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MRCPsych Part B - Al-Rashid Hospital > Learning Disability > Flashcards

Learning Disability Flashcards

1
Q

What term does ICD 10 use for LD?

A

Mental retardation

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2
Q

What term will ICD 11 use for LD?

A

Intellectual disability

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3
Q

What term does DSM V use for LD?

A

Intellectual disability

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4
Q

What defines LD?

A

IQ <70
Presentation in early life - within developmental period
Associated with deficits in social and adaptive functioning

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5
Q

What is the Valuing People 2001’s definition of LD?

A

Significantly reduced ability to understand new or complex information or learn new skills
Reduced ability to cope independently
Impairment that started before adulthood with lasting effect on development

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6
Q

Define Mild LD

A

IQ50-69

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7
Q

Define moderate LD

A

IQ 35-49

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8
Q

Define severe LD

A

IQ 20-34

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9
Q

Define profound LD

A

IQ <20

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10
Q

What does adaptive functioning refer to?

A

How effectively individuals cope with common life demands how well they meet standards of personal independence expected of someone of their age group

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11
Q

How can we measure Adaptive functioning?

A

Vineland Adaptive Behaviour Scale

Information from more than 1 source

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12
Q

When can borderline intellectual functioning be used according to DSM V?

A

When an individuals borderline intellectual functioning if the focus of clinical attention or has an impact on the individuals treatment or prognosis

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13
Q

Characteristics of mild LD

A

Delay in acquiring speech but can develop social and communication skills
Main problems in academic settings but can learn academic skills
Independent with self-care
May be in paid employment

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14
Q

Characteristics of moderate LD

A

Delays in acquiring speech with deficits in use of language and comprehension
Not able to achieve academically
Can profit from training in social and occupational skills
May achieve self-maintenance in unskilled/semiskilled work with appropriate support and supervision
Majority have identifiable organic aetiology

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15
Q

Characteristics of severe LD

A

Poor motor development, social skills and minimal verbal speech
Marked motor impairment and associated deficits
May contribute partially to self-maintenance under close supervision
May adapt well to supervised living situations and perform work-related tasks under supervision

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16
Q

Characteristics of profound LD

A

Comprehension and use of language very limited
Will require assistance with most ADLs
Require nursing care or life support under structured environment
Organic aetiology is usually clear
Commonly associated with neurological and physical disabilities affecting mobility
Other conditions such as epilepsy, visual/hearing impairments

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17
Q

Prevalence of LD

A

1-3%

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18
Q

Highest incidence of LD is in which age group

A

School-aged children

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19
Q

Peak incidence of LD

A

10-14 years

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20
Q

M:F ratio of LD

A

1.5:1

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21
Q

Most common type of LD

A

Mild - 85%

Moderate - 10%

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22
Q

How many people with LD have severe LD?

A

4%

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23
Q

How many people with LD have profound LD?

A

1-2%

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24
Q

How many people with LD have MH issues?

A

66%

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25
Q

Correlation of MH issues with LD?

A

Greater severity correlated with increased chance of MH issues
Greater MH issues in those with neurological conditions

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26
Q

Which MH problems occur more commonly in mild LD?

A

Disruptive and conduct disorders

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27
Q

What MH problems are higher in severe LD?

A

ASD

Pervasive developmental disorder

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28
Q

What sx can occur in people with LD outside the context of a mental disorder?

A

Hyperactivity
Self-injurious behaviour
Short attention span

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29
Q

How many people with LD have hearing loss?

A

25-42%

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30
Q

Age of death of LD compared to general population

A

13 years (men) and 20 years (women) earlier than general population

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31
Q

Genetic factors linked to LD

A

Sex chromosome disorders
Deletions and duplications
AD and AR disorders
X-linked recessive and dominant conditions
Polygenetic conditions (neural tube defects, pervasive developmental disorders)
Mitochondrial and metabolic disorders

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32
Q

External prenatal factors linked to LD

A

Infections
Exposure to medication, alcohol, drugs and toxins
Maternal illness

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33
Q

Perinatal factors linked to LD

A 
Premature infants
Low birth weight
Infections
Problems during delivery
Newborn complications
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34
Q

Post-natal factors linked to LD

A 
CNS infections
IC tumours
Hypoxic brain injury
HI
Exposure to toxic agents
Psychosocial environment
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35
Q

Examples of psychosocial environments linked to LD

A

Family instability
Frequent moves
Multiple but inadequate carers may deprive infant of emotional relationships leading to failure to thrive

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36
Q

Primary prevention of LD

A

Immunization
Provision of Folic acid at time of conception
Good medical follow-up, identification and intervention to reduce risks during pregnancy, delivery and childhood
Lead intoication
Iodine deficiency
Fetal alcohol syndrome
Acidents at home and traffic accidents

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37
Q

What is secondary prevention of LD?

A

Early recognition, diagnosis, good medical care and rehab of injuries or diseases that can avoid or prevent permanent damage which could lead to development of LD

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38
Q

Examples of secondary prevention of LD

A

Screening and early treatment f congenital hypothyroidism and PKU
Planning or genetic counselling after birth of child with genetic disorder

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39
Q

What is tertiary prevention of?

A

Helping individual attain their full developmental potential

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40
Q

Examples of tertiary prevention for LD

A

Biopsychosocial support
Family support
Education
Environmental adjustment and aids

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41
Q

Who introduced the concept of subcultural handicap?

A

EO Lewis - 1933

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42
Q

What is subcultural mental retardation?

A

Lower extreme variant of normal IQ distribution in population

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43
Q

What did EO Lewis divide LD into?

A

Pathological

Subcultural

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44
Q

In which social classes is pathological LD seen in?

A

Evenly distributed

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45
Q

In which social classes is subcultural LD seen in?

A

V

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46
Q

Which type of LD is subcultural LD associated with?

A

Mild

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47
Q

Features of subcultural LD

A 
Mild
More common in low socioeconomic groups
Family members may have borderline IQ
Dysmorphic characters less likely
Syndromic features not seen
Behavioural phenotypes rare
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48
Q

Features of pathological LD

A 
Moderate, severe or profound type
Evenly distributed across social classes
Family members have normal IQ
Dysmorphic features common
Syndromic features seen
Behavioural phenotypes are frequent
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49
Q

What does subcultural LD imply?

A

Psychosocial causation

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50
Q

What is normalisation?

A

Social principle that aims to enable the intellectually disabled to experience normal patterns of daily life

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51
Q

When was principle of normalisation developed and by whom?

A

In Scandinavia in the 1960s by Bengt Nirje

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52
Q

Who furthered the work of normalisation in LD?

A

Wolf Wolfensberger

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53
Q

What did Wolfensberger argue re normalisation?

A

Many of the problems with institutions arose from the way they were designed and run.
Residents were dehumanised

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54
Q

Who created Social Role Valorisation?

A

Wolfensberger

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55
Q

What did Wolfensberger suggest via SRV?

A

Poor attitudes towards people with LD could be countered through inclusion and opportunities to take on valued social roles.

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56
Q

How has SRV been developed?

A

Includes key ideas of respect, opportunities, development of competence, independent living and individual choice

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57
Q

What key principles for LD were set out in the Valuing Paper 2001?

A

Rights
Independence
Choice
Inclusion

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58
Q

When did institutionalisation start being phased out?

A

1980s

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59
Q

Factors to protect against isolation in the community of those with LD

A

Development of robust community services with person-centred approach
Collaborative working between primary care, health and social services

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60
Q

Prevalence of LD amongst offenders

A

1-10%

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61
Q

How many offenders have a LD?

A

30%

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62
Q

How many prisoners have IQ <70

A

7%

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63
Q

How many prisoners have IQ <80

A

25%

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64
Q

What did Bradley’s report on review of people with MH problems or LD in CJS state in 2009?

A

More information needs to be available on needs and abilities of people with LD in all stages of the CJS.

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65
Q

What is the aim of criminal justice liaison teams?

A

Link between criminal justice agencies and health and social care services

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66
Q

Prevalence of MH problems in adults with LD

A

30-50%

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67
Q

Prevalence of epilepsy in LD patients

A

22.1%

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68
Q

Poor eyesight in LD patients?

A

19%

8% - general population

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69
Q

How many LD patients are obese?

A

23.6%

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70
Q

Prevalence of schizophrenia in LD patients

A

3%

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71
Q

Prevalence of bipolar in LD patients

A

1.5%

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72
Q

Prevalence of depression in LD patients

A

4%

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73
Q

Prevalence of agoraphobia in LD patients

A

1.5%

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74
Q

Prevalence of OCD in LD patients

A

2.5%

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75
Q

Prevalence of autism in LD patients

A

7%

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76
Q

Prevalence of severe problem behaviour in LD patients?

A

10-15%

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77
Q

Difficulties in diagnosing patients with LD with psychiatric diagnoses

A

Current classifications based on studies that exclude LD patients
Deficits in language and abstract thinking make emotional sx more difficult to identify
MH problems can present differently

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78
Q

What is diagnostic overshadowing?

A

Once a diagnosis is made of a major condition, there is a tendency to attribute all other problems to that diagnosis, leaving co-existing conditions undiagnosed

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79
Q

What is PAS-ADD?

A

Psychiatric Assessment Schedule for Adults with Developmental Disabilities

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80
Q

Structure of PAS-ADD

A

Semi-structured interview

Allows diagnoses under ICD 10

81
Q

What is mini PAS-ADD used for?

A

Determining psychiatric sx without need for interviewing

82
Q

What is PAS-ADD checklist?

A

Questionnaire for carers and staff to help decide if individual needs further assessment

83
Q

Risk of schizophrenia in LD

A

3x increase compared to general population

84
Q

Differences in schizophrenia in LD

A

Age of onset earlier (23)

Severe LD: unexplained aggression, bizarre behaviour, increased mannerism

85
Q

Sx of schizophrenia in moderate LD

A

Hallucinations with persecutory delusions may lead to fear, social withdrawal and aggressive outbursts

86
Q

Which LDs is self-talk common?

A

Downs

Autism

87
Q

Conditions associated with psychosis

A

Ushers
Velo-cardio-facial
Prader WIlli

88
Q

What does Ushers syndrome consist of?

A 
Retinitis pigmentosa
Congenital deafness
Vestibulo-cochlear ataxia
LD - 23%
Psychosis - 15%
89
Q

Who suggested Velo-cardio-facial syndrome might be associated with high rate of psychotic illness?

A

Goldberg et al 1993

90
Q

Clinical features of bipolar in LD

A 
Elevation of mood not expressed verbally
Hyperactivity
Wandering
Mutism
Unexplained challenging behaviour
91
Q

Sx of depression in LD

A 
Vegetative symptoms
Observable depressed mood
Irritability
Self-injurious behaviour
Marked biological sx
92
Q

Behavioural markers of depression in LD patients who are nonverbal

A 
Self-absorption
Regressive behaviours; pica, rocking
Psychomotor retardation
Tearfulness
Refusal to eat
Sleep disturbance
93
Q

Which type of LD might have suicidal thoughts?

A

Borderline-moderate LD

94
Q

What anxiety disorders are children with LD more likely to have?

A

Simple fears e.g. loud noises, the dark

95
Q

Most commonly reported anxiety disorder in LD

A

Simple phobia
Social phobia
GAD

96
Q

What is the most common compulsion in LD patients with OCD

A

Ordering

97
Q

Genetic associations of social anxiety disorder

A

Fragile X

98
Q

Genetic association with OCD and LD

A

Prader Willi

Rubinstein-Taybi

99
Q

Genetic associated with phobias and LD

A

Williams Syndrome

100
Q

Genetic association with LD and compulsive behaviour

A

Cornelia de Lange syndrome

101
Q

Prevalence of CD and ODD in mild/borderline LD

A

30%

102
Q

What influences antisocial behaviour and low verbal intelligence skills in LD?

A

Family

Sociocultural deprivation

103
Q

How many children with autism have LD?

A

75-80%

104
Q

What psychiatric sx is autism associated with?

A

Emotional and behavioural problems
ADHD
OCD

105
Q

How many people with mild LD have ASD?

A

10%

106
Q

How many people with severe LD have ASD?

A

40%

107
Q

Rate of dementia in those aged 30-39 with Downs

A

1 in 50

108
Q

Rate of dementia in those aged 40-49 with Downs

A

1 in 10

109
Q

Rate of dementia in those aged 50-59 with Downs

A

1 in 3

110
Q

Rate of dementia in those over 60 with Downs

A

> 50%

111
Q

Why is diagnosis of dementia difficult in LD?

A

Pre-existing baseline cognitive, functional & behavioural impairment
Dementia presents atypically in LD

112
Q

Behavioural problems noticeable in patients with dementia who have LD

A

Nocturnal confusion
Transient psychosis
Late-onset epilepsy

113
Q

Medical risk factors of dementia in LD

A

Hx of HTN, ischaemic episodes, neurological sx, organic brain damage
FHx of dementia

114
Q

What is the definition of challenging behaviour

A

Culturally abnormal behaviour of such intensity, frequency or duration that physical safety of the person or others is in jeopardy, or behaviour which is to seriously limit use of or result in person being denied access to ordinary community facilities

115
Q

What is challenging behaviour a result of?

A

Communication of ones frustration, anxiety or stress

116
Q

Criteria for clinically significant challenging behaviour

A

Behaviour caused more than minor injuries to themselves or others or destroyed immediate living or working environment
At least weekly behaviours requiring intervention by staff; placed them in danger; caused damage that could not be rectified; caused at least 1 hour of disruption
Behaviour has caused over a few minutes disruption on at least a daily basis

117
Q

Predisposing factors for behavioural problems in LD

A 
Sensory disabilities
Poor communication
Epilepsy
Physical illnesses
Medication
Limited coping strategies
Abuse
Environmental factors
118
Q

How many people with LD have challenging behaviour?

A

7%

119
Q

Rate of challenging behaviour in hospital settings for LD

A

14%

120
Q

Age range of challenging behaviour

A

15-34

121
Q

What is aggressive behaviour often a feature of?

A

Mental disorder such as psychosis, depression or antisocial PD

122
Q

Which genetic conditions show aggressive behaviour?

A

Fragile X
Prader Willi
Klinefelters

123
Q

How many people with LD at some point display self-injurious behaviour?

A

10-50%

124
Q

What influences prevalence of self-injurious behaviour in LD?

A 
Sensory deficits
Profound D
Autism
Limited expressive communication skills
Ambulatory difficulties
125
Q

Which type of LD sees more self-injurious behaviour?

A

Moderate

Severe

126
Q

Most common age group in which self-injurious behaviours occur?

A

10-30

127
Q

Peak ages of self-injurious behaviour in LD

A

15 and 20 years of age

128
Q

Common forms of self-injurious behaviour in LD

A 
Head banging
Banging other body parts
Punching
Biting
Hair pulling
Pica
129
Q

Which genetic conditions increase risk of self-injurious behaviour?

A 
Prader-Willi
Fragile X
Tourettes
Smith-Magenis
Cornelia de Lange
Lesch-Nyhan
130
Q

Who identified that endogenous opioids produce morphine-like effect that accounts for development of some self-injurious behaviour?

A

Wiseley et al 2002

131
Q

What are the hypothesis for self-injurious behaviour and endorphins

A

Self-injurious behaviour stimulates production of endogenous opioids creating analgesic effect and pleasurable feelings and euphoria.

132
Q

What backs up the theory of self-injurious behaviour and endorphins?

A

Opiate blockers and endogenous opiates serotonin and dopamine reduce self-injurious behaviour

133
Q

How many patients with Prader WIlli show self-injury?

A

81%

134
Q

Most prevalent form of self-injury in Prader Willi?

A

Skin picking

135
Q

Most common mode of self-injury in Lesch-Nyan?

A

Biting of lips and fingers

136
Q

How many children aged 1-4 have pica?

A

30%

137
Q

Prevalence of pica in adults with LD in hospital and the community

A

10-20% - hospital

5% - community

138
Q

Risk factors for pica

A

Male
Poor cognitive functioning
Autism
Non-verbal

139
Q

Protective factor for pica?

A

Good level of ADLs

140
Q

Age and pica?

A

Less common in advancing age

141
Q

Treatment of pica

A

Based on behavioural principles

142
Q

What type of SEs are people with LD more susceptible to?

A

CNS side effects

143
Q

What are antipsychotics used for in patients wit LD?

A

Psychosis
Acute behavioural disturbance
Reduce stereotypies

144
Q

What are antidepressants used for in patients with LD?

A

Depression
OCD
Self-injury
Anxiety

145
Q

What are anticonvulsants useful for in LD patients?

A

Episodic dyscontrol
Epilepsy
Rapid cycling mood disorder
Mixed affective states

146
Q

What is Lithium used for in LD patients?

A

Bipolar
Augmentation of antidepressant
Reducing agressive outbursts
Self-harm

147
Q

What is required before Lithium can be started in LD patients

A

Baseline EEG, renal function and FBC

148
Q

Why is extra monitoring required in LD patients on Lithium?

A

It can induce seizures in those with epilepsy

149
Q

Which patients with LD are more prone to lithium toxicity?

A

Pre-existing EEG changes

150
Q

Common causes of dysphagia in LD

A

Medications altering levels of alertness
Medications altering muscle tone/coordination e.g. baclofen, benzos
Antipsychotic medications that delay swallow process or increase salivation

151
Q

Who carried out a survey on therapies used in LD?

A

Nagel and Leiper

152
Q

What did Nagel and Leiper find re therapies used in LD?

A

80% use behavioural interventions
35% use CBT
17% use psychodynamic methods

153
Q

What conditions is CBT helpful for in LD?

A

Anxiety
Depression
Anger management
Sex offending

154
Q

What are behavioural treatments useful for?

A

Teach basic skills like feeding, toileting
Establishing normal behavioural patterns
Learning complex skills like social skills
Changing maladaptive patterns of behaviour

155
Q

What can behavioural analysts be used for?

A

Analyse challenging behaviour and develop management guidelines

156
Q

Behavioural phenotype of Downs in childhood

A

Oppositional, attention deficit problems

157
Q

Behavioural phenotype of Downs in adulthood

A

Early onset dementia

158
Q

Behavioural penotype of Fragile X

A 
Shy
Gaze avoidance
Social anxiety
Schizotypal
Hyperactive
159
Q

Behavioural phenotype of Prader Willi

A 
Food obsession
Insatiable appetite
Hyperphagia
Obesity
Impulsivity
Skin picking
Aggression
OCD
160
Q

Behavioural phenotype of Smith Magenis

A 
Hyperactive
Impulsive
Aggressive
Stereotypic movements
Self0injury
161
Q

Behavioural phenotype of Williams syndrome

A 
Elfin-face
Endearing
Affectionate
Phobias & anxiety
Hyperactivity
Sleep disorder
Hyperacusis
Visuospatial/motor deficits
162
Q

Behavioural phenotype of Cornelia de Lange

A

Self-injury

163
Q

Behavioural phenotype of Lesch Nyhan

A

Compulsive and severe self-injury

164
Q

Behavioural phenotype of velo-cardio facial syndrome

A

Schizophrenia

Schizoaffective disorder

165
Q

Behavioural phenotype of Retts syndrome

A

Stereotypic hand movements

Reduced interest in play in early infancy followed by autistic like sx

166
Q

Behavioural phenotype of Angelman syndrome

A

Puppet like gait
Attraction to water
Happy disposition
Laughing at minimal provocation

167
Q

Behavioural phenotype of Cri du chat

A

Inappropriate laughter

Cat-like cry during infancy

168
Q

Behavioural phenotype of Sanflippo syndrome

A

Prominent sleep disorder

169
Q

Most common genetic cause of LD

A

Downs

170
Q

Major risk factor of having Downs child

A

Maternal age over 40

171
Q

Incidence of Downs

A

1/1000 live births

172
Q

Incidence of Downs in women <30

A

1:2500

173
Q

Incidence of Downs in women >40

A

1:80

174
Q

Incidence of Downs in women aged >45

A

1:32

175
Q

How many Downs are due to non-disjunction

A

92-95%

176
Q

How many Downs are due to Robertsonian translocation

A

2-4%

177
Q

What happens in Robertsonian translocation for Downs

A

Long arm of chromosome 21 is attached to another chromosome, usually 14.

178
Q

What is Mosaic Downs?

A

Mixture of normal and trisomic cell lines

179
Q

How many cases are Mosaic Downs?

A

1-2.5%

180
Q

Signs of Downs in newborn

A 
General hypotonia
Oblique palpebral fissures
Small flattened skull
High cheekbones
Protruding tongue
181
Q

General features of Downs

A 
Short
Overweight
Hypotonia
Upward slanting palpebral fissures
Flat wide nasal bridge
High arched palate
Instability of atlanto-axial joints
Close togethre eyes
Brushfields spots
Epicanthic folds
ow set ears
Simian crease
Syndactyly
Clincodactyly
182
Q

What are Brushfields spots

A

Grey or light yellow spots of the iris

183
Q

What is syndactyly?

A

Webbed fingers

184
Q

What is clinodactyly?

A

Incurving of fingers

185
Q

Congenital GI defects in Downs

A

Oesophageal atresia, Hirschprungs

Inguinal and umbilical hernias

186
Q

Eye defects in Downs

A

Strabismus

Myopia

187
Q

Hearing defects in Downs

A

Otitis media

Sensorineural deafness

188
Q

Endocrine defects in Downs

A

Hypothyroidism

Diabetes

189
Q

Average IQ in Downs

A

50

190
Q

When does mental development appear to progress normally in Downs?

A

Birth to 6 months

191
Q

When do IQ scores decrease in Downs

A

From near normal at 1 year to about 30 at older ages

192
Q

Neuropathology in Downs

A

Changes similar to Alzheimers in those >40

High incidence of senile plaques and neurofibrillary tangles

193
Q

What scale can be used for dx of dementia in Downs

A

Dementia Questionnaire for persons with mental retardation

194
Q

Epilepsy in Downs

A

10%

195
Q

Epilepsy in those >40 years in Downs

A

40%

196
Q

Epilepsy in those with Downs and Alzheimers

A

80%

197
Q

Cause of death <1 year of age in Downs

A

Congenital heart disease

198
Q

When is there highest absolute risk of death in Downs?

A

<1 year

199
Q

Cause of death up to age 30 in Downs

A

Bronchopneumonia

MRCPsych Part B - Al-Rashid Hospital (64 decks)

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