Path XI Flashcards
most common dementia
alzheimer disease
onset >65 yrs
Signs Sx alzheimer
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
alzheimer assoc with
some familial
some downs
most sporadic
type of atrophy in alzheimer
cortical atrophy
amyloid plaques appear where in alzheimers
outside of neuron
where does tau collect in alzheimer
inside neuron
what enzyme is problem in collection of plaques and tau proteins in alzheimers
beta amyloid converting enzyme
or
beta secretase cleavage
most cases of alzeimers disease are
spontaneous
mutations with familial alzheimers
beta amyloid precursor protein 21q21 (downs region)
presenilin— early onset AD
mutation of presenilin or beta amyloid precursor protein increase what
beta amyloid precursor protein
what apo protein is associated with alzheimers
ApoE epsilon4 form
increase 25% for late onset AD
promotes beta amyloid precursor protein formation
tau protein encoded where
17q21.1
plaques in neurites with amyloid cores
alzheimers
role of tau
stabilizes microtubules
most common initial Sx of alzheimers
impaired memory
personality and judgment affected too
binding domains of tau
3 or 4. 4 is stable
high levels tau protein in CSF after head trauma linked to
poor recovery
inflammatory reactin oto amyloid plaques and tau tangles causes what in brain
gliosis leading to grey matter loss
tau protein is stained with what
silver stain
what condition is almost always present with alzheimers
cerebral amyloid angiopathy
cerebral amyloid angiopathy is due to
deposition of beta amyloid Abeta in small vessels
ischemic lesions of CAA cause
dementia
imaging for alzheimers
CSF
MRI
nuclear scans
what is decreased or increased in alzheimers
decreased beta amyloid
increased tau
part of brain most affected in alzheimers
CA1 hippocampus
frontotemporal lobar degenerations inclue what?
pick disease
progressive supranuclear palsy
corticobasalar degeneration
differences of frontolobar degenerations than AD
personality behavior and language changes appear before memory problems
what cellular inclusions are associated with frontotemporal lobar degenerations
FTLD-tau group: picks, PSP, CD
FTLD-TDP43
which cellular inclusion is associated with ALS patients that present with FTD Sx
FTLD TDP43
what aggregations are present in the FTLD tau goup
tau aggregations
no beta amyloid
tau aggregates in tangles
FTLD tau
tau aggregates in smoothed contoured inclusions
pick disease
areas with most prominent atrophy in frontotemporal lobar degeneration
frontal and temporal lobes
Picks disease
45-65 years with confusion and FTLD
Sx have progressive course 2-5 yrs
advanced same as AD
Pick bodies
tau positive
spherical cytoplasmic neuronal inclusions
Progressive supranuclear palsy signs
taupathy with progressive truncal rigidity, disequilibrium with frequent falls and difficulty with voluntary eye movements
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
onset progressive supranuclear palsy
5-7 decades
males>females
fatal within 5-7 years
Parkinsons presents with
hypokinetic movement disorder
loss of dopaminergic neurons in substantia nigra
dementia with lewy bodies
parkinson disease
severity of motor syndrome in parkinson is proportional to
dopamine deficiency
pars compacta involves what dopamine group
A9
guam parkinson dementia
toxic amino acid in seed of cycad plant used to make flour and staple in local diet
aluminum role in parkinson
can disrupt the neuronal cytoskeleton and cause neurofibrillary pathology
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
all inputs to basal ganglia arrive where
striatum (caudate putamen and nucleus accumbens)
outputs basal ganglia
internal segment of globus pallidus and related to substantia nigra pars compacta and reticulata
basal ganglia controls what
general motor control
eye movements
cognitive functions
emotional functions
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
central triad of parkinsonism
tremor
rigidity
bradykinesia(in absence of toxic or other known underlying etiology)
clincal syndrome of parkinsonism includes
diminished facial expression “masked facies”
stooped posture
slowing voluntary movement
festinating gait (short progressive steps)
rigidity
pill rolling tremor
Dx parkinsons confirmed by
Symptomatic response to L dopa replacement therapy
deep brain stimulation in parkinsons
physiological and chemical changes that ameliorate motor Sx
Tx unilateral tremor and rigidity in parkinsons
stereotactic ablation of contralateral globus pallidus, ventrolateral thalamus or subthalamic nucleus
