dementia and ataxia Flashcards
What chromosome is the APP gene on for ALZ?
21
what is apraxia?
inability to execute motor tasks despite having the strength and general motor required to do it.
dementia has many frontal release signs evident on exam, what are frontal releas signs?
reflexes that are present in infants
what infant reflex is often seen in parkinson’s disease?
myerson’s sign, tapping forehead and blinking eyes
INFANT REFLEX SIGNS ARE ALL SIGNS OF DAMAGE TO WHERE?
FRONTAL SUBCORTICAL MATTER
what is dementia/
cognitive impairment severe enough to impact daily activities and must be different from baseline
what is one step before dementia, impairment but doesn’t disrupt daily functions?
mild cognitive impairment MCI
what are hallmark path findings of ALZ?
neurofibrillary tangles (intracell) and amyloid plaques (extracell)
global cortical atrophy is often seen in ALZ, where is it often most pronounced?
mesial temporal lobe and parietal
what is the most common clinical presentation of ALZ?
amnestic dementia
what may hasten ALZ progression
illnesses or loss of psychosocial support
what is the most common variant of frontotemporal or picks dementia? features?
behavioral
- changes to personality and executive function, disinhibition, loss of motivation, empathy, compulsiveness
frontotemporal dementia spares what? average age of onset?
memory or visuospatial
50
primary progressive aphasia is associated with what?
FTD picks
key ingredient of dementia with lewy bodies?
alpha synuclein- distributed throughout the cortex
what is the most common form of VaD?
extensive small vessel ischemic disease
what is leukoaraiosis?
extensive loss of areas of white matter—> dementia from this is binswangers
what are key findings of vasculat dementia?
executive dysfunction, easy laughing or crying (pseudobulbar affect), loss of motivation (often taken as depression), urinary incontinence, and parkinsonian gait.
what is treatmetn of NPH?
shunting
if the diagnosis of dementia has an onset of 2years or just a couple months, what should we be thinking,
CJD prion disease- sporadic in 1- 1000000
cortical and deep gray matter chandes- 5 months from onset death.
what does MRI show on CJD?
DWI restriction- bright throughout cortex, basal ganglia and thalamus
what dementias can cholinesterase inhibitors help a little?
dementia of alz
DLB
what does memantine help in ?
ALZ
what is memory loss under 24 hours associated with stress in older people? patient repeats same questions but doesn’t remeber saying them but what isn’t effected?
transient global amnesia
autobiography and identity
what is asomatognosia?
left side of own body neglect
what is anosognosia?
lasck of insight to the fact there is a deficit
ocular apraxia or not coordinating eye movement happens with lesion where?
parietal fields
what is simultanagnosia?
deficits seeing the whole for the parts and piece together many individual images to see entire thing
what is balint syndrome?
ocular apraxia
optic ataxia
simultagnosia
posterior watershed bilateral infarction
what is ataxia not do to?
weakness or involuntary movements
what are the eye movements of ataxia?
nystagmus
saccadic intrusions
undershooting or overshooting
what is dysmetria?
imprecise targeting of distal limb movements
what is dyssynergia?
errors in the relative timing of components
what is dysdiadochokinesia?
errors in rate and regularity of rapid alternating movements
what part of the cerebellum causes nystagmus, balance problems due to can’t tell position?
flocculonodular lobe
where in cerebellum do we dysarthria? dysmetria,? dysdiadochokinesia? intention tremor?
all in the hemispheres
what two systems can lead to sensory ataxia?
posterior columns, peripheral nerves
what accounts for 1/3 of all cases of acute ataxia in children?
drug ingestion
bimodal- kids accidental teens with substance abuse
what is the most common cause of childhood
postifectious, autoimmune phenomenon (varicella, mumps, EBV)
where are 60% of all childhood tumors found?
in the brainstem or cerebellum–> ataxia
a cyst with an enhancing mural nodule describes what? what pathology is key?
pilocytic astrocytoma
rosenthal fibers
what is the most common malignant brain tumor in children? what is the pathology?
medulloblastoma
rosettes or pseudorosettes
what is the most common inherited ataxia?
friedrichs
what is freidrich’s repeat? how does it work
GAA- FRDA gene
suppressed gene expression–> iron accumulation
what is onset at around 2 with ataxia, progression of ambulation loss, oculomotor apraxia, oculocutaneous marks, sinopulmonary infections? what is a common lab?
ataxia telangiectasia
increased alpha-fetoprotein. IgA deficiency
