pathology final

Question 1

true or false
All degenerative diseases show fast
deterioration

Answer 1

false , slow deterioration

Question 2

how do the aggregated proteins cause the dysfunction

Answer 2

1) they have a direct toxic effect

2) the more they accumulate the less there are proteins which perform their function

Question 3

true or false
protein aggregates behave like
prions (INFECTIOUS FROM PERSON TO
PERSON)

Answer 3

false , they are not infectious from person to person

Question 4

if proteins accumulate in the motor neurons this will lead to?

Answer 4

motor weakness , the main disease is amyotrophic lateral sclerosis

Question 5

causes of dementia?

Answer 5

1) Alzheimer disease
2) Lewy body disease
3) frontotemporal dementia
4) infections
5) metabolic and endocrine disorders
6) nutritional deficiencies
7) reactions to medications
8) subdural hematoma
9) poisoning
10) brain tumors
11) anoxia

Question 6

complications of dementia

Answer 6

1) inadequate nutrition
2) pneumonia
3) inability to perform daily tasks
4) personal safety challenges
5) death

Question 7

what is the most important risk factor for Alzheimer

Answer 7

Age

Question 8

The most commonly recognized symptom of

Alzheimer is

Answer 8

inability to acquire new memories and not difficulty in recalling recently observed facts

Question 9

what is the affect of the accumulation of both AB amyloid proteins and tou protein

Answer 9

they both cause neuronal death and dysfunction, they accumulate due to increased production and decreased removal

Question 10

true or false

APP is a cell surface protein with multiple transmembrane domains

Answer 10

false, it only has a single transmembrane domain

first cleavage is in the extracellular domain , the second cleavage is in the intra-membranous domain

Question 11

what is the function of APP ?

Answer 11

it’s primary function is unknown but it has been implicated as a regulator of synapse formation and iron export

Question 12

which pathways cut in the middle of the APP and which pathway cuts at the N terminus

Answer 12

the non-amyloidogenic pathway cuts in the middle while

the amyloidogenic pathway cuts at the N terminus

Question 13

true or false

aggregation of beta amyloid leads to alteration in neurotransmission, damage or even death of neuron, inflammation

Answer 13

true

Question 14

true or false

aggregation of tau causes neuronal damage and it causes microtubules instability

Answer 14

true

Question 15

true or false
Certain polymorphisms in this locus on chromosome 19
increases the risk of inherited AD

Answer 15

false, sporadic AD

note: Point mutations in APP are a cause of familial
AD

Question 16

true or false
diagnosis of AD
needs both clinical and histological findings

Answer 16

true

Question 17

the amyloid plaques are divided into ?

Answer 17

focal and diffuse plaques

Question 18

how does the FTLD differ from AD

Answer 18

changes in personality and language precede

memory loss .

Question 19

When Tau is hyperphosphorylated two changes

occur:

Answer 19

1) decrease binding ability to microtubules

2) it’s ability to aggregate increases

Question 20

true or false
INHERITED forms have mutations in Tau protein
causing increased accumulation

Answer 20

true

Question 21

FTLD is a neurodegenerative disease where the primary abnormality is in?

Answer 21

Tau protein

Question 22

parkinsonism is characterized by:

Answer 22

bradykinesia, instability, rigidity, tremors

Question 23

causes of parkinsonism :

Answer 23

1) dopamine antagonists ,
2) toxins
3) Parkinson disease

Question 24

true or false

AD is the most common cause of dementia nd it is the most common cause of neurodegenerative disease

Answer 24

true

Question 25

true or false | the incidence of Parkinson's increases with age

Answer 25

true

Question 26

the rigidity and the tremor in Parkinson's are known as

Answer 26

cogwheel rigidity, resting tremor ( pin-rolling tremor, postural tremor , lower limb tremor(in supine position)) note: the tremors diminish completely during sleep

Question 27

if dementia occurs within the FIRST year in Parkinson's then it is known as

Answer 27

Lewy body dementia

Question 28

what protein accumulates in Parkinson's

Answer 28

alpha synuclein

Question 29

Lewy bodies are?

Answer 29

alpha synuclein inclusions

Question 30

true or false alpha synuclein has prion like pattern , it starts in the brain stem then the basal ganglia, then the cerebral cortex where it causes cognitive impairment

Answer 30

true note: the first alpha synuclein aggregates are found in the medulla

Question 31

Characteristic features of this PD with dementia are

Answer 31

fluctuating course and hallucinations

Question 32

true or false | Huntington's disease had a sporadic form

Answer 32

false

Question 33

what is the role of the huntingtin protein

Answer 33

it has a role in long term memory storage

Question 34

VERY IMPORTANT true or false the course of Huntington's disease is not affected by the number of repeats

Answer 34

true

Question 35

huntingtin's disease causes what histologic changes

Answer 35

intra-nuclear inclusions

Question 36

the Huntington's protein is cleaved by ?

Answer 36

caspases

Question 37

true or false | Huntington disease can cause memory loss , severe dementia and behavioral changes such as suicide

Answer 37

true

Question 38

all spinocerebellar ataxia are caused by

Answer 38

trinucleotide repeat expansion

Question 39

true or false | ataxia plays a role in cognitive and mood problems

Answer 39

true, because the cerebellum plays a role in some forms of thinking

Question 40

what are the symptoms of Fredrich ataxia ?

Answer 40

1)gait ataxia 2) hand clumsiness 3) dysarthria it is associated with cardiac abnormalities and DM

Question 41

what is the function of frataxin?

Answer 41

a protein that regulates cellular iron level especially in the mitochondria

Question 42

very imp | true or false all spinocerebellar ataxias cause neuronal damage by protein accumulation

Answer 42

false, in Fredrich ataxia it is caused by protein loss

Question 43

true or false | ALS is 100% lethal

Answer 43

true

Question 44

true or false in ALS : because there are several mutations, there are also several types of misfiled proteins and several types of inclusions

Answer 44

true

Question 45

can vitamin B 12 cause brain atrophy

Answer 45

yes, which causes the dementia

Question 46

the main cells affected by hypoglycemia in the brain

Answer 46

hippocampal neurons and Purkinje cells

Question 47

what are the most common primary tumors

Answer 47

gliomas note: 80% of gliomas are diffuse astrocytoma's

Question 48

true or false | grade 1 diffuse astrocytoma is known as pilocytic astrocytoma

Answer 48

false, there is no DIFFUSE astrocytoma

Question 49

the characteristics of glioblastoma multiform

Answer 49

1) necrotic( pesudopalisading) OR 2)vascular proliferation note: looks like an anaplastic pus

Question 50

if the vascular proliferation is marked and clear it forms?

Answer 50

glumeruloid body

Question 51

pilocytic astrocytoma's are most commonly found in

Answer 51

cerebellum

Question 52

pilocytic astrocytoma has the same mutation as diffuse astrocytoma ?

Answer 52

false, it doesn't have the IDH mutation but it has the BRAF mutation

Question 53

are the Rosenthal fibers specific for pilocytic astrocytoma

Answer 53

no, they can also be found in chronic gliosis

Question 54

true or false | oligodendrogliomas are mainly found in the cerebral hemispheres in the gray matter.

Answer 54

false,. in the WHITE matter

Question 55

which one has a better prognosis astrocytoma or Oligodendroglioma

Answer 55

oligodendroglioma

Question 56

describe the appearance of cell in oligodendrogliomas

Answer 56

fried egg appearance

Question 57

histology of ependymoma

Answer 57

Rosette around canals and pseudo-Rosette around blood vessels

Question 58

what is the origin of Embryonal neoplasms

Answer 58

they have a neuroendocrine origin

Question 59

the most common embryonal neoplasm is ?

Answer 59

medulloblastoma

Question 60

medulloblastoma occurs Exclusively in

Answer 60

cerebellum | occurs predominantly in children

Question 61

which medulloblastoma has a better prognosis, MYC amplification or WNT pathway

Answer 61

WNT pathway

Question 62

true or false | NUCLEAR beta catenin is a 100% indicator for the presence of a mutation in the WNT pathway

Answer 62

true

Question 63

true or false | WNT tumors arise exclusively in an older age group of children over the age of three years

Answer 63

true

Question 64

meningiomas arise in

Answer 64

ADULTS

Question 65

what are the 4 histologic types of meningiomas

Answer 65

1) syncytial 2) fibroblastic 3) transitional 4) Psammomatous

Question 66

which type of brain tumors resemble sarcoma

Answer 66

anaplastic meningioma

Question 67

what tumor is the most common CNS tumor in immunocompromised individual's

Answer 67

primary CNS lymphoma

Question 68

manifestations of paraneoplastic syndromes

Answer 68

dementia, ataxia, psychosis, sensory neuropathy

Question 69

Von Hippel Lindau syndrome mutation?

Answer 69

mutation in VHL tumor suppressor gene note: Increase risk of renal cell carcinoma