6.1 - Cortical Dementias Flashcards
What are the 2 Dementia Taxonomies?
Cortical Dementias
Subcortical Dementias
What are the 4 Cortical Dementias?
Alzheimer’s Disease
Lewy Body Dementia
Frontotemporal Lobar Dementia
Vascular Dementia
What are the 4 Subcortical Dementias?
Corticobasal Degeneration
Parkinson’s Dementia
Huntington’s Dementia
Progressive Supranuclear Palsy
What is the most common form of dementia?
Alzheimer’s Disease
How many Americans are affected by Alzheimer’s Disease?
More that 5 million
What causes a significant prevalence and incidence of Alzheimer’s Disease?
Age
What are 11 risk factors for Alzheimer’s Disease?
Aging
Genetics/Family History
Gender
Education
Down syndrome
Head trauma
Depression
Vascular risk
Sedentary lifestyle
High fat diets
Low vitamin intake
Who is more at risk for developing Alzheimer’s Disease: men or women?
Women
Who is more at risk for developing Alzheimer’s Disease: those with more or less education?
Less
What are three Characteristics of Alzheimer’s Disease?
Insidious onset
Progressive course
Heterogeneous
What are three Pathological Changes in Alzheimer’s Disease?
The cortex shrivels up
Shrinkage in the hippocampus is especially severe
Ventricles increase in size
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
Billions
Each other
Repair themselves
What two abnormal structures are seen abundantly in individuals with Alzheimer’s Disease?
Beta-Amyloid Plaques
Neurofibrillary Tangles
What are Beta-Amyloid Plaques?
2
Dense deposits of protein and cellular material
They accumulate outside and around nerve cells
What are Neurofibrillary Tangles?
Twisted fibers that build up inside the nerve cell
Neurons have an internal support structure partly made up of _______.
Microtubules
A protein called ______ helps stabilize microtubules.
Tau
In Alzheimer’s Disease, tau changes, causing microtubules to ______, and tau proteins __________ to form __________
Collapse
Clump together
Neurofibrillary tangles
What 4 Common Neuropathology are seen in Alzheimer’s Disease?
Neurofibrillary tangles
Amyloid plaques
Granuvacuolar degeneration
Accumulation of large, double membrane-bound bodies within certain neurons
Ventricular enlargement
Is there a single test to diagnosis Alzheimer’s Disease?
No
What 3 health arenas are used to diagnosis Alzheimer’s Disease?
Physical examinations
Psychiatric examinations
Neurologic examinations
How can examinations be definitively diagnosed?
Pathology examination of brain tissue during autopsy
What 2 symptoms are seen in the Early Stage of Alzheimer’s Disease?
Mild word retrieval
Mild decrease in
comprehension
What 3 symptoms are seen in the Middle Stage of Alzheimer’s Disease?
Frequent word retrieval deficits
Ungrammatical sentences
Reduced conversation
What 4 symptoms are seen in the Late Stages of Alzheimer’s Disease?
Non-functional reading/writing
Limited comprehension
Speech limited to single words
Bizarre and devoid of meaning
What are the 7 stages of Alzheimer’s Disease?
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
What are the 6 levels of Language Progression in Alzheimer’s Disease?
Reduced verbal fluency
Anomic aphasia
Transcortical sensory aphasia
Semantic aphasia
Wernicke’s aphasia
Unrecognizable output, mutism
With what other disorder does Lewy Body Dementia share common neuropathologic and neurogenetic features?
Parkinson’s disease
What 3 features distinguish Lewy Body Dementia from other neurodegenerative diseases?
Fluctuating attention
Visual hallucinations
Parkinsonism
Lewy Body Dementia presents in ___-___% of all autopsy confirmed dementias.
15-20%
Lewy Body Dementia is characterized by _______ and ______ disease.
Cortical
Subcortical
Lewy Body Dementia has less __________ involvement than Alzheimer’s Disease.
Medial temporal
Where are Lewy Bodies found in Lewy Body Dementia?
6
Substantia nigra
Temporal lobe
Frontal lobe
Parietal lobe
Limbic cortex
Brainstem
Lewy bodies can occur in ______ and ______ areas
Cortical
Subcortical
What are the 3 Common Presentations of Lewy Body Dementia?
Type 1
Type 2
Type 3
What is seen in Type 1 Lewy Body Dementia?
2
Memory impairment or cognitive disorder similar to Alzheimer’s Disease
Over time two or more distinctive Lewy Body Dementia features appear
What 9 symptoms can be used to distinguish Lewy Body Dementia from Alzheimer’s Disease?
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
What is seen in Type 2 Lewy Body Dementia?
2
Movement disorder that leads to Parkinson’s Disease
This then develop symptoms of dementia common in Lewy Body Dementia
What is seen in Type 3 Lewy Body Dementia?
2
Neuropsychiatric symptoms
These then develop DLB
What 3 Neuropsychiatric Symptoms are seen in Type 3 Lewy Body Dementia?
Allucinations
Behavioral problems
Difficulty with complex mental activities
What are the 4 Core Clinical Features seen in Lewy Body Dementia?
Fluctuating Cognition
Neuropsychiatric Symptoms
Motor Features of Parkinsonism
Supportive Features
How is Fluctuating Cognition seen in Lewy Body Dementia?
3
Fluctuating attention
Daytime drowsiness
Transient confusion
What Neuropsychiatric Symptoms are seen in Lewy Body Dementia?
Recurrent, detailed and persistent hallucinations of animate + inanimate figures and scenes
What Motor Features of Parkinsonism are seen in Lewy Body Dementia?
(6)
Rigidity
Bradykinesia
Hypophonic speech
Masked facies
Stooped posture
Shuffling gait
What Supportive Features are seen in Lewy Body Dementia?
8
Repeated falls
Syncope
Transient loss of consciousness
Neuroleptic sensitivity
Systematized delusions
Hallucinations of other modalities
Depression
REM sleep behavior disorder
What is the Course of Lewy Body Dementia?
2
Course is variable and patient dependent
Mean disease duration is 5-6 years (range 2-20)
What are the Early Motor Signs in Lewy Body Dementia related to?
Nigrostriatal changes
What are early Hallucinations related to?
Cortical and limbic involvement
How frequent is Frontotemporal Dementia?
Very rare
At what age does Frontotemporal Dementia tend to develop?
Between 35-75
How long do patients usually live after a diagnosis of Frontotemporal Dementia?
2-10 years
What is Frontotemporal Dementia linked to?
Chromosome 17 (FTDP-17)
What is a common characteristic among Frontotemporal Dementia types?
“Cortical atrophy”
What neuro structures are primarily affected in Frontotemporal Dementia?
(2)
Frontal lobes
Temporal lobes
What symptoms are seen in Frontotemporal Dementia?
6
Reasoning
Social behavior
Personality
Speech/language
Movement
Memory
What do the Neurological Changes in Frontotemporal Dementia depend on?
Whether damage is primarily on the left or right front of the brain
What is the Neuropathology of Frontotemporal Dementia?
4
Accumulation of abnormal “tau” protein in nerve cells (Tau proteins also present in AD)
Gliosis
Vacuolation
Picks Bodies
In Frontotemporal Dementia “tau” becomes ______ and aggregates into ______; this disrupts normal nerve cell processes and ultimately leads to __________.
Abnormal
Tangles
Cell death
In Frontotemporal Dementia, where is the progressive loss of nerve cells seen?
In the frontal and temporal lobes
What is Gliosis?
A form of tissue scarring in the CNS
What is Vacuolation?
Process in which holes form in the outer layer of
the brain
What are Picks Bodies?
Abnormal cell inclusions begin to form in the brain
What are 8 Clinical Characteristics of Frontotemporal Dementia?
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
What are 4 specific types of Frontotemporal Dementia?
Pick’s Disease
FIDP-17
Supranuclear Palsy
Corticobasal Degeneration (May also be classified as subcortical dementias)
How does Frontotemporal Dementia differ from Alzheimer’s Disease?
(5)
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
What are 3 Historical Definitions of Vascular Dementia?
Multi-infarct dementia
Vascular dementia
Vascular cognitive impairment
How is Vascular Dementia clinically defined?
Memory decline
Two more cognitive deficits
What can the “two more cognitive deficits” be that can be seen in Vascular Dementia?
(6)
Orientation
Attention
Language
Visuospatial functions
Executive functions
Motor control or praxis
Those with Vascular Dementia typically have history of what 3 things?
HTN
Heart Disease
HTN + Heart Disease
Those with Vascular Dementia have usually had multiple ______.
Strokes
What kind of onset is seen in Vascular Dementia?
An abrupt onset of focal neurological signs
What causes the Slow Stepwise Progression seen in Vascular Dementia?
Multiple strokes
How does Vascular Dementia affect Personality and Intellect?
Preserved until late stages
What are the 8 Pathology Locations in Vascular Dementia?
Bilateral large vessels (MCA, PCA) territories
Multiple lacunes
Thalamus
Frontal white matter
Basal forebrain
Basal ganglia
Internal capsule
Hippocampus
What are the 4 Clinical Features in Vascular Dementia?
Sudden onset of neurological deficits
Stepwise deterioration
Fluctuating course
90% of cases have history of stroke
What is Primary Progressive Aphasia?
A decline in one or more language functions
At what age does Primary Progressive Aphasia tend to occur?
2
Can occur in individuals under the age of 65
Sometimes occurs in individuals as young as 40
What causes Primary Progressive Aphasia?
Brain cell degeneration
What is the course of Primary Progressive Aphasia?
4
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
In Primary Progressive Aphasia, isolated language deficits occur for typically for ___-___ years.
2-5
Is Primary Progressive Aphasia more prevalent in males than females?
Females
Many patients with Primary Progressive Aphasia progress to ___________.
Global deterioration
What are the 5 Language Deficits seen in Primary Progressive Aphasia?
Increased difficulty thinking of words
Problems reading or writing
Reduced ability to understand speech
Decreased use of language
Problems in arithmetic and calculations
What is the most common sign of Primary Progressive Aphasia?
Anomia
What do the Language Deficits seen in Primary Progressive Aphasia correlate with?
Anomalies in anatomical regions
