McHugh: Dementia Flashcards by Megan Foggia

At what age does the prevalence of dementia begin to double every 5 yrs?

after age 65

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What’s this?

syndrome of acquired, persistent intellectual impairment that is due to brain dysfunction

dementia

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What does it mean that dementia is persistent?

it does not get better or worse…

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Operationally, dementia implies impairment in three or more of the following domains of mental capacity:

memory
language
perception
praxis (knowing how to do things)
calculations
semantic knowledge
executive function
personality
emotional expression or awareness

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What’s this?
onset after 65
slowly progressive decline in recent memory, language, visuospatial impairment, executive dysfunction

Alzheimer’s disease

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What’s this?
fluctuating course
dementia followed by spontaneous parkinsonism
visual hallucinosis and/or psychosis neuroleptic sensitivity

dementia w Lewy Bodies

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abrupt cognitive loss, stepwise decline Infarcts and/or vascular disease by imagingfocal neurologic signs

vascular dementia

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onset before 65
prominent impairment of behavior, social conduct, judgment
early disturbance of language, progressive aphasia

frontotemporal dementia

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Is dementia a global impairment of intellectual function?

no, not global

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Does dementia always impair memory?

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Does dementia always impair insight?

no; pts are usu aware of their dementai

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Is dementia only a cognitive disorder and never primarily a behavioral disorder?

no, can be behavioral, too

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Is dementia inevitable and synonymous w senility? Is it = to Alzheimer’s disease?

no!

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Can dementia have an acute onset?

yes

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Is dementia treatable?

it is

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What's this?
memory loss
preserved cognition
preserved ADL (activities of daily living)
not demented

mild cognitive impairment

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What’s this?
memory loss
at least 2 cognitive domains
diminished ADL

dementia

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What's this?
acute onset
lasts hrs to days
fluctuating course
lethargy upon arousal
prominent distractability
memory impaired by inattention
dysarthric speech
frequent misperceptions
fearful, suspicious aspect

delirium

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What's this?
Onset most often insidious
Lasts months to years
Usually constant
Normal arousal and memory
dysnomic or aphasia
no misperceptions

dementia

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What are these associated w?
Decreased complex or sustained attention
Interference from redundant or irrelevant material
Preserved crystallized intelligence (old solutions)
Decreased fluid intelligence (new information for novel solutions)
Relatively stable verbal IQ
Decline in performance IQ
Decreased working memory
Slowed retrieval of stored memory

normal aging

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can distinguish the intellectual changes of dementia from those associated to delirium, isolated cognitive deficits, normal aging and other conditions
Identifies patterns and profiles of neurobehavioral dysfunction which suggest specific dementing diseases
Establishes and communicates severity of dementia and follow course of patients over time

mental status assessment

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What MMSE score is appropriate for those ages 85+ w more than 12 years of education?

What MMSE score is appropriate for those ages 70-74 w more than 12 years of education?

What MMSE score is appropriate for those ages 65-69 w 0-4 years of education?

What test is looking at temporal lobe semantic storage, and assessing category fluency?

animal naming test

How do you perform the animal naming test? What is the animal naming test highly sensitive to?

1 min to name as many animals as you can - you get 1 pt for each animal named w/i the minute, avg performance is 18/minute; Alzheimer's disease

At 3 yrs, impairment of instrumental activities of daily living is a predictor of the incidence of (blank)

dementia

Dementia can be sub-acute or chronic. Over what duration is it sub-acute?

less than 6mo

What are 3 causes of sub-acute dementia?

1. tumors 2. auto-immune disease 3. prions

``` What's this? Prion disorder of sheep Onset 3-4 years Intense itching Ataxia and death Altered prion protein Spongiform encephalopathy Transmitted by milk, urine through enviornment ```

scrapie

``` What's this? Prion disorder of cows and other ungulates Affects cows 4-5 years of age Weakness and ataxia Spongiform encephalopathy Sporadic vs. enviornment/diet ```

mad cow disease

progressive dememtia and myoclonic jerks. Onset late middle age, most cases sporadic, rarely familial

CJ disease

progressive insommnia and dementia

familial fatal insomnia

``` What prion form is this? 209 AA, Alpha Helix Copper binding Soluable Protease digestible ```

normal cellular

``` What prion form is this? 209 AA beta sheet Function if any unknown Insoluable Protease resistant ```

abnormal form

How does the prion model work?

Normal cellular protein misfolds Misfolded protein cannot be cleared It becomes the template to cause other proteins to misfold Misfolded protein kills the cell Dead cell releases misfolded protein Remaining cell engulfs the abnormal protein & transports it by axonal transport to synapse

What is this? Closed head injury causes tau protein to be released into CSF It is converted to a fibrillar form which is taken up by nearby cells and transported along axons It acts as a template to misform normal tau Aggregates cross synaptic space to cortical neurons

chronic traumatic encephalopathy

Chronic traumatic encephalopathy occurs when there is atrophy of the frontal and temporal lobes. (blank) deposits in those lobes and in the basal ganglia, causing dementia (decreased memory, executive function, depression, and aggressive behavior.

TAU

What are the pure tauopathies?

CBD: cortico-basal ganglionic degeneration PSP: progressive supra-nuclear palsy FTLD: fronto-temporal lobar degeneration

``` What are these symptoms of? Parkinson’s Alien hand Apraxia Aphasia 4 repeat Tau and overlaps with PSP ```

CBD

What are these symptoms of? Axial rigidity Loss of vertical and terminally all EOM Dementia/dysphagia/dysarthia 4repeat Tau

PSP

What the heck is this? Behavioral variant-loss of executive function, apathy Primary progressive aphasia Semantic variant-loss of concepts with preserved vocabulary Many but not all due to mutation on Tau gene on chromosome 17 Pick’s disease 3repeat Tauopathy

FTLD

20% of pre-senile Alzheimer’s are (blank)

FTLD

Alzheimer's disease doubles in prevalence every 5 yrs after age (blank)

What is the major amyloidopathy?

Alzheimer's disease

In what portion of the brain will amyloid first build up in Alzheimer's?

medial temporal lobes

What is this? LEWY BODY FORMATION IN THE BRAIN FOLLOWS CAUDAL-ROSTRAL PATH PHENOTYPE FOLLOWS STAGES DEFINITE STAGES ARE DEFINED

Braak hypothesis | *protein spreads from medulla to pons to basal ganglia to cortex

Eosinophilc, round intracytoplasmic inclusions, particularly numerous in the substantia nigra pars compacta

lewy bodies

In the Braak stages 1 and 2, what two things are most affected?

olfactory neurons *nose | motor nucleus of the vagus *gut

What symptom of Parkinson's precedes the onset of motor disease by 10 years?

constipation | *death to neurons in myenteric plexus

Pre-motor symptoms in Parkinson's disease?

olfactory loss - stage 1 Braak dysautonomia (gastroparesis, constipation, urinary urgency, erectile dysfuntion) - stage 1 Braak depression/anxiety - stage 2 Braak REM sleep behavior - stage 2

By Braak stage 6, what structures are affected? What does this lead to?

thalamus, neocortex; cognitive loss

``` SUB-CORTICAL DEMENTIA IMPAIRED CONCENTRATION SLOWED RESPONSES PERSONALITY CHANGE VARIABLE COGNITIVE LOSS AUDITORY-BANGS, KNOCKS, OCC. VAGUE VOICES TACTILE-ANIMAL CONTACT VISUAL ILLUSIONS-INANIMATE SEEN AS LIVING PASSAGE –BRIEF, PASSING BY PATIENT SENSE OF PRESENCE ```

BRAAK STAGE 6

Both Parkinson's disease and Alzheimer's have a common (blank) deficit

cholinergic