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NMSP > degenerative diseases > Flashcards

degenerative diseases Flashcards

1
Q

these are diseases of _ matter

there is a progressive loss of _ with assoicated secondary changes in white matter tracts

A

gray

neurons

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2
Q

degernative diseases have protein aggregates that are resistant to degredation and are recognized histologically as _

A

inclusions

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3
Q

dementia

A

progressive loss of congitive function INDEPENDENT of the state of attention

NOT apartof normal aging and is also pathologic

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4
Q

_ is the most common cause of dementia in the elderly

A

Alzheimers disease

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5
Q

Alzhemier disease is insidious impairement of _ _ functions with alreration in mood and behavior

(AD)

A

higher intelectual

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6
Q

late clinical course of AD

A

disorientation, memory loss, aphasia

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7
Q

5-10 years in AD

A

diables, mute, immobile

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8
Q

AD is rarely symptomatic before the age of _

incidence rises with age and roughly double every _ years

A

50

5 years

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9
Q

most cases of AD are _

_ examination is necessary and definitve for the diagnosis of AD

A

sporadic

pathological

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10
Q

in AD there is global _ atrophy

there is widening of the _ mostly in the frontal, temporal amd later the _ lobes

A

cortical

pariteal

cloudy leptomeninges

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11
Q

becuase there is loss of cortex in AD the ventricles enlarge and create a compensatory _ _ _

A

hydrocephalus ex vacuo

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12
Q

2 pathologic hallmarks of AD

A

amyloid plaques and neurofibrillary tangles

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13
Q

the # of _ correlated better with the degree of dementia in AD

A

neurofibrillary tangles

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14
Q

plaques in AD are aggregates of _ peptides in the neuropil

(outside of the neuron)

A

Alpha B peptides

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15
Q

tangles are aggregates of microtubulule binding protein _ they develop intracellularly and after neuroal death they persist

A

tau

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16
Q

_ generation is the critical initiating event for the development of AD

A

AB plaques

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17
Q

diseases in which Tau deposits appear and AB plaques do not AD _ (does or does not develop)

A

does not

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18
Q

what encodes for TAU

A

MAPT

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19
Q

the genetic locus on chromosome _ that encodes for _ has a strong influence on the risk of developing AD

A

19

Apolipoprotein E

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20
Q

what is a neuritic plaque in AD

where are these located

also known as senile plques

A

neuritic processes around a amyloid core

HAN: hippocampus, amygdala and neocortex

amyloid core will stain with the congo red stain (derived from APP)

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21
Q

what are the diffuse plaques in AD and where are the located

A

no amyloid core

CBS: cerebellar cortex, basal ganglia, superifical cortex

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22
Q

down syndrome has an early onset of AD because

A

the gene encoding APP is on chromosome 21

presents the 2nd or third decade of life

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23
Q

neurofibrillary tangles are bundles of _ in cytoplasm of neurons that displace the nucleus (not specific to AD)

they stain on

A

filaments

bielschowsky stain

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24
Q

what is tau?

A

hyperphosphorylated axonal microtubule associated proteins that enhance microtubule assembly causing a tangle

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25
Q

stain for TAU

A

MAP2 and ubiquitin

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26
Q

fundamental abnormaility in AD is deposition of _ peptides which are derived through processing of _

large burden of plaques and tangles is associated with severe cognitive dyfxn.

tangles> plaques

A

AB peptide

APP (amyloid precursor protein)

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27
Q

histology of AD (besides tangles and palques)

A

granulovacuolar degeneration

hirano bodies

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28
Q

what are granulovacular degeneration

A

small clear intraneuronal cytoplasmic vacuoles (contain aygyrophilic granules)

  • apart of normal aging byt abundant in AD
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29
Q

what are hirano bodies

major component

A

elongated glassy eosinophilic bodies with actin as its major compnent

found in AD

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30
Q

_ _ _ invariably accompanied AD it has AB40 amyloid plaques and **thick vesseled walls **

A

cerebral amyloid angiopathy

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31
Q

frontotempoeral lobar degenerations are a group of disorders with focal degeneration of _ and _ lobes

also known as frontotemporal dementia, share clinical findings like AD need to evalute post mortem to known the difference

FTLD- Tau has _ only and the mutations may be in _ the . An example of this is _

TDP-43 has no _ and has 3 different genes (similar to ALS)

A

frontal and temporal

tau , MAPT, pick disease and prgressive supranuclear palsy

no tau

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32
Q

pick disease is a FTLD-Tau with lobar atrophy

it is rare by has progressive _

early onset of _ changes with alterations in personality this is a _ sign and language distuburbances with is a _ sign

A

dementia

behavioral changes ; frontal sign

temportal sign

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33
Q

pick disease is atrophy of the frontal and temporal lobes with sparing of the _

A

posterior 2/3 of superior temporal gyrus

34
Q

buzz words for pick disease

A

knife edge gyri (thin)

pick cells (swollen)

pick bodies (cytoplasmic innclusions, basophilic and stain with silver stain)

35
Q

in picks disease only the _ lobes are affected not global like _

A

frontal

AD

36
Q

what is progressive supranuclear palsy

A

truncal rigidity and disequilirium

difficulty wit heye movements

nuchal dystonia, demenita, abnormal speech

37
Q

progressive supranuclear palsy is a taupathy (PSP)- FTLD-TAU

it is more common in _ (women/men)

there is widespread _ loss

there are _ neurofibirllary tangles with _ Tau straight filaments

also considered a ?

A

men

neuronal

globose

4R

atypical parkisonian syndrome/Parkinson Plus syndrome

38
Q

vascular dementia is due to _ and imporves with treatment

it is a progressive _ disorder associated with _ injury

it affects the _ matter (white/gray/both)

there will be areas of widespread _

clinical syndrome: _, _ abnormalities, _ signs, and focal neurologic defecits

A

vassculitis

cognitive

vascular injury

both

infarction (due to ischemia)

dementia, gait abnormalities. pseudobulbar signs and nerologic defecits

39
Q

what are psuedobulbar signs

A

frequent, involuntary and uncontrollable outbursts of crying or laughing that are exaggerated or not connected to your emotional state.

40
Q

degenerative diseases of the basal ganglia and brainstem, are associated with _ disorders

sympotms: (3)

A

movement

rigidity, abnormal posturing, chorea

41
Q

parkinsonism is damage to the _ _ system

clinical sydrome of parkinsonism (4)

A

nitrostriatial dopaminergic system

  1. diminished facial expression
  2. slowness of movement (bradykinesia_
  3. fenestrating gait
  4. rigidity and pill rolling tremor
42
Q

wjat are the types of parkinsonisms

A

parkinsons disease
Multiple system atrophy
Post encephaltic parkeinsonism
progressive supranuclear palsy

43
Q

parkinsons disease is associated with protein accumulation, mitochondiral abnormalities, and neuronal loss in the _ _

hypokinetic movement is due to ?

there is a progressive loss of _ response that is why we can treat with _. _ is adminsited in order to inhibit peripheral metabolism of _

A

substantia nigra

loss of dopanergic neurons from the substantia nigra

L-Dopa

L-Dopa

Carbidopa

LEvadopa

44
Q

what gene encodes for a synculein in PD (parkinsons disease)

what chromosme is it on

A

SNCA

chromosome 4a21

45
Q

mitochondiral dysfunction in PD is caused b y mutations in ?

A

DJ-1, PINK1 and parkin

46
Q

a synculein makes

A

lewy bodies

47
Q

_ accounts for the juvinile autosomal recessive parkinsons disease

A

parkin

48
Q

what is the most common gene causing of autosomal dominant PD

A

LRRK2

49
Q

heterogenous mutations in _ are the most important risk factor for the development of PD

A

glucocerebrosides

50
Q

in parkinsons there is pallor of the _ and _

there is loss of pigmented _ neurons associated with gliosis

there are _ bodies that are located in the cytoplasm are eosinophilic. They are elongated and have a dense core surrounded by a pale _

A

substantia nigra and locus ceruleus

catecholaminergic

lewy

halo

(also a-synculein is present)

51
Q

parkinsons disease CAN be accompanied by _ dysfunction

A

congitive

52
Q

15% of PD patients develop _ and the incidence increases with age

A

dementia

53
Q

there is a flutuating course of _ and prominent _ signs in dementia with lewy bodies

A

hallucinations

frontal

54
Q

dementia with lewy bodies has lewy bodies in _ locations and lewy _ that contain a syncuclein, there is depigmentation of the _ and _

A

cortical

nitrites (dystrophic processes)

substantia nigra and locus cerelus

there is preservation in the cortex, hippocampus, and amygdala

55
Q

the diagnosis is lewy body dementia when

  1. dementia symptoms consitent with lewy body dementia develop _
  2. when both _ and _ symtpoms are present at the time of diagnosis
  3. when dementia appears within _ _ after movement symptoms
A

first

dementia and motor

1 year

56
Q

the diagnosis of parkinsons disease dementia when a person is orginally diagnosed with parkinsons based on _ symptoms and dementia symptoms occur more than a year later

A

movement

57
Q

atypical parkinsonian/ parkisonian plus syndromes

A

progressive supranuclear palsy
corticobasal degeneration
multisystem atrophy

58
Q

what is the hallmark of atypical parkinsonian/ parkisonian plus syndromes

A

they all include parkinsonism as a component but they are minimmally responsive to L-dopa and they have additional signs and symptoms

59
Q

corticobasal degernation is a progressive _ with extraprymidal _, asymmetric _ distuburbances (jerking of limbs) and imapired higher _ function (difficulty with skilled movement)

_ decline will present later in disease

tau lesions in the _

A

taupathy

rigidity

motor

cortical

cognitive decline later

cerebral cortex

60
Q

progressive subnuclear palsy has tau lesions in what regions?

A

brainstem and deep gray matter

61
Q

CBD (corticobasal degeneration) Tau can be found in _ ( tufted) and _ (coiled bodies)

A

astrocytes

oligodendrocytes

62
Q

presence of _ positive threads in gray and white matter may be the most specific pathologic finding in corticobasal degernation

A

tau

63
Q

in CBD the affected regions of the cortex shows _ neurons

A

ballooned

64
Q

what is multiple system atrophy

A

an atypical sporadic syndrome that is sporadic and involves differeny systems in the brain

65
Q

in multiple system atrophy there is _ cytoplasmic inclusions in _

A

alpha synclein

oligodendrocytes

66
Q

what are the three distinct neuroataomic systems involved in MSA

A

striatonigral circuit–> parkisonism

olivopontencerebellar-> ataxia

ANS–> autnomic dysfunction (orthostatic hypotension)

67
Q

MSA has severe atrophy of the

A

basis pontis

68
Q

huntingtons disease is autosomal _ it is a progressive _ disorder and _

A

dominant

movement and dementia

69
Q

huntingtons is degeneration of ?

A

striatal neurons

70
Q

movement disorder in huntingtons

A

jerky. hyperkinetic involving all parts of the body (chorea)

71
Q

HD repeat

A

polyglutamine trinucleotide on chromosome 4p16.3

CAG (10-35 copies)

72
Q

average age of huntingtons disease

A

4-5th decade with an average of a 15 year death

73
Q

COD of HD

A

penumonia and suicide

74
Q

HD CAG repeates

40-50= _ onset

> 60= _ onset

A

adult

juvenille

75
Q

HD shows anticipation which are repeat expansions during _ leads to _ onset with _ transmission

A

spermatogenesis

earlier

parental

76
Q

HD pathogenesis

A

loss of medium spiny striatal neurons leads to dysregulation of basal ganglia circuitry that increases motor output

motor proceeds cognitive

77
Q

med spiny striatal neurons normally _ motor activity

A

dampen

78
Q

cognitice changes in HD is due to neurnal loss from the _

A

cortex

79
Q

expansion in HD produces a toxic _ of function

there are _ inclusions

A

gain

intranuclear

80
Q

in HD the brain is _ and there is striking atrophy of the _ _ and _ later

globus pallidus is scondarily atrophied

atrophy of _ lobes

severity of symptoms is directly related to the degree of degeneration

A

small

caudate nucleus

putamen

frontal

caudate nucleus is decreased

NMSP (13 decks)

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