Intellectual disability Flashcards

1
Q

How do people woth learning disabilities often express pain

A

‘Aggressively’
eg tooth pain may hit jaw

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1
Q

How do people woth learning disabilities often express pain

A

‘Aggressively’
eg tooth pain may hit jaw

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2
Q

Levels of intellectual disability

A

Mild
Moderate
Severe
Profound

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3
Q

What is IQ in mild disability?

A

50 to 70

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4
Q

Moderate intellectual disability IQ?

A

35 to 50
3-4 Standard deviations below mean

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5
Q

Severe intellectual disability IQ

A

20-35

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6
Q

Profound intellectual disability

A

<20

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7
Q

Why is IQ no longer used to make diagnosis of intellectual disability?

A

Overall score IQ doesnt indiccate strengths or weaknesses, social adaptation and function

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8
Q

Mild intellectual disability features

A

Lower end normal IQ distribution
Communicate with spoken language
Multifactorial genetic and environemtnal factors
Higher rates in social class IV and V
-Definite organic, subtle genetic, perinatal facotrs

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9
Q

Perinatal factors for intellectual disability

A

Alcohol, drugs, medications, maternal infections etc

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10
Q

Features of moderate to severe intellectual disability

A

Greater degree of care often including physical care to assist with feeding, contincence etc
Nearly always organic pathology for severe

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11
Q

Distribution of causes of severe and profound intellectual disability

A

Chromosomal 40%
Other genetic 15%
pre & peri-natal 10%
post-natal 10%
unknown 25%

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12
Q

Profound intellectual disability features

A

Excess numbers at more severe levels (‘bump’ on IQ curve)
Due to organic or pathological group with cluster of disorders of definable aetiology
Genetic
Environmental
Some will have syndromes like cerebral malformation syndromes or cerebral palsy, without yet defined cause

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13
Q

Chromosomal abnormalities causing intellectual disability

A

Down’s Syndrome (trisomy 21), Prader-Willi Syndrome (microdeletion on chromosome 15)

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13
Q

Chromosomal abnormalities causing intellectual disability

A

Down’s Syndrome (trisomy 21), Prader-Willi Syndrome (microdeletion on chromosome 15)

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14
Q

Genetic disorders causing intellectual disability

A

Fragile X Syndrome (X-linked), PKU (autosomal recessive), tuberous sclerosis (autosomal dominant)

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15
Q

Causes of intrauterine and neonatal damage causing intellectual disability

A

infections (rubella), foetal alcohol syndrome, prematurity, labour complications

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16
Q

Developmental and anatomical abnormalities of CNS - intellectual disability

A

e.g. neural tube defects (Intellectual disability usually secondary to hydrocephalus or infection), cerebral palsies

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17
Q

Late post natal causes of intellectual disability

A

e.g. encephalitis, meningitis, trauma, hypoxia (for example, due to severe epilepsy)

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18
Q

Examples of complex disorders

A

Retts syndrome

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19
Q

What is the commonest identifiable cause of intellectual disability?

A

Downs syndrome

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20
Q

Common physcial problems with Downs

A

Early onset dementia -alzeihmers
depression
Congenital heart defects
Hypothyroidism
Visual disorders - cataracts + keratococonus
Hearing imapriment - middle ear infections, conductive hearing loss, early onset of presbyacusis
Obesity
Epilepsy
Degenerative spine disorders
Atlanto axial joint subluxation
DM
Infections

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21
Q

Prader-Willi on mothers side

A

Angelmans syndrome

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22
Q

What trisomy is Downs?

A

21

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23
Q

Commonest inheritbale cause of intellectual disability

A

Fragile X

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24
Q

How severe is Downs syndrome?

A

Moderate to severe

25
Q

Fragile X severity

A

Borderline to moderate

26
Q

Face appearnace fragile X

A

Large head circumfernece, long and prominenet ears

27
Q

Physical features of Fragile X

A

mitral valve prolapse
lax joints
scoliosis
flat feet
testicular enlargement

28
Q

Behavioural features of Fragile X

A

poor concentration, hyperkinetic behaviour, avoidance of eye contact and significant social anxiety

29
Q

What is Prader Willi syndrome>

A

Genetically determined neurodevelopmental disorder
microdeletions on chromosome 15

30
Q

Infants with prader willi syndrome

A

Extremely hypotonic, failure to thrive, feeding difficulties

31
Q

Prader Willi syndrome from age 2

A

mild developmental delay, tendency to skin pick, obesity
Difficulty controlling eating behaviour

32
Q

Prader Willi syndrome physically

A

Short stature, failure of normal secondary sexual development

33
Q

Complications of Prader Willi

A

diabetes mellitus, sleep and respiratory disorders

34
Q

IQ with Prader Willi

A

60

35
Q

Clozapine GI effects

A

Constipation

36
Q

EHCP

A

Educational Health Care Plan
til 25

37
Q

Difficuties accessing healthcare

A

inability to recognise symptoms
realise their significance
know what service to seek help from
difficulty in communicating these symptoms effectively
diagnostic overshadowing

38
Q

Psychiatric co morbidities with intellectual disability

A

ASD, ADHD, Attachment Difficulties, Depression/Anxiety, Psychosis and Dementia
neurodevelopmental disorders, gender dysphoria, dementia in Down’s Syndrome, social anxiety in Fragile X syndrome etc.

39
Q

Causes of new symptoms

A

Physical causes– pain or discomfort, e.g. from ear infection, toothache, constipation, reflux oesophagitis, sensory impairments.
Psychiatric causes – depression, anxiety, psychosis, dementia.
Social/environmental causes – change in carers, disruption to routines, bereavement, abuse.

40
Q

Causes of new symptoms

A

Physical causes– pain or discomfort, e.g. from ear infection, toothache, constipation, reflux oesophagitis, sensory impairments.
Psychiatric causes – depression, anxiety, psychosis, dementia.
Social/environmental causes – change in carers, disruption to routines, bereavement, abuse.

41
Q

Presentation of psychiatric problems in intellectual disability

A

Level of disability: presentation in people with mild ID is often similar to general population whilst more severe to profound often present with more complexity
Communication difficulties: result in the need to have a high index of suspicion to diagnose illnesses due to reduced reports of symptoms
Challenging behaviour: is often reflective of anxiety rather than being “naughty” or oppositional
Emotional regulation difficulties: irritability and angry outbursts could be underpinned by sleep difficulties/insomnia

42
Q

What is intellectual disability?

A

Disorder of intellectual development (originates in developmental period) characterised by significant below average intellectual and adaptive behaviour that is
-2, 4 SDs below mean or 0.003rd percentile

43
Q

What below the mean is significant in intellectual disability?

A

2 - 3 standard deviations = mild
3-4 SDs = moderate
0.003rd percentile - profound

44
Q

What is equivalent of 2 standard deviations from the mean in %?

A

2.5

45
Q

If IQ under 70 but no problems functioning do they ahve an intellectual disability?

A

No

46
Q

Why is the distribution curve for IQ skewed at the bottom?

A

Addition of pathological causes of intellectual disability

47
Q

What is the test for functioning?

A

ABAS III (Adaptive Behaviour assessment system)

48
Q

Skills assessed in ABAS-III

A

4 domains: Conceptual, Social, Practical, and General Adaptive Composite (GAC).
Skills assessed:
Communication
Community use
Functional academics
School/home living
Health and safety
Leisure
Self-care
Self-direction
Social
Motor
Work

49
Q

What is the GAC?

A

General Adaptive Composite score - one of four domains taken from ABAS III. compares a person’s global adaptive skills to the adaptive skills of others in the same age group from the standardization sample.

50
Q

What is WAIS?

A

Wechsler Adult Intelligence Scale - is an IQ test designed to measure intelligence and cognitive ability in adults and older adolescents.

51
Q

Causes of profound intellectual disability

A

Environmental – hypoxia, trauma, infections
Genetic
Sydnromes eg cerebra malformation or cerebral palsy

52
Q

What genetic disorder causing intellectual disbailitiy is autosomal recessive vs dominant?

A

PKU - recessive
Tuberous sclerosis - autosomal dominant

53
Q

Facial appearance Downs syndrome

A

Small ears, eyes and head
Protruding tongue
Short stature and neck
Brushfield spots
Flattened nasal bridge
Epicanthal fold

54
Q

What age do people woth Downs syndrome often get dementia?

A

40

55
Q

What are brushfield spots?

A
  • small, white spots in a ring in iris of eye seen in Downs syndrome
56
Q

What is an epicanthal fold?

A

Upper eyelid skin fold covers inner corner of the eye seen in Downs

57
Q

Which geneder is Retts syndrome seen in?

A

Girls - X linked

58
Q

Signs of Retts syndrome

A

Wheelchair
Girl
Hand ringing

59
Q

MDT around people with intellectual disbaility

A

Mental Health: Psychiatry, Psychology, CPN
Nurses - RNs, RMNs, RLDNs
Education and social care: schools, specialist provisions, respite provisions, social worker, support worker from social services
Physical Health: GP, Paediatricians, Nurses, Physiotherapists, Podiatrists, Dieticians, Cardiology, Neurology
Communication: Speech and Language Therapists
Activities: Occupational Therapists
Support: Carers, Family, Personal Assistants

60
Q

What is difficulty in accessing health care often due to in intellectual disability?

A

inability to recognise symptoms
realise their significance
know what service to seek help from
difficulty in communicating these symptoms effectively
diagnostic overshadowing

61
Q

Communication support for intellectual disabilities

A

Easy read documents
Communication passport when in hospital
PECS - picotrial aids
Makaton (sign language almost)