6.1 - Cortical Dementias Flashcards

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1
Q

What are the 2 Dementia Taxonomies?

A

Cortical Dementias

Subcortical Dementias

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2
Q

What are the 4 Cortical Dementias?

A

Alzheimer’s Disease

Lewy Body Dementia

Frontotemporal Lobar Dementia

Vascular Dementia

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3
Q

What are the 4 Subcortical Dementias?

A

Corticobasal Degeneration

Parkinson’s Dementia

Huntington’s Dementia

Progressive Supranuclear Palsy

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4
Q

What is the most common form of dementia?

A

Alzheimer’s Disease

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5
Q

How many Americans are affected by Alzheimer’s Disease?

A

More that 5 million

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6
Q

What causes a significant prevalence and incidence of Alzheimer’s Disease?

A

Age

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7
Q

What are 11 risk factors for Alzheimer’s Disease?

A

Aging

Genetics/Family History

Gender

Education

Down syndrome

Head trauma

Depression

Vascular risk

Sedentary lifestyle

High fat diets

Low vitamin intake

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8
Q

Who is more at risk for developing Alzheimer’s Disease: men or women?

A

Women

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9
Q

Who is more at risk for developing Alzheimer’s Disease: those with more or less education?

A

Less

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10
Q

What are three Characteristics of Alzheimer’s Disease?

A

Insidious onset

Progressive course

Heterogeneous

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11
Q

What are three Pathological Changes in Alzheimer’s Disease?

A

The cortex shrivels up

Shrinkage in the hippocampus is especially severe

Ventricles increase in size

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12
Q

The brain has _______ of neurons, each with an axon and many dendrites.

To stay healthy, neurons must communicate with ___________, carry out metabolism, and ___________.

AD disrupts all three of these essential jobs

A

Billions

Each other

Repair themselves

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13
Q

What two abnormal structures are seen abundantly in individuals with Alzheimer’s Disease?

A

Beta-Amyloid Plaques

Neurofibrillary Tangles

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14
Q

What are Beta-Amyloid Plaques?

2

A

Dense deposits of protein and cellular material

They accumulate outside and around nerve cells

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15
Q

What are Neurofibrillary Tangles?

A

Twisted fibers that build up inside the nerve cell

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16
Q

Neurons have an internal support structure partly made up of _______.

A

Microtubules

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17
Q

A protein called ______ helps stabilize microtubules.

A

Tau

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18
Q

In Alzheimer’s Disease, tau changes, causing microtubules to ______, and tau proteins __________ to form __________

A

Collapse

Clump together

Neurofibrillary tangles

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19
Q

What 4 Common Neuropathology are seen in Alzheimer’s Disease?

A

Neurofibrillary tangles

Amyloid plaques

Granuvacuolar degeneration

Accumulation of large, double membrane-bound bodies within certain neurons

Ventricular enlargement

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20
Q

Is there a single test to diagnosis Alzheimer’s Disease?

A

No

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21
Q

What 3 health arenas are used to diagnosis Alzheimer’s Disease?

A

Physical examinations

Psychiatric examinations

Neurologic examinations

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22
Q

How can examinations be definitively diagnosed?

A

Pathology examination of brain tissue during autopsy

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23
Q

What 2 symptoms are seen in the Early Stage of Alzheimer’s Disease?

A

Mild word retrieval

Mild decrease in
comprehension

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24
Q

What 3 symptoms are seen in the Middle Stage of Alzheimer’s Disease?

A

Frequent word retrieval deficits

Ungrammatical sentences

Reduced conversation

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25
Q

What 4 symptoms are seen in the Late Stages of Alzheimer’s Disease?

A

Non-functional reading/writing

Limited comprehension

Speech limited to single words

Bizarre and devoid of meaning

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26
Q

What are the 7 stages of Alzheimer’s Disease?

A

Stage 1: No Cognitive Decline

Stage 2: Very Mild Cognitive Decline

Stage 3: Mild Cognitive Decline

Stage 4: Moderate Cognitive Decline

Stage 5: Moderate-to-Severe Cognitive Decline

Stage 6: Severe Cognitive Decline

Stage 7: Very Severe Cognitive Decline

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27
Q

What are the 6 levels of Language Progression in Alzheimer’s Disease?

A

Reduced verbal fluency

Anomic aphasia

Transcortical sensory aphasia

Semantic aphasia

Wernicke’s aphasia

Unrecognizable output, mutism

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28
Q

With what other disorder does Lewy Body Dementia share common neuropathologic and neurogenetic features?

A

Parkinson’s disease

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29
Q

What 3 features distinguish Lewy Body Dementia from other neurodegenerative diseases?

A

Fluctuating attention

Visual hallucinations

Parkinsonism

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30
Q

Lewy Body Dementia presents in ___-___% of all autopsy confirmed dementias.

A

15-20%

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31
Q

Lewy Body Dementia is characterized by _______ and ______ disease.

A

Cortical

Subcortical

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32
Q

Lewy Body Dementia has less __________ involvement than Alzheimer’s Disease.

A

Medial temporal

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33
Q

Where are Lewy Bodies found in Lewy Body Dementia?

6

A

Substantia nigra

Temporal lobe

Frontal lobe

Parietal lobe

Limbic cortex

Brainstem

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34
Q

Lewy bodies can occur in ______ and ______ areas

A

Cortical

Subcortical

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35
Q

What are the 3 Common Presentations of Lewy Body Dementia?

A

Type 1

Type 2

Type 3

36
Q

What is seen in Type 1 Lewy Body Dementia?

2

A

Memory impairment or cognitive disorder similar to Alzheimer’s Disease

Over time two or more distinctive Lewy Body Dementia features appear

37
Q

What 9 symptoms can be used to distinguish Lewy Body Dementia from Alzheimer’s Disease?

A

Unpredictable levels of cognitive ability

Attention or alertness

Changes in walking or movement

Visual hallucinations

Sleep disorders

Hallucinations

Impaired in all areas of cognition and with higher variability compared to healthy controls.

Less severe memory impairment

More visuo-perceptual, visuospatial, and constructional disabilities

38
Q

What is seen in Type 2 Lewy Body Dementia?

2

A

Movement disorder that leads to Parkinson’s Disease

This then develop symptoms of dementia common in Lewy Body Dementia

39
Q

What is seen in Type 3 Lewy Body Dementia?

2

A

Neuropsychiatric symptoms

These then develop DLB

40
Q

What 3 Neuropsychiatric Symptoms are seen in Type 3 Lewy Body Dementia?

A

Allucinations

Behavioral problems

Difficulty with complex mental activities

41
Q

What are the 4 Core Clinical Features seen in Lewy Body Dementia?

A

Fluctuating Cognition

Neuropsychiatric Symptoms

Motor Features of Parkinsonism

Supportive Features

42
Q

How is Fluctuating Cognition seen in Lewy Body Dementia?

3

A

Fluctuating attention

Daytime drowsiness

Transient confusion

43
Q

What Neuropsychiatric Symptoms are seen in Lewy Body Dementia?

A

Recurrent, detailed and persistent hallucinations of animate + inanimate figures and scenes

44
Q

What Motor Features of Parkinsonism are seen in Lewy Body Dementia?

(6)

A

Rigidity

Bradykinesia

Hypophonic speech

Masked facies

Stooped posture

Shuffling gait

45
Q

What Supportive Features are seen in Lewy Body Dementia?

8

A

Repeated falls

Syncope

Transient loss of consciousness

Neuroleptic sensitivity

Systematized delusions

Hallucinations of other modalities

Depression

REM sleep behavior disorder

46
Q

What is the Course of Lewy Body Dementia?

2

A

Course is variable and patient dependent

Mean disease duration is 5-6 years (range 2-20)

47
Q

What are the Early Motor Signs in Lewy Body Dementia related to?

A

Nigrostriatal changes

48
Q

What are early Hallucinations related to?

A

Cortical and limbic involvement

49
Q

How frequent is Frontotemporal Dementia?

A

Very rare

50
Q

At what age does Frontotemporal Dementia tend to develop?

A

Between 35-75

51
Q

How long do patients usually live after a diagnosis of Frontotemporal Dementia?

A

2-10 years

52
Q

What is Frontotemporal Dementia linked to?

A

Chromosome 17 (FTDP-17)

53
Q

What is a common characteristic among Frontotemporal Dementia types?

A

“Cortical atrophy”

54
Q

What neuro structures are primarily affected in Frontotemporal Dementia?

(2)

A

Frontal lobes

Temporal lobes

55
Q

What symptoms are seen in Frontotemporal Dementia?

6

A

Reasoning

Social behavior

Personality

Speech/language

Movement

Memory

56
Q

What do the Neurological Changes in Frontotemporal Dementia depend on?

A

Whether damage is primarily on the left or right front of the brain

57
Q

What is the Neuropathology of Frontotemporal Dementia?

4

A

Accumulation of abnormal “tau” protein in nerve cells (Tau proteins also present in AD)

Gliosis

Vacuolation

Picks Bodies

58
Q

In Frontotemporal Dementia “tau” becomes ______ and aggregates into ______; this disrupts normal nerve cell processes and ultimately leads to __________.

A

Abnormal

Tangles

Cell death

59
Q

In Frontotemporal Dementia, where is the progressive loss of nerve cells seen?

A

In the frontal and temporal lobes

60
Q

What is Gliosis?

A

A form of tissue scarring in the CNS

61
Q

What is Vacuolation?

A

Process in which holes form in the outer layer of

the brain

62
Q

What are Picks Bodies?

A

Abnormal cell inclusions begin to form in the brain

63
Q

What are 8 Clinical Characteristics of Frontotemporal Dementia?

A

Uninhibited and socially inappropriate behaviors

Inappropriate sexual behavior

Loss of awareness of concern about changes in behavior

Major increase in appetite

Loss of speech/language

Compulsive and repetitive behaviors

Oral fixation

Memory loss

64
Q

What are 4 specific types of Frontotemporal Dementia?

A

Pick’s Disease

FIDP-17

Supranuclear Palsy

Corticobasal Degeneration (May also be classified as subcortical dementias)

65
Q

How does Frontotemporal Dementia differ from Alzheimer’s Disease?

(5)

A

Episodic memory loss = AD

Semantic memory loss = FTD

Early behavioral changes = FTD

Early personality changes = FTD

FTD tends to emerge one decade but the overall risk is not increased

66
Q

What are 3 Historical Definitions of Vascular Dementia?

A

Multi-infarct dementia

Vascular dementia

Vascular cognitive impairment

67
Q

How is Vascular Dementia clinically defined?

A

Memory decline

Two more cognitive deficits

68
Q

What can the “two more cognitive deficits” be that can be seen in Vascular Dementia?

(6)

A

Orientation

Attention

Language

Visuospatial functions

Executive functions

Motor control or praxis

69
Q

Those with Vascular Dementia typically have history of what 3 things?

A

HTN

Heart Disease

HTN + Heart Disease

70
Q

Those with Vascular Dementia have usually had multiple ______.

A

Strokes

71
Q

What kind of onset is seen in Vascular Dementia?

A

An abrupt onset of focal neurological signs

72
Q

What causes the Slow Stepwise Progression seen in Vascular Dementia?

A

Multiple strokes

73
Q

How does Vascular Dementia affect Personality and Intellect?

A

Preserved until late stages

74
Q

What are the 8 Pathology Locations in Vascular Dementia?

A

Bilateral large vessels (MCA, PCA) territories

Multiple lacunes

Thalamus

Frontal white matter

Basal forebrain

Basal ganglia

Internal capsule

Hippocampus

75
Q

What are the 4 Clinical Features in Vascular Dementia?

A

Sudden onset of neurological deficits

Stepwise deterioration

Fluctuating course

90% of cases have history of stroke

76
Q

What is Primary Progressive Aphasia?

A

A decline in one or more language functions

77
Q

At what age does Primary Progressive Aphasia tend to occur?

2

A

Can occur in individuals under the age of 65

Sometimes occurs in individuals as young as 40

78
Q

What causes Primary Progressive Aphasia?

A

Brain cell degeneration

79
Q

What is the course of Primary Progressive Aphasia?

4

A

Begins gradually and initially results in difficulty thinking of common words while speaking or writing

Results in limited verbal communication and reduction in comprehension

Memory, reasoning and visual perception are not affected by the disease in early stages

Mental abilities decline during disease progression

80
Q

In Primary Progressive Aphasia, isolated language deficits occur for typically for ___-___ years.

A

2-5

81
Q

Is Primary Progressive Aphasia more prevalent in males than females?

A

Females

82
Q

Many patients with Primary Progressive Aphasia progress to ___________.

A

Global deterioration

83
Q

What are the 5 Language Deficits seen in Primary Progressive Aphasia?

A

Increased difficulty thinking of words

Problems reading or writing

Reduced ability to understand speech

Decreased use of language

Problems in arithmetic and calculations

84
Q

What is the most common sign of Primary Progressive Aphasia?

A

Anomia

85
Q

What do the Language Deficits seen in Primary Progressive Aphasia correlate with?

A

Anomalies in anatomical regions