Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

2. Geriatric Psychiatry + Neuroanatomy > Major/Mild Frontotemporal Neurocognitive Disorder > Flashcards

Major/Mild Frontotemporal Neurocognitive Disorder Flashcards

1
Q

criterion A for M/M NCD due to Frontotemporal NCD

A

criteria are met for major or mild NCD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

criterion B for M/M NCD due to Frontotemporal NCD

A

disturbance has INSIDIOUS onset and GRADUAL progression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the two variants of M/M NCD due to Frontotemporal NCD

A

behavioural variant

language variant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

criterion C for M/M NCD due to Frontotemporal NCD

A

either (1) or (2)

  1. behavioural variant–> THREE or more of the following symptoms:
    -behavioural disinhibition
    -apathy or inertia
    -loss of sympathy or empathy
    -perseverative, stereotyped or compulsive/ritualistic behaviour
    -hyperorality and dietary changes
    AND prominent decline in SOCIAL COGNITION and/or executive abilities
  2. language variant–> prominent decline in language ability, in the form of speech production, word finding, object naming, grammar or word comprehension
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

criterion D for M/M NCD due to Frontotemporal NCD

A

relative SPARING of learning and memory and perceptual-motor function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

criterion E for M/M NCD due to Frontotemporal NCD

A

the disturbance is not better explained by cerebrovascular disease, another neurdegenerative disease, the effects of a substance or another mental, neurological or systemic disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

by what criteria do you diagnose probable Frontotemporal NCD

A

Frontotemporal NCD is probable if either of the following is present:

  1. evidence of a causative frontotemporal neurocognitive disorder genetic mutation, from either family history or genetic testing
  2. evidence of DISPROPORTIONATE frontal and/or temporal lobe involvement from NEUROIMAGING
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

when is possible Frontotemporal NCD diagnosed

A

if theres no evidence of a genetic mutation, and neuroimaging has not been performed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the three language variants of M/M NCD due to Frontotemporal NCD

A

semantic

agrammatic/nonfluent

logopenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the core features of M/M NCD due to Frontotemporal NCD overall (though the disorder comprises a number of syndromic variants)

A

progressive development of behavioural and personality change and/or language impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what distinguishes the behavioural and language variants of M/M NCD due to Frontotemporal NCD, beyond their symptoms

A

they each have distinct patterns of brain atrophy and some distinctive neuropathology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

why might medical consultation usually be delayed in those with the behavioural variant of M/M NCD due to Frontotemporal NCD

A

insight is often impaired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

how do those with the behavioural variant of M/M NCD due to Frontotemporal NCD often present

A

with varying degrees of APATHY or DISINHIBITION

they may lose interest in socialization, self care and personal responsibilities, or display socially inappropriate behaviours

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what other changes are often observed in those presenting with the behavioural variant of M/M NCD due to Frontotemporal NCD

A

changes in social style

changes in religious and political beliefs

repetitive movements

hoarding

changes in eating behaviour

hyperorality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what physical symptom may occur in later stages of the behavioural variant of M/M NCD due to Frontotemporal NCD

A

loss of sphincter control

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what domain is often preserved, especially in the early stages, of the behavioural varient of M/M NCD due to Frontotemporal NCD

A

cognition–> formal testing may show relatively few deficits in the early stages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what are common neurocognitive symptoms in those with the behavioural variant of M/M NCD due to Frontotemporal NCD

A

lack of planning and organization

distractibility

poor judgment

deficits in executive function (poor performance on tests of mental flexibility, abstract reasoning, and response inhibition are present but learning and memory are relatively spared and perceptual-motor abilities are almost always preserved in the early stages)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

how do those with the language variant of M/M NCD due to Frontotemporal NCD often present

A

with primary progressive APHASIA with GRADUAL onset

each of the three subtypes have distinctive features and corresponding neuropathology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

list three other symptoms that may be present and may support a diagnosis of M/M NCD due to Frontotemporal NCD

A
  1. EPS features
    - -may be prominent in some cases, with overlap of syndromes such as PSP and corticobasal degeneration
  2. features of motor neuron diseases
    - -in some cases
    - -i.e muscle atrophy, weakness
  3. visual hallucinations
    - -in a subset of individuals
20
Q

in which population is M/M NCD due to Frontotemporal NCD a common cause of NCD

A

M/M NCD due to Frontotemporal NCD is a common cause of EARLY ONSET NCD in those younger than 65

21
Q

what is the population prevalence estimate for M/M NCD due to Frontotemporal NCD

A

2-10 per 100 000

22
Q

what % of M/M NCD due to Frontotemporal NCD occur in those over age 65

A

20-25%

23
Q

in an unselected autopsy series, what % of dementias were attributable to M/M NCD due to Frontotemporal NCD

A

5%

24
Q

which subtypes of M/M NCD due to Frontotemporal NCD are more prevalent in men? in women?

A

men–> behavioural and semantic language variants

women–> nonfluent language variant

25
Q

in what decade of life do people with M/M NCD due to Frontotemporal NCD usually present

A

6th decade

*age at onset varies between 3rd decade to ninth decade of life

26
Q

what is the median survival of those diagnosed with M/M NCD due to Frontotemporal NCD

A

6-11 years post symptom onset

3-4 years after diagnosis usually

27
Q

how does the natural course of M/M NCD due to Frontotemporal NCD compare to that seen in alzheimers disease

A

survival is shorter and decline is faster in M/M NCD due to Frontotemporal NCD than in typical alzheimers disease

28
Q

what % of those with M/M NCD due to Frontotemporal NCD have a family history of early onset NCD

A

40%

*10% show an autosomal dominant inheritance pattern

29
Q

mutations in which genes have been associated with M/M NCD due to Frontotemporal NCD

A
  1. the gene encoding the microtubule associated protein tau (MAPT)
  2. the granulin gene (GRN)
  3. the C9ORF72 gene
    * though some causative genes have been IDed, many people with known familial transmission do not have a known mutation
  4. transactive response DNA binding protein f 43 kDa (TDP-43 or TARDBP)
  5. valosin-containing protein (VCP)
  6. chromatin modifying protein 2B (CHMP2B)
  7. fused in sarcoma protein (FUS)
30
Q

what associated finding is associated with more rapid deterioration in M/M NCD due to Frontotemporal NCD

A

presence of motor neuron disease

31
Q

what findings from CT and MRI are associated with the BEHAVIOURAL variant of M/M NCD due to Frontotemporal NCD

A

both FRONTAL LOBES (especially the medial frontal lobes) are atrophic

32
Q

what findings on CT and MRI are associated with the SEMANTIC language variant of M/M NCD due to Frontotemporal NCD

A

the middle, inferior, and anterior TEMPORAL LOBES are atrophic bilaterally but ASYMMETRICALLY

(LEFT side usually more affected)

33
Q

what findings on CT or MRI are associated with the NONFLUENT language variant of M/M NCD due to Frontotemporal NCD

A

predominantely LEFT posterior FRONTAL-INSULAR atrophy

34
Q

what findings on CT or MRI are associated with the LOGOPENIC language variant of M/M NCD due to Frontotemporal NCD

A

predominantely LEFT posterior PERISYLVIAN or PARIETAL atrophy

35
Q

what do you see on functional imaging in the various variants of M/M NCD due to Frontotemporal NCD

A

HYPOPERFUSION and/or cortical HYPOMETABOLISM in the corresponding brain regions

*functional findings may present in early stages in the ABSENCE of structural abnormality

36
Q

the logopenic variant of M/M NCD due to Frontotemporal NCD is often a manifestation of what other NCD

A

alzheimers

37
Q

how do you distinguish between FTD and alzheimers other than with symptoms?

A

emerging biomarkers for alzheimers, like CSF amyloid beta and tau levels, and amyloid imaging, MAY help in the ddx, but distinction can remain difficult

38
Q

why is function often more severely impacted early on in the course of M/M NCD due to Frontotemporal NCD, compared, per se, with other NCDs

A

because of the involvement of language and/or behaviour, and its younger age at onset

39
Q

what behaviours, associated wtih M/M NCD due to Frontotemporal NCD, can be severely disrupting (Often leading to placement)

A

hyperorality, impulsive wandering and other disinhibited behaviours

even in structured settings can be severely disruptive–> especially when the individual is otherwise healthy, nonfrail and free of other comorbidities

40
Q

ddx of M/M NCD due to Frontotemporal NCD

A
  1. other NCDs
  2. other neurological conditions
  3. other mental disorders and medical conditions
41
Q

what % of those presenting with syndrome suggestive of M/M NCD due to Frontotemporal NCD are found at autopsy to have alzheimer’s disease pathology

A

10-30%

*occurs more in individuals who present with progressive dysexecutive syndromes in the absence of behavioural changes or movement disorder or in those with the logopenic variant

42
Q

with which neurological disorders do the symptoms of M/M NCD due to Frontotemporal NCD overlap

A

progressive supranuclear palsy

corticobasilar degeneration

motor neuron disease

*overlap clinically as well as pathologically

43
Q

what characterizes progressive supranuclear palsy

A

supranuclear gaze palsies and axial-predominant parkinsonism

pseudobulbar signs may be present and retropulsion is often prominent

44
Q

what is seen on neurocognitive assessment of those with progressive supranuclear palsy

A

psychomotor slowing

poor working memory

executive dysfunction

45
Q

how does corticobasal degeneration present

A

asymmetric rigidity

limb apraxia

postural instability

myoclonus

alien limb phenomenon

cortical sensory loss

2. Geriatric Psychiatry + Neuroanatomy (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions