Introduction to Intellectual Disabilities MHA / MCA in ID

1
Introduction to Intellectual DisabilitiesMHA /
MCA in ID

• Dr Simon Bonell
• Consultant Psychiatrist in Learning Disabilities
• Plymouth Community Healthcare (CIC)

MRCPsych Course Jan 2015
2
Overview

• Housekeeping
• Definition and Epidemiology of ID
• Causes of ID and relevance to psychiatric
  disorders
• Pathoplastic effect of ID on psychopathology
• Assessment and diagnostic challenges
• MHA and MCA in ID

3
True or False

• The definition of Learning Difficulties is an IQ
  below 70
• More males are affected by ID than females
• There is no difference in rates of severe ID by
  socio-economic group
• By definition, someone with a significant
  impairment in their cognitive functioning and
  adaptive social functioning has LD
• Successive generations have a slightly lower IQ
  than previous ones

4
First a note on terminology
Learning Difficulties
Learning Disabilities
Intellectual Disability
5
First a note on terminology
Subnormal
Mental retardation
Imbecile

• Handicapped

6
What is the definition of ID?
7
What is the definition of ID?

• Reduced level of intellectual functioning
• Reduced ability to adapt to the daily demands of
  a normal social environment
• Manifested during the developmental period

8
How do you diagnose ID?
9
How do you diagnose ID?

• Clinical assessment
• Adaptive behaviour
• Psychometric test performance

10
Clinical assessment

• Developmental history
• Family history
• Childhood / educational history
• Occupational history
• Social history
• Level of functioning
• Past medical and psychiatric history
• Forensic history
• Pre-morbid personality

11
Clinical assessment

• Appearance and Behaviour
• Speech
• Mood
• Thoughts
• Perceptions
• Cognition
• Insight

12
The role of IQ

• Intelligence Quotient (IQ) measures a range of
  different intellectual abilities
• In most people skills develop to a similar level
• Large discrepancies can occur esp. in ID
• IQ scores summated to give verbal and performance
  score and a full scale IQ
• Normal distribution

13
The role of IQ

• Mean IQ of population is 100
• IQ of 70 represents 2 standard deviations from
  the mean
• Mild ID 50 69
• Moderate ID 35 49
• Severe ID 20 34
• Profound ID lt20

14
Models of Disability

• Medical model
• Social model
• video
• Normalisation and de-institutionalisation
• Social role valorisation
• Person centred planning

15
Epidemiology

• 2.27 of the population have IQlt70
• Add those with specific causes
• Overall prevalence rate between 2 3
• 75 90 have mild ID

16
A note on mental age

• Adult with profound ID has an equivalent mental
  age of 0 3 years
• Unable to understand abstract concepts
• However, has lifelong experiences, skills
  development and adult biological urges and drives
• Mental age should not be used for adults

17
The Flynn Effect

• Flynn found IQ increases with each successive
  generation
• Particularly fluid intelligence problem
  solving
• Cause remains unclear
• ?environmental / educational
• ?cultural
• ?nutrition

18
Clinical descriptors of ID

• Mild ID
• The majority of people with ID
• Verbal communication usually reasonable but note
  receptive / expressive mismatch and
  overestimation of understanding and abilities
• Usually only minimal support with basic self care
  but some deficits in more complex aspects of
  social functioning (finance / employment)
• Support level ranges from none to very high
  levels (if significant risks are present)

19
Clinical descriptors of ID

• Moderate ID
• Verbal communication more reduced. Can generally
  understand short simple sentences. Need to use
  adapted communication
• Higher levels of support with self care usually
  required. Difficulties in adapting to new
  situations (e.g. may learn to use a bus
  independently but not able to work out a new
  route)
• Moderate to high levels of support required

20
Clinical descriptors of ID

• Severe / profound ID
• Little expressive verbal ability but note
  understanding may be better
• Needs high levels of support with basic self care
• Increasing rates of sensory deficits and physical
  disabilities

21
Why do we label a group of people as having ID
22
Vulnerability

• To mental illness
• 40 of people with ID have mental disorder
• To physical illness
• To poor treatment by society

23
(No Transcript)
24
(No Transcript)
25

• https//www.youtube.com/watch?vsubMgwyJOK8

26
True or False

• The definition of Learning Difficulties is an IQ
  below 70 F
• More males are affected by ID than females T
• There is no difference in rates of severe ID by
  socio-economic group F
• By definition, someone with a significant
  impairment in their cognitive functioning and
  adaptive social functioning has LD F
• Successive generations have a slightly lower IQ
  than previous ones F

27
Cause of ID true or false

1. Down syndrome is the most common genetically
   inherited cause of ID
2. Down syndrome is sometimes not caused by trisomy
   21
3. Fragile X is the most common single gene cause of
   ID
4. Prader Willi syndrome is associated with high
   rates of psychosis

28
Causes of ID

• Idiopathic ID accounts for 30 50 of cases
• Most common inherited cause of ID Fragile X
• Most common chromosomal defect Down Syndrome
• Why is the cause of ID relevant to the
  psychiatrist?

29
Behavioural phenotypes

• Prader-Willi Hyperphagia, food ideation
• Lesch-Nyhan Extreme self mutilation
• Smith-Magenis Objects in orifices, self hugging
• 22q11.2 deletion Psychotic illness
• Rett Stereotypic hand movements
• Angelman Puppet-like gait, attraction to
  water
• 5p- (Cri du chat) Inappropriate laughter,
  cat-cry during infancy

30
Causes of ID
Prenatal
Inherited disorders (such as phenylketonuria, Tay-Sachs disease, neurofibromatosis, tuberous sclerosis, hypothyroidism, and fragile X syndrome) Chromosome abnormalities Down syndrome Trisomy 21, mosaic, translocation, Prader-Willi, Klinefelters syndrome, Cri-du-chat etc
31
Causes of ID
Intra-uterine
Severe maternal malnutrition Infections (HIV, cytomegalovirus, herpes simplex, toxoplasmosis, rubella virus) Toxins (such as alcohol, lead, and mercury) Drugs (such as phenytoin, valproate, chemotherapy) Abnormal brain development (such as porencephalic cyst, grey matter heterotopia, hydrocephalus, neural tube defects and encephalocele) Pre-eclampsia and multiple births Placental dysfunction (IUGR, toxaemia)
32
Causes of ID
Perinatal
Birth asphyxia/Hypoxic brain damage Extreme prematurity Kernicterus
33
Causes of ID
Postnatal
Brain infections (such as meningitis and encephalitis) Severe head injury Malnutrition of the child Severe emotional neglect or abuse Toxins (such as lead and mercury) Brain tumours and their treatments
34
Down Syndrome

• Trisomy 21
• 95 caused by non-disjunction
• 88 maternal non-disjunction
• 2-3 Robertsonian translocation
• Long arm chromosome 21 attached to chromosome 14
• Not linked to maternal age
• 1-2 Mosaic
• Some cells have normal karyotype and others have
  trisomy 21

35
Down Syndrome

• Typical physical appearance
• Congenital heart disease
• Haematological malignancies
• Thyroid disorder
• Eye and hearing disorders
• Gastrointestinal abnormalities
• Average IQ 50

36
Down Syndrome

• Increased risk of Alzheimers type dementia
• Increased risk of autism
• Possible increased risk of depressive disorder
• Conduct disorder in childhood

37
Prader Willi syndrome

• Results from failure of expression of paternally
  derived gene on chromosome 15
• 15q11-13
• Failure of maternally derived gene leads to
  Angelman syndrome

38
PWS genetics

• Interstitial deletions 70
• Uniparental disomy 25
• Imprinting centre defects lt5
• Chromosomal translocations 1

39
Clinical features

• Infant
• Lethargy
• Hypotonia
• Poor feeding / failure to thrive
• Childhood
• Short stature
• Hypogonadism
• Hyperphagia and obesity

40
Clinical features
41
Psychosis and PWS

• Case reports of cyclic affective disorders and
  psychosis
• Boer et al (2002) Lancet
• Identified all people with PWS in Oxford/Anglia
• 25 adults with genetic confirmation of PWS
• 15 had detailed psychiatric assessment

42
Psychosis and PWS

• Boer 2002
• 28 of adults with PWS have severe affective
  disorder psychotic symptoms
• 8 of adults with deletion
• 62 of adults with uniparental disomy
• Later study (Soni 2007)

43
Medication in PWS

• Those on antipsychotic or antidepressant
  medication significantly less likely to have
  relapse
• Those on mood stabilising medication more likely
  to have a relapse
• Sodium valproate carbamazepine may not be
  effective for people with PWS
• Mood stabilisers only used in more severe,
  relapsing conditions (BPAD)

44
Cause of ID true or false

• Down syndrome is the most common genetically
  inherited cause of ID F
• Down syndrome is sometimes not caused by trisomy
  21 T
• Fragile X is the most common single gene cause of
  ID T
• Prader Willi syndrome is associated with high
  rates of psychosis T

45
Diagnostic and Assessment Issues in ID
46
Diagnostic Challenges in ID

• ICD-10 assumes a certain level of cognitive /
  verbal ability
• Level of ID important in assessment of possible
  mental health problems
• Short, simple questions
• Risk of suggestibility
• Repeat and check answers
• Use time anchors

47
Diagnostic challenges in ID

• Same range of psychopathology is not experienced
  in people with ID as general population
• ICD/DSM weighted heavily towards verbal items and
  intellectually complex concepts (guilt, body
  image etc.)

48
Diagnostic Challenges in ID

• Darren is a 35 year old man with severe
  intellectual disability and longstanding
  self-injurious behaviour (hand biting and banging
  his head against walls). He is taken to the GP
  due to increased self injurious behaviour. The GP
  starts risperidone and refers him to the LD
  psychiatrist due to his behaviour.

49
Diagnostic Challenges

• Diagnostic overshadowing- behaviour /
  presentation is attributed to ID rather than a
  diagnosable condition
• Baseline exaggeration - high levels of unusual
  behaviour prior to the onset of a condition make
  it difficult to recognise the onset of a new
  disorder
• Loss of skills and impairment of communication
  may also be signs of mental illness

50
Diagnostic Challenges in ID

• Mike is a 48 year old man with moderate learning
  disabilities. He is referred to the psychiatrist
  after being seen outside his new home waving at
  passing cars and making gestures. His support
  staff are concerned about him talking to himself
  and think he might have developed psychosis.

51
Impact of ID on MH presentations

• Psychosocial masking
• Impoverished social skills and life experiences
  result in unsophisticated presentation of a
  disorder or misdiagnosis of unusual behaviour as
  a psychiatric disorder
• Cognitive disintegration
• Decreased ability to tolerate stress leading to
  anxiety-induced decompensation (often
  misdiagnosed as a psychotic illness)
• Misdiagnosis of developmentally appropriate
  phenomenon
• Developmentally appropriate behaviours that are
  inappropriate for chronological age are
  misdiagnosed as psychiatric disorder (solitary
  play, talking to oneself and imaginary friends
  taken as evidence of psychosis)

52
Impact of functional impairment on mental health
presentations

• Implications for mental state examination
• Assessment of decline in abilities
• Working with carers
• Sensory impairments
• Common (deafness 40 x more common and blindness
  8.5 x more common than general population)

53
Diagnostic classificatory systems

• DC-LD
• Provides operationalised diagnostic criteria for
  psychiatric disorders in people with ID
• Works in a complementary way with ICD-10
• Developed by the RCPsych
• Hierarchical system to reach diagnoses

54
DC-LD
Axis I Axis II Axis III Level A Level B Level C Level D Level E Level of ID Cause of ID Psychiatric disorders Developmental disorders Psychiatric illnesses Personality disorders Problem behaviours Other disorders
55
Example of DC-LD hierarchical approach

• Jane is a 24 year old lady with moderate ID and
  excessive over eating
• Use the hierarchical approach to consider the
  differential diagnosis

56
Example of DC-LD hierarchical approach

• Jane is a 24 year old lady with moderate ID and
  excessive over eating
• Axis I level of ID does not account for the over
  eating
• Axis II ascertain cause of ID. If Prader-Willi
  syndrome present then eating a feature of this.
  If not continue to Axis III
• Axis III
• Level A does Jane have a developmental disorder?
  Eg. is the over-eating a ritual associated with
  autism?
• Level B is there evidence of a psychiatric
  illness (e.g. depression or an eating disorder)
• Level C is a personality disorder present? Does
  this account for her over-eating?

57
Summary

• ID is defined as a significant impairment in
  cognitive abilities and adaptive social
  functioning that arises during the developmental
  period
• Increased risk of physical and mental disorders
• The cause of ID can shed light on MH presentation
• Assessment must include consideration of the
  biological, psychological, social and
  developmental issues
• The DC-LD provides a hierarchical structure to
  reach psychiatric diagnoses in people with ID

58
MHA and MCA in ID True or False

• LD must be associated with abnormally aggressive
  or seriously irresponsible behaviour for
  detention under Section 3
• Autism must be associated with abnormally
  aggressive or seriously irresponsible behaviour
  for detention under Section 3

59
Guardianship orders True or False

1. The Local Authority is always the Guardian
2. Allow the Guardian to specify the persons
   residence
3. Give the power to recall a person to hospital
4. Give the power to require the person to attend
   places for medical treatment
5. Allow the deprivation of a persons liberty in a
   residential home
6. Are less restrictive than CTOs

60
MHA and MCA in ID

• LD must be associated with abnormally aggressive
  or seriously irresponsible behaviour for
  detention under Section 3 T
• Autism must be associated with abnormally
  aggressive or seriously irresponsible behaviour
  for detention under Section 3 F

61
Guardianship orders True or False

• The Local Authority is always the Guardian F
• Allow the Guardian to specify the persons
  residence T
• Give the power to recall a person to hospital F
• Give the power to require the person to attend
  places for medical treatment T
• Allow the deprivation of a persons liberty in a
  residential home F
• Are less restrictive than CTOs ?

62
Deprivation of Liberty true / false

1. The MCA code of practice clearly defines
   deprivation of liberty
2. The Deprivation of Liberty safeguards were
   introduced to fill the Winterbourne gap
3. If any person is under continuous supervision and
   control and not free to leave they are being
   deprived of their liberty
4. If a person does not object to the restrictions
   they are not deprived of their liberty

63
DoLs True / False cont.

1. Deprivation of Liberty Safeguards (DoLS) are part
   of the updated MHA
2. DoLS only apply in hospital or residential care
   settings
3. The House of Lords have recommended DoLS are
   scrapped as not fit for purpose

64
Deprivation of Liberty true / false

1. The MCA code of practice clearly defines
   deprivation of liberty F
2. The Deprivation of Liberty safeguards were
   introduced to fill the Winterbourne gap F
3. If any person is under continuous supervision and
   control and not free to leave they are being
   deprived of their liberty T
4. If a person does not object to the restrictions
   they are not deprived of their liberty F

65
DoLs True / False cont.

1. Deprivation of Liberty Safeguards (DoLS) are part
   of the updated MHA F
2. DoLS only apply in hospital or residential care
   settings T
3. The House of Lords have recommended DoLS are
   scrapped as not fit for purpose T

66
Reading

• Books
• Intellectual Disability Psychiatry a practical
  handbook 2009 Eds Hassiotis, Barron Hall
• DC-LD 2001 Royal College of Psychiatrists
• Psychiatric and Behavioural Disorders in
  Intellectual and Developmental Disabilities 2007
  Eds Bouras and Holt
• Intellectual Disability and Ill Health 2010 Eds.
  OHara, McCarthy and Bouras
• Mental Health Act Code of Practice
• Mental Capacity Act Code of Practice

67
Reading

• Papers
• Cooper et al (2007) Mental ill-health in adults
  with intellectual disabilities prevalence and
  associated factors BJPsych
• Boer et al (2002) Psychotic illness in people
  with Prader Willi syndrome due to chromosome 15
  maternal uniparental disomy The Lancet 350
• Soni et al (2007) The course and outcome of
  psychiatric illness in people with PraderWilli
  syndrome implications for management and
  treatment JIDR 51

68
Questions

• Email s.bonell_at_nhs.net