Week 2 Flashcards
Chapter 19, chapter 20 (109 cards)
1
Q
What is Alzheimer’s disease?
A
A neurodegenerative disease and the most common cause of dementia, marked by progressive cognitive decline.
2
Q
What is dementia?
A
A clinical syndrome of significant cognitive decline that interferes with independent functioning.
3
Q
What percentage of dementia cases is due to Alzheimer’s?
A
Approximately 60–70%.
4
Q
What is the first and most prominent symptom of Alzheimer’s disease?
A
Gradually progressive memory loss.
5
Q
Name atypical symptoms of Alzheimer’s disease.
A
Language, visual, executive, or behavioral impairments.
6
Q
What neuropsychiatric symptoms are common in Alzheimer’s?
A
Depression, apathy, anxiety.
7
Q
What happens in the final stage of Alzheimer’s disease?
A
Complete cognitive loss and full dependence on care.
8
Q
What are the DSM-5 criteria for dementia (major neurocognitive disorder)?
A
Significant cognitive decline, interference with independence, not explained by delirium or another mental disorder.
9
Q
What defines probable Alzheimer’s dementia (NIA-AA criteria)?
A
Gradual onset, impairment in ≥2 cognitive domains, no alternative explanation.
10
Q
What additional evidence increases diagnostic certainty for Alzheimer’s?
A
Documented decline, neuroimaging/CSF biomarkers, or genetic mutations.
11
Q
What is the difference between MCI and dementia?
A
MCI has modest cognitive decline without loss of independence.
12
Q
What is amnestic MCI?
A
MCI with a prominent memory deficit, often a prodrome to Alzheimer’s dementia.
13
Q
What is the chance of progressing from MCI to dementia in 5–10 years?
A
About 50%, varying with age and other factors.
14
Q
How many people had dementia in the Netherlands in 2020?
A
Around 290,000.
15
Q
How many worldwide had dementia in 2020?
A
Over 50 million
16
Q
What is the global dementia diagnosis rate?
A
One every 3 seconds.
17
Q
What percentage of people aged 90+ have dementia?
A
Over 40%.
18
Q
How many people under 65 had dementia worldwide in 2020?
A
About 3.9 million.
19
Q
Who is more likely to develop dementia—men or women?
A
Women (1 in 3 vs. 1 in 7 men).
20
Q
What is the average survival after a dementia diagnosis?
A
7–10 years.
21
Q
What are the two hallmark brain pathologies of Alzheimer’s disease?
A
Amyloid-beta plaques (extracellular) and tau neurofibrillary tangles (intracellular).
22
Q
A
23
Q
What is the ultimate consequence of plaque and tangle accumulation in the brain?
A
Neurodegeneration and brain atrophy.
24
Q
How is Alzheimer’s disease definitively diagnosed?
A
Post-mortem neuropathological examination.
25
Which imaging techniques can assess Alzheimer’s pathology in vivo?
Structural (CT, MRI), molecular (amyloid/tau PET), and CSF biomarker analysis.
26
What does the amyloid cascade hypothesis propose?
Amyloid-beta accumulation triggers tau pathology, leading to neurodegeneration and dementia.
27
Which gene mutation supports the amyloid cascade hypothesis?
APP (Amyloid Precursor Protein) mutation.
28
Why might anti-amyloid drugs fail clinically despite reducing plaques?
Intervention may be too late or amyloid alone may not be sufficient to cause Alzheimer’s.
29
What does the vascular hypothesis of Alzheimer’s suggest?
Vascular risk factors (e.g., hypertension, diabetes) lead to brain hypoxia and promote amyloid overproduction and neurodegeneration.
30
How can cardiovascular health influence Alzheimer’s risk?
Improved cardiovascular health (e.g., exercise, less smoking) reduces Alzheimer's risk.
31
How do amyloid and vascular factors interact in Alzheimer’s disease?
They may have additive or synergistic effects on neurodegeneration and cognitive decline.
32
What are non-modifiable risk factors for Alzheimer’s disease?
Older age, female sex, genetic predisposition.
33
What is familial Alzheimer’s disease?
A rare, inherited form caused by mutations in APP, PSEN1, or PSEN2 genes (<1% of cases).
34
What gene is the strongest genetic risk factor for sporadic Alzheimer's disease?
APOE ε4 allele (risk ↑), APOE ε2 allele (protective).
35
Does having the APOE ε4 allele guarantee Alzheimer’s disease?
No, it increases risk but is not deterministic.
36
Name 12 modifiable risk factors linked to dementia.
Low education, hypertension, hearing loss, smoking, obesity, depression, inactivity, diabetes, small social network, alcohol misuse, brain injury, air pollution.
37
What proportion of dementia cases might be preventable through risk factor management?
Around 40%.
38
What is the typical clinical progression of Alzheimer’s disease?
A continuum from subtle cognitive changes → persistent cognitive decline → functional impairment → dementia syndrome.
39
What is the importance of informant interviews in Alzheimer's diagnosis?
Crucial, especially in dementia stage, because the patient may lack insight into their deficits.
40
What are the clinical stages of Alzheimer’s disease (based on CDR)?
1) Mild Cognitive Impairment (MCI)
2) Mild dementia
3) Moderate dementia
4) Severe dementia
41
What scale is commonly used to stage Alzheimer’s disease?
Clinical Dementia Rating (CDR) scale.
42
What are typical early cognitive symptoms of Alzheimer’s disease?
Episodic memory deficits, especially anterograde amnesia; word-finding difficulties.
43
What is typically impaired in mild dementia (CDR 1)?
Semantic memory, time/place orientation, planning, and executive functioning; affects instrumental ADLs.
44
What impairments occur in moderate dementia (CDR 2)?
Memory for distant past, visuoconstruction, apraxia; dependency in basic daily activities increases.
45
What characterizes severe dementia (CDR 3)?
Severe cognitive loss, communication difficulties, disorientation, motor issues (e.g., swallowing), incontinence, and high dependency.
46
What distinguishes Alzheimer’s neuropsychological profile from other dementias?
Early episodic memory deficits + semantic, language, working memory, and executive function decline, with progression to visuoconstruction and praxis deficits.
47
What is the typical pattern of brain atrophy in Alzheimer's seen on MRI?
Starts in medial temporal lobe (especially hippocampus), spreads to posterior/frontal cortex, then global atrophy.
48
What is the MTA scale used for?
Visually assessing hippocampal atrophy on MRI (Scheltens scale).
49
What EEG finding is typical in Alzheimer’s disease?
Slowed dominant alpha rhythm (reduced peak frequency).
50
What does FDG-PET measure and what is seen in Alzheimer’s?
Measures glucose metabolism; reduced activity especially in temporo-parietal regions.
51
What does amyloid PET scan show?
Amyloid-beta deposition, correlates well with pathology post-mortem, but not with symptom severity.
52
What CSF profile is typical for Alzheimer’s?
↓ Amyloid-beta, ↑ Total tau, ↑ Phosphorylated tau (P-tau).
53
When is CSF analysis preferred over amyloid PET?
It's quicker, cheaper, and more accessible, though it lacks spatial information.
54
Why is structural imaging (CT/MRI) still most used in clinical diagnosis?
To rule out other causes and identify characteristic atrophy, especially in older adults with MCI or dementia.
55
What distinguishes atypical variants of Alzheimer’s from the typical form?
Atypical variants have the same pathology (amyloid and tau) but start in different cortical regions, causing different initial symptoms.
56
What are the core symptoms of Posterior Cortical Atrophy (PCA)?
Prominent visuospatial deficits like impaired visuoconstruction and visual agnosia; memory is initially preserved.
57
Which brain regions are affected first in PCA?
Parietal and occipital lobes.
58
What characterizes the behavioural/dysexecutive variant of Alzheimer’s?
Early behavioral changes and impaired executive function; frontal lobe atrophy is most pronounced.
59
How does Logopenic Variant PPA (lvPPA) typically present?
With word-finding problems and impaired sentence repetition; atrophy mainly in the left temporal lobe.
60
What defines early-onset Alzheimer's disease?
Onset before age 65, often with atypical symptoms and faster progression.
61
Name common neuropsychiatric symptoms in Alzheimer’s disease.
Agitation, apathy, depression, anxiety, delusions, hallucinations, and sleep impairment.
62
Why are neuropsychiatric symptoms clinically important?
They affect quality of life, increase caregiver burden, and speed up disease progression.
63
What is the mechanism of cholinesterase inhibitors in Alzheimer’s?
They reduce acetylcholine breakdown, enhancing communication between neurons.
64
What are common side effects of cholinesterase inhibitors?
Rivastigmine and galantamine.
Nausea and vomiting.
65
What is memantine used for, and how does it work?
Used for moderate-to-severe AD; it increases glutamate release and may improve daily functioning.
66
What are the most accepted hypotheses about Alzheimer’s pathogenesis?
The amyloid cascade hypothesis and the vascular hypothesis.
67
What are the key risk and protective factors in Alzheimer's development?
Age is the biggest risk factor; modifiable lifestyle factors may influence onset and progression.
68
What are promising directions in Alzheimer’s research?
Early detection, better biomarkers, and lifestyle-based prevention strategies.
69
What is the most common form of frontotemporal dementia (FTD)?
Behavioural variant FTD (bvFTD)
70
What are the main clinical symptoms of FTD?
Changes in behaviour, language, and/or motor function
71
Why is early diagnosis of FTD difficult?
Slow onset, early age, symptom overlap with other dementias and psychiatric diseases
72
What is the average time from first FTD symptoms to diagnosis?
More than six years
73
What are the three diagnostic certainty levels for bvFTD?
Possible bvFTD, probable bvFTD, definite bvFTD
74
What symptoms are required for possible bvFTD?
Progressive deterioration of behaviour and/or cognition
75
What symptoms are typical in bvFTD?
Disinhibition, apathy, changes in eating, executive dysfunction, social withdrawal
76
What behaviours are considered disinhibition in bvFTD?
Socially inappropriate acts, loss of manners, impulsivity (e.g., gambling, risky driving)
77
What imaging is needed for probable bvFTD?
Neuroimaging evidence (MRI/CT atrophy or PET/SPECT hypometabolism) and functional decline
78
What confirms definite bvFTD?
Histopathology or known pathogenic mutation
79
What are the PPA diagnostic steps (Gorno-Tempini criteria)?
Step 1 – Language is main problem; Step 2 – Subtype classification (svPPA, nfvPPA, lvPPA)
80
Key feature of semantic variant PPA (svPPA)?
Fluent but empty speech, loss of word meaning, semantic paraphasias
81
Key feature of non-fluent variant PPA (nfvPPA)?
Effortful speech, agrammatism, preserved word understanding
82
Key feature of logopenic variant PPA (lvPPA)?
Word-finding difficulty, impaired sentence repetition, often Alzheimer's pathology
83
What percentage of ALS patients show FTD symptoms?
35–40% show executive/behavioural decline; 10–15% have comorbid FTD
84
What gene is commonly mutated in familial ALS/FTD?
C9orf72 repeat expansion
85
Which cognitive domain is most affected in ALS?
Executive function (also memory, visuospatial, language, social cognition)
86
What are common misdiagnoses in bvFTD?
Psychiatric disorders (depression, bipolar, OCD, schizophrenia, autism, ADHD).
87
What is bvFTD phenocopy syndrome?
Fulfills bvFTD criteria but with no progression, normal imaging, and mild/no impairments.
88
What percentage of FTD cases have a positive family history?
40–60%
89
What is the inheritance pattern in genetic FTD?
Autosomal dominant in 10–40%
90
What pattern of brain atrophy is typical in FTD?
Asymmetric frontal and temporal lobe atrophy.
91
Which brain regions are affected in bvFTD?
Prefrontal, orbitofrontal cortex, anterior cingulate, insula, and subcortical structures.
92
Where is atrophy located in SVPPA?
Left anterior/inferior temporal lobe.
93
What symptoms are seen in right-sided SVPPA?
Semantic deficits, behavioral changes (OCD), prosopagnosia.
94
Where is atrophy in nfvPPA?
Left inferior frontal gyrus, insula, premotor/supplementary motor areas.
95
Where is atrophy in lvPPA?
Posterior temporal and parietal areas (e.g., posterior cingulate, precuneus).
96
Is there a curative treatment for FTD?
no
97
What do current treatments for FTD focus on?
Managing behavioral and motor symptoms.
98
What are the key cognitive deficits in bvFTD?
Executive dysfunction, language issues, and impaired social cognition.
99
Which executive functions are commonly impaired in bvFTD?
Working memory, inhibition, flexibility, planning, abstraction.
100
Is the MMSE a good screening tool for bvFTD?
No, it lacks executive function assessment.
101
What kind of language disturbances can occur in bvFTD?
Stereotyped speech, echolalia, mutism, and reduced fluency.
102
What social cognition deficits are found in bvFTD?
Impaired emotion recognition, Theory of Mind, and moral reasoning.
103
Are memory impairments exclusionary for bvFTD diagnosis?
No, they can be present, even without executive dysfunction.
104
What are the hallmark symptoms of behavioural variant frontotemporal dementia (bvFTD)?
Marked personality and behavioural changes (e.g. apathy, disinhibition, rigid routines), lack of insight, inappropriate social behaviour, and normal MoCA despite cognitive impairments in executive function, memory, and social cognitio
105
What characterizes non-fluent variant primary progressive aphasia (nfvPPA)?
Effortful, agrammatic speech with preserved comprehension; speech apraxia and syntactic deficits; later progression to motor symptoms like limb dystonia, ideational and ideomotor apraxia, often linked to corticobasal syndrome
106
What are the core symptoms of semantic variant primary progressive aphasia (svPPA)?
Progressive word-finding and naming difficulties, impaired semantic fluency and word knowledge, intact phonology and grammar initially, later developing object and face recognition issues, surface dysgraphia, and behavioural changes.
107
What cognitive and behavioral changes, besides language impairments, can occur in different PPA variants as the disease progresses?
In svPPA, semantic loss affects sensory modalities, object and face recognition, and behavior similar to bvFTD. In nfvPPA, impairments develop in attention, memory, executive function, and praxis. In lvPPA, phonological loop deficits impact working memory and repetition. Language issues can mask other cognitive problems in assessments.
108
What is Pick’s disease and what historical contributions did Arnold Pick and Alois Alzheimer make to its understanding?
Pick’s disease, described by Arnold Pick, involves progressive language and behavioral decline with frontal/temporal atrophy. Alzheimer later found distinct neuropathology, including Pick bodies. It contributed to defining the frontotemporal dementia spectrum.
109
What are key diagnostic challenges and current limitations in managing frontotemporal dementia (FTD)?
FTD overlaps with other disorders, often delaying diagnosis. Memory issues may occur, contrary to past beliefs. Diagnosis relies on clinical interview, neuropsychology, imaging, and genetics. There are no curative treatments or diagnostic biomarkers yet.
