Learning Disability Flashcards
What term does ICD 10 use for LD?
Mental retardation
What term will ICD 11 use for LD?
Intellectual disability
What term does DSM V use for LD?
Intellectual disability
What defines LD?
IQ <70
Presentation in early life - within developmental period
Associated with deficits in social and adaptive functioning
What is the Valuing People 2001’s definition of LD?
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
Define Mild LD
IQ50-69
Define moderate LD
IQ 35-49
Define severe LD
IQ 20-34
Define profound LD
IQ <20
What does adaptive functioning refer to?
How effectively individuals cope with common life demands how well they meet standards of personal independence expected of someone of their age group
How can we measure Adaptive functioning?
Vineland Adaptive Behaviour Scale
Information from more than 1 source
When can borderline intellectual functioning be used according to DSM V?
When an individuals borderline intellectual functioning if the focus of clinical attention or has an impact on the individuals treatment or prognosis
Characteristics of mild LD
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
Characteristics of moderate LD
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
Characteristics of severe LD
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
Characteristics of profound LD
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
Prevalence of LD
1-3%
Highest incidence of LD is in which age group
School-aged children
Peak incidence of LD
10-14 years
M:F ratio of LD
1.5:1
Most common type of LD
Mild - 85%
Moderate - 10%
How many people with LD have severe LD?
4%
How many people with LD have profound LD?
1-2%
How many people with LD have MH issues?
66%
Correlation of MH issues with LD?
Greater severity correlated with increased chance of MH issues
Greater MH issues in those with neurological conditions
Which MH problems occur more commonly in mild LD?
Disruptive and conduct disorders
What MH problems are higher in severe LD?
ASD
Pervasive developmental disorder
What sx can occur in people with LD outside the context of a mental disorder?
Hyperactivity
Self-injurious behaviour
Short attention span
How many people with LD have hearing loss?
25-42%
Age of death of LD compared to general population
13 years (men) and 20 years (women) earlier than general population
Genetic factors linked to LD
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
External prenatal factors linked to LD
Infections
Exposure to medication, alcohol, drugs and toxins
Maternal illness
Perinatal factors linked to LD
Premature infants Low birth weight Infections Problems during delivery Newborn complications
Post-natal factors linked to LD
CNS infections IC tumours Hypoxic brain injury HI Exposure to toxic agents Psychosocial environment
Examples of psychosocial environments linked to LD
Family instability
Frequent moves
Multiple but inadequate carers may deprive infant of emotional relationships leading to failure to thrive
Primary prevention of LD
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
What is secondary prevention of LD?
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
Examples of secondary prevention of LD
Screening and early treatment f congenital hypothyroidism and PKU
Planning or genetic counselling after birth of child with genetic disorder
What is tertiary prevention of?
Helping individual attain their full developmental potential
Examples of tertiary prevention for LD
Biopsychosocial support
Family support
Education
Environmental adjustment and aids
Who introduced the concept of subcultural handicap?
EO Lewis - 1933
What is subcultural mental retardation?
Lower extreme variant of normal IQ distribution in population
What did EO Lewis divide LD into?
Pathological
Subcultural
In which social classes is pathological LD seen in?
Evenly distributed
In which social classes is subcultural LD seen in?
V
Which type of LD is subcultural LD associated with?
Mild
Features of subcultural LD
Mild More common in low socioeconomic groups Family members may have borderline IQ Dysmorphic characters less likely Syndromic features not seen Behavioural phenotypes rare
Features of pathological LD
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
What does subcultural LD imply?
Psychosocial causation
What is normalisation?
Social principle that aims to enable the intellectually disabled to experience normal patterns of daily life
When was principle of normalisation developed and by whom?
In Scandinavia in the 1960s by Bengt Nirje
Who furthered the work of normalisation in LD?
Wolf Wolfensberger
What did Wolfensberger argue re normalisation?
Many of the problems with institutions arose from the way they were designed and run.
Residents were dehumanised
Who created Social Role Valorisation?
Wolfensberger
What did Wolfensberger suggest via SRV?
Poor attitudes towards people with LD could be countered through inclusion and opportunities to take on valued social roles.
How has SRV been developed?
Includes key ideas of respect, opportunities, development of competence, independent living and individual choice
What key principles for LD were set out in the Valuing Paper 2001?
Rights
Independence
Choice
Inclusion
When did institutionalisation start being phased out?
1980s
Factors to protect against isolation in the community of those with LD
Development of robust community services with person-centred approach
Collaborative working between primary care, health and social services
Prevalence of LD amongst offenders
1-10%
How many offenders have a LD?
30%
How many prisoners have IQ <70
7%
How many prisoners have IQ <80
25%
What did Bradley’s report on review of people with MH problems or LD in CJS state in 2009?
More information needs to be available on needs and abilities of people with LD in all stages of the CJS.
What is the aim of criminal justice liaison teams?
Link between criminal justice agencies and health and social care services
Prevalence of MH problems in adults with LD
30-50%
Prevalence of epilepsy in LD patients
22.1%
Poor eyesight in LD patients?
19%
8% - general population
How many LD patients are obese?
23.6%
Prevalence of schizophrenia in LD patients
3%
Prevalence of bipolar in LD patients
1.5%
Prevalence of depression in LD patients
4%
Prevalence of agoraphobia in LD patients
1.5%
Prevalence of OCD in LD patients
2.5%
Prevalence of autism in LD patients
7%
Prevalence of severe problem behaviour in LD patients?
10-15%
Difficulties in diagnosing patients with LD with psychiatric diagnoses
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
What is diagnostic overshadowing?
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
What is PAS-ADD?
Psychiatric Assessment Schedule for Adults with Developmental Disabilities
Structure of PAS-ADD
Semi-structured interview
Allows diagnoses under ICD 10
What is mini PAS-ADD used for?
Determining psychiatric sx without need for interviewing
What is PAS-ADD checklist?
Questionnaire for carers and staff to help decide if individual needs further assessment
Risk of schizophrenia in LD
3x increase compared to general population
Differences in schizophrenia in LD
Age of onset earlier (23)
Severe LD: unexplained aggression, bizarre behaviour, increased mannerism
Sx of schizophrenia in moderate LD
Hallucinations with persecutory delusions may lead to fear, social withdrawal and aggressive outbursts
Which LDs is self-talk common?
Downs
Autism
Conditions associated with psychosis
Ushers
Velo-cardio-facial
Prader WIlli
What does Ushers syndrome consist of?
Retinitis pigmentosa Congenital deafness Vestibulo-cochlear ataxia LD - 23% Psychosis - 15%
Who suggested Velo-cardio-facial syndrome might be associated with high rate of psychotic illness?
Goldberg et al 1993
Clinical features of bipolar in LD
Elevation of mood not expressed verbally Hyperactivity Wandering Mutism Unexplained challenging behaviour
Sx of depression in LD
Vegetative symptoms Observable depressed mood Irritability Self-injurious behaviour Marked biological sx
Behavioural markers of depression in LD patients who are nonverbal
Self-absorption Regressive behaviours; pica, rocking Psychomotor retardation Tearfulness Refusal to eat Sleep disturbance
Which type of LD might have suicidal thoughts?
Borderline-moderate LD
What anxiety disorders are children with LD more likely to have?
Simple fears e.g. loud noises, the dark
Most commonly reported anxiety disorder in LD
Simple phobia
Social phobia
GAD
What is the most common compulsion in LD patients with OCD
Ordering
Genetic associations of social anxiety disorder
Fragile X
Genetic association with OCD and LD
Prader Willi
Rubinstein-Taybi
Genetic associated with phobias and LD
Williams Syndrome
Genetic association with LD and compulsive behaviour
Cornelia de Lange syndrome
Prevalence of CD and ODD in mild/borderline LD
30%
What influences antisocial behaviour and low verbal intelligence skills in LD?
Family
Sociocultural deprivation
How many children with autism have LD?
75-80%
What psychiatric sx is autism associated with?
Emotional and behavioural problems
ADHD
OCD
How many people with mild LD have ASD?
10%
How many people with severe LD have ASD?
40%
Rate of dementia in those aged 30-39 with Downs
1 in 50
Rate of dementia in those aged 40-49 with Downs
1 in 10
Rate of dementia in those aged 50-59 with Downs
1 in 3
Rate of dementia in those over 60 with Downs
> 50%
Why is diagnosis of dementia difficult in LD?
Pre-existing baseline cognitive, functional & behavioural impairment
Dementia presents atypically in LD
Behavioural problems noticeable in patients with dementia who have LD
Nocturnal confusion
Transient psychosis
Late-onset epilepsy
Medical risk factors of dementia in LD
Hx of HTN, ischaemic episodes, neurological sx, organic brain damage
FHx of dementia
What is the definition of challenging behaviour
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
What is challenging behaviour a result of?
Communication of ones frustration, anxiety or stress
Criteria for clinically significant challenging behaviour
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
Predisposing factors for behavioural problems in LD
Sensory disabilities Poor communication Epilepsy Physical illnesses Medication Limited coping strategies Abuse Environmental factors
How many people with LD have challenging behaviour?
7%
Rate of challenging behaviour in hospital settings for LD
14%
Age range of challenging behaviour
15-34
What is aggressive behaviour often a feature of?
Mental disorder such as psychosis, depression or antisocial PD
Which genetic conditions show aggressive behaviour?
Fragile X
Prader Willi
Klinefelters
How many people with LD at some point display self-injurious behaviour?
10-50%
What influences prevalence of self-injurious behaviour in LD?
Sensory deficits Profound D Autism Limited expressive communication skills Ambulatory difficulties
Which type of LD sees more self-injurious behaviour?
Moderate
Severe
Most common age group in which self-injurious behaviours occur?
10-30
Peak ages of self-injurious behaviour in LD
15 and 20 years of age
Common forms of self-injurious behaviour in LD
Head banging Banging other body parts Punching Biting Hair pulling Pica
Which genetic conditions increase risk of self-injurious behaviour?
Prader-Willi Fragile X Tourettes Smith-Magenis Cornelia de Lange Lesch-Nyhan
Who identified that endogenous opioids produce morphine-like effect that accounts for development of some self-injurious behaviour?
Wiseley et al 2002
What are the hypothesis for self-injurious behaviour and endorphins
Self-injurious behaviour stimulates production of endogenous opioids creating analgesic effect and pleasurable feelings and euphoria.
What backs up the theory of self-injurious behaviour and endorphins?
Opiate blockers and endogenous opiates serotonin and dopamine reduce self-injurious behaviour
How many patients with Prader WIlli show self-injury?
81%
Most prevalent form of self-injury in Prader Willi?
Skin picking
Most common mode of self-injury in Lesch-Nyan?
Biting of lips and fingers
How many children aged 1-4 have pica?
30%
Prevalence of pica in adults with LD in hospital and the community
10-20% - hospital
5% - community
Risk factors for pica
Male
Poor cognitive functioning
Autism
Non-verbal
Protective factor for pica?
Good level of ADLs
Age and pica?
Less common in advancing age
Treatment of pica
Based on behavioural principles
What type of SEs are people with LD more susceptible to?
CNS side effects
What are antipsychotics used for in patients wit LD?
Psychosis
Acute behavioural disturbance
Reduce stereotypies
What are antidepressants used for in patients with LD?
Depression
OCD
Self-injury
Anxiety
What are anticonvulsants useful for in LD patients?
Episodic dyscontrol
Epilepsy
Rapid cycling mood disorder
Mixed affective states
What is Lithium used for in LD patients?
Bipolar
Augmentation of antidepressant
Reducing agressive outbursts
Self-harm
What is required before Lithium can be started in LD patients
Baseline EEG, renal function and FBC
Why is extra monitoring required in LD patients on Lithium?
It can induce seizures in those with epilepsy
Which patients with LD are more prone to lithium toxicity?
Pre-existing EEG changes
Common causes of dysphagia in LD
Medications altering levels of alertness
Medications altering muscle tone/coordination e.g. baclofen, benzos
Antipsychotic medications that delay swallow process or increase salivation
Who carried out a survey on therapies used in LD?
Nagel and Leiper
What did Nagel and Leiper find re therapies used in LD?
80% use behavioural interventions
35% use CBT
17% use psychodynamic methods
What conditions is CBT helpful for in LD?
Anxiety
Depression
Anger management
Sex offending
What are behavioural treatments useful for?
Teach basic skills like feeding, toileting
Establishing normal behavioural patterns
Learning complex skills like social skills
Changing maladaptive patterns of behaviour
What can behavioural analysts be used for?
Analyse challenging behaviour and develop management guidelines
Behavioural phenotype of Downs in childhood
Oppositional, attention deficit problems
Behavioural phenotype of Downs in adulthood
Early onset dementia
Behavioural penotype of Fragile X
Shy Gaze avoidance Social anxiety Schizotypal Hyperactive
Behavioural phenotype of Prader Willi
Food obsession Insatiable appetite Hyperphagia Obesity Impulsivity Skin picking Aggression OCD
Behavioural phenotype of Smith Magenis
Hyperactive Impulsive Aggressive Stereotypic movements Self0injury
Behavioural phenotype of Williams syndrome
Elfin-face Endearing Affectionate Phobias & anxiety Hyperactivity Sleep disorder Hyperacusis Visuospatial/motor deficits
Behavioural phenotype of Cornelia de Lange
Self-injury
Behavioural phenotype of Lesch Nyhan
Compulsive and severe self-injury
Behavioural phenotype of velo-cardio facial syndrome
Schizophrenia
Schizoaffective disorder
Behavioural phenotype of Retts syndrome
Stereotypic hand movements
Reduced interest in play in early infancy followed by autistic like sx
Behavioural phenotype of Angelman syndrome
Puppet like gait
Attraction to water
Happy disposition
Laughing at minimal provocation
Behavioural phenotype of Cri du chat
Inappropriate laughter
Cat-like cry during infancy
Behavioural phenotype of Sanflippo syndrome
Prominent sleep disorder
Most common genetic cause of LD
Downs
Major risk factor of having Downs child
Maternal age over 40
Incidence of Downs
1/1000 live births
Incidence of Downs in women <30
1:2500
Incidence of Downs in women >40
1:80
Incidence of Downs in women aged >45
1:32
How many Downs are due to non-disjunction
92-95%
How many Downs are due to Robertsonian translocation
2-4%
What happens in Robertsonian translocation for Downs
Long arm of chromosome 21 is attached to another chromosome, usually 14.
What is Mosaic Downs?
Mixture of normal and trisomic cell lines
How many cases are Mosaic Downs?
1-2.5%
Signs of Downs in newborn
General hypotonia Oblique palpebral fissures Small flattened skull High cheekbones Protruding tongue
General features of Downs
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
What are Brushfields spots
Grey or light yellow spots of the iris
What is syndactyly?
Webbed fingers
What is clinodactyly?
Incurving of fingers
Congenital GI defects in Downs
Oesophageal atresia, Hirschprungs
Inguinal and umbilical hernias
Eye defects in Downs
Strabismus
Myopia
Hearing defects in Downs
Otitis media
Sensorineural deafness
Endocrine defects in Downs
Hypothyroidism
Diabetes
Average IQ in Downs
50
When does mental development appear to progress normally in Downs?
Birth to 6 months
When do IQ scores decrease in Downs
From near normal at 1 year to about 30 at older ages
Neuropathology in Downs
Changes similar to Alzheimers in those >40
High incidence of senile plaques and neurofibrillary tangles
What scale can be used for dx of dementia in Downs
Dementia Questionnaire for persons with mental retardation
Epilepsy in Downs
10%
Epilepsy in those >40 years in Downs
40%
Epilepsy in those with Downs and Alzheimers
80%
Cause of death <1 year of age in Downs
Congenital heart disease
When is there highest absolute risk of death in Downs?
<1 year
Cause of death up to age 30 in Downs
Bronchopneumonia
