Path XI Flashcards

1
Q

most common dementia

A

alzheimer disease

onset >65 yrs

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2
Q

Signs Sx alzheimer

A
slowly progressive memory dysfunction
dysphagia and dyspraxia
prgression to akynetic mutism
confusion with time and place
misplacing objects
decreasing judgement
social withdrawal
mood personality changes
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3
Q

alzheimer assoc with

A

some familial
some downs
most sporadic

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4
Q

type of atrophy in alzheimer

A

cortical atrophy

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5
Q

amyloid plaques appear where in alzheimers

A

outside of neuron

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6
Q

where does tau collect in alzheimer

A

inside neuron

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7
Q

what enzyme is problem in collection of plaques and tau proteins in alzheimers

A

beta amyloid converting enzyme
or
beta secretase cleavage

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8
Q

most cases of alzeimers disease are

A

spontaneous

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9
Q

mutations with familial alzheimers

A

beta amyloid precursor protein 21q21 (downs region)

presenilin— early onset AD

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10
Q

mutation of presenilin or beta amyloid precursor protein increase what

A

beta amyloid precursor protein

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11
Q

what apo protein is associated with alzheimers

A

ApoE epsilon4 form
increase 25% for late onset AD
promotes beta amyloid precursor protein formation

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12
Q

tau protein encoded where

A

17q21.1

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13
Q

plaques in neurites with amyloid cores

A

alzheimers

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14
Q

role of tau

A

stabilizes microtubules

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15
Q

most common initial Sx of alzheimers

A

impaired memory

personality and judgment affected too

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16
Q

binding domains of tau

A

3 or 4. 4 is stable

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17
Q

high levels tau protein in CSF after head trauma linked to

A

poor recovery

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18
Q

inflammatory reactin oto amyloid plaques and tau tangles causes what in brain

A

gliosis leading to grey matter loss

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19
Q

tau protein is stained with what

A

silver stain

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20
Q

what condition is almost always present with alzheimers

A

cerebral amyloid angiopathy

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21
Q

cerebral amyloid angiopathy is due to

A

deposition of beta amyloid Abeta in small vessels

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22
Q

ischemic lesions of CAA cause

23
Q

imaging for alzheimers

A

CSF
MRI
nuclear scans

24
Q

what is decreased or increased in alzheimers

A

decreased beta amyloid

increased tau

25
part of brain most affected in alzheimers
CA1 hippocampus
26
frontotemporal lobar degenerations inclue what?
pick disease progressive supranuclear palsy corticobasalar degeneration
27
differences of frontolobar degenerations than AD
personality behavior and language changes appear before memory problems
28
what cellular inclusions are associated with frontotemporal lobar degenerations
FTLD-tau group: picks, PSP, CD | FTLD-TDP43
29
which cellular inclusion is associated with ALS patients that present with FTD Sx
FTLD TDP43
30
what aggregations are present in the FTLD tau goup
tau aggregations | no beta amyloid
31
tau aggregates in tangles
FTLD tau
32
tau aggregates in smoothed contoured inclusions
pick disease
33
areas with most prominent atrophy in frontotemporal lobar degeneration
frontal and temporal lobes
34
Picks disease
45-65 years with confusion and FTLD Sx have progressive course 2-5 yrs advanced same as AD
35
Pick bodies
tau positive | spherical cytoplasmic neuronal inclusions
36
Progressive supranuclear palsy signs
taupathy with progressive truncal rigidity, disequilibrium with frequent falls and difficulty with voluntary eye movements
37
Sx progressive supranuclear palsy
nuchal dystonia pseudobulbar palsy and a mild progressive dementia progressive truncal rigidity disequilibrium with frequent falls and difficulty with voluntary eye movements
38
onset progressive supranuclear palsy
5-7 decades males>females fatal within 5-7 years
39
Parkinsons presents with
hypokinetic movement disorder | loss of dopaminergic neurons in substantia nigra
40
dementia with lewy bodies
parkinson disease
41
severity of motor syndrome in parkinson is proportional to
dopamine deficiency
42
pars compacta involves what dopamine group
A9
43
guam parkinson dementia
toxic amino acid in seed of cycad plant used to make flour and staple in local diet
44
aluminum role in parkinson
can disrupt the neuronal cytoskeleton and cause neurofibrillary pathology
45
parkinson syndrome can develop in course of other conditions like what
striatonigral degeneration, postencephalitic parkinsonism, manganese poisoning, CO poisoning, hypoxic ischemic encephalopathym traumatic brain injury and stroke
46
all inputs to basal ganglia arrive where
striatum (caudate putamen and nucleus accumbens)
47
outputs basal ganglia
internal segment of globus pallidus and related to substantia nigra pars compacta and reticulata
48
basal ganglia controls what
general motor control eye movements cognitive functions emotional functions
49
how do you prepare a section to visualize basal ganglia
locate superior colliculus after cut off cerebellum | cut mid brain across superior colliculus to see substantia nigra
50
central triad of parkinsonism
tremor rigidity bradykinesia(in absence of toxic or other known underlying etiology)
51
clincal syndrome of parkinsonism includes
diminished facial expression "masked facies" stooped posture slowing voluntary movement festinating gait (short progressive steps) rigidity pill rolling tremor
52
Dx parkinsons confirmed by
Symptomatic response to L dopa replacement therapy
53
deep brain stimulation in parkinsons
physiological and chemical changes that ameliorate motor Sx
54
Tx unilateral tremor and rigidity in parkinsons
stereotactic ablation of contralateral globus pallidus, ventrolateral thalamus or subthalamic nucleus