Cognitive Disorders- Dementia Types/Forms Flashcards

1
Q

Alzheimer’s Disease or Dementia of the Alzheirmer’s Type (DAT)

A
  • most common etiology of dementia
  • typically impacts those 65+ years
  • can be difficult to diagnose
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2
Q

DAT diagnostic criteria

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”
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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
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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
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5
Q

Neuropathology of DAT

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
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6
Q

Motor impairments associated with 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
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7
Q

Microscopic Changes with 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

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8
Q

Neuritic Plaques with DAT

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
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9
Q

Neurofibrillary Tangles and DAT

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
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10
Q

DAT and 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
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11
Q

Granulovacuolar degeneration and DAT

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
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12
Q

DAT 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
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13
Q

Preclinical DAT

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
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14
Q

Predictors of disease progression in DAT

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

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15
Q

Cognitive/communication effects of DAT

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
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16
Q

DAT early stage 1

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
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17
Q

DAT early stage 2

A
  • basic ADLs: can complete basic ADLs
  • MMSE: 16-24 points (Mini mental status exam)
  • 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

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18
Q

DAT Middle Stage 1

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
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19
Q

DAT Middle Stage 2

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

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20
Q

DAT 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 of 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
Q

Diagnostic criteria for possible VaD

A

possible VaD:

  • dementia
  • CVD
  • onset of dementia w/in 3 months of stroke
  • abrupt deterioration in cognitive functions
26
Q

Diagnostic criteria for probable VaD

A

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
Q

VaD impact on cognition

A
  • 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
Q

VaD impact on communication

A
  • 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
Q

Mixed Dementia

A
  • presence of both AD pathology and vascular disease
  • produces earlier and more severe cognitive impairment
  • shorter length of survival
30
Q

Lewy Body Dementia

A
  • 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
Q

Diagnostic criteria for lewy body dementia 1

A
  • 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
Q

Diagnostic criteria for lewy body dementia 2

A
  • 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
Q

Diagnostic criteria for lewy body dementia 3

A
  • 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
Q

Diagnostic criteria for lewy body dementia 4

A
  • 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
Q

Diagnostic criteria for lewy body dementia 5

A
  • 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
Q

LBD vs. PD

A
  • 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
Q

Cognition and communication LBD

A
  • 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
Q

Frontotemporal Dementia

A
  • 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
Q

Pick’s Disease

A
  • 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
Q

Cognitive changes in Pick’s Disease 1

A
  • 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
Q

Cognitive changes in Pick’s Disease 2

A
  • 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
Q

Primary progressive aphasia (PPA)

A
  • 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
Q

Language characteristics of PPA

A
  • 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
Q

Nonfluent PPA

A
  • 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
Q

Fluent PPA

A
  • anomia
  • poor comprehension
  • normal articulation
  • reading/spelling skills deteriorate
  • paraphasias
  • some suggest that fluent PPA is synonymous with semantic dementia
46
Q

Neuropathology of PPA

A
  • 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
Q

Semantic Dementia

A
  • 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
Q

Language deficits in semantic dementia

A
  • 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
Q

Neuropathology of semantic dementia

A
  • MRI scans reveal bilateral but asymmetric pathology of the temporal lobes w/left hemisphere more affected