NP: Chapter 20 Frontotemporal Dementia

Question 1

frontotemporal dementia =

Answer 1

progressive neurodegenerative disorder, develops before the age of 65 years

Question 2

what is the most common type of dementia after alzheimers

Answer 2

vascular dementia

Question 3

what is the most common clinical manifestation of ftd

Answer 3

the behavioural variant.

Question 4

behavioural variant frontotemporal dementia (bv-ftd)

Answer 4

changes in personality and social behaviour, cognitive impairments, language impairments

Question 5

changes in personality bij bv-ftd

Answer 5

decreased emotional involvement
emotional blunting
lack of initiative
apathy
impaired judgement
hyperactivity
restlessness

Question 6

wat gebeurt er met language bij bv-ftd

Answer 6

in early stages, language remains fluent.

but in later stages: economy of speech, whcih means that the patients tend to speak less as the disease advances. they initiate less conversation, and answer with yes/no. eventually may become mute

Question 7

3 types of bv-ftd

Answer 7

1. profile of disinhibition, hyperactivity and distractability
2. profile of apathy, behavioural withdrawal, lack of initiative
3. profile with stereotypy and compulsive behaviour

Question 8

ftd met mdn

Answer 8

frontotemporale dementie met motorneuronziekte (dus normale ftd + de manifestaties van motorische neuronziekte zoals neurogene spieratrofie)

Question 9

3 subtypes of frontotemporal dementia

Answer 9

behavioural variant
progressive non-fluent aphasia
semantic dementia

Question 10

behavioural variant =

Answer 10

• deterioration in social behaviour + emotional blunting and anosognosia (loss of insight)
• impairment in regulation of personal behaviour at an early stage
• increasing neglect of personal care
• speech and language impairment

Question 11

progressive non-fluent aphasia

Answer 11

non fluent speech: agrammatism, phonemic paraphrasias, word finding problems

Question 12

wat hebben mensen met pnfa niet

Answer 12

no behavioural or cognitive dysfunctions.

(heel soms tijdens eindfases wat behavioural)

Question 13

semantic dementia

Answer 13

prominent verbal or visual semantic impairments which are often manifested as a fluent, empty speech and word finding problems.
visuo-perceptual impairments
initially no impairments in other domains

Question 14

kijken naar prevalentie model dementias in docs

Answer 14

oke

Question 15

wanneer is de onset van ftd

Answer 15

before the age of 65, and peaks between 50 and 60

Question 16

about 10% of patients are above 70 years

Answer 16

oke

Question 17

what is the average duration of the disease

Answer 17

8 years (3 years for ftd-mnd)

Question 18

prevalence of ftd

Answer 18

ongeveer 20 per 100.000 ppl

Question 19

genetics of ftd

Answer 19

40%

Question 20

waar is een autosomal dominant inheritence pattern gevonden

Answer 20

bij bv-ftd (minder bij sd en pnfa)

Question 21

welke genes bij ftd

Answer 21

MAPT gene, progranulin (GRN) and C90RF72

Question 22

welke proteins bij ftd

Answer 22

tau, tdp-43 and fus

Question 23

waar zie je atrophy bij bv ftd

Answer 23

frontal and or temporal, soms hippocampal.

Question 24

bij early stages wat kan je zien bij ftd

Answer 24

not geen atrophy, dan via spect or pet scan kijken

Question 25

pnfa wat zie je

Answer 25

assymetrical atrophy: left frontotemporal region

Question 26

wat zie je bij sd

Answer 26

temporal lobe atrophy and hippocampal atrophy

Question 27

welke cognitive domains affected by bv ftd

Answer 27

attention
concentration
executive functions
abstract thinking
language

Question 28

wat voor impairments nog meer bij bv ftd

Answer 28

sociocognitive functioning; such as emotion perception and theory of mind, en planning, organizing,, structuring and mental flexibility

Question 29

wat is lastig bij deze patienten met testen

Answer 29

patients are willing to be tested, but easily distracted and only minimal effort (=economy of effort).
poor insight into performance and effort
= daardoor missing test results

Question 30

responses of bv ftd are often…

Answer 30

arbitrary, impulsive, and lacking in basis/arguments

Question 31

wat blijft unaffected bij bv ftd tot de advanced stages of the disease

Answer 31

memory and orientation

Question 32

pnfa main symptoms

Answer 32

hestitation, stuttering use of language (flagging?)

Question 33

insight in disease bij pnfa

Answer 33

goed, want patienten proberen zichzelf te corrigeren.

Question 34

wat is nog meer impaired bij pnfa

Answer 34

ook reading and writing. other cogn domains remain unimpaired until the final stages

Question 35

wat is bij semantic dementia de main symptoms

Answer 35

comprehension of the meaning of the content of words, naming things etc. ook soms visual agnosia (geen voorwerpen herkennen).

Question 36

wat remains intact bij sd

Answer 36

repetition of words, and sentences. memory voor een groot deel ook, behalve de semantic memory. autobiografie blijft meestal goed.