Dementia Types Flashcards
1
Q
Alzheimer’s Disease or Dementia of The Alzheimer’s Type (DTA)
A
- most common etiology of dementia
- typically impacts those 65+ years
- can be difficult to diagnose
2
Q
Diagnostic Criteria (DTA)
A
- build upon the basic criteria for diagnosing dementia
- gradual onset and progressive cognitive deterioration
- cognitive deficits are not due to: other CNS issues, systemic conditions, substance-induced conditions
- cognitive deficits are not due to an axis I disorder (schizophrenia or major depressive disorder)
- diagnosis of AD is presumptive until autopsy or brain biopsy can be completed; focus is on criteria leading to “degree of confidence”
3
Q
“Possible” Alzheimer’s Disease
A
- dementia in the absence of other dementia-producing causes but w/atypical onset, presentation or course
- dementia presenting with another systemic or brain disorder sufficient enough to produce dementia but which is not considered to be the cause of the dementia
- severe progressive decline of a single cognitive function in the absence of another specific cause
4
Q
“Probable” Alzheimer’s Disease
A
-dementia is established by clinical exam documented by mental status examinations and confirmed by neuropsychological tests
-deficits exist in 2+ areas of cognition
progressive worsening of memory and other cognitive functions has occurred
-no disturbance of consciousness
-onset was b/w 40-90; most often after age 65
-absence of systemic disorders or other brain disease that in of themselves could account for the progressive deficits in memory and cognition
5
Q
Neuropatholgy of AD
A
-typically begins in areas most related to episodic memory
(perirhinal cortex, hippocampal complex in temporal lobes and basal forebrain)
-as disease worsens, changes in frontal lobe are noted and working memory is affected
-once disease gets to the temporal and parietal areas, sensory memory becomes impacted
-motor and occipital functions are typically spared
-since motor is not usually affected, speech remains intact
6
Q
Motor Impairment (DAT)
A
-motor symptoms can develop as the disease progresses
typical EPS s/s
-change in muscle tone, cogwheel rigidity, postural instability and difficulty with gait
-the presence of EPS s/s are associated with greater dementia severity
7
Q
Microscopic Changes (DAT)
A
microscopic examination of brain tissue, often postmortem reveals the presence of:
- neuritic plaques
- neurofibrillary tangles
- atrophy
- areas of granulovacular degeneration
- amyloid deposits may also be seen in the blood vessels
8
Q
Neuritic Plaques
A
- bits and pieces of degenerating neurons that clump together and have a beta-amyloid core
- beta-amyloid is a protein fragment that has been separated from a larger protein
- disjoined beta-amyloid fragments aggregate and mix with other molecules, neurons and non-nerve cells
- most prevalent in the outer half of the cortex where the number of neuronal connections is largest
9
Q
Neurofibrillary Tangles
A
-disintegrating microtubules
microtubules are part of the internal structure of healthy neurons
-they break down due to changes in the protein tau (these stabilize the microtubules)
-as they break down they become entangled
-they are a signature morphological sign of AD
10
Q
Atrophy
A
- shrinking of brain tissue
- may not show up on CT if Pt is in the early stages of AD
- better visualization using PET and MRI scans
11
Q
Granulovacular Degeneration
A
- refers to fluid-filled spaces within cells that contain granular debris
- along with all of the other microscopic changes in the brain facilitate interruption of intercellular communication and thus information processing
12
Q
AD Risk Factors
A
age family history less education head trauma gender maternal age having two copies of the type 4 allele of apolipoprotein E having MCI
13
Q
Preclinical AD
A
- AD occurs many years before a clinical diagnosis is made
- cognitive decline may occur 6y before clinical diagnosis of AD
- preclinical deficits are apparent for both verbal and nonverbal information
- lower scores on measures of memory and abstract reasoning are particularly strong predictors of probable AD
14
Q
Predictors of Disease Progression
A
Average duration of AD is 8y+
More rapid decline is associated with:
- early age at onset
- presence of delusions or hallucinations
- presence of EPS s/s
15
Q
Cognitive/Communication Effects
A
Consistent features of AD include:
- impairment of episodic and working memory
- other executive function declines
Longitudinal studies using the MMSE indicate that the average amount of decline per year is 2-4 points
16
Q
AD Early Stage
A
Lasts from 2-4y
Caregivers report changes with:
- difficulty handling finances
- memory problems
- concentration problems
- difficulty w/complex tasks
- forgetting the location of objects
- decreased awareness of recent events
Mental status: disoriented for time
Motor function: good, ambulatory
Memory: problems w/episodic memory and working memory
17
Q
AD Early Stage Cont…
A
Basic ADLs: can complete basic ADLs
MMSE: 16-24 points
Linguistic skills:
- fluent speech
- spelling/grammar errors are common in written language
- increased number of empty words (thing, it)
- anomia
- vocabulary shrinks
- difficulty with sarcasm and understanding jokes
18
Q
AD Middle Stage
A
4-10y post diagnosis
MMSE: 8-15 points
- Mental status: becomes disoriented for place and time
- Motor: good, restlessness is common
- Memory: episodic memory worsens, pt is easily distractible
incontinence: mostly bladder - Basic ADLs: can complete w/supervision; managing finances and driving becomes difficult
19
Q
AD Middle Stage (Linguistic skills)
A
Linguistic skills:
- fluent speech, but is slower and halting
- use fewer nouns than verbs
- vocabulary continues to decrease
- diminished comprehension of written and spoken language
- anomia
- difficulty repeating phrases
- problems defining words
20
Q
AD Late Stage
A
MMSE: 0-9 points
Mental status: place, time, person disorientation
Motor: impaired; some pts are non-ambulatory
Incontinence: both bowel and bladder
Basic ADLs: unable to complete
Linguistic skills:
-fluent speech but is slow/halting w/meaningful expression reduced
-some pts are mute; some have palilalia; some have jargon
-reading comprehension is severely impaired
-considerable variability exits among late-stage AD
21
Q
Vascular Dementia
A
VaD
- cerebrovascular disease is the second most common cause of dementia
- more common in men
- caused by disease to the smaller blood vessels of the brain
- median survival time after onset of vascular dementia is 3.3y
- has also been referenced as multi-infarct dementia
- greater risk of morbidity and mortality than AD
22
Q
Neuropathology (VaD)
A
- defined as the loss of cognitive functions to a degree that interferes w/ADLs resulting from ischemic or hemorrhagic CVD
- type of vascular disease, its location, and the amount of brain damage dictate the clinical presentation of the dementia
23
Q
Major Etiologic Subtypes of VAD
A
- large vessel disease associated w/multiple infarcts in cortex, white matter, and basal ganglia
- small blood vessel disease associated w/multiple infarcts
- multilacunar state
- hypoperfusion in border zones and granular cortical activity
- post-ischemic encephalopathy
24
Q
Risk Factors for VAD
A
- more common in males
- incidence/prevalence increases with age
- history of stroke or a first-degree relative w/a history of stroke
- poor life-style choices: dietary habits, lack of exercise, alcohol abuse and smoking
- HTN: single most important risk factor for VaD
- diabetes; 3x more likely to develop stroke-related dementia
25
Diagnostic Criteria for Possible VAD
Possible VaD:
- dementia
- CVD
- onset of dementia w/in 3 months of stroke
- abrupt deterioration in cognitive functions
26
Diagnostic Criteria for Probable VAD
Probable VaD:
- dementia
- CVD
- onset of dementia w/in 3 months of stroke
- abrupt deterioration in cognitive functions
- gait disturbance
- falls (unsteadiness)
- urinary symptoms
- personality/mood changes
- absence of other disorders capable of producing dementia
27
VAD Impact on Cognition
- no one pattern of cognitive decline
- executive dysfunction is the most common cognitive consequence of CVD whereas memory impairment is typically associated with AD
28
VAD Impact on Communication
- communicative function is impacted most in pts with VaD
- will vary depending upon the type of vascular disease
- significant aphasia
- comprehension deficits
- difficulty w/language formulation
- dysarthria
- motor weakness
- changes in pitch, melody, and rate of articulation
29
Mixed Dementia
- presence of both AD pathology and vascular disease
- produces earlier and more severe cognitive impairment
- shorter length of survival
30
Lewy Body Dementia
- possibly the second most common type of dementia
- first described in 1961
- between 15% and 25% of pts with dementia have diffuse cortical lewy bodies
- lewy body: abnormal aggregation of protein in the cell processes of neurons
- age of onset for LBD is 50-83y
According to the LBD association, LBD is now considered a spectrum disorder; it now includes:
1 dementia w/lewy bodies
2 parkinson’s disease dementia
31
Diagnostic Criteria (LBD) Central Feature
Central feature:
Essential for a diagnosis of possible or probable lewy body dementia:
-dementia defined as progressive cognitive decline that interferes w/normal social/occupational functions
-persistent memory impairment is evident w/progression noted
-deficits in attention, executive function, and visuospatial ability
32
Diagnostic Criteria (LBD) Core Feature
Core feature:
Two core features are sufficient for a diagnosis of probable lewy body dementia, one for possible lewy body dementia:
- fluctuating cognition w/pronounced variations in attention and alertness
- recurrent visual hallucinations that are typically well formed/detailed
- spontaneous features of parkinsonism
33
Diagnostic Criteria (LBD) Suggestive Features
Suggestive Features:
reference p. 107 for criteria
-REM sleep behavior disorder [pt acts out dreams; not paralyzed]
-severe neuroleptic sensitivity [medication that blocks dopamine receptors]
-low dopamine uptake in the basal ganglia
34
Diagnostic Criteria (LBD) Supportive Features
```
Supportive features:
Commonly present but not proven to have diagnostic specificity:
-repeated falls
-transient LOC
-severe autonomic dysfunction
-hallucinations in other modalities
-systematized delusions
-depression
```
35
Diagnostic Criteria (LBD)
A diagnosis of lewy body dementia is less likely if:
- pt has CVD
- any other illness or disorder can account for the clinical picture
- parkinsonism only appears for the first time at the stage of severe dementia
Lewy body dementia should be:
-diagnosed when dementia occurs before or concurrently w/parkinsonism
Parkinson disease dementia should be:
-used to describe dementia that occurs in the context of well established parkinson’s disease
36
LBD vs. PD
- fluctuation of cognitive symptoms is a defining feature of lewy body dementia
- 50%+ of pts with lewy body dementia do not respond to treatment w/Ldopa
- in lewy body dementia, dementia signs precede parkinsonism
- in PD, parkinsonism signs usually precede dementia
37
Cognition and Communication in LBD
- cognitive impairment is present, severity fluctuates
- hallucinations are present
- sleep disorders, daytime drowsiness, and apathy develop
- aphasia and apraxia may appear in the later stages
- issues arise regarding fluency
- abnormal gait
- slow movements may be present
38
Frontotemporal Dementia
describes a clinical syndrome associated with various degenerative conditions
SLPs are likely to work with pts who have:
- pick’s disease
- primary progressive aphasia
- semantic dementia
39
Pick's Disease
- behavioral component
- affects more women than men; usually in their 50s
- disease duration can last from 3-17y
- presence of pick bodies
- secondary to frontal lobe issues, changes in personality are present
- pts demonstrate: poor judgment, are emotionally blunted, compulsively explore their environment, hyperorality, altered dietary preferences, changes in sexual behavior, visual/auditory agnosia
- 78% exhibit repetitive behaviors
40
Cognitive Changes in Pick's Disease
- attention deficits
- executive function issues
- some memory issues as well, however, most pts can track day-to-day events until late in the disease
- there is preservation of visuospatial abilities
41
Communication Changes in Pick's Disease
- majority of pts w/pick’s disease do not have aphasia early in the disease
- many do develop communication disorders that take the form of nonfluent aphasia with phonological and articulatory impairments
- agrammatism is also present
- slow speech
42
Primary Progressive Aphasia (PPA)
- diagnosis is made for those that demonstrate progressive aphasia in the absence of other cognitive/behavioral problems
- although aphasia may interfere with performance on memory and reasoning tests, the pt w/PPA will have no difficulty recalling day-to-day events or in problem solving
43
Language Characteristics of PPA
- high level of variability
- researchers usually break PPA down into the taxonomies of fluent vs. nonfluent
- kertesa, et al., (2003) examined 67 pts with PPA and found that most were fluent initially but became agrammatic and nonfluent later in the course of the disease
- some prefer to use the term “PPA” only for those individuals who are nonfluent and the term “semantic dementia” for those w/fluent aphasia
- our class will use fluent PPA and nonfluent PPA
44
Nonfluent PPA
- anomia
- effortful, agrammatic speech that lacks function words
- good comprehension w/the exception of more grammatically complicated sentences
- occasional oral apraxia, neurogenic stuttering, impaired repetition, dyslexia, dysgraphica, and sometimes mutism
45
Fluent PPA
- anomia
- poor comprehension
- normal articulation
- reading/spelling skills deteriorate
- paraphasias
- some suggest that fluent PPA is synonymous with semantic dementia
46
Neuropathology of PPA
- left perisylvian atrophy (most common pathology)
- nonfluent PPA: pathology is inferior frontal lobes and anterior temporal lobe
- fluent PPA: pathology is in the parietal lobe and temporal lobe
- pathology is greatest in the left hemisphere
47
Semantic Dementia
- pts progressively lose their semantic “conceptual” knowledge
- have difficulty understanding the meaning of words, objects, faces, nonverbal sounds, tastes, smells, etc
- nonsemantic aspects of cognition remain intact
- difficulty arises when attempting to attach meaning to perception
Preserved:
- visuoperceptual and spatial abilities
- nonverbal reasoning
- executive functions
- episodic memory
48
Language Deficits in Semantic Dementia
- typically complaint is “loss of words”
- verbally fluent w/intact grammar and syntax
- testing reveals anomia (greater for nouns than verbs)
- comprehension deficit for single words and difficulty providing definitions
- occasionally anomia is masked in conversation by circumlocutions
49
Neuropathology of Semantic Dementia
MRI scans reveal bilateral but asymmetric pathology of the temporal lobes w/left hemisphere more affected
