Dementia Flashcards
What is the prevalence of dementia at 85+ years of age?
40%
T/F Our population is getting older & therefore our population of demented patients is getting larger.
Yeah, true.
What is dementia?
a syndrome of acquired, persistent intellectual impairment that is due to brain dysfunction.
Do patients with Down’s Syndrome have dementia?
No. It must be acquired. Those with congenital mental & developmental delays do not have dementia.
T/F Delirium & dementia are the same.
False. They are different. one of the main distinguishing characteristics of dementia is that it is persistent. Delirium is not.
If you have amnesia or aphasia & nothing else—>do you have dementia?
No. b/c dementia does not consist of isolated deficits, but of multiple deficits. A syndrome.
The diagnosis of dementia requires diminishment in 3 or more domains of mental capacity. What are some options for domains?
**there are 9 domains memory language: receptive & expressive perception praxis calculations semantic knowledge: conceptual knowledge executive functions personality emotional expression/awareness
What is praxis?
knowledge of how to do things–>use a screwdriver
Name 4 common dementia syndromes.
Alzheimer’s Disease
Dementia with Lewy Bodies
Vascular Dementia
Frontotemporal Dementia
What is the usual age of onset of Alzheimer’s disease? Which deficit usu occurs first? What are the other losses that often happen?
usual age of onset–>age 65
usu memory goes first.
slowly progressive decline in recent memory, language, visuospatial impairment, executive dysfunction
Describe what happens in dementia with lewy bodies.
fluctuating course
dementia followed by spontaneous parkinsonism
visual hallucinosis and/or psychosis
neuroleptic sensitivity
What are the signs of vascular dementia?
abrupt cognitive loss, stepwise decline
Infarcts and/or vascular disease by imaging
focal neurologic signs
What is the usual age of onset for frontotemporal dementia? What are the beginning losses? What is eventually lost?
age of onset less than age 65
prominent impairment of behavior, social conduct, judgment
early disturbance of language, progressive aphasia
begins with language loss or behavioral control loss or loss of concepts.
What is the difference b/w dementia & mild cognitive impairment?
Dementia: memory loss & loss of at least 2 cognitive domains; diminished ADL
Mild Cognitive Impairment: memory loss but persevered condition & ADL
T/F If you can do activities of daily living–you are NOT demented.
True.
Does mild cognitive impairment ever progress to dementia?
Yes-1/3 of the time
1/3 of the time you remain at mild cognitive impairment
1/3 of the time you get better! yay!
What is the onset, duration, and course of delirium like?
acute onset.
duration of hours–days
fluctuating course
What is the onset, duration, and course of dementia like?
can’t really notice when it starts (insidious)
duration of months to years
usually a constant course
What is the arousal, attention, and memory like for patients with delirium?
Arousal: lethargic–agitated
Attention: prominent distractibility
Memory: impaired by inattention
What is the arousal, attention, and memory like for patients with dementia?
Arousal: normal
Attention: normal or mildly abnormal
Memory: amnesia present, but attentional function intact
What is speech/language, perception & affect like for patients with delirium?
Language: dysarthric, incoherent, dysgraphia prominent
Perception: frequent misperceptions
Affect: fearful or suspicious
What is speech/language, perception & affect like for patients with delirium?
Language: dysnomic, aphasia
Perception: normal or mildly abnormal
Affect: normal or mildly abnormal
What are the motor signs of delirium? What does the EEG show?
postural tremor
myoclonus-occurs late in the game
maybe asterixis (tremor of hand when wrist extended)
EEG: diffusely slow
What are the motor signs of dementia? What does the EEG show?
usually none until late progression of dementia
EEG: normal or mildly slow
When old people get lost while driving should you report them? Is this a motor problem or memory problem?
usu a memory problem
only if it is a motor problem are they really endangering other people & needed to be reported to the DMV
What does normal aging look like?
of cognitive and behavioral changes
Most consistently manifests as a generalized slowing of both intellectual and physical performance
preserved crystalline intelligence (old solutions)
decreased fluid intelligence
stable verbal IQ
decreased working memory
What is praxis?
Integration and performance of learned, complex motor act
What is a normal median score for the MMSE for a person with these stats?
>85 years and >12 years of education
28
What is a normal median score for the MMSE for a person with these stats?
70-74 and >12 years of education
29
What is a normal median score for the MMSE for a person with these stats?
65-69 and 0-4 years of education
22
What is the animal naming test? What is this a highly sensitive test for?
name as many animals as you can in 1 minute.
highly sensitive for alzheimer’s disease-these people can often only name 12
average normal is 18.
Which things count as activities of daily living?
Bathing Dressing Toileting Transfer Continence Feeding Independent Assistance Dependent
Which things count as instrumental activities of daily living?
Traveling Telephone Shopping Preparing meals Housework Medication Money Independent, Assistance, or Dependent
Which is a better 3 year predictor of incidence of dementia: ADL or IADL impairment?
IADL is a better predictor
What are the 2 categories of dementia?
subacute & chronic
Describe subacute dementia.
duration is less than 6 mo
can be caused by prions, tumors or auto-immune
What are some tumors that can cause subacute dementia?
primary tumors
metastatic tumors
lymphoma
What are some autoimmune conditions that can cause subacute dementia?
Hashimoto’s Disease
K channel antibodies
Paraneoplastic process
What are some neurodegenerative diseases that are caused by normal proteins gone bad?
Alzheimer’s Disease
Frontal Temporal Lobe Dementia
DLD?
Creutzfeld-Jakob Disease
Which protein is messed up in Alzheimer’s?
Amyloid (Abeta42)
Tau
Which protein is messed up in Frontal Temporal Lobe Dementia?
Tau
Which protein is messed up in DLD?
Synuclein
T/F CJ disease includes prions.
True.
What are some tauopathies?
CTE: chronic trauma encephalopathy PSP: progressive supranuclear palsy AD: Alzheimer's FTLD: Frontal Temporal Lobe Dementia CBD: Chronic Basal Ganglionic Degeneration
What are some amyloidopathies?
AD: Alzheimer’s
What are some synucleinopathies?
MSA: multiple system atrophy
DLB: Dementia with Lewy bodies
PD: Parkinson’s Disease
Describe the idea behind protein recycling.
old or abnormal protein has a signal on it…ubiquitin attaches to it & it gets put in the trash
proteases (which use ATP) degrade the abnormal protein.
What is kuru?
seen in people of New Guinea who practiced cannibalism as a part of funeral ritual
women & children ate brain & were most affected
**get an instead gait & dysarthria–eventually get ataxia & uncontrollable laughter
incubation period of 14 yrs…
Describe the onset & symptoms of Creutzfeld-Jakob disease.
onset-late middle age
usu sporadic
progressive dementia & myoclonic jerks
Describe the onset & symptoms of familial fatal insomnia.
onset: late middle age
see progressive insomnia & dementia
see spongiform changes in the brain w/o inflammation (spongiform encephalopathy)
death in months following symptoms
A lot of these prion disorders started in animals. Describe the deal with scarpie-prion disorder of sheep.
onset is 3-4 years caused by altered prion protein get intense itching, ataxia, death shows spongiform encephalopathy transmitted into the environment thru urine & milk
A lot of these prion disorders started in animals. Describe the deal with Mad Cow Disease.
affects cows at 4-5 yo
experience weakness, ataxia, spongiform encephalopathy
people can get it when they eat bad cow meat
What was the variant of CJD that affect young people in Britain?
looked like a psychiatric disorder
due to ingestion of beef that had spongiform encephalopathy
long duration of illness (14 mo)
T/F Prions are protease resistant & insoluble.
True.
Describe prion conversion.
normal protein changes & enucleates starts stacking into beta sheets it elongates & a piece breaks off autocatalytic process by which one bad protein starts making a bunch of other proteins bad too fast progression of disease
What is a good test to pick up dead brain tissue from a stroke within an hour of onset?
diffusion weight axial CT
How do prions spread from cell to cell?
Misfolded protein kills the cell-cell membrane damage, excessive energy usage etc
Dead cell releases misfolded protein
Remaining cells engulf the abnormal protein and transport it by axonal transport to synapse
Describe how tau protein can undergo prion conversion with head trauma. Which condition does this describe?
- *head trauma can cause micro-hemorrhages
- *tau holds things together with axonal transport from soma to synapse.
- *when tau is released b/c of trauma->it is picked up by another cell & misfolds into fibrillar form
- *this is what happens in chronic traumatic encephalopathy
What is the end result of CTE?
atrophy of frontal & temporal lobes
tau deposition in these lobes & in the basal ganglia
Cortico-basal-ganglionic degeneration is a pure tauopathy. Which things fall into this category?
Parkinson’s
Alien Hand
Apraxia-disorder of motor planning
Aphasia-language problem
Progressive supra-nuclear palsy is a pure tauopathy. Which things fall into this category?
Axial rigidity messed up ocular movements dementia dysphasia dysarthria
20% of Pre-Senile Alzheimer’s is ______.
80% of Dementia is _______.
Frontal Temporal Lobar Dementia
Alzheimer’s
What are some of the pathological things that observed upon autopsy of Alzheimer’s patients?
amyloid plaques
tau & neurofibrillary tangles
What is the amyloid precursor protein?
transmembrane protein
involved in cell adhesion
has a G protein
Why can the amyloid precursor protein turn evil?
secretases will eat the APP up & spit out external & internal portions of it.
they will leave a beta amyloid peptide (40-42AA)
Beta amyloid can misfold & aggregate
Which area of the brain is first targeted by Alzheimer’s?
medial temporal lobe is the first to go!
T/F Parkinson’s is a systemic disease, not a brain disease.
True…
Describe Braak’s staging of Lewy bodies.
the protein that is evil gets into the brainstem
then it gets into the basal ganglia
then it moves to the cortex
What are the symptoms of stage 1 of Braak? Which structures are damaged during this stage?
constipation & dysautonomia & orthostatic hypotension
anosmia-b/c the protein is found in the nasal epithelium
**DMX & Olfactory nucleus are damaged during this stage.
What are Lewy bodies?
eosinophilic & intracytoplasmic inclusions
they are particularly numerous in the substantia nigra pars compacta
Which structures are damaged during stages 3 & 4? What are the symptoms of this?
Structures: locus ceruleus, raphe nucleus, amygdala, limbic cortex–>Symptoms: depression, anxiety
Structures: nucleus subceruleus, peduculopontine nucleus, thalamus, hypothalamus–>symptoms: Sleep Rem disturbance
**substantia nigra is also damaged in stage 4
Which structures are damaged in stage 6? What are the symptoms of this stage?
thalamus & neocortex compromised. get hallucinatory experiences. impaired concentrations. personality changes **dementia
A cholinergic deficit can often be seen in which 2 separate disorders? What is the treatment for this?
Parkinson’s Disease (Lewy bodies in cholinergic basal forebrain)
Alzheimer’s Disease (neurofibrillary tangles in cholinergic basal forebrain)
**use cholinesterases
