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

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2
Q

criterion B for M/M NCD due to Frontotemporal NCD

A

disturbance has INSIDIOUS onset and GRADUAL progression

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3
Q

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

A

behavioural variant

language variant

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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
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5
Q

criterion D for M/M NCD due to Frontotemporal NCD

A

relative SPARING of learning and memory and perceptual-motor function

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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

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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
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8
Q

when is possible Frontotemporal NCD diagnosed

A

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

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9
Q

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

A

semantic

agrammatic/nonfluent

logopenic

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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

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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

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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

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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

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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

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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

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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

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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)

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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

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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

23
Q

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

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
in what decade of life do people with M/M NCD due to Frontotemporal NCD usually present
6th decade *age at onset varies between 3rd decade to ninth decade of life
26
what is the median survival of those diagnosed with M/M NCD due to Frontotemporal NCD
6-11 years post symptom onset | 3-4 years after diagnosis usually
27
how does the natural course of M/M NCD due to Frontotemporal NCD compare to that seen in alzheimers disease
survival is shorter and decline is faster in M/M NCD due to Frontotemporal NCD than in typical alzheimers disease
28
what % of those with M/M NCD due to Frontotemporal NCD have a family history of early onset NCD
40% *10% show an autosomal dominant inheritance pattern
29
mutations in which genes have been associated with M/M NCD due to Frontotemporal NCD
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
what associated finding is associated with more rapid deterioration in M/M NCD due to Frontotemporal NCD
presence of motor neuron disease
31
what findings from CT and MRI are associated with the BEHAVIOURAL variant of M/M NCD due to Frontotemporal NCD
both FRONTAL LOBES (especially the medial frontal lobes) are atrophic
32
what findings on CT and MRI are associated with the SEMANTIC language variant of M/M NCD due to Frontotemporal NCD
the middle, inferior, and anterior TEMPORAL LOBES are atrophic bilaterally but ASYMMETRICALLY (LEFT side usually more affected)
33
what findings on CT or MRI are associated with the NONFLUENT language variant of M/M NCD due to Frontotemporal NCD
predominantely LEFT posterior FRONTAL-INSULAR atrophy
34
what findings on CT or MRI are associated with the LOGOPENIC language variant of M/M NCD due to Frontotemporal NCD
predominantely LEFT posterior PERISYLVIAN or PARIETAL atrophy
35
what do you see on functional imaging in the various variants of M/M NCD due to Frontotemporal NCD
HYPOPERFUSION and/or cortical HYPOMETABOLISM in the corresponding brain regions *functional findings may present in early stages in the ABSENCE of structural abnormality
36
the logopenic variant of M/M NCD due to Frontotemporal NCD is often a manifestation of what other NCD
alzheimers
37
how do you distinguish between FTD and alzheimers other than with symptoms?
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
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
because of the involvement of language and/or behaviour, and its younger age at onset
39
what behaviours, associated wtih M/M NCD due to Frontotemporal NCD, can be severely disrupting (Often leading to placement)
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
ddx of M/M NCD due to Frontotemporal NCD
1. other NCDs 2. other neurological conditions 3. other mental disorders and medical conditions
41
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
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
with which neurological disorders do the symptoms of M/M NCD due to Frontotemporal NCD overlap
progressive supranuclear palsy corticobasilar degeneration motor neuron disease *overlap clinically as well as pathologically
43
what characterizes progressive supranuclear palsy
supranuclear gaze palsies and axial-predominant parkinsonism pseudobulbar signs may be present and retropulsion is often prominent
44
what is seen on neurocognitive assessment of those with progressive supranuclear palsy
psychomotor slowing poor working memory executive dysfunction
45
how does corticobasal degeneration present
asymmetric rigidity limb apraxia postural instability myoclonus alien limb phenomenon cortical sensory loss