Dementia

Question 1

TEST?: key features of CJD:

Answer 1

very rapid decline (over weeks to months) this is NOT present in AD, and marked myoclonus on exam

Question 2

TEST: 3 D’s
Dementia, is characterized by:
Delirium:

Answer 2

memory impairment that has a gradual progression;
delirium is an acute confusional state that is brought on, usually by an underlining medical condition, marked by inattention being the main feature, highly disorganized thinking, waxing waining, etc.
To tell the difference between delirium and dementia, you can’t have a conversation with someone who is delirius because they won’t stop and focus, they’re constantly doing crazy things all the time; AD can have a whole conversation with you about what’s going on in 1974 but they can’t remember that it’s not 1974

Question 3

TEST: The most common form of dementia is _____, the second most come is ______

Answer 3

AD, followed by vascular, followed by Lewy body although they’re underdiagnosed so we’re not sure, MANY DIAGNOSED WITH AD PROBALBY HAVE LEWY BODY

Question 4

TEST: Stage 1 is characterised by _________, Stage 2 is characterised by _________, Stage 3 is characterised by _______

Answer 4

memory disturbances, poor judgement, carelessness in work habits, disorientation to space and time, etc;
restlessness, pacing, incontinence;
worsening language function, motor disturbances, incontinence of urine and feces

Question 5

neuropathology: Early signs_____, then reduced ____ levels in ______ with increased buden in brain; location is important and the ______ is always effected

Answer 5

synaptic loss in limbic structures, B-amyloid levels in csf; Increase in Tau protein buildup and the temporal lobe is always affected (the time course involvement of other areas varies)

Question 6

TEST: It is the concentration of tau proteins in the _____ that distinguishes the diagnoses of AD

Answer 6

Medial Temporal lobe (or temporal lobe)

Question 7

Over _____of AD is sporadic and is later onset

Answer 7

Over 90% of AD is sporadic and is later onset

Question 8

Family history _____ risk of AD in patients with first degree relative

Answer 8

Family history 10-30% risk of AD in patients with first degree relative

Question 9

Genetic component of AD:

Answer 9

Not c/w Mendelian inheritance

Question 10

______accounts for relationship with Down syndrome in their 40s

Answer 10

21q APP accounts for relationship with Down syndrome in their 40s

Question 11

Late-onset AD:

Answer 11

Apolipoprotein E (ApoE) status:
Late onset associated with ApoE4 allele (chromosome 19)
ApoE2 allele protective

Question 12

Early-onset AD:

Answer 12

Autosomal dominant:
Amyloid precursor protein (APP) (chromosome 21) (Down)
Presenilin 1 (PSEN1) (chromosome 14) (most severe)
Presenilin 2 (PSEN2) (chromosome 1)

Question 13

if there is an acute drop in memory and then stability, it may be_____, as one of the diagnositc criteria for AD is _____

Answer 13

trauma or vascular event, progressive worsening of memory and cognition, with no disturbance of activity, or hyper active, etc

Question 14

62 y/o man, right handed, w/ MA in Ed, retired teacher and realtor
5-6 year hx of memory problems, insidious onset, gradual progression. Word finding problems, forgetting events and conversations, getting lost in familiar areas, forgetting appointments. Retired because unable to perform his duties as realtor.

Answer 14

Early Stage AD:
Cardinal symptom: impaired learning of new information, reflecting earliest involvement of medial temporal region
Verbal memory tests – most sensitive
decreased delayed recall
decreased immediate but NORMAL delayed recall is NOT likely dementia
Impairment should be judged relative to estimated premorbid status

Question 15

Mild cognitive impairment: prognosis

Answer 15

80% of adults with amnestic MCI progress to AD within 6 years
Best predictors of progression: 
poor memory performance
APOE-e4 genotype
hippocampal atrophy
existing metabolic disease

Question 16

Depression vs Dementia

Answer 16

If person with depression can internalize the information, they’ll recall it (maybe need some cueing) but AD they won’t;
Impaired attention and effort
May perform better on difficult tests
Memory impairment 2 to impaired attention; poor immediate memory, no additional loss with delay; pronounced benefit of cueing
Late-life depression = risk factor of AD
Important to differentiate cognitive symptoms (avoiding challenge) from affective symptoms (tearful, sadness)

Question 17

TEST: “step-wise progression”

Answer 17

if someone takes a hit to their cognition (i.e. overnight) and then are stable, then a hit, then stable, etc, it is Vascular dementia, NEED TO HAVE EVIDENCE VIA IMAGINING; used to think there had to be a significant stroke, etc but actually there is more of a chance of small vessel disease

Question 18

someone isn’t doing their shopping anymore; are they not shopping because they can’t walk and carry their basket/because it’s easier to have somone else do it, or bc they can’t manage a list and know how to find things

Answer 18

Vascular dementia:
distinction executive function deficit
reduced verbal fluency
motor slowing
depression, irritability or apathy often present

Question 19

cognitive impairment with fluctuations and vivd visual hallucinations: not stressful, but vivid and well formed. Woman believes that a family of young children moved into the house, and so she cooks food for the kids to feed them, no idea that the hallucination isn’t real; could be about small animals that come into the room, parkinsonism, COGWHEEL, sleep problems, bradykinesia.

Answer 19

lewy body dementia, adverse reaction to l/dopa (falls syncope, autonomic dysfunctino)

Question 20

diff between pd and lewy body

Answer 20

all in the timing: PD dementia, parkinsonism symptoms have been there at least a year or longer, in LBD, the cognitive changes come first or come in conjunction with the motor symptoms

Question 21

TEST: diff between pd and lewy body

Answer 21

all in the timing: PD dementia, parkinsonism symptoms have been there at least a year or longer, in LBD, the cognitive changes come first or come in conjunction with the motor symptoms

Question 22

TEST: 87 y.o. male, 8 yrs of education, retired ironworker
progressive memory loss for 2-3 years:
repeating questions
word finding difficulties
difficulty with IADLs (e.g., finances, cooking, driving)
visual hallucinations for 5 months; animals, people invading home paranoia, irritability
relevant med hx: atrial fib, macular degeneration, cataracts,
hearing loss, freq dizziness + falls, recent CT: generalized atrophy
meds: warfarin, furosemide, zinc, risperidone for hallucinations
Prominent motor symptoms on exam: cogwheeling, rigidity

Answer 22

Lewy Body DM

Question 23

Differential Diagnosis: Fronto-temporal lobar degeneration

Answer 23

Earlier onset (45-65) than most AD, with exceptions
May represent 20% of all cases <65 years of age seen in dementia clinic
4x more common in men, + FH in 1/3 cases
Personality changes: withdrawal, irritability, social inappropriateness
Often misdiagnosed as depression

Question 24

TEST: if younger person, male, presents with completely impaired cognitive functioning but memory completely intact

Answer 24

Think FTLD- Picks disease

Fronto-temporal lobar degeneration

Question 25

Gait disturbance, urinary incontinence, cognitive deficits (wobbly, wet, & wacky)
Cognitive deficits involve attention & executive function

Answer 25

Normal Pressure Hydrocephalus
Radiologist guides distinction between: atrophy leading to AD type dimentia, or enlarged ventricleswith preservation which point to nph

Question 26

Additional conditions that can impair cognition:

Answer 26

Cardiovascular disease
Nutritional deficiencies: B12, folate
Type 2 diabetes & impaired glucose tolerance
COPD/sleep apnea
Thyroid dysfunction
Hypo: memory recall, visuospatial, psychomotor speed
Hyper: executive function