Executive Deficits And Their Various Causes: Behavioural Variant Frontotemporal Dementia, Acquired Brain Impairment And The Effect Of The Personality Disorders

Front 1

What are Lichter & Cummings’ (2001) three behavioural syndromes?

Back 1

1. A dorso-lateral pre-frontal (DLPFC)
convexity syndrome
2. An orbital pre-frontal syndrome (OPFC)
3. Medial frontal/anterior cingulate syndrome

Front 2

What are Lichter & Cummings’ (2001) three behavioural syndromes:

Characteristics of a dorso-lateral pre-frontal (DLPFC) convexity syndrome? (6)

Back 2

1. compromise in executive functions
2. Decreased verbal and design fluency,
3. Abnormal motor programming
4. Impaired set shifting
5. Reduced learning and memory retrieval
6. Poor problem solving

Front 3

What are Lichter & Cummings’ (2001) three behavioural syndromes:

Characteristics of orbital pre-frontal syndrome (OPFC)? (7)

Back 3

1. Disinhibition
2. Irritability
3. Impulsivity
4. Emotional lability
5. Poor insight
6. Poor judgment
7. Distractibility

Front 4

What are Lichter & Cummings’ (2001) three behavioural syndromes:

Medial frontal/anterior cingulate syndrome

Back 4

1. Apathy
2. Diminished initiative (abulia)

Front 5

The frontal syndromes also include regions of the
distributed frontal-subcortical circuitry including the:

Back 5

1) Striatum

2) Pallidum

3) Mediodorsal thalamus

Front 6

Back 6

Front 7

Frontal subcortical Circuits:
1. Dorsolateral Prefrontal Circuit

Back 7

Front 8

Roles of the Dorsolateral Prefrontal Circuit? (4)

Back 8

Executive functions:
1. planning,
2. organising,
3. deciding which stimuli to attend to,
4. shifting cognitive sets

Front 9

Dysfunction of the Dorsolateral Prefrontal Circuit results in? (4)

Back 9

1. concretedness,
2. perseverative,
3. impaired reasoning,
4. poor mental flexibility

Front 10

What are the two functional syndromes?

Back 10

Disorders of drive and Disorders of control

Front 11

Characteristics of disorders of drive? (6)

Back 11

1. Apathy
2. Inertia
3. Lack of initiative
4. Inflexibility
5. Rigidity
6. Cognitive slowing

Front 12

Characteristics of disorders of control? (6)

Back 12

1. Restlessness
2. Hyper-reactivity
3. Disinhibition
4. Impulsivity
5. Irresponsibility
6. Cognitive acceleration

Front 13

Orbitofrontal Syndrome is attributable to? which are associated with?

Back 13

OFC lesions associated with emotional disturbances, personality changes (“Positive Symptoms”)

Front 14

Components of Orbitofrontal Syndrome?

Back 14

1. Inappropriate laughter, crying

2. Emotional lability (mood swings)

3. Disinhibition

4. Impulsivity
5.
Criminal behaviour

6. Unusual sexual behaviour

7. Often lack awareness

Front 15

Frontal subcortical Circuits:
3. Anterior Cingulate Circuit

Back 15

Front 16

What is the main function of the Anterior Cingulate Circuit?

Back 16

Mediates motivation

Front 17

Anterior Cingulate Circuit dysfunction results in? (4)

Back 17

1. Abulia
2. Akinetic mutism,
3. Apathy,
4. lack of initiation

Front 18

What are the personality changes in Traumatic Brain Injury? (7)

Back 18

1. Impulsiveness (acting before thinking)

2. Disinhibition (lack of self-control)

3. Inappropriate sexual activity

4. Apathy/Poor initiation (trouble getting started)

5. Frustration and loss of temper

6. Poor insight (lack of self-awareness of changes)

7. Emotional problems: depression, anxiety, irritability, anger, mood swings

Front 19

What is the second most common young-onset dementia

Back 19

Frontotemporal dementia

Front 20

What are the main features of FTD? (3)

Back 20

Characterised by (1) progressive behaviour change,
(2) executive dysfunction and (3) language difficulties

Front 21

What are the three FTD clinical syndromes?

Back 21

Three clinical syndromes :
1. Behavioural variant FTD
2. Semantic dementia
3. Progressive and non fluent aphasia

Front 22

The frontotemporal dementias (FTD) are a
heterogenous group of neurodegenerative
conditions that account for __ to __% of all
dementia.

Back 22

The frontotemporal dementias (FTD) are a
heterogenous group of neurodegenerative
conditions that account for 3 to 10% of all
dementia.

Front 23

DLDH =

Back 23

Dementia lacking distinctive histopathology

Front 24

PSP =

Back 24

Progressive supranuclear palsy

Front 25

FTDP-17 =

Back 25

FTD with parkinsonism linked to chromosome 17

Front 26

Dysexecutive (bvFTD): What are the three key characteristics?

Back 26

1. Behavioural disturbances

2. Disturbances on tests of executive functioning

3. Usually loss of insight into disturbances

Front 27

Key characteristic of Semantic Dementia & Progressive Non-Fluent Aphasia?

Back 27

disruptions of language

Front 28

bvFTD =

Back 28

behavioural variant FTD

Front 29

SD =

Back 29

Semantic dementia

Front 30

PNFA =

Back 30

Progressive Non-fluent Aphasia

Front 31

CBS =

Back 31

Corticobasal syndrome. A rare progressive neurodegenerative disease involving the cortex and the basal ganglia.

Front 32

Personality Changes in FL Dementias.

Impairments in social skills (in what two ways?)

Back 32

1. inappropriate or bizarre social behaviour
2. “loosening" of normal social restraints (e.g., using
obscene language or making inappropriate sexual
remarks)

Front 33

Personality Changes in FL Dementias.

Change in motivation (in what 4 ways?)

Back 33

1. apathy
2. withdrawal
3. lack of interest
4. and initiative

Front 34

Change in motivation in FL dementia may appear to be depression but the patient does not...

Back 34

experience sad feelings.

Front 35

Personality Changes in FL Dementias:

Decreased judgment might result in what two issues?

Back 35

1. Impairments in financial decision- making (e.g.,
impulsive spending)
2.
Difficulty recognizing consequences of behaviour

lack of appreciation for threats to safety (e.g.,
inviting strangers into home)

Front 36

Personality Changes in FL Dementias:

Changes in personal habits (3)

Back 36

1. lack of concern over personal appearance
2. Irresponsibility
3. compulsiveness (need to carry out repeated
actions that are inappropriate or not relevant to
the situation at hand).

Front 37

Patients with definite frontotemporal dementia have a poor prognosis which is worse if __________ ___________are also present.

Back 37

Patients with definite frontotemporal dementia have a poor prognosis which is worse if language deficits are also present.

Front 38

What % of the bvFTD do not progress?

Back 38

37%! (those with phenocopy syndrome fare better)

Front 39

Antisocial Personality Disorder (ASPD)? (16)

Back 39

1. Superficial charm
2. Absence of delusions and irrational thinking
3. Absence of “nervousness”
4. Unreliability
5. Untruthfulness and insincerity
6. Lack of remorse or shame
7. Inadequately motivated antisocial behaviour
8. Poor judgment and failure to learn by
experience
Pathological egocentricity and incapacity for
love
10. General poverty in major affective reactions
11. Specific loss of insight
12. Unresponsiveness in general interpersonal
relations
13. Fantastic and uninviting behaviour with drink
14. Suicide rarely carried out
15. Sex life impersonal, trivial, and poorly
integrated
16. Failure to follow any life plan

Front 40

Most antisocial adults were antisocial ___ __________

Back 40

Found that most antisocial adults were
antisocial in childhood

Front 41

Most antisocial children ___ ___ _________ ____ ______.

Back 41

Most antisocial children are not antisocial as
adults

Front 42

ASPD vs. criminality (4 key points?)

Back 42

- “criminal” is a legal term denoting
conviction for breaking a law:

-
Not all people with ASPD are criminals
(or in jails)

-
Not all people in jail or considered
criminal have ASPD


- Not all people with ASPD are
psychopaths

Front 43

Prevalence of ASPD?

Back 43

prevalence is 3% in men; lower in women

sex difference is probably real, but may be
inflated by clinician bias

Front 44

Onset of ASPD?

Back 44

-
Onset in childhood (by definition)


-CD portion may start as early as age 3-5

Front 45

Course of ASPD?

Back 45

Course of all PDs is chronic, but overt
antisocial behaviour seems to  after 40

could still show ASPD features (e.g., lying; poor
work habits)

Front 46

Acquired sociopathy: READ

Blair (2001) considers that acquired sociopathy is most likely the consequence of impairment in the brain systems that respond to threat. He contends that these behaviours are “a consequence of inability to socialize due to an impairment in the capacity to form associations between emotional unconditioned stimuli (particularly distress cues) and conditioned stimuli (specifically representations of transgressions). If the person is raised in a social environment (for example, poverty) where there are advantages for engaging in antisocial behaviour, they will engage in this behaviour but will not experience aversion to the distress of their victims” (p. 730).

Back 46

Acquired sociopathy: READ

Blair (2001) considers that acquired sociopathy is most likely the consequence of impairment in the brain systems that respond to threat. He contends that these behaviours are “a consequence of inability to socialize due to an impairment in the capacity to form associations between emotional unconditioned stimuli (particularly distress cues) and conditioned stimuli (specifically representations of transgressions). If the person is raised in a social environment (for example, poverty) where there are advantages for engaging in antisocial behaviour, they will engage in this behaviour but will not experience aversion to the distress of their victims” (p. 730).

Front 47

Acquired sociopathy is most likely the consequence of impairment in the brain systems that __________ ____ _____.

Back 47

respond to threat.

Front 48

Blair argues that acquired sociopathy is a consequence of “a consequence of inability to socialize due to an impairment in the capacity to form associations between what two things?

Back 48

emotional unconditioned stimuli (particularly
distress cues) and conditioned stimuli (specifically
representations of transgressions).

If the person is raised in a social environment (for example, poverty) where there are advantages for engaging in antisocial behaviour, they will engage in this behaviour but will not experience aversion to the distress of their victims” (p. 730).

Front 49

Psychopaths do not react as negatively as controls to
_________ _________Gray et al, 2003)

Back 49

Psychopaths do not react as negatively as controls to
_________ _________Gray et al, 2003).

Front 50

MRI studies of grey matter in PFC of psychopaths reveal what?

Back 50

MRI indicates that grey matter in PFC of psychopaths is 11% smaller than control (Raine et al, 2002).

Front 51

One needs to be very cautious in calling anything a “frontal lobe” anything without good _________ _______ _________.

Back 51

One needs to be very cautious in calling anything a “frontal lobe” anything without good quality imaging support

Front 52

Which circuit mediates executive functions: planning, organising, deciding which stimuli to attend to, shifting cognitive sets?

Back 52

. Dorsolateral Prefrontal Circuit

Front 53

Which circuit mediates emotional life and personality structure, mediates arousal, motivation, affect; mediates socially modulated civil behaviour?

Back 53

. Lateral Orbitofrontal Circuit

Front 54

Which circuit mediates motivation?

Back 54

Anterior Cingulate Circuit

Front 55

When is typical onset in FTD?

Back 55

Onset typically younger (less than 65 years)

Front 56

Predominant changes/disturbances in behaviour in FTD

what is the hallmark? When does this occur?

Back 56

 Personality change is a hallmark
 Changes occur early and progress

Front 57

Non-fluent, expressive aphasia common

What will you sometimes see?

Back 57

 Words remain but are presented in nonsensical
format

Front 58

What will you see on MRI in FTD?

Back 58

Frontal and/or temporal atrophy on MRI

Front 59

Neurological signs in the early stages of FTD?

Back 59

Early ABSENCE of neurologic signs, neurologic
signs occur with progression

Front 60

Kipps, Hodges & Hornberger (2010)

“Despite indistinguishable clinical profiles, studies in a cohort of bvFTD patients showed a particularly good prognosis for the subgroup of predominantly male patients in whom initial structural
imaging was normal. This could not be explained by differences in
disease duration, and was confirmed by subsequent PET studies. Retrospective review of clinical data in these groups verify that the current clinical diagnostic criteria are both insensitive to true
progressive bvFTD, particularly in the early stages, and also poorly specific. In contrast, measures of the activity of daily living
performance, executive function and tests of social cognition appear to have better discriminatory value for patient who show the clinical progression, with many individual diagnoses verified by post mortem examination in this group.

Their conclusion?!

Back 60

Summary: it remains doubtful that the
non progressive group have a neurodegenerative disease“ .

Front 61

Determinants of survival in behavioural variant frontotemporal dementia, Garcin et al, (2009). found that


 Methods: We analyzed survival in a large group of clinically
diagnosed bvFTD patients (n = 91) with particular attention
to demographic and clinical features at presentation. Of the
91 cases, 50 have died, with pathologic confirmation in 28.

 Conclusions: Patients with definite frontotemporal dementia have a poor prognosis which is worse if language deficits are also present. This contrasts with the extremely good outcome in those with the phenocopy syndrome: of our 24 patients only 1 has died (of coincident pathology) despite, in some cases, many years of follow-up

This means that...

Back 61

i.e. 37% of the bvFTD do not progress!!!

Front 62

Lee Robins’ work in mid-1960’s formed basis of
current ASPD criteria... two main findings

Back 62

1Found that most antisocial adults were antisocial in
childhood
2 Most antisocial children are not antisocial as adults

Front 63

Psychopaths do not react as negatively as controls to ______ _________ words (Gray et al, 2003)

Back 63

Psychopaths do not react as negatively as controls to violent words
(Gray et al, 2003)

Front 64

(Raine et al., 1999; 2000)

41 normal individuals (non murderers) matched for sex and age including 6 ‘murdering’ schizophrenia patients who were matched with 6 ‘non murdering’ schizophrenia patients

Findings?!

PET scans of 41 murderers revealed reduced activity in the frontal lobes. In a follow‐up study repeat offenders had 11% less frontal lobe activity compared to normals (Raine et al., 1999; 2000).

Back 64

1. Reduced activity in the frontal lobes on PET
2. Repeat offenders had 11% less activity than normals


 Reduced activity in prefrontal cortex, parietal region
and corpus callosum
 Left hemisphere less activity than right
 Abnormal asymmetries in amygdala and thalamus
 Both groups performed similarly on performance task

Front 65

Criticisms of Raine's studies include (3)

Back 65

6 murderers were left handed
 14 murderers were non white
 23 murderers had history of head injury

Front 66

Anterior tertiary area is responsible for what three things?

Back 66

1. planning
2. anticipating
3.restraint and error utilization

Front 67

Lesions to anterior tertiary area have serious implications for what?

Back 67

Lesions in these areas have a serious implication
for independent behaviour