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Management > Frontotemporal Dementia > Flashcards

Frontotemporal Dementia Flashcards

1
Q

What is the second most common young onset degenerative dementia?

A

Frontotemporal dementia

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2
Q

What is the prevalence and incidence of FTD?

A

Prevalence
– 10-20 per 100,000 (ages 45-64)

Incidence
– 3-4 per 100,000 (ages 45-64)

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3
Q

FTD is a ________ disease

A

Heterogeneous

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4
Q

What are the 2 variants of FTD?

A

Behavioral (bvFTD) and language (non fluent variiant nfvPPA, semantic variant svPPA)

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5
Q

What do the language variants lead to?

A

Primary progressive aphasia (PPA)

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6
Q

What other conditions does FTD overlap with?

A

Atypical parkinsonian syndromes:
– Progressive supranuclear palsy (bvFTD, nfvPPA)
– Corticobasal (degeneration) syndrome (bvFTD, nfvPPA)

  • Motor neurone disease/amyotrophic lateral sclerosis
    (bvFTD&raquo_space; nfvPPA and rare descriptions of svPPA)
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7
Q

What are the consensus criteria for FTD?

A

*Lund-Manchester criteria 1994
*Neary criteria 1998
* McKhann criteria 2001
* Mesulam PPA criteria 1998/2001

New criteria:
*Rascovsky
International bvFTD
Consortium criteria
for bvFTD
(Brain, 2011)

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8
Q

What are the features of the behavioral variant of FTD?

A
  1. Disinhibition
    – Socially inappropriate behaviour
    – Loss of manners or decorum
    – Impulsive, rash or careless actions
  2. Apathy/inertia
  3. Loss of sympathy/empathy
  4. Perseverative, stereotyped and compulsive behaviour
    – Simple repetitive movements
    – Complex, compulsive or ritualistic behaviours
  5. Hyperorality/dietary changes
    – Altered food preference – sweet tooth, binge eating
  6. Executive dysfunction
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9
Q

What are the other symptoms not in bvFTD criteria?

A
  • Loss of insight
  • Impaired social cognition
  • Altered sensitivity to pain and/or temperature
  • Delusions and/or hallucinations (usually visual)
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10
Q

What are the subtypes of PPA?

A

3 (rather than 2)
subtypes:
– svPPA
– nfvPPA
– lvPPA
= logopenic
aphasia (first
described 2004)

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11
Q

What are the features of svPPA? (semantic)

A
  • Multimodal loss of semantic knowledge: verbal initially
    then… visual, auditory, olfactory, gustatory
  • Fluent speech with semantic errors
  • Anomia
  • Single word comprehension problems
  • Surface dyslexia
  • Later, nonverbal impairment e.g. visual agnosia
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12
Q

What is the problem with nonfluent aphasia nfvPPA?

A

Problem: nfvPPA very mixed
– Agrammatism
– Apraxia of speech
– Anomia
– Word-finding pauses
* New classification splits “nonfluent” patients into nfvPPA
and lvPPA

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13
Q

What are the features of nfvPPA?

A
  • ‘Motor’ speech impairment
  • Can be v slow and
    misdiagnosed as
    functional
  • Agrammatism
  • May develop CBS/PSP
  • More anterior (left inferior
    frontal and insula) atrophy
  • Underlying pathology is
    FTLD: tau&raquo_space; TDP-43
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14
Q

What are the features of ivPPA?

A
  • Hard to characterise as
    ‘fluent’ vs ‘nonfluent’
  • Word-finding difficulties
  • Motor speech/grammar
    normal
  • More posterior atrophy
  • Underlying pathology AD
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15
Q

What are the pathological subtypes of frontotemporal lobar degeneration?

A

Tau positive (commonest) or ubiquitin positive tau negative (FTLD-U)

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16
Q

What are the genetics of FTD?

A
  • Up to 50% of patients with FTD describe a family history of
    a dementia
  • But… a smaller number have an autosomal dominant
    inheritance (~a third)
  • And variable across clinical syndromes
17
Q

What are the main genetic findings?

A

2011 – Chromosome 9 open reading frame 72 (C9ORF72)

2006 – Progranulin (GRN)

1998 – Microtubule-associated protein tau (MAPT)

In our series: C9ORF72 9%, MAPT 10%, GRN 7%…
but variable across the world

18
Q

What to test?

A

On family history
– Autosomal dominant family history commonly present… but
not always
* On clinical syndrome:
– bvFTD/FTD-MND > nfvPPA > CBS&raquo_space; PSP&raquo_space; svPPA
– bvFTD: any of the genes (C9orf72 if delusions)
– FTD-MND: C9orf72
– nfvPPA: GRN&raquo_space;C9orf72
– CBS: MAPT > GRN mutations
– PSP syndrome: rare cases of MAPT mutations

19
Q

What allows us to test all the genes at the same time?

A

next generation sequencing

but multiple variants in same person? whats pathogenic? some people carry double mutations

20
Q

Imaging of bvFTD involves which lobes

A

variable
not just frontal and temporal lobes but
also insula and anterior cingulate; usually asymmetrical
* Whitwell et al, 2009, Brain suggest 4 subtypes:
– Frontal predominant
– Temporal predominant
– Frontal = temporal
– Temporofrontoparieta

21
Q

What is the problem?

A

we cannot diagnose underlying pathology in life

22
Q

What are the FTD biomarkers?

A
  • Tau pathology
    – PSP syndrome (highly likely)
    – MAPT mutations
    – Tau PET ligand
    – Tau isoforms in CSF
  • TDP pathology
    – FTD-MND syndrome/svPPA syndrome (highly likely)
    – GRN mutations or serum GRN level
    – TDP-43 levels in serum or CSF
  • FUS pathology
    – Very young onset
23
Q

Management directed to

A

controlling symptoms and helping patients and caregivers cope with impact of their illness

24
Q

What are the non pharmacological managements?

A

– Simple interventions (puzzles, music, controlled access to food/drink) may help govern troublesome behaviours and contribute to a stable routine

– Speech and language therapy can provide communication aids, plus
assessment of swallowing and advice on dietary modification

– Programmed respite, counselling, and psychological support for
caregivers

– Genetic counselling of at-risk family members

25
Q

Clinical trial evidence of FTD includes

A
  • Phase 3:
    – LMTM (TauRx) FAILED
  • Phase 2:
    – HDAC inhibitor (FORUM) – GRN mutation carriers FAILED
  • Phase 1:
    – ASOs (Ionis) – C9orf72 mutation carriers/MAPT mutation
    carriers
    – ASO (Wave) – C9orf72 mutation carriers
    – Sortilin antibody (Alector) – GRN mutation carriers
25
Q

What are the pharmacological management options for FTD?

A

No clear symptomatic benefit
for cholinesterase inhibitors or memantine; these agents may aggravate behavioural
disturbance

– Small, double blind, controlled trials have provided some evidence for modest improvement of behavioural symptoms with selective serotonin reuptake inhibitors

Management (4 decks)

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