d/t subacute hemorrhage & somewhat atrophic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Thiamine is what vitamin?

A

B1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Thiamine deficiency can lead to what condition?

A

Wernicke-Korsakoff Syndrome

&

Wernicke encephalopathy (acute condition)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Chronic severe vitamin B12 deficiency can lead to what condition?

A

degeneration of the axones in ascendign tractsof posterior columns & descending pyramidal tracts

also, degeneration of brain, nerves of eyes, & peripheral nerves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What conditoin is caracteristic of the shown spinal cord segment with myelin stain?

A

severe demyelination of the posterior & lateral columns

chronic severe vitamin B12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

The provided pathology is likely caused by what?

A

CO poisoning

bilateral necrosis of globus pallidus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the chronic CNS sequelae of chronic excessive alcohol use?

A

cerebellar dysfunction - truncal ataxia, unsteady gait, nystagmus

atrophy & granular cell loss in anterior vermis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Degenerative diseases affect what type of matter?

A

gray matter disease - loss of neurons

secondary changes in white matter tracts

20
Q

General cause of degenerative diseases?

A

protein aggregates that aer resistant to degradation via the ubiquitin-proteasome system

“cellular inclusions”

21
Q

What is the most common neurodegenerative disease?

A

Alzheimer’s

most common cause of dementia in the elderly

22
Q

Almost all individuals in what demographic get Alzeimer disease if they live past 45?

A

trisomy 21

23
Q

What pathology is shown in the provided image?

A

Cortical atrophy (consistent with Alzheimer disease)

24
Q

What pathology is shown in the provided image?

A

Cortical atrophy (consistent with Alzheimer disease)

25
Q

What are the 2 main processes of Alzheimer Disease?

A
  • Extracellular
    • deposition of AmyloidB -> senile plaques
  • Intracellular
    • accumulation Tau proteins -> neurofibrillary tangles
26
Q

The left is an H&E stain and the right is a Beta amyloid stain. What is the nme of the structures on the slides? They are characteristic of what disease?

A

senile plaques (extracellualr accumulation amyloid B)

Alzheimer Disease

27
Q

The left is an H&E stain and the right is a silver stain. What is the nme of the structures on the slides? They are characteristic of what disease?

A

Neurofibrillary tangles

Alzheimer Disease

28
Q

What is the name of the structures shown in the provided congo red stain?

They are seen in what condition?

They increase risk of what situation?

A

Cerebral Amyloid Angiopathy

Alzheimers disease (but not only seen in AD)

  • narrows lumen ; loss of SM - (increases risk of infarct & hemorrhage)
29
Q

What are they overarching sympoms of frontotemporal dementias?

A

disorders with progressive deterioration of language & personalty changes

30
Q

Pick Disease age of onset?

A

45-65 yr

(younger than Alzheimer)

31
Q

What is the caue of Pick disease?

Symptoms?

A

tauopathy: abnormal deposits of hyperphosphorylated tau

alterations in personality (frontal) & language disturbances (temporal)

32
Q

The provided image is indicative of what pathology?

A

Pick disease

Frontotemporal atrophy “walnut brain”

33
Q

The stain on the left is H&E; the stain on the right is Tau immunostain

What is the name of the characteristic cell shown in the samples & what is the probable disease?

A

Balooned Cell (Pick Bodies)

Pick Disease

34
Q

What is is the age of onset for Parkinson Disease?

What nerurological symptom is uncommon in Parkinson Disease?

A

>60 yr

dementia is uncommon

35
Q

Parkinson Disease is generally responsive to what therapies?

A

L-DOPA

antichoninergic drugs

36
Q

What is the name of the characteristic cell shown in the samples & what is the probable disease?

A

Lewy Body - ubiquitin & a-synuclein round inclusions

Parinkson Disease

37
Q

A. is a normal brain.

What pathology is shown in the B?

A

depigmented substantia nigra

Parkinson Disease

38
Q

What are the symptoms of Diffuse Lewy Body Disease?

A

combined features of Parkinsonism & dementia

Motor dysfunction

fluctuating attention & cognition and hallucinations

depression, sleep disorder & autonomic dysfunction

39
Q

If this is a sample from a patient with Diffuse Lewy Body diseae, what immunostain is being used?

A

alpha-synuclein

small, inconspicuous Lewy bodies seen throughout the brain

40
Q

A large proportion of patients with Diffuse Lewy Body Disease also show pathology for what disease?

A

Alzheimer Disease

41
Q

What is the age of onset for Huntington disease?

Inheritance pattern?

Symptoms?

A
  • 30-40s
  • autosomal dominant - fatal
  • behavioral changes, chorea, dementia
42
Q

What is the genetic cause of Huntington Disease?

A

HD gene on 4p16.3 -> encode huntington protein

normally 6-35 CAG repeat in first exon (higher number -> HD ; higher the number, the earlier the onset)

43
Q

What condition is depicted in the diseased brain shown on the right of the image?

A

Huntington Disease

atrophy of basal ganglia & cerebral cortex, dilation of ventricle

44
Q

The top spinal segment shows the anterior motor roots & the lower spinal segment shows the posterior sensory roots. What do you notice? This is indicative of what condition?

A

Attenuation of the surface of the anterior motor root (look atrophied)

ALS

45
Q

ALS is what type of disease?

A

Degenerative disease affecting motor neurons

Neuro II (30 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions