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NDDs/Stroke > William's > Flashcards

William's Flashcards

1
Q

William’s Syndrome is caused by

A

Deletion of 26-28 genes on Chromosome 7

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2
Q

Stromme et al., (2002)

Prevalence of William’s?

A

1 in 7,500

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3
Q

What are the ‘distinct facial features’ found in WS?

A

Elfin like features –> broad brow, flat nasal bridge, wide mouth etc

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4
Q

What health complications might be found in WS? (2)

A

Cardiovascular disorders

Kidney/bladder issues

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5
Q

Bullugi, Klima & Wang (1996)

WS children possess mild to moderate…

A

Intellectual deficits

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6
Q

Bullugi, Klima & Wang (1996)

The intellectual deficits found in WS are _____ to _____

A

Mild to moderate

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7
Q

How is WS diagnosed?

A

Genetic Diagnosis (FISH)

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8
Q

What does FISH stand for?

A

Fluorescent in Situ Hybridisation

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9
Q

Lowery et al., (1996)

In WS, one copy of the elastin gene is deleted in over ____ of patients

A

96%

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10
Q

Which NDD has ‘elfin-like’ features?

A

WS

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11
Q

Which brain imaging technique is generally used to detect abnormalities in WS?

A

MRI

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12
Q

What are the main structural differences in WS brains?

A

Total brain + occipital grey matter volumes significantly reduced
Ventral prefrontal cortex & amygdala grey matter significantly enlarged

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13
Q

Martens, Wilson + Reutens (2008)

What are the differences in terms of the cerebrum/cerebellum in WS?

A
  1. Reduction in cerebral volume

2. Preservation of cerebellar volume

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14
Q

WS

How is the amygdala different?

A

Increase in volume

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15
Q

WS

Grey matter volume in PREFRONTAL cortex?

A

Increase in volume

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16
Q

Levitin et al., (2003)

Musical stimuli resulted in widespread…. activation

A

Cortical + subcortical, amygdala

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17
Q

Martens, Wilson + Reutens (2008)

How does the brain of a WS patient respond to seeing threatening scenes?

A

Increased amygdala activation

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18
Q

Martens, Wilson + Reutens (2008)

How does the brain of a WS patient respond to seeing unfamiliar/threatening faces?

A

Reduced amygdalar activation

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19
Q

Martens, Wilson + Reutens (2008)

“Thus they proposed this abnormal circuity may underlie the HYPERSOCIABLE and ANXIOUS characteristics of many individuals with WS”

What abnormal circuitry is this referring to?

A
  1. Increased amygdalar activation (threatening scenes)

2. Reduced amygdalar activation (threatening faces)

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20
Q

Bellugi et al., (1999)

In WS, Mean IQ is between ____, with a range of ______

A

50 + 60

40-100

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21
Q

Martens, Wilson + Reutens (2008)

Is IQ stable or unstable over time?

A

Stable

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22
Q

Martens, Wilson + Reutens (2008)

Which is strongest for WS, verbal IQ or performance IQ?

A

Verbal IQ

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23
Q

3 strengths (cognitive) of WS?

A
  1. Language
  2. Face processing
  3. Musical ability
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24
Q

3 language strengths of WS?

A
  1. Speech production
  2. Receptive vocab
  3. Grammatical abilities
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25
Q

Bellugi et al., (1990)

Found that WS children’s _______ exceeded their mental age, as well as that of age-matched controls

A

Receptive vocab

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26
Q

2 Language WEAKNESSES in WS?

A
  1. Pragmatics

2. Reciprocal conversation

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27
Q

Laing et al., (2002)

Compared to mental age-matched controls….

A

Toddlers with WS use fewer gesturing skills

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28
Q

Why might difficulty with pragmatics hinder somebody with WS?

A

They may take metaphors/sarcasm at face value, missing out on important convo

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29
Q

Four general cognitive WEAKNESSES of WS?

A
  1. Visuospatial abilities
  2. Attention
  3. EF
  4. Fear
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30
Q

Farron + Iarrold (2003)

Found that WS’s struggle with visuospatial tasks such as.. (2)

A

Block Design task or Pattern Construction

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31
Q

Bihrle et al., (1989)

Children/adults have a deficit in processing the ______

A

Global aspects of visuospatial stimuli

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32
Q

Hoffman et al., (2003)

The WS deficit in global processing…

A

May reflect deficits in planning + execution of motor responses

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33
Q

Cornish et al., (2007)

Found that WS have problems with ______ and ________

A

Attentional control

Visual orienting

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34
Q

Cornish et al., (2007)

Children with WS display poor performance on _____ task and ______ task

A

Anti-saccade

Orientating

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35
Q

Anti-saccade tasks tap into…

A

Voluntary control of gaze

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36
Q

Mervis (2003)

Posed that attentional difficulties pose problems with interactions with peopole, if there is a

A

Greater degree of distractibility

37
Q

Rhodes et al., (2010)

Which task can assess EF PLANNING?

A

Tower of Hanoi

38
Q

Rhodes et al., (2010)

Found WS children have difficulties with (3)

A

Planning (frontal lobe)
WM (frontal lobe)
Attention (frontal lobe)

39
Q

COMORBIDITY of WS (3)

A
  1. ADHD
  2. Specific phobia
  3. GAD
40
Q

65% of WS children also have

A

ADHD

41
Q

54% of WS children have a specific phobia, resulting from….

A

Enlargement of grey matter in amygdala/prefrontal cortex

42
Q

WS children have enlargement of grey matter in amygdala/prefrontal cortex. This affects

A

Risk/fear processing

43
Q

Differences in WHERE affects risk/fear processing?

A

Amygdala/prefrontal cortex

44
Q

Example of a specific fear seen in WS?

A

Getting lost (spatial navigation difficulties?)

45
Q

Jones et al., (2000)

Found that WS patients are unusually….

A

Sociable, friendly and empathetic

46
Q

Riby et al., (2014)

WS individuals show little restraint towards…

A

Unfamiliar people

47
Q

WS children are more anxious, ______ and ______

A

Distractible

Hyperactive

48
Q

Gosch + Pankau (1997)

As WS individuals reach adulthood, they are…

A

More reserved/less active, but still more extroverted than NT controls

49
Q

Bellugi (1999)

Adults with WS rated unfamiliar faces…

A

Significantly more approachable than NT controls

50
Q

What is the main behavioural phenotype for WS?

A

Hypersociability

51
Q

WS children have a clear, innate affinity with

A

Music

52
Q

Lehnoff et al., 2001

WS children have…

A

Perfect pitch

53
Q

Levitin + Bellugi, 1998

A

WS children’s rhythmic production abilities may be similar to musically trained children

54
Q

How are WS children DIFFERENT to autistic children?

A

WS are hypersociable and have strengths in language

55
Q

How are Autistic children SIMILAR to WS? (3)

A

Distractibility
Inflexibility
Pragmatic deficits

56
Q

Martens, Wilson + Reutens (2008)

________ deficits confirmed across a large body of studies

A

Visuospatial deficits

57
Q

Difference between WS and FXS?

A

FXS tend to be socially withdrawn/have poor social eye gaze

58
Q

WS is caused by the deletion of ____ on _______

A

26-28 genes

Chromosome 7

59
Q

WS is caused by _________ on Chromosome 7

A

The deletion of 26-28 genes

60
Q

Aortic stenosis is

A

Narrowing of arteries to the heart

61
Q

Aortic _______ is associated with WS

A

Stenosis

62
Q

Despite a reduction in overall brain volume in WS, which areas are significantly increased?

A

Amygdala

Ventral prefrontal grey matter

63
Q

Reiss et al., 2004 found….(2)

A
  1. Reduction in overall grey matter/occipital cortex

2. Increase in amygdalr/prefrontal grey matter

64
Q

Martens, Wilson & Reutens found that in WS, IQ ________ time

A

Is stable over

65
Q

One example of a grammatical ability?

A

Understanding syntax

66
Q

Farran & Jarrold (2003) –> investigated visuospatial abilities in WS by administering…

A

Pattern construction task

Block design task

67
Q

Anti-saccade tasks require…

A

Considerable voluntary eye-movement control

68
Q

Cornish et al., (2007) (WS)

What were the results of the anti-saccade task?

A

Problems with attention disengagement

69
Q

Cornish et al., 2007 (WS)

What were the results of the orienting task?

A

Greater interference effects of invalid cues –> problems with attentional disengagement and visual orienting

70
Q

Cornish et al., (2007)

Suggested that as age increases,

A

Distractibility may also increase

71
Q

Martens et al., (2008) found that WS children have a really clear talent for

A

Music

72
Q

Don et al., 1999

__% of the sample were either indifferent or displayed an intense dislike to

A

15%

Music

73
Q

Bellugi et al., 1999

A

IQ between 50-60, with a range of 40-100

74
Q

Bellugi et al., 1990

A

Found WS children have a receptive vocab that exceeds mental age/age-matched controls

75
Q

Martens et al., (2008) concurred that _______ consistute a cognitive weakness in WS

A

Visuospatial abilities

76
Q

Jones et al., 2000

A

WS are unusually sociable, friendly and empathetic

77
Q

____% of WS children have a specific phobia

A

54%

78
Q

Bellugi et al., 1999 found that adults with WS…

A

Rated unfamiliar faces as significantly more approachable than NT controls

79
Q

Martens et al., 2008

Speech & Language in WS

A

WS children show speech & language delays

80
Q

Krasneger, 1991

A

Expressive lang is often elaborate and gramatically correct

81
Q

Martens et al., 2008

Language DEFICITS were found in

A

Pragmatics
Reciprocal convo.
Complex language skills

82
Q

“The visuospatial deficit in WS may reflect a deficit in planning & execution of motor responses” who said this?

A

Hoffman et al., 2003

83
Q

Martens et al., (2008)

Evidence of a _______ that is distinguishable from….

A

Hypersociability phenotype

General friendliness

84
Q

Don et al., (1999)

Music & WS

A

15% of sample were either indifferent or displayed an intense dislike to music

85
Q

Heal & Sigelman (1995)

Why might researchers be wary using self-report methods with WS?

A

Sociable personality may result in response bias

86
Q

Which studies provides evidence of impaired executive function in WS?

A

Rhodes et al., 2010

87
Q

Rhodes et al., 2010

What were the non-executive deficits found?

A

STM and memory span

88
Q

WS exhibit a distinct profile of proficient _________ and severe __________

A

Verbal skills

Severe visuospatial processing impairment

NDDs/Stroke (9 decks)

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