pathophysiology Flashcards
(27 cards)
what diseases show Lewy bodies? what are they made of?
PD and Dementia with Lewy Bodies
alpha-synuclein
what diseases show neurofibrillary tangles?
progressive supranuclear palsy, AD, Pick disease (Pick bodies)
tangles of filaments around hyper-phosphorylated tau protein
what are 6 clinical features of PSP that differentiate it from PD?
early symmetry, early postural instability, no tremor, supranuclear gaze palsy, dementia, neck extension
which parkinsonian diseases show pyramidal tract involvement? how is that manifested
progressive supranuclear palsy and multiple system atrophy
positie Babinski sign
which parkinsonian diseases often show respiratory stridor?
multiple system atrophy
what are four clinical features of multiple system atrophy that differentiate it from PD?
early postural instability, no tremor, worse autonomic dysfunction, cerebellar dysfunction
what could exclude a diagnosis of multiple system atrophy?
dementia
what diseases show glial cytoplasmic inclusion bodies? what do they contain?
multiple system atrophy
alpha-synuclein
which two diseases show dementia characterized by executive dysfunction (impaired planning, organizing, reasoning, etc)?
PD and HD
which striatal interneurons are preserved in HD?
all but parvalbuminergic
what is the cause of primary generalized dystonia?
AD mutation in DY1 gene for Torsin A protein
which dystonia shows a dromedary appearing gait?
primary generalized dystonia
what is the genetic basis of Wilson’s Disease?
AR mutation in ATP7B gene for Cu-transporting ATPase
how does Wilson’s Disease cause movement disorders? what is the most common feature and what are some others?
basal ganglia dysfunction
tremor
dysarthria, chorea, dystonia, parkinsonism
what is an Opalski cell and what diseases show it?
altered glial cell in basal ganglia
Wilson’s Disease
what shows difficulty naming objects, difficulty reading/writing, right-left disorientation, difficulty identifying own individual fingers? where is the lesion?
Gerstmann Syndrome
left angular gyrus
which regions of brain show most atrophy in AD?
parietal and temporal lobes
hippocampal formation
what are the two histologic signs of AD? what are they?
neuritic plaques: dystrophic neurites containing tau protein aggregated around core of beta-amyloid
neurofibrillary tangles: pyramidal cells with tangled filaments and hyper-phosphorylated tau protein
where are the tangles and neuritic plaques found in AD?
tangles: temporal lobe, hippocampal formation
plaques: parietal and temporal lobes
what are two lab findings in AD?
increased CSF tau protein
low CSF amyloid beta42
what are three imaging findings in AD?
parieto-temporal and hippocampal atrophy on MRI
increased amyloid on PET imaging
decreased glucose utilization on FDG-PET imaging
why is there a cholinergic deficit in AD?
atrophy of nucleus basalis of Meynert
what are 3 characteristics of Pick’s disease?
(a frontotemporal dementia)
frontal lobe atrophy->cognitive, executive dysfunction
temporal atrophy->language deficits (~Wernicke aphasia)
Pick bodies->neurofibrillary tangles with tau protein
what is Wernicke triad? what is Korsakoff syndrome?
- ophthalmoparesis 2. gait ataxia (central, heel-shin) 3. confusion
Korsakoff: amnesia and confabulation
