Brainscape's Knowledge GenomeTM

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


See full index

Pathology 3 > neurodegeneration and dementia > Flashcards

neurodegeneration and dementia Flashcards

1
Q

features shared by NDD

A

progressive - once disease process has begun it continues relentlessly
fatal outcome
associtaed with aging
degeneration of neurones - dysfunction and cell death

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

many of these diseases are caused by

A

abnormal accumulation of proteins in the CNS

mostly unknown causes - genetic and environmental factors

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

common pathological factors

A

cmmonest in old age
each disease affects specific neuronal groups
clinical features relate to the anatomy and function of the affected areas
atrophy of affected areas with disease progression - seen on imaging or macroscopically at PM
regional pattern of atrophy may be pathognomonic in some cases

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

microscope appearance of NDD

A

histopathology shows neuronal loss with variable gliosis
microscopy may show distinctiive cellular inclusions containing specific proteins in either neurones of glial cells eg. NFT, lewy bodies

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

cortical degenerations

A

alzhiemer disease
frontotemporal lobar degeneration
dementia with lewy bodies

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

akinetic movement disorders

A

parkinsons disease
progressive supranuclear palsy
corticobasal degeneration
multiple ssystem atrophy (striatonigral degeneration)

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

hyperkinetic syndroms

A

huntington disease

choreoarthritis

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

cerebellar ataxis

A

inherited and sporadic

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

diseases of motor systems

A

motor neurone disease

hereditary spastic paraperesis

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

autonomic disorders

A

parkinosns disease

multiple system atrophy (shy-drager syndrome)

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

hippocapmus diseases

A

congnitive changes, memory, behaviour, language

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

basal ganglia diseases

A

movement disorders
hypokinetic
hyperkinetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

cerebellum diseases

A

ataxia - psinocerebellar ataxia

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

motor system diseases

A

weakness, difficulty swallowing, respiration, amyotropic lateral sclerosis

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

protein accumulation

A

Ab amyloid - alzhiemers
Tau - pick disease (3 repeat) or others with 4 repeats
alpha-synuclein
TDP-43
polyglutamine repeat expansions - huntington disease
SOD1, TDP-43
prions

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

dementia

A

an acquired progressive global impairment of intellect, memory and personality, without impairment of consciousness

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

the definition of dementia excludes

A

acute confusional state
impairment of consciousness
delirium or depression
mental impairment due to maldevelopment of the brain or due to a brain insult during development

18
Q

main causes of demntia

A

alziemerhs
dementia with lewy bodies
frontotemporal lobar degeneration
vescular pathology

19
Q

alzheimers disease

A

progressive and inevitably fatal within 5-15 years
immidiate cause of death often terminal infection eg. pneumonia
3rd most common death in developed world (after vascular disease and cancer)

20
Q

ealry onset alzheimers

A

<65 yo

21
Q

gold standard for alzeimhers diagnossis

A

post mortem pathology

22
Q

changes in the brain

A

reduction in size and atrophy
ventricle increases in size due to decrease volume of white matter
sulci appear expanded and gyri are narrowed
reduced cerebral weight
atrophy of medial temporal lobe

23
Q

histopathological features of alzheimers

A

amyloid b-protein accumulation - cortical neuritic plaques and cerebral amyloid angiopathy
tau accumulation - neurofibrillary tangles, plaque - associated dystrophic neurites and neuropil threads

24
Q

the amyloid hypothesis

A

abnormal accumulation of amyloid b protein through to play a key role in initiating and perpetuating the neurotoxic events that culminate in dementia (by reduced clearance rather than increased production
released into the ECS where it aggregates to form plaques and accumulates in vessel walls as CAA

25
Q

genetic risks for alzheimers

A

ApoE4 allele is the only recognised risk factor
familial form - mutations in presenilin 1 or presenilin 2 leading to early onset alzheimers
trisomy 21 - alzheimers by 35yrs

26
Q

diagnostic criteria fro AD

A

ABC score
thal phase - of amyloid plaques (beta-amylloid stain)
braak stage - of neurofibillary tangles (tau AT8 stain)
CERAD stage of neuritic plaques (bielschowsky stain)

27
Q

frontotemporal lobar degeneration (FTLD)

A

pathological correlate of FTD
progressive frontal lobe dysfunction that may be followed by temporal lobe symptomatology
behaviour and language problems precede memory problems
onset younger than for alzheimers

28
Q

FTLD is divided by

A

divided into several subgroups that are distinguished according to type of protein deposited on immunohistochemistry (Tau, TDP-43, FUS, UPS)

29
Q

FTLD macroscopic

A

severe frontotemporal atrophy
variable other findings
pallor of pigmented nuclei in the brainstem in some entities

30
Q

NDD with clinical features of parkinsonism

A

several different disease affecting the same circuitries in the network of neurones in the brain can result n similar clinical findings
many of these movement disorders are sassociated with dementia
only able to be distinguished on neuropathological examination

31
Q

clinical features of parkinosnism

A 
head bent forward 
tremors of the head 
mask-like facial expression 
drooling 
rigidity 
stooped posture 
weight loss 
tremor 
akinesia 
loss of postural reflexes 
done demineralization 
shuffling and propulsive gait 
bradykinesia 
pill-rolling tremor 
festinant gait
32
Q

causes of parkinsonism

A 
parkinsons disease 
dementia with lewy bodies 
multiple system atrophy 
tauopathies 
trinucleotide repeat diseases 
symptomatic parkinsomism - toxic eg. carbon monoxide, carbon sulphide, manganese 
drug induced - butyrophenones, phenotiazines, fluraridine, reserpine 
vascular parkinonsim
33
Q

synucleinopathies

A

alpha-synuclein found to be major component in several cytoplasmic inclusions
protein involved in synaptic transmission

34
Q

dementia with lewy bodies

A

2nd most common dementia, 10-25% of cases
fluctuating cognitive impairment, visual hallucinations, parkinsonism
autonomic dysfunction
sleep difficulty - REM sleep behaviour disorder
depression
apathy
DLB = cognitive impairment first, movement later

35
Q

huntington disorder

A 
AD movement disorder - hyperkinetic 
degeneration of the striatum 
relentless progression 
CAG trinucleotide repeat 
anticipation
36
Q

progression of huntington disorder

A

early cognitive symptoms, mood disorder
progression to severe dementia
death in 15 years on average

37
Q

the prion hypothesis

A

PrPc is a normal cellular tranmembrane protein of uncertain function
prion disease is due to an abnormally folded form of the protein (PrPsc) with has a tendancy to aggregate in the ECS and causing spongiform change

38
Q

creutzfelt jakob disease

A

most common prion disease
myoclonus and characteristic EEG changes occur late
can be inherited as AD trait, can bbe acquired by tranmission or occur spontaneosly

39
Q

tranmission of cruetzfelt jakob disease

A

person to person tansmission requires parenteral innoculation of infected tissue (iatrogenic) - incubation period ranges from years to decades

40
Q

diagnosis of cruetzfelt jakob disease

A

requires histology

41
Q

CJD autopsiess

A

requires special decontamination procedurres

needs full PPE and self contained breathing devices

42
Q

CJD histology

A

microscopic vacuolaton of gray matter - spongiform change
extra cellular accumulation of protease resistant peptide (PrP - prio protein)
neuronal loss and gliosis

Pathology 3 (11 decks)

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