Learning Disabilities

Question 1

What is a learning disability?

Answer 1

Learning disability is a general umbrella term encompassing a range of different conditions that affect the ability of the child to develop new skills.

Learning disabilities are heterogeneous conditions but are defined by 3 core criteria:
lower intellectual ability (usually defined as an IQ of less than 70)
significant impairment of social or adaptive functioning a
onset in childhood

Significant impairment in IQ and adaptive living with onset in childhood

Question 2

What are the types of learning difficulty - which may co-exist with learning disability?

Answer 2

Dyslexia refers to a specific difficulty in reading, writing and spelling.

Dysgraphia refers to a specific difficulty in writing.

Dyspraxia, also known as developmental co-ordination disorder, refers to a specific type of difficulty in physical co-ordination. It is more common in boys. It presents with delayed gross and fine motor skills and a child that appears clumsy.

Auditory processing disorder refers to a specific difficulty in processing auditory information.

Non-verbal learning disability refers to a specific difficulty in processing non-verbal information, such as body language and facial expressions.

Profound and multiple learning disability refers to severe difficulties across multiple areas, often requiring help with all aspects of daily life.

Question 3

Within what IQ is a learning disability classified as mild?

Answer 3

50-69

Question 4

Within what IQ is a learning disability classified as moderate?

Answer 4

35 – 49

Question 5

Within what IQ is a learning disability classified as severe?

Answer 5

20 – 34

Question 6

Within what IQ is a learning disability classified as profound?

Answer 6

Under 20

Question 7

Conditions that have a strong association with learning disability?

Answer 7

Genetic disorders such as Downs syndrome

Antenatal problems, such as fetal alcohol syndrome and maternal chickenpox

Problems at birth, such as prematurity and hypoxic ischaemic encephalopathy

Problems in early childhood, such as meningitis

Autism

Epilepsy

Question 8

What does MDT management of learning disability involve?

Answer 8

Health visitors
Social workers
Schools (children)
Educational psychologists
Paediatricians, GPs and nurses
Occupational therapists
Speech and language therapists

Question 9

What must a patient be able to demonstrate the ability to do to be considered to have capacity?

Answer 9

Understand the decision that needs to be made
Retain the information long enough to make the decision
Weight up the options and the implications of choosing each option
Communicate their decision

Question 10

What factors may increase the risk of learning disability?

Answer 10

A family history of learning disability increases the risk.

Environmental factors such as abuse, neglect, psychological trauma and toxins can all increase the risk.

Question 11

What is diagnostic overshadowing?

Answer 11

Presenting complaint attributed to existing diagnosis rather than considering an additional underlying differential

Question 12

What is autism

Answer 12

Autism is a lifelong neurodevelopmental
condition, the core features of which are
persistent difficulties in social interaction
and communication and the presence of
stereotypic (rigid and repetitive)
behaviours, resistance to change or
restricted interests

Question 13

What genetic factors make someone more prone to both learning disability and autism

Answer 13

Tuberous sclerosis
Fragile X syndrome

Question 14

What is wing and Gould’s Triad

Answer 14

The Triad is made up of three areas of difficulty in autism

SOCIAL COMMUNICATION

not appreciate the social uses or pleasure of communication.
talk at people rather than to them.
expression of own emotions/feelings.
understanding the emotions/beliefs of others.
reading the meaning of gestures, facial expressions or tone of voice.

SOCIAL INTERACTION

inappropriate touching
difficulty understanding / using non verbal body language
personal space issues
unaware of different social relationships / hierarchy
struggle to initiate and maintain relationships
not understanding / reading social cues / rules

SOCIAL IMAGINATION

doesn’t understand others views / feelings/ lack of empathy
difficulties with Theory Of Mind
agitated by changes in routine
unable to generalise information
special interests / obsessive behaviour
literality
rigid thought patterns

Question 15

What problems are patients with learning disabilities more likely to have?

Answer 15

Language difficulties
Sensory impairment
Epilepsy
Mobility problems
Physical ill health
Limited coping strategies
Limited social networks
Limited choices and opportunities
Limited or adverse life experiences (repeated broken relationships, hospital care, multiple moves between foster homes, bullying and harassment)

Question 16

Examples of reasonable adaptations for a person with learning disability?

Answer 16

Allocate more time - for communication and understanding
Talk to informants
Reduce stress - see at home if appropriate, avoid long waits
Help with communication difficulties (SLT)

Question 17

What is challenging behaviour and how can the concept have problems?

Answer 17

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

Vague, numerous meanings, used as a diagnosis so risk of diagnostic overshadowing, encourages consideration of causes rather than reasons

Question 18

What kind of behaviour migh be considered challenging behaviour?

Answer 18

Aggressive behaviour
Self injury
Stereotypic behaviour
Destructive or dangerous behaviour
Loud behaviour that makes others frightened
“Absconding” or behaviour making the individual vulnerable
Inappropriate sexual behaviour
Spitting, smearing

Question 19

Understanding the physical environment for a person with autism

Answer 19

Difficulties in relation to: central coherence, sensory overload (hypo or hyposentivitiy to sensory stimulants such as sound, vision, touch smell, may demonstrated sensory blocking (tune outs, humming)
Making sense of physical space
Understanding structure and routine
Understanding transitions

Question 20

Examination and investigations in challenging behaviours

Answer 20

MSE
physical examination: investigating as appropriate, hearing or eye checks
Observe: environment and interaction with carers, functional analysis (ABC charts etc)
SALT, psychology, OT assesments

Consider capacity and consent

Question 21

Causes and reasons for challenging behaviour

Answer 21

Physical illness
Pain
Mental DISORDERS (mental illness, OD, dementia, autism)
Communication of need or distress
Learnt behaviour
Sensory impairment
Communication difficulties
Behaviour phenotypes
Medications or substances
Developmentally normal
Environment
Abuse

Question 22

NICE recommendations of use of antipsychotics in patients with learning disabilities

Answer 22

Other interventions should be tried first
Only intimidated by specialists
Monitor response and use lowest dose possible

Question 23

MILD LEARNING DISABILITY FEATURES

Answer 23

expressive language is delayed but everyday speech is normal
Able to hold conversation
Comprehension is reasonable, abstract concepts e.g. time are difficult
Good non verbal communication
Full independence in wash and dressing, eating, toileting (normal continence)
Independent living possible
Academic work includes difficulties but able to read, write and do simple maths
Adult work - capable of work demanding practice rather than academic skills
Normal mobility
Social immaturity but otherwise normal social develops
Associated difficulties are as for the non LD population

Question 24

MODERATE LEARNING DISABILITY FEATURES

Answer 24

Expressive language is delayed but simple phrases used
Comprehension is limited to simple phrases and requests
Basic communication skills
Non verbal communication is limited
Self care is limited supervision often required
Often continent
Lives under supervision often with family or supported accommodation
Academic work - limited achievements but may develop some reading, writing and maths skills
Can do simple practical adult work with usoerbision
Delayed mobility but usually fully mobile
Social development is limited but interaction usual
Associated defects: increased risk of neurological disorders, particularly epilepsy

Question 25

SEVERE/PROFOUND LEARNING DISABILITIES

Answer 25

Severe delay in expressive language, with only a few words or absent speech Comprehension very limited Non verbal communication is rudimentary Self care is very limited supervision is always required, mainly in ongoing Live with 24 hour supervision - family, group/nursing home Some simple visousoatial skills Mostly not capable of adult work Frequent MSK abnormalities Social development may be very limited, autism common Associated deficits include neurological disorders and sensory deficits are prevalent Multiple medical problems Inner world largely unavailable to others because of communication difficulty

Question 26

Common physical health problems in people with intellectual disability?

Answer 26

Constipation Dental problems Epilepsy GORD H pylori Infections: ear UTI RTI Mobility problems Obesity Sensory impairments - visual/hearing impairment Cerebral palsy Fracture Incontinence Caries Edentulus Swallowing soreness Injuries - self inflicted, abuse, accidental Syndrome specific associations - Down’s syndrome: sensory, hypothyroid, CVS, respiratory Hypothyroidism

Question 27

Key issues in treating with Physical Health needs in people with intellectual disability

Answer 27

Understanding / communicating symtpoms - changing in behaviour - visual aids/scales Capacity to consent - easy read information / visits - best interest process - IMCA

Question 28

LD history

Answer 28

Demographics details History of presenting complaint *** Psychiatry history *** PMH FHx Medication history and allergies *** Developmental history *** Forensic history Personal history Current social circumstances - functioning, package of care *** Risk assessment ***

Question 29

How is learning disability assessed?

Answer 29

Functional history (ALDs) Developmental history (was the onset in the developmental period) Functioning tests (ABAS - adaptive behaviour assessment) IQ testing for the minority (many pts already had it and more suitable for milder learning disabilities)

Question 30

Learning difficulty vs disability

Answer 30

Difficulty Isolated problems with specific skills (reading, writing, numeracy) Emotional/behavioural problems that may have disrupted schooling Neurodevelopmental disorders such as ASD or ADHD Head injury or cognitive decline in adult life Diasbility Significant impairment in IQ and adaptive living with onset in childhood

Question 31

What all doctors can do to help patients with LD

Answer 31

Treat underlying health conditions - physical or psychiatric disorders - sensory impairments - consider medication side effects Consider social and physical environment - are carers trained, well and supervised - avoiding reinforcing behaviour - any need to adjust environment LD services: referral or call for advice - SLT - psychotherapies, behavioural support etc - assesment and treatment of mental health conditions

Question 32

What 3 criteria define learning disabilities?

Answer 32

- lower intellectual ability (usually defined as an IQ of less than 70) - significant impairment of social or adaptive functioning a - onset in childhood

Question 33

What might be considered adaptive skills?

Answer 33

* Communication * Self-care * Home living * Social skills * Community use * Self-direction * Health and safety * Functional academics * Leisure * Work

Question 34

What IQ is considered borderline for learning disability?

Answer 34

70 - 84

Question 35

Limitations in the use of IQ in determining the classification of learning disability?

Answer 35

These categories have been criticized on the grounds that they omit any measure of social or adaptive functioning. In addition, it has been argued that in practice IQ scores are often uncertain in people with more severe learning disabilities. It is also widely recognised that IQ scores are not fixed throughout life, so provide only an approximate guide to intellectual ability. Accordingly, many health and social care professionals object to subdividing learning disabilities because such subdivisons create labels that are then used to describe people, often inaccurately. Whatever subdivisions are used, a person with a milder degree oflearning disability may need support in only some areas (for example, budgeting, planning and time management). The more severe a person’s learning disability, the more likely the person is to have very limited communication skills and a very significantly reduced ability to learn new skills. Likewise, the more severe the learning disability, the more likely the person is to need support with daily activities such as dressing, washing, eating, and mobility. It is widely agreed that it is important to treat each person as an individual, with their own specific needs, and it is recognised that a broad and detailed assessment of needs is essential.

Question 36

Borderline Intellectual Functioning

Answer 36

* Not classified as LD but still vulnerable due to cognitive status * Living independently * Subtle communication difficulties * High-school ‘drop-out’ or in special education * Difficulty keeping a job, receiving government assistance * At risk of abusive relationships, challenges rearing children

Question 37

What sub-classification of learning disability is most common (75% of LD population, 2% general population)

Answer 37

Mild (IQ of 50-69)

Question 38

Most common inherited cause of LD

Answer 38

Fragile X syndrome

Question 39

Most common known genetic cause of LD

Answer 39

Down's syndrome

Question 40

Most common cause of LD wordwide?

Answer 40

Malnutrition

Question 41

Chromosomal Aberrations - Autosomal abnormalities

Answer 41

Downs syndrome - Trisomy 21 Edwards syndrome - Trisomy 18 Pataus syndrome - Trisomy 13

Question 42

Chromosomal Aberrations - sex chromosomal abnormalities

Answer 42

Fragile X syndrome X q 27-28 Klinefelters syndrome 47 XXY Turners syndrome 45 XO

Question 43

Autosomal dominant conditions associated with LD?

Answer 43

– Tuberous sclerosis – Neurofibromatosis – Sturge–Weber syndrome

Question 44

Autosomal recessive conditions associated with LD?

Answer 44

* Disorders of protein metabolism (hereditary aminoacidurias) – PKU * Disorders of carbohydrate metabolism – Galactosaemia * Disorders of fat metabolism (lipidoses) – Cerebromacular degeneration – Niemann-Pick disease * Mucopolysaccharidoses – Hurlers disease

Question 45

Challenging behaviors?

Answer 45

Some people with learning disabilities display behaviour that challenges. ‘Behaviour that challenges’ describes actions that often result from the interaction between individual and environmental factors. It includes aggression toward others, self-injury, stereotypic behaviour (such as rocking or hand flapping), disruptive or destructive behaviour and withdrawn behaviour. It can also include violence, arson or sexual abuse, thereby bringing the person into contact with the criminal justice system. The most widely used definition of such behaviour is ‘culturally abnormal behaviour(s) of such intensity, frequency or duration that the physical safety of the person or others is likely to be placed in serious jeopardy, or behaviour which is likely to seriously limit use, or result in the person being denied access to, ordinary community facilities .Such behaviours increase the likelihood that restrictive and aversive management strategies will be used and can result in people being excluded from services and from ordinary community life.

Question 46

Deletion syndromes associated with LD?

Answer 46

Velo-cardio-facial (Di George) syndrome - Micro deletion of 22 Smith Magenis - Deletion of 17 Prader-Willi syndrome - Micro deletion of paternal 15q Angelmans syndrome - Micro deletion of maternal 15q Williams syndrome - Micro deletion of 7 Cri du chat syndrome - Partial deletion of 5

Question 47

X-linked disorders?

Answer 47

* Fragile X syndrome * Lesch-Nyhan syndrome * Haemoglobin (HbH) disease with mental retardation * ATR-X syndrome (α–thalassaemia/mental retardation syndrome)

Question 48

Perinatal causes of learning disability?

Answer 48

* Iatrogenic: – Radiation, chemotherapy, medication * Infections – TORCH group * Delivery – Anoxic brain damage – Prematurity * Others – Hyperbilirubinaemia – Foetal Alcohol Syndrome

Question 49

What maternal infections during the perinatal period may cause LD? (TORCH group)

Answer 49

Toxoplasmosis Other (HIV, syphilis, VZ, Zika, fifth disease) Rubella CMV HSV

Question 50

Post natal causes of LD?

Answer 50

* Infections - Encephalitis * Metabolic - Hypoglycaemia * Endocrine - Hypothyroidism (cretinism) * Cerebro-vascular - Thrombo-embolism * Toxins - Lead poisoning * Trauma - Head injury * Neoplasms - Meningioma * Psychosocial - Low socio-economical background, malnutrition, deprivation

Question 51

Multisystem disorder features of Down Syndrome?

Answer 51

Hypotonia (reduced muscle tone) Brachycephaly (small head with a flat back) Short neck Short stature Flattened face and nose Prominent epicanthic folds ( folds of skin covering the medial portion of the eye and eyelid) Upward sloping palpebral fissures (gaps between lower and upper eyelid) Single palmar crease

Question 52

Potential complications/physical associations with Downs Syndrome?

Answer 52

Learning disability Recurrent otitis media Recurrent respiratory tract infection Obesity Obstructive sleep apnoea Deafness. Eustachian tube abnormalities lead to glue ear and conductive hearing loss. Visual problems such myopia, strabismus and cataracts Hypothyroidism (occurs in 10 – 20%) Cardiac defects (affect 1 in 3) particularly ASD, VSD, patent ductus arteriosus and tetralogy of Fallot C spine abnormalities - Atlantoaxial instability Leukaemia is more common in children with Down’s Dementia is more common in adults with Down’s

Question 53

What does the management of Downs Syndrome involve?

Answer 53

Management involves supportive care from the multidisciplinary team to help them meet their needs: Occupational therapy Speech and language therapy Physiotherapy Dietician Paediatrician GP Health visitors Cardiologist for congenital heart disease ENT specialist for ear problems Audiologist for hearing aids Optician for glasses Social services for social care and benefits Additional support with educational needs Charities such as the Down’s Syndrome Association Routine follow up investigations (thyroid, echo, audometry, eye checks)

Question 54

What routine follow up investigations are important for children with Down’s syndrome?

Answer 54

Regular thyroid checks (2 yearly) Echocardiogram to diagnose cardiac defects Regular audiometry for hearing impairment Regular eye checks

Question 55

Psychiatric co-morbidities in LD?

Answer 55

* Depression * Hyperactivity * Conduct disorder * OCD * Autism * Dementia, with unusually early onset

Question 56

Issues in communication faced by patients with LD

Answer 56

* Understanding and processing: – Can mask comprehension difficulties – Difficulty understanding medical terms – Will say ‘yes’ to appease – Suggestible – Sensory issues * Expression: -Content of conversation can be superficial -Likely to contain ‘fillers’ such as ‘as you know’. -Articulation may be difficult -May speak too slowly or loudly, find it difficult to use conventional syntax or grammar * Pragmatics: - Problems understanding and applying social conventions in conversations - Difficulty waiting their turn - Communication style may seem self-centered - Sensitive to criticism or negative approach in conversation * Sensory function: - Hearing impairment - Visual impairment - Other physical disabilities * Cognitive function: - Attention difficulties - Retention difficulties * Environment - Too many people in room - Temperature - Lighting - Sound - Other environmental trigger

Question 57

How might a clincian improve communication with a patient with an LD?

Answer 57

Look at the Communication Passport Environmental alterations Use simple language, check if they have understood, clarify, avoid yes or no questions Phrase questions carefully in order to avoid interrogative suggestibility Give enough time for responses Write things down / draw pictures to aid understanding If the patient uses sign language (BSL, Signalong and Makaton) Minimise wait times and increase consultation time Explain the basics – Who you are, why you are seeing them, how long it will take Speak to patient before speaking to carer * Get supporting information from carer If anxious consider a short break, eat/drink, favourite book/object, offer reassurance, one person takes lead Continuity of care particularly important

Question 58

Why might psychiatric illness present differently in a patient with a LD?

Answer 58

* Limited communication skills * Difficult to assess thought disorder & hallucinations esp. in IQ<50 * Diagnostic overshadowing ( automatically attributed the symptoms or disturbance to LD)

Question 59

Why might a patient with LD be more susceptible to psychiatric illness?

Answer 59

* Limited coping strategies * Limited choices and opportunities * Limited social networks * Limited or adverse life experiences including discrimination, victimisation, multiple moves

Question 60

How might a patient with LD present with psychiatric problems (ie. more atypically)?

Answer 60

* Change in behaviour e.g. self harm * Loss of skills e.g. incontinence * Withdrawal/isolating self * Not doing things they used to enjoy doing * Biological symptoms: – Sleep disturbance – Weight changes

Question 61

Principles of treatment of psychiatric illness in people with Learning Disability

Answer 61

* Same options as general population, but: * Capacity/best interests * Extra support – Accessible information – Psychotherapies may need adapting – Community learning disability team involvement * Go ‘low and slow’ with medication

Question 62

Autism - sensory sensitivities

Answer 62

*Hyper / hypo sensitivity to sensory stimuli – Hyposensitivity more common in LD * Sound most common sense affected * Also vision, touch, smell and taste * Propensity to sensory overload * Sensory blocking or ‘tune outs’ e.g. humming

Question 63

Primary (associated) physical health problems in people with LD?

Answer 63

Hypothyroidism Epilepsy Visual/hearing impairment Cerebral palsy Motor problems

Question 64

Secondary physical health problems in people with LD?

Answer 64

Fracture Obesity GORD Caries Edentulus Incontinence Infection Constipation

Question 65

Epilepsy in people with learning disability?

Answer 65

* More common than the general population (20-30% in comparison to 1%). * Multiple types exist in one person at the same time * More chance of being treatment resistant * Rate of polypharmacy is higher * Mortality rate is high especially because of SUDEP

Question 66

4 key principles of improving health in patients with LD

Answer 66

4 key principles: Inclusion Choice Independence Rights

Question 67

What can help to reduce morbidity and mortality in LD?

Answer 67

* Acute liaison nurses-based in hospitals, facilitating reasonable adjustments * Primary care liaison nurse-based at GP surgeries * Annual Health Check up and Health Action (management) Plan: – Preventative: cancer screening, immunizations – Healthy life style – Active case finding and screening – Regular monitoring (annual health monitoring)

Question 68

Why do people with learning diasbilities have worse health than people without learning disabilities?

Answer 68

Diagnostic overshadowing ( automatically attributed the symptoms or disturbance to LD) Limited social networks Vulnerable to abuse Limited of adverse life expereicnes Institutionalizations Predisposition to both primary and secondary co morbidities

Question 69

Somatic phenotypes of Downs syndrome?

Answer 69

AD CVD Respiratory disease Sensory defects Hypothyroidism

Question 70

Behavioural phenotypes of Prader Willi Syndrome?

Answer 70

Food seeking Poor impulse control Psychiatric illness Self injury (skin picking) Sleep abnormalities

Question 71

Hx in LD?

Answer 71

P/C - verbatim and further detail Collateral hx History of childhood illness, birth history, developmental history, significant adverse events Motor, communication, and social milestones Current functional ability Schooling history Occupation Current medications PMH Past psychiatric hx Forensic hx Specific behavioral history Premorbid personality

Question 72

Mental state examination in LD

Answer 72

Appearance and behavior (level of cooporation, manner, dress/self care, posture/movements, facial expression etc, rapport/appropriateness, gesture/non verbal communication Mood (objective/subjective, biological features, cognitive inc. suicide) Though content (concerns/preoccupations, obsessions/complucions) Abnormal perception Abnormal perception Talk/communication (nature/speed, structure/complexity, repetition, vocabulary, disorders of speech) Cognition [adapted to ability]: orientation, concentraion (serial 7s, months of year fwd/rev, days of week fwd/rev, memory (immediated recall/STM) General knowledge/intelligence (receptive/expressive ability, constructional dyspraxia, execution of sequential task, literacy, numeracy) Insight (Ill? Need rx? Attitude to rx?)