Neurocognitive Degenerative Disorder

Question 1

What is the most common cause of dementia?

Answer 1

Alzheimer’s disease

Question 2

What are the domains of cognitive decline?

Answer 2

Complex attention
Execute function
Learning and memory
Language
Perceptual motor
Social cognition

Question 3

What is the diagnosis of AD per DSM-5?

Answer 3

The patient meets criteria for mild/major neurocognitive disorder
Onset is insidious and there is a gradual progression of impairment in one or more cognitive domains. At least 2 of the cognitive domains are impaired in order to classify as major here

Question 4

What are the sx of AD?

Answer 4

ABCs
ADLs
Behavior
Cognition

Question 5

What are other potential causes of memory loss?

Answer 5

Vit B12 and folate deficiency, hypothyroidism, syphilis, stroke

Question 6

What is a way to diagnose AD?

Answer 6

Autopsy of brain

Question 7

What are ways to diagnose AD when they are alive?

Answer 7

Neuropsychiatric testing

MRI, CT, SPECT, PET

Question 8

What are the biomarkers for AD?

Answer 8

A(beta)1-42 decreased level in CSF when they accumulate into insoluble plaques
Tau increased level when released from damage and dying neurons

Question 9

What are the causes of AD?

Answer 9

Alterations in brain structure
Amyloid plaques and neurofibrillary tangles
Cholinergic hypothesis
Amyloid hypothesis
Genetics

Question 10

How is the brain altered in AD?

Answer 10

AD destroys neurons in the cortex and other structures of the brain, particularly the basal forebrain, amygdala, hippocampus, and cerebral cortex. These areas are responsible for higher learning, memory, reasoning, behavior, and emotional control

Question 11

What are the hallmark of lesions in AD?

Answer 11

Amyloid plaques and neurofibrillary tangles

Question 12

What are neurofibrillary tangles (NFT)?

Answer 12

Paired helical filaments that aggregate in bundles

Formed from tau proteins

Question 13

What is a tau protein?

Answer 13

Structural support (microtubules/cytoskeleton)
Abnormally phosphorylated tau leads to microtubular collapse and eventual cell death

Question 14

What are amyloid plaques?

Answer 14

Extracellular lesions found in the brain and cerebral vasculature
Comprised of a core of beta amyloid and rings of abnormal neurites

Question 15

What is the cholinergic hypothesis in AD?

Answer 15

Idea that cholinergic cell less was the source of memory and cognitive impairment in AD
Cholinergic pathways are profoundly affected in AD

Question 16

What is the amyloid hypothesis of AD?

Answer 16

Misprocessing of amyloid precursor protein
Deposition of insoluble beta amyloid plaques (A-beta)
Leads to: synaptic failure, neuronal failure, hyperphosphorylated tau protein, neuronal apoptosis

Question 17

What genes are linked strongly with early-onset AD?

Answer 17

Autosomal dominant
APP mutations
Presenillin 1 (PSEN1) mutations
Presenillin 2 (PSEN2) mutations

Question 18

What genes are linked with late onset AD?

Answer 18

Apolipoprotein E-4 (APOE4) allele

Question 19

What causes a normal amyloid plaque?

Answer 19

when amyloid precursor protein is converted to A(beta)40 via alpha-secretase

Question 20

What causes a abnormal amyloid plaque?

Answer 20

When amyloid precursor protein is converted to A(beta)42via beta- and gamma-secretase

Question 21

How is frontotemporal lobar degeneration diagnosed?

Answer 21

The patient meets criteria for mild or major neurocognitive disorder

Question 22

What is the onset of FTD?

Answer 22

Insidious and progression is gradual

Question 23

What are the two types of FTD?

Answer 23

Behavioral variant

Language variant

Question 24

What is FTD behavioral variant?

Answer 24

3 or more of the following sx are present and there is a prominent decline in social cognition and/or executive abilities:
Behavioral disinhibition
Apathy
Loss of sympathy or empathy
Preservative, stereotyped or compulsive/ritualistic behavior
Hyperorality and dietary changes

Question 25

What is FTD language variant?

Answer 25

Prominent decline in language ability (speech production, word finding, object naming, grammar, word comprehension)

Question 26

What is Lew Body disease?

Answer 26

The patient meets criteria for mild or major neurocognitive disorder

Question 27

What is the onset of Lewy Body Disease?

Answer 27

Insidious and progression is gradual

Question 28

What is a probable diagnosis of lewy body disease?

Answer 28

Two core features or one of each a core and suggestive feature is present

Question 29

What is a possible diagnosis of lewy body disease?

Answer 29

One core feature or one or more suggestive features are present

Question 30

What are the core features of lewy body disease?

Answer 30

Fluctuating cognition with pronounced variation in attention and alertness
Recurrent visual hallucinations that are well-formed and detailed
Spontaneous features of parkinsonism, onset is subsequent to the development of cognitive decline [sx onset for the two conditions (LBD and parkinsonism) typically occur w/in 12 months of each other]

Question 31

What are the suggestive features of lewy body disease?

Answer 31

Meets criteria for rapid eye movement sleep behavior disorder
Severe neuroleptic sensitivity

Question 32

What is vascular disease?

Answer 32

The patient meats criteria for mild or major neurocognitive disorder

Question 33

What are the clinical features for vascular disease?

Answer 33

Onset of cognitive deficits are temporally associated to one or more cerebrovascular events
Evidence for decline is prominent in complex attention and frontal-executive function

Question 34

How can you find evidence of vascular disease?

Answer 34

Present on history, exam and/or neuroimaging and is sufficient enough to cause the cognitive defects

Question 35

What is the onset of dementia associated with Parkinson’s disease?

Answer 35

Insidious and progression is gradual

Question 36

What is a probably diagnosis of dementia associated with PD?

Answer 36

Has BOTH:
No evidence of mixed etiology
PD clearly precedes the onset of cognitive defects

Question 37

What is a possible diagnosis of dementia associated with PD?

Answer 37

Has one or the other but not both:
No evidence of mixed etiology
PD clearly precedes the onset of cognitive defects