CM: Dx of Neurodegenerative

Question 1

What cognitive changes can occur normally in aging?

Answer 1

memory problems, sleep apnea and certain meds may contribute, depression may affect cognitive fxn
variability in performance during timed tests
more difficulty selectively attending to info while inhibiting irrelevant info/stimuli
decline in fluid intellectual abilities
crystallized abilities, general knowledge and vocab are stable

Question 2

What cognitive changes are seen in MCI?

Answer 2

impairment in one or more cognitive domains w preservation of fxn in daily life
on testing - lower performance than expected in one or more domain
=middle ground between nl and neurodegenerative

Question 3

What is the most common form of MCI?

Answer 3

amnestic - early stage of Alzheimers

impaired episodic memory - inability to learn and retain new verbal info, list learning & logical memory

Question 4

What are biomarkers for amnestic MCI?

Answer 4

beta amyloid and tau in CSF
F-FDG PET can look for hypometabolism (of glucose)
PET scanning w amyloid tracer
measure hippocampal atrophy
genetic testing

Question 5

When is dementia considered to occur?

Answer 5

when dz affects multiple areas of cognitive fxning rendering pt unable to independently carry out activities of daily living

Question 6

What is the general prevalence of Alzheimers?

Answer 6

1% at 60 and doubles every 5 yrs

Question 7

What NINCDS-ADRDA criteria suggest PROBABLE Alzheimers?

Answer 7

dementia, defects in at least two cog domains, progressive cog decline, normal consciousness, onset 40-90, no other explanation

Question 8

What NINCDS-ADRDA criteria suggest POSSIBLE Alzheimers?

Answer 8

atypical onset, sudden, focal signs, seizures or gait disorder early that normally occur later

Question 9

What NINCDS-ADRDA criteria are necessary to determine DEFINITE Alzheimers?

Answer 9

meets criteria for probably AND histopathologic evidence

Question 10

What are the DSM-IV criteria for diagnosing Alzheimers?

Answer 10

both memory impairment and one or more other cognitive disturbance
impairment in social or occupational functioning
gradual onset and continuing decline
no other cause, deficits don’t occur exclusively

Question 11

What are the current 3 stages of Alzheimers dz that are recognized and what can be done to diagnose at each stage?

Answer 11

pre clinical - biomarkers
MCI - biomarkers, testing
Alzheimers w dementia

Question 12

What is typically the first symptom of Alzheimers?

Answer 12

short term memory loss - esp episodic, difficulty encoding and storing new memories
most pts w isolated aMCI will progress to dementia

Question 13

What is aphasia (anomia) and how do you test for it?

Answer 13

word finding difficulties in spontaneous speech - test w LOW frequency words

Question 14

What is the typical order of appearance of language deficits in Alzheimers?

Answer 14

aphasia (anomia) first
comprehension later - no meaning behind words
transcortical aphasia if repetition intact

Question 15

What findings suggest perceptual deficits and agnosia in Alzheimers?

Answer 15

environmental disorientation, getting lost
constructional apraxia - assess in office by drawing pictures - frontal executive fxning and visuospatial needed to be normal
agnosia = difficulty recognizing objects
prosopagnosia = difficulty recognizing faces

Question 16

What is apraxia and how can it be tested in general?

Answer 16

loss of ability to perform learned motor task despite having intact basic sensory and motor function - ask pt to pantomime an action

Question 17

What is ideomotor apraxia?

Answer 17

inability to pantomime gestures to command

Question 18

What are transitive gestures?

Answer 18

involving use of tool or utensil - more sensitive than intransitive - watch for spatial or timing errors, using body part as tool sub

Question 19

What are intransitive gestures?

Answer 19

communicative - always better preserved

Question 20

What is ideational apraxia?

Answer 20

inability to properly sequence acts of a multistep action

Question 21

What is conceptual apraxia?

Answer 21

loss of semantic knowledge involving tool use - inability to select proper tool or make subs

Question 22

What sorts of behavior changes may be seen in pts with neurodegenerative disorders?

Answer 22

apathy/indifference - frontal lobe involvement, frequently misinterpreted as depression
irritability/agitation
delusions = fixed false beliefs, Capgras = think spouse is imposter
hallucinations

Question 23

If hallucinations occur earlier vs later in the course of the dz, what dz is expected?

Answer 23

earlier - dementia w lewy bodies

later - Alzheimers

Question 24

Which neurodegenerative disorders presents w behavior disturbances at onset?

Answer 24

frontotemporal lobar

Question 25

What problems with learning are seen with cortical dementia vs subcortical dementia?

Answer 25

cortical - can’t learn

subcortical - can learn, just can’t get info back out

Question 26

What CSF findings can be diagnostic of Alzheimers?

Answer 26

increased levels of tau and decreased amyloid

Question 27

What is the general treatment for Alzheimers?

Answer 27

ACHEIs for all forms, NMDA receptor antagonist for moderate to severe
may slow progression and help w behavioral symptoms
medical food in APOE eta4 negative pts
all three may be used together

Question 28

What is the differential of Alzheimers and what are features of each that can suggest it rather than Alzheimers?

Answer 28

FTD
dementia w lewy bodies
vascular dementia
CJD
Parkinsons dz dementia
infectious - subacute rather than chronic presentation
inflammatory/autoimmune (Hashimotos encephelopathy)

Question 29

What findings suggest FTD as opposed to Alzheimers?

Answer 29

younger onset, something other than memory as presenting symptom, tauopathy

Question 30

What findings suggest dementia w lewy bodies rather than Alzheimers?

Answer 30

visual hallucinations, REM behavioral disorders

Question 31

What findings suggest vascular dementia rather than Alzheimers?

Answer 31

hx of strokes, vascular lesions on imaging, onset of dementia w/i 3 mos of stroke

Question 32

What findings suggest CJD?

Answer 32

prion dz, rapid progression, ataxia, myoclonus, abnormal EEG, severe visuoperceptive derangements
CSF positive for 14-3-3

Question 33

What are some reversible causes of dementia?

Answer 33

structural causes on neuroimaging (hydrocephalus, subdural hematoma, benign tumors)
subacute encephalitis, depressive psuedodementia (but may still develop dementia later), metabolic disorders (B12 def, thyroid dz, folate, thiamine, niacin def)
nonconvulsive status epilepticus - continual seizure activity on EEG