L6 Pathology of PD Flashcards
where can it start
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
in what brainstem regions are there predominance of lewy body pathology
medulla pons oblongata
what basal forebrain regions is there lewy body pathology
parahippocampal gyrus
amygdala
meynert
anterior cingulate
in what neocortical regions is there lewy body pathology
frontal cortex
occipital
parietal
what is typical pathology
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
in what patients is deviation from Braak stage seen
patients with no parkinsonism
why might patients who develop pd later die quicker?
might have more active and toxic alpha synuclein compared to patients who have the disease earlier
where is striatonigral degeneration found
glial cells
what is the dominant system in MSA-P (multiple system atrophy) and what pathological diagnosis corresponds to it
parkinsonism dominant,
MSA-striatonigral degeneration
what problems is MSA-C dominant with and what pathological diagnosis corresponds to it
ataxic problems in cerebellar
MCA - olivopontocerebellar degeneration
what is seen in pathology on MSA-P
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
what is seen in MSA-C
- putamen fine
- substantia nigra pale
- asymmetric cerebellum from different hemispheres
- base of pons shrunken
- atrophic olivary nucleus
- little wm remaining
- superior cerebellar peduncle fine
what are some rare clinical phenotypes of MSA
MSA-CBS = cortico basal
MSA-PPA = primary progressive aphasia
MSA-bvFTD
what ethnic groups are MSA-P AND MSA-C seen in
both in caucasian
MSA-C in japanese
what does MSA minimal change look like
parkinsons but not under microscope
what contributes to the increased likelihood of MSA
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
how many times are you more likely to have MSA than Lewy body related pathology
8.8
what signs can you show to have Lewy body
orofacial dystonia inspiratory signs contractures of hands or feet poly-mini-myoclonus severe dysarthria pathological laughter or crying cold hand or feet
what symptoms are more common in MSA than lewy body disease
dysphagia, stridor, falls
how many times more likely is it MSA than PSP
4.8
what are symptoms of PSP
orofacial dystonia inspiratory signs contractures of hands or feet poly-mini-myoclonus severe dysphonia jerky myoclonic postural or action tremor snoring
what is more common in MSA than PSP
ataxia
stridor
which disease can have 3 repeat tau
pick’s disease
which diseases can have 4 repeat tau
AGD GGT CBD PSP CTE/artag
compare pathology in PD, PSP and MSA-OPCA
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
what can be seen in PSP pathology
atrophy of subthalamic nucleus
tufted astrocytes
4R tau
what are clinical phenotypes of PSP in typical
PSP-RS
what are specific phenotypes of atypical PSP in brainstem, cortical, cerebellum
brainstem: PSP-P, PSP-PAGF
cortical: PSP-CBS, PSP-PNFA, PSP-bvFTD, PSP PLS
cerebellum: PSP-C
what sign are you looking for in PSP in radiology
hummingbird sign in midbrain
mickey mouse sign
what is pathology of corticobasal
astrocytic plaque, 4R tau
what pathology is CBD with PSP clinical phenotype characterised by and what is it associated with
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
why are referrals made to qs brain bank
confirm or establish diagnosis
research to improve clinical accuracy
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