McHugh: Dementia Flashcards
At what age does the prevalence of dementia begin to double every 5 yrs?
after age 65
What’s this?
syndrome of acquired, persistent intellectual impairment that is due to brain dysfunction
dementia
What does it mean that dementia is persistent?
it does not get better or worse…
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
What’s this?
onset after 65
slowly progressive decline in recent memory, language, visuospatial impairment, executive dysfunction
Alzheimer’s disease
What’s this?
fluctuating course
dementia followed by spontaneous parkinsonism
visual hallucinosis and/or psychosis neuroleptic sensitivity
dementia w Lewy Bodies
abrupt cognitive loss, stepwise decline Infarcts and/or vascular disease by imagingfocal neurologic signs
vascular dementia
onset before 65
prominent impairment of behavior, social conduct, judgment
early disturbance of language, progressive aphasia
frontotemporal dementia
Is dementia a global impairment of intellectual function?
no, not global
Does dementia always impair memory?
no
Does dementia always impair insight?
no; pts are usu aware of their dementai
Is dementia only a cognitive disorder and never primarily a behavioral disorder?
no, can be behavioral, too
Is dementia inevitable and synonymous w senility? Is it = to Alzheimer’s disease?
no!
Can dementia have an acute onset?
yes
Is dementia treatable?
it is
What's this? memory loss preserved cognition preserved ADL (activities of daily living) not demented
mild cognitive impairment
What’s this?
memory loss
at least 2 cognitive domains
diminished ADL
dementia
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
What's this? Onset most often insidious Lasts months to years Usually constant Normal arousal and memory dysnomic or aphasia no misperceptions
dementia
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
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
What MMSE score is appropriate for those ages 85+ w more than 12 years of education?
28
What MMSE score is appropriate for those ages 70-74 w more than 12 years of education?
29
What MMSE score is appropriate for those ages 65-69 w 0-4 years of education?
22
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?
- tumors
- auto-immune disease
- 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)
65
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
