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Intellectual disability Flashcards

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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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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
Commonest inheritbale cause of intellectual disability
Fragile X
24
How severe is Downs syndrome?
Moderate to severe
25
Fragile X severity
Borderline to moderate
26
Face appearnace fragile X
Large head circumfernece, long and prominenet ears
27
Physical features of Fragile X
mitral valve prolapse lax joints scoliosis flat feet testicular enlargement
28
Behavioural features of Fragile X
poor concentration, hyperkinetic behaviour, avoidance of eye contact and significant social anxiety
29
What is Prader Willi syndrome>
Genetically determined neurodevelopmental disorder microdeletions on chromosome 15
30
Infants with prader willi syndrome
Extremely hypotonic, failure to thrive, feeding difficulties
31
Prader Willi syndrome from age 2
mild developmental delay, tendency to skin pick, obesity Difficulty controlling eating behaviour
32
Prader Willi syndrome physically
Short stature, failure of normal secondary sexual development
33
Complications of Prader Willi
diabetes mellitus, sleep and respiratory disorders
34
IQ with Prader Willi
60
35
Clozapine GI effects
Constipation
36
EHCP
Educational Health Care Plan til 25
37
Difficuties accessing healthcare
inability to recognise symptoms realise their significance know what service to seek help from difficulty in communicating these symptoms effectively diagnostic overshadowing
38
Psychiatric co morbidities with intellectual disability
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
Causes of new symptoms
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
Causes of new symptoms
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
Presentation of psychiatric problems in intellectual disability
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
What is intellectual disability?
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
What below the mean is significant in intellectual disability?
2 - 3 standard deviations = mild 3-4 SDs = moderate 0.003rd percentile - profound
44
What is equivalent of 2 standard deviations from the mean in %?
2.5
45
If IQ under 70 but no problems functioning do they ahve an intellectual disability?
No
46
Why is the distribution curve for IQ skewed at the bottom?
Addition of pathological causes of intellectual disability
47
What is the test for functioning?
ABAS III (Adaptive Behaviour assessment system)
48
Skills assessed in ABAS-III
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
What is the GAC?
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
What is WAIS?
Wechsler Adult Intelligence Scale - is an IQ test designed to measure intelligence and cognitive ability in adults and older adolescents.
51
Causes of profound intellectual disability
Environmental – hypoxia, trauma, infections Genetic Sydnromes eg cerebra malformation or cerebral palsy
52
What genetic disorder causing intellectual disbailitiy is autosomal recessive vs dominant?
PKU - recessive Tuberous sclerosis - autosomal dominant
53
Facial appearance Downs syndrome
Small ears, eyes and head Protruding tongue Short stature and neck Brushfield spots Flattened nasal bridge Epicanthal fold
54
What age do people woth Downs syndrome often get dementia?
40
55
What are brushfield spots?
- small, white spots in a ring in iris of eye seen in Downs syndrome
56
What is an epicanthal fold?
Upper eyelid skin fold covers inner corner of the eye seen in Downs
57
Which geneder is Retts syndrome seen in?
Girls - X linked
58
Signs of Retts syndrome
Wheelchair Girl Hand ringing
59
MDT around people with intellectual disbaility
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
What is difficulty in accessing health care often due to in intellectual disability?
inability to recognise symptoms realise their significance know what service to seek help from difficulty in communicating these symptoms effectively diagnostic overshadowing
61
Communication support for intellectual disabilities
Easy read documents Communication passport when in hospital PECS - picotrial aids Makaton (sign language almost)

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