Neurocognitive Degenerative Disorder Flashcards
What is the most common cause of dementia?
Alzheimer’s disease
What are the domains of cognitive decline?
Complex attention Execute function Learning and memory Language Perceptual motor Social cognition
What is the diagnosis of AD per DSM-5?
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
What are the sx of AD?
ABCs
ADLs
Behavior
Cognition
What are other potential causes of memory loss?
Vit B12 and folate deficiency, hypothyroidism, syphilis, stroke
What is a way to diagnose AD?
Autopsy of brain
What are ways to diagnose AD when they are alive?
Neuropsychiatric testing
MRI, CT, SPECT, PET
What are the biomarkers for AD?
A(beta)1-42 decreased level in CSF when they accumulate into insoluble plaques
Tau increased level when released from damage and dying neurons
What are the causes of AD?
Alterations in brain structure Amyloid plaques and neurofibrillary tangles Cholinergic hypothesis Amyloid hypothesis Genetics
How is the brain altered in AD?
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
What are the hallmark of lesions in AD?
Amyloid plaques and neurofibrillary tangles
What are neurofibrillary tangles (NFT)?
Paired helical filaments that aggregate in bundles
Formed from tau proteins
What is a tau protein?
Structural support (microtubules/cytoskeleton) Abnormally phosphorylated tau leads to microtubular collapse and eventual cell death
What are amyloid plaques?
Extracellular lesions found in the brain and cerebral vasculature
Comprised of a core of beta amyloid and rings of abnormal neurites
What is the cholinergic hypothesis in AD?
Idea that cholinergic cell less was the source of memory and cognitive impairment in AD
Cholinergic pathways are profoundly affected in AD
What is the amyloid hypothesis of AD?
Misprocessing of amyloid precursor protein
Deposition of insoluble beta amyloid plaques (A-beta)
Leads to: synaptic failure, neuronal failure, hyperphosphorylated tau protein, neuronal apoptosis
What genes are linked strongly with early-onset AD?
Autosomal dominant APP mutations Presenillin 1 (PSEN1) mutations Presenillin 2 (PSEN2) mutations
What genes are linked with late onset AD?
Apolipoprotein E-4 (APOE4) allele
What causes a normal amyloid plaque?
when amyloid precursor protein is converted to A(beta)40 via alpha-secretase
What causes a abnormal amyloid plaque?
When amyloid precursor protein is converted to A(beta)42via beta- and gamma-secretase
How is frontotemporal lobar degeneration diagnosed?
The patient meets criteria for mild or major neurocognitive disorder
What is the onset of FTD?
Insidious and progression is gradual
What are the two types of FTD?
Behavioral variant
Language variant
What is FTD behavioral variant?
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
What is FTD language variant?
Prominent decline in language ability (speech production, word finding, object naming, grammar, word comprehension)
What is Lew Body disease?
The patient meets criteria for mild or major neurocognitive disorder
What is the onset of Lewy Body Disease?
Insidious and progression is gradual
What is a probable diagnosis of lewy body disease?
Two core features or one of each a core and suggestive feature is present
What is a possible diagnosis of lewy body disease?
One core feature or one or more suggestive features are present
What are the core features of lewy body disease?
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]
What are the suggestive features of lewy body disease?
Meets criteria for rapid eye movement sleep behavior disorder
Severe neuroleptic sensitivity
What is vascular disease?
The patient meats criteria for mild or major neurocognitive disorder
What are the clinical features for vascular disease?
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
How can you find evidence of vascular disease?
Present on history, exam and/or neuroimaging and is sufficient enough to cause the cognitive defects
What is the onset of dementia associated with Parkinson’s disease?
Insidious and progression is gradual
What is a probably diagnosis of dementia associated with PD?
Has BOTH:
No evidence of mixed etiology
PD clearly precedes the onset of cognitive defects
What is a possible diagnosis of dementia associated with PD?
Has one or the other but not both:
No evidence of mixed etiology
PD clearly precedes the onset of cognitive defects