Dementia and Prion-related Diseases Flashcards
Every 4 years after the age of 65-69, SDAT prevalence increase by how much?
it doubles (starting at 2% for ages 65-69)
The populations of numerous countries has a higher percentage of people that are getting (blank) than past years
older
(blank) is a syndrome of acquired, persistent intellectual impairment that is due to brain dysfunction.
dementia
What kind of dementia is this:
conenital mental retadation, and developmental delay
acquired dementia
What kidn of dementia is this:
delirium
persistent dementia
What kind of dementia is this: isolated deficiets (amnesia or aphasia)
multiple deficits dementia
Operationally, dementia implies impairment in (blank) or more of the following domains of mental capacity: Memory Praxis Executive Functions Language Calculations Personality Perception Semantic Knowledge Emotional Expression Awareness
three
How can you figure out if someone has dementia?
mental status assessment, clinical rating scales, neuropsychological testing
When do you get alzeheimers?
after 65
What is this:
slowly progressive decline in recent memory, language, visuospatial impairment, executive dysfunction
alzheimers
What is this: fluctuating course dementia followed by spontaneous parkinsonism visual hallucinosis and/or psychosis neuroleptic sensitivity
dementia with lewy bodies
What is this:
abrupt cognitive loss, stepwise decline
Infarcts and/or vascular disease by imaging
focal neurologic signs
vascular dementia
Dementia is abrupt or insiduous?
insiduous
Is delirium constant or fluctuating?
fluctuating
What is this:
onset BEFORE 65
prominent impairment of behavior, social conduct, judgement
early disturbance of lanuage, progressive aphasia
frontotemporal dementia
T/F
dementia is a global impairment of intellectual function
F
T/F
dementia always impairs memory?
F
T/F
dementia always impairs insight; patients aware of their deficits dont have dementia
F
T/F dementia is a cognitive disorder and never primarily a behavioral disorder
F
Dementia is synonymous with alzheimers disease
T/F
F
Dementia can have an acute onset
T
Can you treat dementia?
no, but you can treat the symptoms
Is this dementia or mild cognitive impairment: memory loss preserved cognition preseved ADL not demented
mild cognitive impairment
Is this dementia or not:
memory loss
at least 2 cognitive domains
diminished ADL
dementia
Is this delirium or dementia: Onset is acute duration is hours to days course is fluctuating arousal is lethargic to agitated distracted memory impaired by inattention dysarthric, incoherent speech and language frequent misperceptions fearful postural tremor myoclonus or asterixis EEG is slow
delirium
What is this:
most often insidious, last months to years, usually is constant, normal arousal and attnetion but abnormal amnesic memory, aphasia an dysnomic speech and language skills, mormal perception and fine motor skills until later in life and a normal EEG
dementia
What is this:
Learning is slower
Learned material is retained
Language, math, visual-spatial ability is preserved
Problem solving using shifting strategy is impaired, but experience aids
normal aging :)
(blanK) 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 exam
Alzheimers is associated with what protein gone bad?
amyloids
What are the elements you test in mental status evaluation?
arousal and attention language memory perception and construction personality and emotion
Median scores based on age and educational level for MMSE are what:
>85 years and >12 years of education = (blank)
70-74 and >12 years of education = (blank)
65-69 and 0-4 years of education = (blank)
28
29
22
what is the mini cog?
When are you considered demented on this exam?
combines 3 item recall w/ clock drawing test
if you get a 0/3 on recall or if you get a 1 or 2/3 and mess up clock drawing
memories are not stored in the (blank), it is just a pointer to the neural populations that are engaged during the memory phenomenon.
hippocampus
is it normal when you’re older to get interference form redundant or irrelevant material?
yes
What is preserved as you age, crystallized or fluid intelligence?
crystalllized
What are the categories of Katz index of ADLs? How do you rank each category?
bathing dressing toileting transfer continence feeding independent, assistance, dependent
What are these: Traveling Telephone Shopping Preparing meals Housework Medication Money
instrumental activites of daily living
At 3 years, instrumental activites of daily living impairment is a predictor of incidence of (blank).
dementia
greater the impairment, the greater the likelihood of dementia
What is the amount of time that is considered subacute dementia?
less than 6 months
What can cause sub-acute dementia?
prions, tumors, autoimmune
What are these:
Prions, Tumors, Auto-immune
Tumors- primary, metastatic and lymphomas
Auto-immune-Hashimoto’s, K channels antibodies, paraneoplastic
Prions –Hereditary, Acquired, Iatrogenic
causes of sub acute dementia
What is the cellular protein that goes bad in SDAT (alzheimers)?
amyoloid (abeta 42) and Tau
What is the cellular protein that goes bad in FTLD (frontal temporal lobe dementia)?
Tau
What protein goes bad in parkinsons disease dementia with lewy bodies (DLD)?
synuclein
What protein goes badin creutzfeld jakob disease (CJD)?
prions
What kind of neurogenerative diseases are these:
Multiple system atrophy
Parkinsons’ Disease
Dementia with lewy bodies
synucleinopathies
What kind of neurodegenerative disease does alzheimers belong to?
amyloidopathies
What kind of neurogenerative diseases are these:
Alzheimers disease, progressive supranuclear palsy, chronic traumatic encephalopathy, frontotemporal dementia, corticobasal degeneration
Tauopathies
How does normal protein degredation happen?
with ubiquitination and proteases
What is Kuru?
a neurodegenerative brain disease caused by cannabilizing brain tissue
What disease is this:
progressive dememtia and myoclonic jerks. Onset late middle age, most cases sporadic, rarely familial
CJ disease
What is this:
progressive insomnia and dementia
familial fatal insomnia (FFI)
How long does it take for a person to pass away from CJ disease or familial fatal insomnia?
6-12 months following symptoms
What is this: Onset 3-4 years Intense itching Ataxia and death Altered prion protein Spongiform encephalopathy Transmitted by milk, urine through enviornment
scrapie-prion disorder of sheep
What is 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
What is this:
Young people in Britain-prion disorder
Presents as a psychiatric disorder
Due to ingestion of beef affected by bovine spongiform encephalopathy
Relatively long duration of illness(14 months)
No new cases since control of bovine disease
variant CJD
What is the abnormal form of PrPC and what does it make?
PrPsc
209 beta sheet insoluble, protease resistant, prion
How do you get rid of a prion?
you have to burn it (cant autoclave it and is protease resistant)
How does a prion work?
normal protein undergoes change and forms beta sheets that elongate and trigger normal proteins to misfold-> kills cell membranes
Tau is a microtubular (Blank) protein. So when these get messed up and aggregate its bad.
stabilizing
What is chronic traumatic encephalopathy (CTE)?
closed head injury disrupts fiber tract and you get tau protein release that misfolds proteins
Whats scary about concussions?
if you get one and dont let it heal properly you cant get misfolded proteins!
What is this: can cause parkinsons alien hand apraxia aphasia 4 repeat tau and overlaps with PSP
CBD (cortio-basal ganlgionic degeneration)
What is this: Axial rigidity Loss of vertical and terminally all EOM Dementia/dysphagia/dysarthia 4repeat Tau
PSP (progressive supra-nuclear palsy)
What 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
20% of pre-senile Alzheimer’s are due to this
3 repeat Tauopathy
FTLD (fronto-temporal lobar degeneration)
The amyloid precursor protein has a (blank) function with (blank) control and cell binding
G protein
calcium
Beta amyloid peptide can misfold into (blank) and (Blank)
fibril
aggregate
B amyloid fibrils can travel in (blank) to neighboring cells forming template for further misfolding
axons
where does degenearation first occur in the brain with alzeheimers?
medial temporal lobe and then travels to pairetal lobe and temporal lobe
Is parkinson a systemic disease or a local disease?
systemic disease
lewy body fromation follows what kind of path?
a caudal - rostral path
What is the Braak staging of lewy bodies?
asymptomatic phase, presymptomatic phase
What is this:
Eosinophilc, round intracytoplasmic inclusions
Described in 1912 by a German neuropathologist
Inclusions particularly numerous in the substantia nigra pars compacta.
lewy bodies
what do you find in parkinsons and dementia?
What do you find in alzheimers disease
synuclein and lewy bodies
amyloid plaque cores
What is affected in BRAAK stage 1 and 2?
(olfactory neurons and motor nucleus of vagus)
DMN-X- MOTOR FUNCTION TO PHARYNX, GUT
SENSORY FUNCTION FROM PALATE , PHARYNX, GUT
HYPOPHONIA, CONSTIPATION PRECEED OTHER MOTOR SYMPTOMS BY YEARS
CARDIAC SYMPATHETIC DENERVATION
ie. think nose and gut!!!!
if you have alot of amyloid you probably have what?
alzeheimers
if you dont have any, you have some form of dementia
when you are in stage one of braak, why do you have olfactory loss?
because you have damage to the olfactory bulb, and ant. olf. nerve, and amygdala is damaged
When you are in stage one of braak stage. why do you have dysautomonia-gastroparesis, constipation, urinary urgency, erectile dysfunction?
because you have damage to dorsal motor nucleus of vagus, symp. ganglia, enteric plexu, intermed/later column cord
When you are in stage 2,3 of braak stage why do you have depression/anxiety?
your locus ceruleus, raphe nucleus, amygdala, limbic cortex
Why do you have disorganized Rem sleep behvaior when stage 2 of braak stage?
nucleus subcerules, peduculopontine nucleus, thalamus, hypothalamus are affected
What are the three components that contribute to the overlapping nature of parasomnias?
sleep phenomena, seizures, psychogenic
What kind of parasomnias are these:
PTSD
nocturnal panic
dissociative disorders
psychogenic
What kind of parasomnias are these:
confusional arousals
sleepwalking
sleep terrors
NREM
What kind of parasomnias are these:
hypnagogic hallucinations
sleep paralysis
REM disorders
Parkinson is associated with what kind of disorder?
affective disorder
What BRAAK stage is this: SUB-CORTICAL DEMENTIA IMPAIRED CONCENTRATION SLOWED RESPONSES PERSONALITY CHANGE VARIABLE COGNITIVE LOSS
BRAAK stage 6
What stage of BRAAK is this:
Hallucinations of…..
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
What are 2 distinct disorders with a common cholinergic deficit?
PDD and AD
How can you differentiate between PDD and AD?
PDD:
lewy body pathology in cholinergic basal forebrain and brainstem PPT
AD: neurofibrillary tangles in cholinergic basal forebrain and brainstem PPT
When you have parkinsons disease, what happens to your blood pressure as you stand up?
it goes down
