Dementia Flashcards

1
Q

What is dementia? (4)

A

o Dementia is the term used to describe the symptoms of a large group of illnesses which cause a progressive decline in a person’s functioning.
o It is a broad term used to describe a loss of memory, intellect, rationality, social skills and physical functioning.
o There are many types of dementia including Alzheimer’s disease, vascular dementia, frontotemporal dementia and Lewy body disease.
o Dementia can happen to anybody, but it is more common after the age of 65.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

How do we diagnose dementia?

A

Of dementia –clinically
Of disease process –on autopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the DSM5 criteria for diagnosing dementia? (9)

A

A diagnosis of Major NeurocognitiveDisorder (formerly Dementia) requires:
1. A significant cognitive decline from a previous level of performance in one or more of the following cognitive domains:
a. Complex attention (which includes sustained attention, divided attention, selective attention and information processing speed)
b. Executive functioning (which includes planning, decision making, working memory, response to feedback, inhibition and mental flexibility)
c. Learning and memory (which includes free recall, cued recall, recognition memory, semantic and autobiographical long term memory, and implicit learning)
d. Language (which includes object naming, word finding, fluency, grammar and syntax, and receptive language)
e. Perceptual motor function (which includes visual perception, visuoconstructional reasoning and perceptual-motor coordination)
f. Social cognition (which includes recognition of emotions, theory of mind, and insight)

  1. The cognitive deficits must be sufficient to interfere with independence in activities of daily living
    ◦If the cognitive impairments do not have a functional impact the person is said to have Mild NeurocognitiveDisorder or a Mild Cognitive Impairment
  2. The cognitive deficits must not be attributable to another medical disorder
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the incidence and prevalence of dementia? (8)

A

WORLDWIDE:
o Over 55 million people are living with dementia (in 2020)
o This number will almost double every 20 years
o These numbers are projected to reach 82 million by the year 2030 and 152 million by 2050
o The annual number of new cases of dementia is 9.9 million, approximately 30% higher than in 2010
o Every three seconds someone in the world develops dementia

AUSTRALIA:
o Dementia is thesecond leading cause of deathof Australians.
o In 2016 dementia became the leading cause of death of Australian women, surpassing heart disease
o In 2022, there is an estimated 487,500 Australians living with dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are 5 cortical dementias?

A

ALZHEIMER’S DISEASE (AD)
FRONTOTEMPORAL DEMENTIA (FTD)
Bv-FTD
Primary progressive aphasia
Semantic dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is AD (+ incidence)? (6)

A

Most common neurodegenerative disease
The majority of cases develop after 65 (although you can get an early onset Alzheimer’s Dementia which has a more rapid progression)
◦Affects 2% of people over 70
◦25% of people over 80
◦50% of people over 90
Slow and insidious progression (often lasting 10+ years)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the diagnostic criteria for AD? (9)

A

Meets clinical criteria for dementia and also has:
◦Insidious onset
◦Clear-cut history of worsening of cognition
◦The initial and most common cognitive deficits are evident on history and during testing in one of the following categories:
◦Amnesic category (most common): deficits in learning and recall of recently learned information with evidence of decline in at least one other cognitive domain
◦Language presentation (word finding difficulties most prominent)
◦Visuospatial presentation (spatial cognition deficit most prominent)
◦Executive dysfunction (impaired reasoning, judgment, problem solving most prominent)
◦No evidence of substantial cerebrovascular disease, or another dementia syndrome, non-neurological medical comorbidity or medication that could substantially affect cognition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is AD’s pathology? (10)

A

“Hallmark” lesions
◦Amyloid plaques
◦Neurofibrillary tangles

Begin in medial temporal lobes
◦Hippocampi
◦Entorrhinalcortex
Progress to parietal and frontal lobes

Neurochemistry:
o Loss of acetylcholine and nicotinic receptors
o Up to 70% loss of choline acetyltransferase (precursor to acetylcholine) in temporal and parietal lobes in AD
o Correlates with number of amyloid plaques and severity of cognitive dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are AD symptoms? (5)

A

Early symptoms vary from person to person
◦Typically memory problems
◦Word finding, spatial issues, impaired reasoning
Often noticed by family and friends not the individual (AD is associated with poor insight)
Usually cope relatively well in established environments early on (but may have difficulty coping with complexity, new situations, changed environments)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is AD’s presentation? (5)

A

o Repetitive in conversation
o Forget recent conversations
o Insidious onset of symptoms
o Deny or minimize deficits
o Difficulty with activities of daily living

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is AD’s progression?

A

Mild AD
Wandering and getting lost
Trouble handling money and paying bills
Repeating questions
Taking longer to complete normal daily tasks
Losing things or misplacing them in odd places
Personality and behavioural changes

Moderate AD
Increased memory loss and confusion
Difficulties recognising family and friends
Inability to learn new things
Self-care/dressing problems
Hallucinations, delusions, paranoia
Impulsive behaviour

Severe AD
Inability to communicate
Weight loss
Seizures
Skin Infection
Difficulty swallowing
Groaning/grunting
Increased sleeping
Incontinence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are 7 risk factors for AD?

A

Ø Age
Ø Family history
Ø Vascular disorder
Ø ApoEe4 allele
Ø Female
Ø History of head injury
Ø Negative relationship with education level

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is APOE? (6)

A

Ø Presence of the ApoEe4 allele of the apolipoprotein gene in 15 % of the population
Ø 60% of AD patients are e4 carriers
Ø E4 increases AD risk 20 fold
Ø Young, non-demented carriers of e4 show lower temporal, parietal and frontal metabolism
Ø Age of onset about 10 years younger
Ø Associated with more severe cholinergic deficit, increased amyloid beta burden, more neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is AD’s cognitive profile? (4)

A
  • Memory
    Anterograde episodic memory is impaired
    Characterised by poor encoding and rapid forgetting
  • Language
    Semantic loss
    Word finding difficulties
    Anomia
  • Visuospatial Impairment
    Apraxia
    Getting lost/driving issues/sewing/puzzles
    Visual agnosia (inability to recognise objects)
  • Executive Dysfunction
    Problem-solving
    Flexible thinking
    Abstraction Planning
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the neuropsychology of AD? (5)

A
  • Behavioural/Affective Changes
    Apathy Social withdrawal
    Agitation Anxiety and Depression
  • Anosagnosia/insight
    Lack of awareness of difficulties
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is frontotemporal dementia (FTD)? (+ onset and course) (5)

A

Umbrella term for neurodegenerative disorders that affect anterior parts of the brain
Clinically and pathologically distinct from AD
Onset typically between 50 –60 years
◦Earlier onset than AD
Insidious onset and rapid progression
◦5-7 year course from diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are FTD’s subtypes? (5)

A

Two broad categories of FTD and several subtypes
Behavioural Variant (bvFTD)
◦Previously known as Pick’s Disease

Primary Progressive Aphasia (i.e., Temporal Variant)
◦Progressive Non-fluent Aphasia
◦Semantic Dementia
◦Logopenic Progressive Aphasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is bvFTD’s neuropathology? (2)

A

Focal atrophy of the orbito-mesial frontal and anterior temporal lobes, extending over time to the hippocampus, DLPFC and basal ganglia
Tau or ubiquitin (TDP43) positive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is PPA’s neuropathology?

A

Progressive non-fluent Aphasia
◦Greater degeneration in the left posterior frontal cortex

Semantic Dementia
◦Polar and inferior temporal cortex atrophy

Logopenic PPA
◦Posterior aspect of the left superior and middle temporal gyriand parietal lobe involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are bvFTD’s symptoms? (6 bvral +5 cog)

A

Behavioural
◦Change in social conduct and personality
◦Lack of empathy, Reduced emotional warmth
◦Apathy; neglect of self-care
◦Disinhibition
◦Perseverative behaviours
◦Excessive eating, change in food preferences

Cognitive
◦Executive dysfunction
◦Reasoning, decision making, judgment impaired
◦Memory impairment variable (poor encoding rather than forgetting)
◦No visuospatial disorientation
◦Lack of insight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are frontal release signs? (3)

A

•Refers to the ‘release’ of primitive reflexes
•These reflexes are normally present at birth, but are inhibited after the early postnatal period
•‘Released’ from inhibition in the event of frontal lobe damage

22
Q

What are PPA’s symptoms? (3)

A

All PPA’s present with reductions in speech/language and/or verbal comprehension
They are typically not associated with personality and behavioural problems
Can be associated with depression and anxiety

23
Q

What are PPA’s neuropsychological features?

A

Progressive Non-fluent Aphasia
Speech is non-fluent and effortful
Comprehension remains intact
Dysarthria

Semantic Dementia
Loss of semantic knowledge
Conversation is fluent and non-dysarthric
Word finding difficulties
Impaired retrieval and comprehension of low frequency single words

Logopenic Primary Progressive Aphasia
Word finding difficulties
Impaired repetition and comprehension of sentences (single word is ok)
Semantic knowledge is intact (spared single-word comprehension and object knowledge)

24
Q

What is semantic dementia (+ neuropathology)? (4)

A

•Initial presentation bilateral atrophy of anterior temporal lobe, more severe on left than right
•About 25% atypical predominant right side presentation
•Typical neuropathology ubiquitin
•A profound loss of semantic memory: general knowledge of objects, words meanings, facts and people without any connection to a time or place

25
Q

What are 3 subcortical dementias?

A

VASCULAR DEMENTIA
PARKINSON’S DISEASE Parkinson’s plus
HUNTINGTON’S DISEASE

26
Q

What are NINDS –AIREN criteria? (8)

A

1.Dementia
2.Cerebrovascular disease
◦defined by the presence of focal signs on neuroloxicexamination (e.g. hemiparesis, lower facial weakness, sensory deficit, hemianopia, dysarthria)
◦Evidence of CVD by brain imaging
◦May be multiple large vessel infarcts, or a single strategically placed infarct, or multiple white matter lesions
3.A relationship between 1 and 2 manifested or inferred by the presence of:
a)Onset of dementia within 3 months following stroke
b)Abrupt deterioration in cognitive functions, or fluctuating stepwise progression of cognitive deficits

27
Q

What are DSM-5’s criteria for subcortical dementia? (4)

A
  • Clinical evidence of vascular cause: Either onset of cognitive deficits is temporally related to one or more cerebrovascular events or evidence for prominent decline in complex attention (including processing speed) and frontal-executive function; there should be evidence of cerebrovascular disease from history, physical examination, and/or neuroimaging
  • No Neuroimaging evidence of cerebrovascular disease required
  • Exclusionary criteria: Deficits are not better explained by another brain disease or systemic disorder
  • Criteria for ‘possible’ VCI: Clinical criteria are met but neuroimaging is not available and the temporal relationship of the neurocognitive syndrome with one or more cerebrovascular events is not established
28
Q

What is atherosclerosis?

A

Atherosclerosis=pathological thickening and hardening of arterial walls; build up of fatty deposits

29
Q

What are infarcts?

A

Ø 80% of strokes are ischaemic
Ø Blockage of the artery causing reduced blood flow
Ø Can be thrombotic or embolic
Embolus = A travelling particle in the arterial bloodstream originating from elsewhere
Thrombus = clump of blood particles and tissue overgrowth

30
Q

What are heamorrages? (+ 2 causes)

A

Blood leakage from arteries into the brain
Approx 20% of all strokes are haemorrhagic
Two primary mechanisms:
1. Weakening of vessel wall
◦ secondary to hypertension in most cases
2. Rupture associated with vascular abnormality
◦ Aneurysm
◦ Arteriovenous malformation
◦ Tumour
Symptoms often include sudden headache, nausea, vomiting

31
Q

What is the natural history of vascular cognitive impairment (VCI)? (5)

A

Age of onset = 65
More frequent in males
Second most common dementia (after AD)
Mixed etiology dementia is common
Prevalence is 1.5% in Western countries and approximately 2.2% in Japan.

32
Q

What are 7 risk factors of VCI?

A

Ø High blood pressure
Ø Smoking
Ø Diabetes
Ø High cholesterol
Ø History of mild warning strokes
Ø Evidence of disease in arteries elsewhere
Ø Heart rhythm abnormalities.

33
Q

What is VCI’s cognitive profile?

A

Ø Depends on the location and severity of vascular brain injury
Ø Differs by individual and by type of cerebrovascular disease.

Ø Cortical (large vessel) infarcts
Ø Lateralised changes
Ø Abrupt onset of cognitive symptoms
Ø Cortical neuropsychological syndrome (e.g. apraxia, agnosia)

Ø Subcortical (small vessel) infarcts
Ø Caused by small vessel disease
Ø White matter lesions
Ø Disrupt fronto-subcortical connections
Ø Insidious onset and slower progression

34
Q

What is parkinsonism?

A

Parkinsonism is a syndrome, not a disease entity. It is characterised by:
•Tremor
•Rigidity
•Akinesia/Bradykinesia
•Postural instability
•Also balance problems, masked facies, hypophonia and
•micrographia

Many disorders can cause parkinsonism:
•e.g., Huntington’s disease, Multiple System Atrophy, drug induced parkinsonism, Parkinson’s disease, Parkinson’s plus syndromes

35
Q

What is PD? (4)

A

Movement disturbance
Onset ~ 60 years
Males at higher risk
Course is variable

36
Q

What are 4 hallmark symptoms of PD?

A
  1. Tremor
    ◦ Resting tremor, regular and rhythmic
  2. Bradykinesia
    ◦ Slowness of voluntary movement
    ◦ Walking, talking & swallowing
  3. Muscle rigidity
    ◦ Cogwheel rigidity
  4. Postural instability
    ◦ Gait changes – shuffling, reduced arm swing
    ◦ Falls risk
37
Q

What are non-motor symptoms of PD? (5)

A

Anxiety and depression
Fatigue
Micrographia
◦ Very small handwriting
Microphonia
◦ Decreased volume of speech
Postural hypotension
◦ Drop in blood pressure on standing up

38
Q

What is PD’s cognitive profile? (9)

A

Fronto-subcortical profile
Impairments in:
◦Processing speed (prominent)
◦Attention
◦Executive functions
◦Working memory
◦Generativity
◦Flexibility
◦Set-shifting
◦Memory (encoding and retrieval)
◦Some visuospatial/visuoperceptual deficits

39
Q

What is PD dementia prevalance? (3)

A

Not all individuals with PD will develop dementia
Lifetime prevalence of dementia in PD is about 20 –30 %
Although 70% will develop dementia after 10 years of motor symptoms

40
Q

What is DLB? (essential + 4 core features + 9 supporting features)

A

Essential: progressive cognitive decline, which interferes with normal social/occupational functions, or usual daily activities.

Core Features (first 3 typically occur early in course)
Fluctuating levels of attention/alertness (hours to days)
Visual hallucinations (recurrent, well-formed, detailed)
REM sleep behavioural disorder (may precede cognitive decline)
Parkinsonian motor features (bradykinesia, resting tremor, rigidity)

Supporting Features:
Repeated falls Postural instability
Syncopy or other transient unresponsiveness
Hallucinations in other modalities
Systematised delusions
Sensitivity to antipsychotic agents
Severe autonomic dysfunction
Hypersomnia
Hyposmia
Apathy, anxiety, depression

41
Q

What is DLB’s onset, progression and prevalence?

A

Onset:
o typically between 50-70 years of age
o prior to age 65 is more common than in AD
Progression:
o Slow and insidious course
o Typicallymore rapid than AD, 5-7 years common
Prevalence:
o Reported as second or third most common
o Estimates 7-27% of dementias

42
Q

What is DLB’s cognitive profile? (5)

A

Neuropsychological Features:
Marked deficits in
◦Attention
◦Executive Functioning (incl. Working Memory)
◦Visuoconstructional deficits
Memory is typically okay early on
Language is not markedly affected (although deficits in verbal fluency and naming can occur)

43
Q

What is Progressive Supranuclear Palsy (PSP) 5 symptoms?

A

Unsteady gait with falls
Bradykinesia
Rigidity
Impaired eye movements
Speech disturbances

44
Q

What is PSP’s cognitive profile? (8)

A

Fronto subcortical dysfunction
Prominent slowing
Executive dysfunction
o Concrete
o Poor set-shifting
o Poor planning and organization
o Poor generativity
o Memory–not markedly impaired
o Encoding deficits (secondary to slowed processing and poor attention)

45
Q

What are 5 symptoms of cortico basal degeneration (CBD)?

A

Ø Rigidity
Ø Bradykinesia
Ø Apraxia
Ø Action tremor
Ø Alien limb

46
Q

What is CBD’s cognitive profile? (4)

A

Fronto subcortical pattern
Prominent ideomotor and ideational apraxia
Prominent impairments in:
o Executive function
o Complex attention
o Verbal fluency

47
Q

What is HD? (3)

A

Rare, autosomal dominant neurodegenerative disorder
Hereditary
Huntington’s chorea: Involuntary, spasmodic, often tortuous movements that become disabling

48
Q

What is the onset, progression and prevalence of HD?

A

Onset:
o Typically develops between 40-50 years of age
o Juvenile cases can occur before age 20
Progression:
o 10 to 20 years
Prevalence:
o About 1-2% of dementias
o Varies greatly between countries and regions within countries

49
Q

What is HD’s clinical presentation? (6)

A
  • Cognitive impairments
    ◦Learning new motor skills
    ◦Executive dysfunction
  • Psychiatric symptoms
    ◦Personality change
    ◦Irritability
    ◦Depression
  • Motor dysfunction
    o Chorea
50
Q

What are the diagnostic criteria for possible AD? (7)

A

Possible AD diagnosed if there is:
◦Atypical course
◦Meets core clinical criteria regarding the nature of cognitive difficulties, but either has sudden onset, or insufficient historical detail or objective cognitive documentation of progressive decline
◦Etiologically mixed presentation
◦Meets core clinical criteria, but has evidence of
a) concomitant cerebrovascular disease,
b) features of Lewy Body disease, or
c) evidence of another neurological disease or non neurological medical condition or medication that could have an impact on cognition