w4: Vascular dementia & Frontotemporal dementia

Question 1

vascular dementia definition

Answer 1

Vascular Dementia (VaD) is a type of dementia caused by reduced blood flow to the brain, leading to damage in brain regions responsible for cognitive functions.

Question 2

vascular cognitive impairments definition

Answer 2

Vascular Cognitive Impairment (VCI) is a broader term encompassing all forms of cognitive decline due to vascular brain damage, ranging from mild impairment to severe dementia.

Question 3

Vascular Dementia - DSM-5

Answer 3

A. the criteria are met for major or mild neurocognitive disorder

B.clinical features are consistent w vascular etiology suggested by:
- cognitive deficit onset related to one or more cerebrovascular event
- decline is complex in attention- including processing speed- and frontal-executive functions

C. evidence of presence of cerebrovascular disease from history, physical examination, neuroimaging

D. symptoms not better explained by other disease or systemic disorder

Question 4

Cerebrovascular Events

Answer 4

There is a huge number of blood vessels in your brain, and if something goes wrong it can lead to vascular dementia.

 vascular dementia is the result of cerebral vascular pathology

Question 5

Neuropathology Vascular Dementia

Answer 5

no definitive pathological criteria - diff from alzh

underlying cerebrovascular pathology is expansive- result of diffuse injury

difficult to define gold standard.

features- presence of:
- small or large vessle disease
- white matter lesions
- infrarcts
- lacunes

absence of:
confounding pathologies
- neurofibilary tangles, amyloid pl, lewy bodies

Question 6

Causes Vascular Dementia

Answer 6

Large Vessel Disease:
Stroke (ischemic or haemorrhagic).
Blockage in major arteries, causing infarcts.
Small Vessel Disease:
Chronic narrowing of small blood vessels.
Leads to lacunar infarcts and white matter lesions.
Mixed Pathology:
Combination of vascular pathology and Alzheimer’s disease processes.

Question 7

White Matter Lesions

Answer 7

= damage in the brain’s white matter

Key Features:
Varied and diffuse: not confined to specific regions.

White matter hyperintensities (leukoaraiosis):
Common in older adults, especially those with vascular risk factors (e.g., hypertension, diabetes).
Visible as hyperintensities on MRI scans.

Diffuse demyelination: Damage to the myelin sheath that insulates nerve fibres, impairing communication between brain regions.

Causes:
Chronic ischemia (lack of blood flow).

Stroke or lacunes (small, localised areas of tissue death due to blockages in tiny blood vessels).

Question 8

Stroke (Ischaemic)

Answer 8

occlusion of major cerebral blood vessle or a series of small cerebral blood vessles

due to:
- acute blockage due to embolism
- thickening of the vascular wall

Question 9

Haemorrhagic Stroke

Answer 9

rupture of large or small cerebral blood vessle

**Large vessel stroke or large vessel disease **
- One or more arteries supplying blood to the brain rupture or experience blockage.
- Effects are relatively focal.

**Small vessel stroke/ disease or lacunar stroke **
- Blockage of the cerebral microvessles – strongly associated with hypertension
- Effects are relative diffuse- because of the build up of dsamage in small vessels.
- = more often associated with vascular dementia.

Question 10

Vascular Dementia - the result of

Answer 10

1. extensive white matter lesions and lacunar infarcts due to small vessle disease = VaD has slow gradual onset, gradual decline

OR

2. one or more strokes to the main cebrebral arteries (large vessel disease)
   = VaD has an abrupt onset and stepwise decline

OR.
combination of 1 and 2

Question 11

Clinical Manifestation VaD

Answer 11

heterogeneity is the rule = all different
- contrast to alzh

neuropsychological manifestation is driven by extent of focal and diffuse vascular lesions- what is affected depends on what area and how much of it is affected.

Question 12

CLinical Manifestation

Middle cerebral artery

Answer 12

• hemiplegia -Paralysis of one side of the body
• aphasia - Impairment of language abilit
• hemianesthesia -Loss of sensation on one side of the body

Question 13

Clinical Manifestation

Anterior Cerebral Artery

Answer 13

• paraplegia -Paralysis of the lower half of the body
• abulia -lack of willpower or motivation
• executive dysfunctions -higher-order cognitive processes like planning
• personality changes - Alterations in behaviour, emotional responses

Question 14

clinical manifestations

posterior cerebral artery

Answer 14

• alexia with or without agraphia
  with:Inability to read and write
  without:Inability to read while retaining the ability to write
• visual agnosia - Inability to recognise or interpret visual information
• balint syndrome - inability to percieve multiple objects, inability to reach for objects
• prosopagnosia - recognise familiar faces

Question 15

People with vascular dementia can have impairments in:

Answer 15

• Learning or memory
• Executive functions.
• Attention
• Language
• Visuoperceptual function
• Psychomotor skills

Impairments in attention and executive functions are most salient.
- Impaired performance on test of:
- Planning
- Set-shifting
- Inhibition
- Selective attention
- Information processing

Question 16

other typical features VaD

Answer 16

Language
- Naming difficulties- worsen as vascular dementia progresses

Visuoperceptual skills:
- Impaired performance on visual organisation tasks

Psychomotor function:
- Impaired performance on test of psychomotor speed

Neuropsychiatric disturbances:
 not everyone will develop this but some might.
- depression
- anxiety
- apathy

Question 17

Alzh vs Vad

Answer 17

alzh:
onset: gradual
memory: early episodic loss
executive function: impaired later
attention: preserved early
imaging: atrophy in hippocampus
progression: stedy decline

Vad:
onset:absrupt/ stepwise
memory: mild impairment initiallt
executive function: early impairment
attention: early impairment
imaging: ischemic lesions, white matter changes
progression: stepwise decline

Question 18

VaD AND Alzh

Answer 18

Post-mortem findings: pathology of alzh disease is relatively common in people with vascular dementia.
 mixed dementia.
 plaques and tangles

when one or more strokes occurm less pathology is needed to cause dementia

Question 19

Risk Factor VaD

Answer 19

• age
• diabities
• hypertension
• cerebrovascular fragility
• genetics (vacular dementia = CADASIL gene)
• cardiovascular disease
• cardiac arrhythmis

= all because reduced blood flow and therefore oxygen to brain.

Question 20

Frontotemporal Dementia (FTD)

Answer 20

Frontotemporal Dementia (FTD), also called Frontotemporal Lobar Degeneration, is a progressive neurodegenerative disorder primarily affecting the frontal and/or temporal lobes.
Previously known as Pick’s Disease, first described by Arnold Pick in 1892.

Question 21

Key Features FTD

Answer 21

Common in younger individuals, often affecting those under 65 years.
Mean age of onset: 52–56 years.

More prevalent than Alzheimer’s in people under 60.

Caused by the inclusion of Pick bodies (tau-positive protein aggregates).

Question 22

Pathological Causes FTD

Answer 22

• Prick Bodies- intracellular inclusions composed of hyperphosphorylated tau protein.

Impact:
Disrupt normal neuronal functioning.
Commonly found in the frontal and temporal lobes, aligning with the areas of atrophy in FTD.
Distinct from tau pathology in Alzheimer’s, as Pick bodies are more localised.

Frontal Lobe Atrophy: Loss of executive functions: planning, decision-making, and judgement

Temporal Lobe Atrophy: Loss of semantic memory, Language production and fluency issues in non-fluent primary progressive aphasia.

Question 23

Frontotemporal Dementia- subtypes

Answer 23

Frontotemporal dementia:
- behavioural or dysexecutive variant
- primary progressive aphasia

primary progressive aphasia (further subtypes)
- semantic
- logopenic
- non-fluent / agrammatic

Question 24

Frontotemporal dementia- DSM-5

Answer 24

A. the critria are met for major or mild neurocognitive disorder

B. the disturbance has insidious onset and gradual progression.

C. either 1 or 2:
1. behavioural variant:
three or more of the following behavioural symptoms
- behavioural disihibition
- apathy or inertia
- loss of sympathy or empathy
- perseverative, stereotyped or compulsive/ ritualistic behavois
- hyperorality and dietary changes

prominent decline in social cognition and/ or executive abilities

2. language variant:
   - prominent decline in language ability, in the form of speech production, word finding, object naming, grammar, or word comprehension

D. Relation sparing of learning and memory and perceptual-motor function

E. The disturbance is not better explained by cerebovascular disease, another neurodegenerative disorder.

Question 25

Behavioural Variant - FTD

Answer 25

60% of all ppl w it will have behavioural variant.

Orbitofrontal Cortex Dysfunction:
Symptoms:
Disinhibition: Poor impulse control, socially inappropriate behaviour, distractibility.
Antisocial behaviour: Reduced agreeableness, inappropriate actions.
Restlessness, irritability, aggressiveness, and violent outbursts.

Medial Frontal Lobe and Anterior Cingulate Cortex Dysfunction:
Symptoms:
Apathy: Loss of motivation, often mistaken for depression (though depression is rare in bvFTD).
Loss of personal awareness: Reduced concern about hygiene and self-care.
Loss of social awareness: Inappropriate social interactions.
Akinetic mutism: Advanced stages may lead to complete loss of voluntary movement and speech.

Right Hemisphere Dysfunction:
Symptoms:
Severe behavioural changes:
Dramatic changes in beliefs, attitudes, or religious sentiments.
Excessive sentimentality in some cases.

Temporal Lobes Dysfunction:
Symptoms:
Reduced empathy: Emotional coldness, self-centred behaviour, and lack of concern for others.
Hyperorality and dietary changes:
Craving for sweet foods, overeating, and decreased satiety.
Weight gain as a consequence.
Features of Klüver-Bucy syndrome (advanced stages):
Hyperorality: Putting non-food objects in the mouth.
Oral exploratory behaviour

Frontal Lobes Dysfunction (General):
Symptoms:
Compulsive or ritualistic behaviours:
Repetitive actions, obsessional tendencies, and stereotyped behaviours.
Cognitive impairments:
Decline in executive abilities: Poor planning, organising, and decision-making.
Impaired social cognition: Difficulty understanding social norms and cues

Question 27

Behavioural Variant of FTD
- neuropsychological assessment

Answer 27

Impairments in executive functions and social cognition are most often present

memory and visuospatial functions are relatively spared

language impairments may be evident in later stages

Question 28

Behavioural Variant of Frontotemporal Dementia.
- Summary

Answer 28

Characterized by behavioural symptoms.
- Disinhibition
- Apathy
- Loss of sympathy or empathy
- Obsessive compulsive behavior
- Hyperorality and dietary changes.

Decline in social cognition and/ or executive abilities
Related to progressive neurodegeneration of the frontal cortex

Question 29

Primary Progressive Aphasia

Answer 29

neurodegenerative disorder characterised by the progressive deterioration of language abilities, including speaking, understanding, reading, and writing

primary progressive aphasia is characterised by an exlcusive impairment in language during the first 2 years of the disease
- typically report gradual decline in speech fluency, word finding, or object knowledge

Question 30

subtype of Primary Progressive Aphasia

Sematic dementia

Answer 30

Progressive loss of semantic knowledge or knowledge about people, objects, facts and words.
- Results associated agnosia

Presenting complaining involved language:
- Loss of memory of words
- Loss of meaning of words
- Result: progressive fleunt aphasia
- Speech is fluent
- Sematic paraphasia are frequently present
- Substitute phases (thing, stuff) are often used.

People with semantic dementia are aware of their expressive deficits.

People with semantic dementia are often unaware of their comprehension difficulties.

Repetition, prosody, syntax, and verb generation are preserved in people with semantic dementia.
 losing the meaning of words

Question 31

Semantic Dementia - assessment

Answer 31

most impaired in:
- category fluency test (name as many animals as possible)
- naming task
- generation of verbal definitions of words and pictured (early stages: loss of subcortinate knowledge)

Question 32

subtype of Primary Progressive Aphasia

Non-fluent / agrammatic

Answer 32

People present with changes in:
- Agrammatism
- Effortful speech

People report difficulties with
- Loss of function words- speech becomes telegraphic.

Fluency, specifically in:
- Prosody or melody of speech
- Lengths of utterances
- Rate of speech
- Effortfulness

Pronunciation or articulation

Word finding difficulties.
- More significant for verbs than for nouns.

Question 33

Logopogenic Primary progressive Aphasia:

Answer 33

Impairments in:
Single-word retrieval in conversation and on testing of naming to confrontation or to auditory cues

Impaired repetition of sentences and phrases

People with lopogenic primary progressive aphasia report.
Slowness of speech due to word finding difficulties.
Paraphasic errors which tend to be more phonological (substituting words with similar sounds) than semantic.

Question 34

Primary Progressive Aphasia

Answer 34

Behavioural changes are not present until later in the disease
Executive functions and visuospatial functioning are initially preserved but decline as disease progresses.
Explicit memory relatively intact.

Question 35

FTD differentiation from Alzh

Answer 35

alzh disease is also charcterised by:
- language impairments
- neuropsychiatric disturbances
- impairments in executive functions can be present

many cases of FTD diagnosed with alzh.
BUT: memory impairments more significant in alzh

Question 36

FTD- summary

Answer 36

most common dementia in individuals <65 years of age

behavioural variants most common- up to 40% develop the language variant

often misdiagnosed as neurpsychiatric disorder or alzh