dementia and ataxia

Question 1

What chromosome is the APP gene on for ALZ?

Answer 1

21

Question 2

what is apraxia?

Answer 2

inability to execute motor tasks despite having the strength and general motor required to do it.

Question 3

dementia has many frontal release signs evident on exam, what are frontal releas signs?

Answer 3

reflexes that are present in infants

Question 4

what infant reflex is often seen in parkinson’s disease?

Answer 4

myerson’s sign, tapping forehead and blinking eyes

Question 5

INFANT REFLEX SIGNS ARE ALL SIGNS OF DAMAGE TO WHERE?

Answer 5

FRONTAL SUBCORTICAL MATTER

Question 6

what is dementia/

Answer 6

cognitive impairment severe enough to impact daily activities and must be different from baseline

Question 7

what is one step before dementia, impairment but doesn’t disrupt daily functions?

Answer 7

mild cognitive impairment MCI

Question 8

what are hallmark path findings of ALZ?

Answer 8

neurofibrillary tangles (intracell) and amyloid plaques (extracell)

Question 9

global cortical atrophy is often seen in ALZ, where is it often most pronounced?

Answer 9

mesial temporal lobe and parietal

Question 10

what is the most common clinical presentation of ALZ?

Answer 10

amnestic dementia

Question 11

what may hasten ALZ progression

Answer 11

illnesses or loss of psychosocial support

Question 12

what is the most common variant of frontotemporal or picks dementia? features?

Answer 12

behavioral

- changes to personality and executive function, disinhibition, loss of motivation, empathy, compulsiveness

Question 13

frontotemporal dementia spares what? average age of onset?

Answer 13

memory or visuospatial

50

Question 14

primary progressive aphasia is associated with what?

Answer 14

FTD picks

Question 15

key ingredient of dementia with lewy bodies?

Answer 15

alpha synuclein- distributed throughout the cortex

Question 16

what is the most common form of VaD?

Answer 16

extensive small vessel ischemic disease

Question 17

what is leukoaraiosis?

Answer 17

extensive loss of areas of white matter—> dementia from this is binswangers

Question 18

what are key findings of vasculat dementia?

Answer 18

executive dysfunction, easy laughing or crying (pseudobulbar affect), loss of motivation (often taken as depression), urinary incontinence, and parkinsonian gait.

Question 19

what is treatmetn of NPH?

Answer 19

shunting

Question 20

if the diagnosis of dementia has an onset of 2years or just a couple months, what should we be thinking,

Answer 20

CJD prion disease- sporadic in 1- 1000000

cortical and deep gray matter chandes- 5 months from onset death.

Question 21

what does MRI show on CJD?

Answer 21

DWI restriction- bright throughout cortex, basal ganglia and thalamus

Question 22

what dementias can cholinesterase inhibitors help a little?

Answer 22

dementia of alz

DLB

Question 23

what does memantine help in ?

Answer 23

ALZ

Question 24

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?

Answer 24

transient global amnesia

autobiography and identity

Question 25

what is asomatognosia?

Answer 25

left side of own body neglect

Question 26

what is anosognosia?

Answer 26

lasck of insight to the fact there is a deficit

Question 27

ocular apraxia or not coordinating eye movement happens with lesion where?

Answer 27

parietal fields

Question 28

what is simultanagnosia?

Answer 28

deficits seeing the whole for the parts and piece together many individual images to see entire thing

Question 29

what is balint syndrome?

Answer 29

ocular apraxia
optic ataxia
simultagnosia

posterior watershed bilateral infarction

Question 30

what is ataxia not do to?

Answer 30

weakness or involuntary movements

Question 31

what are the eye movements of ataxia?

Answer 31

nystagmus
saccadic intrusions
undershooting or overshooting

Question 32

what is dysmetria?

Answer 32

imprecise targeting of distal limb movements

Question 33

what is dyssynergia?

Answer 33

errors in the relative timing of components

Question 34

what is dysdiadochokinesia?

Answer 34

errors in rate and regularity of rapid alternating movements

Question 35

what part of the cerebellum causes nystagmus, balance problems due to can’t tell position?

Answer 35

flocculonodular lobe

Question 36

where in cerebellum do we dysarthria? dysmetria,? dysdiadochokinesia? intention tremor?

Answer 36

all in the hemispheres

Question 37

what two systems can lead to sensory ataxia?

Answer 37

posterior columns, peripheral nerves

Question 38

what accounts for 1/3 of all cases of acute ataxia in children?

Answer 38

drug ingestion

bimodal- kids accidental teens with substance abuse

Question 39

what is the most common cause of childhood

Answer 39

postifectious, autoimmune phenomenon (varicella, mumps, EBV)

Question 40

where are 60% of all childhood tumors found?

Answer 40

in the brainstem or cerebellum–> ataxia

Question 41

a cyst with an enhancing mural nodule describes what? what pathology is key?

Answer 41

pilocytic astrocytoma

rosenthal fibers

Question 42

what is the most common malignant brain tumor in children? what is the pathology?

Answer 42

medulloblastoma

rosettes or pseudorosettes

Question 43

what is the most common inherited ataxia?

Answer 43

friedrichs

Question 44

what is freidrich’s repeat? how does it work

Answer 44

GAA- FRDA gene

suppressed gene expression–> iron accumulation

Question 45

what is onset at around 2 with ataxia, progression of ambulation loss, oculomotor apraxia, oculocutaneous marks, sinopulmonary infections? what is a common lab?

Answer 45

ataxia telangiectasia

increased alpha-fetoprotein. IgA deficiency