pathology final Flashcards

1
Q

true or false
All degenerative diseases show fast
deterioration

A

false , slow deterioration

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2
Q

how do the aggregated proteins cause the dysfunction

A

1) they have a direct toxic effect

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

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3
Q

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

A

false , they are not infectious from person to person

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4
Q

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

A

motor weakness , the main disease is amyotrophic lateral sclerosis

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5
Q

causes of dementia?

A

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

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6
Q

complications of dementia

A

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

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7
Q

what is the most important risk factor for Alzheimer

A

Age

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8
Q

The most commonly recognized symptom of

Alzheimer is

A

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

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9
Q

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

A

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

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10
Q

true or false

APP is a cell surface protein with multiple transmembrane domains

A

false, it only has a single transmembrane domain

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

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11
Q

what is the function of APP ?

A

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

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12
Q

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

A

the non-amyloidogenic pathway cuts in the middle while

the amyloidogenic pathway cuts at the N terminus

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13
Q

true or false

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

A

true

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14
Q

true or false

aggregation of tau causes neuronal damage and it causes microtubules instability

A

true

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15
Q

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

A

false, sporadic AD

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

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16
Q

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

A

true

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17
Q

the amyloid plaques are divided into ?

A

focal and diffuse plaques

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18
Q

how does the FTLD differ from AD

A

changes in personality and language precede

memory loss .

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19
Q

When Tau is hyperphosphorylated two changes

occur:

A

1) decrease binding ability to microtubules

2) it’s ability to aggregate increases

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20
Q

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

A

true

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21
Q

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

A

Tau protein

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22
Q

parkinsonism is characterized by:

A

bradykinesia, instability, rigidity, tremors

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23
Q

causes of parkinsonism :

A

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

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24
Q

true or false

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

A

true

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25
Q

true or false

the incidence of Parkinson’s increases with age

A

true

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26
Q

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

A

cogwheel rigidity, resting tremor ( pin-rolling tremor, postural tremor , lower limb tremor(in supine position))

note: the tremors diminish completely during sleep

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27
Q

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

A

Lewy body dementia

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28
Q

what protein accumulates in Parkinson’s

A

alpha synuclein

29
Q

Lewy bodies are?

A

alpha synuclein inclusions

30
Q

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

A

true

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

31
Q

Characteristic features of this PD with dementia are

A

fluctuating course and hallucinations

32
Q

true or false

Huntington’s disease had a sporadic form

A

false

33
Q

what is the role of the huntingtin protein

A

it has a role in long term memory storage

34
Q

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

A

true

35
Q

huntingtin’s disease causes what histologic changes

A

intra-nuclear inclusions

36
Q

the Huntington’s protein is cleaved by ?

A

caspases

37
Q

true or false

Huntington disease can cause memory loss , severe dementia and behavioral changes such as suicide

A

true

38
Q

all spinocerebellar ataxia are caused by

A

trinucleotide repeat expansion

39
Q

true or false

ataxia plays a role in cognitive and mood problems

A

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

40
Q

what are the symptoms of Fredrich ataxia ?

A

1)gait ataxia 2) hand clumsiness 3) dysarthria

it is associated with cardiac abnormalities and DM

41
Q

what is the function of frataxin?

A

a protein that regulates cellular iron level especially in the mitochondria

42
Q

very imp

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

A

false, in Fredrich ataxia it is caused by protein loss

43
Q

true or false

ALS is 100% lethal

A

true

44
Q

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

A

true

45
Q

can vitamin B 12 cause brain atrophy

A

yes, which causes the dementia

46
Q

the main cells affected by hypoglycemia in the brain

A

hippocampal neurons and Purkinje cells

47
Q

what are the most common primary tumors

A

gliomas

note: 80% of gliomas are diffuse astrocytoma’s

48
Q

true or false

grade 1 diffuse astrocytoma is known as pilocytic astrocytoma

A

false, there is no DIFFUSE astrocytoma

49
Q

the characteristics of glioblastoma multiform

A

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

50
Q

if the vascular proliferation is marked and clear it forms?

A

glumeruloid body

51
Q

pilocytic astrocytoma’s are most commonly found in

A

cerebellum

52
Q

pilocytic astrocytoma has the same mutation as diffuse astrocytoma ?

A

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

53
Q

are the Rosenthal fibers specific for pilocytic astrocytoma

A

no, they can also be found in chronic gliosis

54
Q

true or false

oligodendrogliomas are mainly found in the cerebral hemispheres in the gray matter.

A

false,. in the WHITE matter

55
Q

which one has a better prognosis astrocytoma or Oligodendroglioma

A

oligodendroglioma

56
Q

describe the appearance of cell in oligodendrogliomas

A

fried egg appearance

57
Q

histology of ependymoma

A

Rosette around canals and pseudo-Rosette around blood vessels

58
Q

what is the origin of Embryonal neoplasms

A

they have a neuroendocrine origin

59
Q

the most common embryonal neoplasm is ?

A

medulloblastoma

60
Q

medulloblastoma occurs Exclusively in

A

cerebellum

occurs predominantly in children

61
Q

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

A

WNT pathway

62
Q

true or false

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

A

true

63
Q

true or false

WNT tumors arise exclusively in an older age group of children over the age of three years

A

true

64
Q

meningiomas arise in

A

ADULTS

65
Q

what are the 4 histologic types of meningiomas

A

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

66
Q

which type of brain tumors resemble sarcoma

A

anaplastic meningioma

67
Q

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

A

primary CNS lymphoma

68
Q

manifestations of paraneoplastic syndromes

A

dementia, ataxia, psychosis, sensory neuropathy

69
Q

Von Hippel Lindau syndrome mutation?

A

mutation in VHL tumor suppressor gene

note: Increase risk of renal cell carcinoma