L6 Pathology of PD Flashcards

1
Q

where can it start

A

starts specifically in medullus, 10th cranial nerve nucleus, might come from guty
then spreads to pons then substantia nigra - hippocampus - neocortical areas - sensory cortex in parietal lobe

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2
Q

in what brainstem regions are there predominance of lewy body pathology

A

medulla pons oblongata

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3
Q

what basal forebrain regions is there lewy body pathology

A

parahippocampal gyrus
amygdala
meynert
anterior cingulate

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4
Q

in what neocortical regions is there lewy body pathology

A

frontal cortex
occipital
parietal

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5
Q

what is typical pathology

A

putamen is normal
subthalamic nucleus normal
cerebellar cortex and wm normal

substantia nigra black substance not present
locus coeruleus missing at top
alpha synuclein positive

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6
Q

in what patients is deviation from Braak stage seen

A

patients with no parkinsonism

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7
Q

why might patients who develop pd later die quicker?

A

might have more active and toxic alpha synuclein compared to patients who have the disease earlier

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8
Q

where is striatonigral degeneration found

A

glial cells

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9
Q

what is the dominant system in MSA-P (multiple system atrophy) and what pathological diagnosis corresponds to it

A

parkinsonism dominant,

MSA-striatonigral degeneration

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10
Q

what problems is MSA-C dominant with and what pathological diagnosis corresponds to it

A

ataxic problems in cerebellar

MCA - olivopontocerebellar degeneration

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11
Q

what is seen in pathology on MSA-P

A

atrophic putamen shrunken and become darker

  • substantia nigra will be pale and not black
  • locus coeruleus is pale
  • inferior olivary nucleus ok
  • wm in cerebellum fine
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12
Q

what is seen in MSA-C

A
  • putamen fine
  • substantia nigra pale
  • asymmetric cerebellum from different hemispheres
  • base of pons shrunken
  • atrophic olivary nucleus
  • little wm remaining
  • superior cerebellar peduncle fine
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13
Q

what are some rare clinical phenotypes of MSA

A

MSA-CBS = cortico basal
MSA-PPA = primary progressive aphasia
MSA-bvFTD

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14
Q

what ethnic groups are MSA-P AND MSA-C seen in

A

both in caucasian

MSA-C in japanese

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15
Q

what does MSA minimal change look like

A

parkinsons but not under microscope

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16
Q

what contributes to the increased likelihood of MSA

A

development of:

  • autonomic dysfunction within the first 3 years of symptom onset: autonomic dysfunction in PD and PSP-P often
  • severe orthostatic hypotension
  • urinary incontinence
  • longer latency to residential care than in PSP
17
Q

how many times are you more likely to have MSA than Lewy body related pathology

A

8.8

18
Q

what signs can you show to have Lewy body

A
orofacial dystonia
inspiratory signs
contractures of hands or feet
poly-mini-myoclonus
severe dysarthria
pathological laughter or crying
cold hand or feet
19
Q

what symptoms are more common in MSA than lewy body disease

A

dysphagia, stridor, falls

20
Q

how many times more likely is it MSA than PSP

A

4.8

21
Q

what are symptoms of PSP

A
orofacial dystonia
inspiratory signs
contractures of hands or feet
poly-mini-myoclonus
severe dysphonia
jerky myoclonic postural or action tremor
snoring
22
Q

what is more common in MSA than PSP

A

ataxia

stridor

23
Q

which disease can have 3 repeat tau

A

pick’s disease

24
Q

which diseases can have 4 repeat tau

A
AGD
GGT
CBD
PSP
CTE/artag
25
Q

compare pathology in PD, PSP and MSA-OPCA

A

in PD - locus coeruleus atrophic in all 3

in PSP- midbrain tegmentum darker discoloured and atrophic, substantia nigra atrophic, superior cerebellar peduncle thin, dentate nucleus atrophic

MSA-OPCA - dentate nucleus atrophic

26
Q

what can be seen in PSP pathology

A

atrophy of subthalamic nucleus
tufted astrocytes
4R tau

27
Q

what are clinical phenotypes of PSP in typical

A

PSP-RS

28
Q

what are specific phenotypes of atypical PSP in brainstem, cortical, cerebellum

A

brainstem: PSP-P, PSP-PAGF
cortical: PSP-CBS, PSP-PNFA, PSP-bvFTD, PSP PLS
cerebellum: PSP-C

29
Q

what sign are you looking for in PSP in radiology

A

hummingbird sign in midbrain

mickey mouse sign

30
Q

what is pathology of corticobasal

A

astrocytic plaque, 4R tau

31
Q

what pathology is CBD with PSP clinical phenotype characterised by and what is it associated with

A

TDP43
the presence of severe TDP43 pathology in the midbrain tectum strongly associated with downward gaze palsy
tau burden in the olivo-ponto-cerebellar system significantly greater in TDP-43 positive CBD cases
-MAPT H1/H1 genotype less frequent in TDP43 positive cases

32
Q

why are referrals made to qs brain bank

A

confirm or establish diagnosis
research to improve clinical accuracy
material for world-wide research