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LEARNING DISABILITIES
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1.
Introduction
In people of working age, learning disability is the commonest disability in the UK.1
1.1
Definition
Learning disability consists of three main components:
1. Impaired
intellectual
function.
2. Onset at birth or in early childhood.
3. Impaired coping and social skills.
Learning disability’s intellectual effects have a particular impact on language and
numeracy skills. Physical disability, epilepsy, mental illness, incontinence and
immobility are important associated problems.
The terminology used to describe learning disability changes with fashion, political
correctness, and attempts to avoid stigmatising terms. In the UK, the term “learning
disability” is used, but the expression “mental retardation” is preferred by the W.H.O.
All the terms mean “arrested or incomplete development of the mind”.2
Intelligence is a broad concept, including the ability to reason, comprehend and
make judgements. Psychometric testing leads to an IQ (intelligence quotient).
Intelligence is distributed in the population along a normal distribution curve.
An IQ of 100 is the centre of the distribution curve. IQs of 70 and over are
considered normal.
IQ scores are used to define categories of learning disability:
1.2
Classification of Learning Disability
EQUIVALENT MENTAL
Classification IQ
Proportion4
AGE3
Mild
50 – 69
8 – 12 years
85%
Moderate
35 – 49
3 – 8 years
10%
Severe
20 – 35
1 – 3 years
3.5%
Profound
< 20
< 1 year
1.5%
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1.3
Functional Effects of Learning Disability
There is considerable overlap of functional ability between the categories of learning
disability.
1.3.1
Mild Learning Disability
This is not usually associated with abnormalities in appearance or behaviour.
Language, sensory and motor abnormalities are mild or absent. Because it is mild,
the problem is not usually apparent until school age. Adults may have difficulty
coping with stress, and may need support with complex functioning such as
parenting and handling their finances. However, the majority are able to live
independently in the community and manage some form of employment.5
1.3.2
Moderate Learning Disability
People with moderate learning disability are rarely able to live independently, but
they may learn to wash, dress and feed themselves. This group has limited but
useful language skills. However, receptive skills tend to be better than expressive
skills, leading to a high incidence of frustration and challenging behaviour. Help is
needed with road sense and finances. Moderate learning disability is often
associated with epilepsy, neurological, and other physical disabilities.
1.3.3
Severe and Profound Learning Disability
This group of claimants have very limited verbal and self-care skills. Severe physical
handicaps are very common. Epilepsy affects 33%, incontinence 10% and inability
to walk 15%. Behavioural disturbance such as purposeless, self-harming or
inappropriate sexual behaviour becomes more common with increasing severity of
learning disability. It occurs in up to 40% of children and 20% of adults in these
categories.5,6
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2.
Prevalence
In England, it is estimated that there are 1.2 million people (2% of the population)
with mild or moderate learning disability, and about 120,000 adults with severe or
profound learning disability.3,7,8,9 About 600,000 require input from specialist
services.9
There are more males with learning disability than females, in a ratio of 1.5:1.
3
This is probably due to the greater prevalence of sex-linked inherited learning
disability in males.
Learning disability is more common in developing countries because of a higher
incidence of birth injury and anoxia, and early childhood infections.2
Mild learning disability is more common in lower socio-economic groups.10
The incidence of severe learning disability is falling due to improvements in
prevention.3
In the UK, the amount spent on services for learning disability is about £3 billion
per annum.11
In the UK, about 7,000 people with learning disability are in
supported
employment.12
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3.
Aetiology
There is an obvious cause for mild learning disability in about half of cases.9 In the
remainder, a combination of social, educational and emotional deprivation are the
main contributory factors.5
As the severity of learning disability increases, there is a rising chance of finding a
specific cause. In severe learning disability, 80% have evidence of organic brain
damage.3,9
Birth injury accounts for 10% of those diagnosed with learning disability.4 It is
estimated that up to 5% of learning disability is due to physical and emotional child
abuse.9
3.1
Genetic Factors
Chromosomal
abnormalities.
Down’s and Fragile X Syndromes are the commonest chromosomal causes of
learning disability.9
Metabolic disorders: Phenylketonuria and Tay-Sachs Disease (recessive) are
examples.
Tuberous Sclerosis (autosomal dominant).
3.2
Intrauterine Factors
Malnutrition.
Fetal alcohol syndrome.
Infections: Rubella, Toxoplasmosis and Cytomegalovirus infections are
examples.
Pre-eclampsia.
3.3
Perinatal Factors
Prematurity.
Hypoxia.
Intracerebral
bleed.
Neonatal
infections.
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3.4
Postnatal Factors
Meningitis and encephalitis.
Head Injury (accidental or physical abuse).
Malnutrition.
Toxins. (e.g. Lead).
Hypothyroidism.
3.5
Environmental Factors
Malnutrition (uncommon in developed countries.)
Socio-economic
deprivation.
Emotional
abuse.
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4.
Co-morbidity
Learning disability is associated with a high prevalence of epilepsy and mental
health problems.13
4.1
Physical Co-morbidity
The more severe the learning disability, the higher the prevalence of serious
physical disabilities.14
Prevalence in severe
Prevalence in mild
Physical Impairment
learning disability
learning disability
Cerebral Palsy
20%
8%
Epilepsy 35%
15%
Severe Visual Impairment
8%
5%
Severe Hearing
9% 4.5%
Impairment
For a child with learning disability, the prognosis is poorer when there are multiple
problems, especially those interfering with social relationships, and those inhibiting
learning and play.13
4.1.1
Cerebral Palsy
Cerebral Palsy causes spasticity and physical disability, but may be associated with
normal intelligence.5
4.1.2
Epilepsy
Making a diagnosis of partial epilepsy can be difficult, as patients with learning
disability may be unable to describe their symptoms. Treatment of their epilepsy is
complicated because they may have difficulty in describing side effects. To address
these problems, there are national specialist education, treatment and assessment
centres for adults with learning disability, including: the Chalfont Centre for Epilepsy
and the David Lewis Centre.
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4.2
Psychiatric Co-morbidity
40% of those diagnosed with learning disability also have a mental illness.4,9 The
risks increase with the severity of the learning disability.9 In adults with learning
disability, schizophrenia, mood disorders, personality disorder and neurotic
disorders are all more common.3,9,14
Learning disability + personality or behavioural disorder – 25 - 30%.
Learning disability + mood disorder – 10 -15%.
Learning disability + obsessive-compulsive disorder – 4%.
Learning disability + schizophrenia – 3%.
Learning disability + dementia – 3%.
Because of difficulty with communication, those with learning disability may not have
the skills to express and describe what they are experiencing, so presentations may
differ from those with a normal IQ. The observation of behavioural changes such as
psychomotor retardation, agitation and possible responses to hallucinations can be
helpful, and information from family and carers is especially important.9
Co-morbid
Effects of Learning Disability
Condition
Delusions are less elaborate and hallucinations are simple
Schizophrenia
and repetitive.
Patients are less likely to express depressive ideas. Carers
Depression
may observe sadness or alterations in behaviour or sleep
pattern. The suicide rate is lower.4
Adjustment
Common when there are changes to routine, such as loss of
Disorders
carers.
Phobias
Easily overlooked because of language difficulties.
Obsessive-
More frequent than in the general population. Over-eating
Compulsive
and unusual dietary preferences are frequent.
Disorder
Personality Disorder Common, and can lead to greater management problems.
Tends to occur at a younger age in those with learning
Dementia
disability.
Sleep Disorder
Common, and may cause significant stress in carers.
Mild learning disability is associated with a higher rate than in
Criminal Behaviour
the general population. Arson and sexual offences
(exhibitionism) are particularly common.
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5.
Autistic Spectrum Disorders
The term 'autistic spectrum disorders' is used to describe a group of developmental
conditions that affect the way the brain processes information. People with autism
are severely affected, while Asperger syndrome describes people at the higher
functioning end of the autistic spectrum.
5.1
Autism
Autism is a lifelong developmental disability that affects the way a person
communicates and relates to people around them. The core feature of autistic
spectrum disorders is difficulty in “making sense of the world.” An autistic person
experiences a confusing mass of events, people, places, sounds and sights without
order or meaning. Thus, a lot of time is spent trying to “work out the pattern behind
everything”.15
The range of intellectual ability extends from severe learning disability to above
average IQ. 15
5.1.1
Epidemiology
The prevalence of autism is about 5 in 10,000.3,9,15,16 More males than females are
diagnosed with autism in a ratio of 4:1.16 The prevalence does not vary with socio-
economic class. 3,14,17
5.1.2
Aetiology
Twin and family studies suggest a genetic component to the development of
autism.3 A family that already has one autistic child has a 3% risk of having
another.15 Autism is also strongly associated with organic causes of learning
disability such as complications of pregnancy and birth. Neurochemical studies of
autism have reported abnormalities in dopamine and serotonin metabolism.
5.1.3
Core Clinical Features
Autism is usually apparent by the age of 3 years. 9
Abnormal Social Interaction.
There is failure to initiate, develop or respond to social situations, poor grasp of
nonverbal social cues and avoidance of eye contact, so people with autism may
appear aloof and indifferent.
Impaired Language and Communication Skills.
This includes delayed or impaired language development, difficulty maintaining
conversation, lack of creativity and lack of imaginative play.
Restricted and Repetitive Behaviour.
This includes a “rigid routine,” interests and activities that have a preoccupation
with dates or numbers, and a stereotyped behaviour pattern such as hand
flapping, nodding or rocking.
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Because they can concentrate on a single task for long periods, people with autism
can become very proficient in those tasks that interest them. About 10% of children
with autistic spectrum disorders have a special skill at a much higher level than the
rest of their abilities - for example, music, art, numerical calculations or jigsaw
puzzles.15
5.1.4
Complications
75% have learning disability.3,9
20% have fragile X syndrome and 6% have tuberous sclerosis.16
25% develop epilepsy.17
Psychiatric co-morbidity is common.
Problems with communication and difficulty in adjusting to change often cause
frustration, which may result in aggressive or challenging behaviour. It is best to talk
to the autistic person in unambiguous terms, and maintain a routine. When
challenging behaviour does occur, it can sometimes be channelled into harmless
activities such as shredding paper or punching a pillow.15
5.1.5
Prognosis
Autism typically runs a steady lifelong course.9 Specialised education and support
aim to help a child to maximise their skills and achieve their full potential. Although
some autistic adults learn to adapt partially to their disability, only 11% gain jobs on
the open market, and only 15% achieve independent living.9,17,18
5.2
Asperger Syndrome
Asperger syndrome has the same core features as autism, but is at the high
functioning end of the autistic spectrum. People with Asperger syndrome find it hard
to read social signals, and as a result, they find it difficult to communicate and
interact with others.15
People with Asperger syndrome can speak fluently, but they may not understand the
reactions of the people listening to them. They may talk on and on, regardless of the
listener's interest, or they may appear insensitive to the listener’s feelings. Jokes,
turns of phrase and metaphors can be confusing to a person with Asperger
syndrome, because they tend to think in an over-literal way.
People with Asperger syndrome often develop an obsessive interest in memorising
facts about a special subject, such as train timetables. They also prefer a set
routine. Any unexpected happening or change in the routine can upset them.
Children with Asperger syndrome usually have normal or above average
intelligence, and they attend mainstream school. Many seem clumsy: they have poor
coordination and difficulties with fine motor control. Adults with Asperger syndrome
can be considered eccentric, and may resemble those with a schizoid or anankastic
personality disorder. (See the protocol
Personality Disorders for further
information.)
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These problems of social interaction are the main cause of disability, and may make
it difficult for a person with Asperger syndrome to cope in a working environment.15
The prevalence of Asperger syndrome is estimated to be about 36 per 10,000 in the
UK.15
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6.
Down’s Syndrome
Down’s syndrome is the commonest specific cause of learning disability.
6.1
Epidemiology
The incidence of Down’s syndrome is falling because of increased antenatal
detection.
Overall, it occurs in 1 in 650 live births. For mothers aged 20 - 25 the incidence is 1
in 2,000 live births, increasing to 1 in 45 for a mother over 45.4
6.2
Aetiology
The vast majority of cases of Down’s syndrome are caused by trisomy 21.
6.3
Clinical Features
Down’s syndrome is associated with a typical facial appearance and short stature.
85% have moderate or severe learning disability.9
5% have autistic features and 25% have hyperkinetic disorder.4,9
Physical health problems are associated with Down’s syndrome:
Congenital heart disease – 40%, of which half require surgery.9,19
Visual and hearing impairment – 50%.19
Hypothyroidism – 30%.19
Oesophageal and duodenal atresia.
20% increased risk of developing infections and leukaemia.20
Atlanto-axial
instability.
Cognitive decline and dementia (similar to Alzheimer’s disease) occurs 30-40 years
earlier than in the general population, and affects 25% of people with Down’s
syndrome.20
6.4
Prognosis
Some live independently in sheltered accommodation, and some find sheltered or
standard employment.20
With improved medical care, survival has improved. At the beginning of the 20th
century, life expectancy was less than 10 years. Now it is close to 50 years, with a
quarter living beyond the age of 50.4
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7.
Fragile X Syndrome
Fragile X syndrome is the second commonest cause of moderate and severe
learning disability after Down’s syndrome, accounting for 20 - 30% of learning
disabilities.
Fragile X syndrome is the commonest
directly inherited cause of learning disability.
7.1
Epidemiology
Fragile X syndrome occurs in 1 in 1000 males. A milder form affects 1 in 2500 girls,
who may have normal intelligence.4,21
7.2
Clinical Features
Fragile X syndrome is associated with a typical appearance, including an elongated
face, large ears and blue eyes. Other features include flat feet, macro-orchidism and
hyper-flexible joints. The physical features usually develop by puberty, with infants
often appearing normal. Females only tend to exhibit large or prominent ears.
The degree of learning disability is similar to that in Down’s syndrome, 80% of males
having an IQ less than 70.22,23 People with fragile X syndrome have particular
problems with language skills. They also have an aversion to loud noise and strong
smells. Difficulty adjusting to change, (particularly environmental change), and mood
instability are also prominent features.22
Boys tend to have more behaviour problems and girls tend to be shy and socially
withdrawn. Girls often suffer from anxiety and depression.
Fragile X syndrome is associated with autism and ADHD.9
80% have mitral valve disease and 20% have seizures. Recurrent ear infections and
squint are more common, and there is an increased incidence of connective tissue
disorders.9,22
7.3
Prognosis
Behavioural problems tend to improve with age.
Some people with fragile X syndrome are employed and are able to live
independently. The majority need day-to-day supervision. They work in a sheltered
environment, and either live at home or in supported accommodation.
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8.
Attention Deficit Hyperactivity Disorder – ADHD
ADHD does not have a significant effect on an individual’s intelligence.24 People
with ADHD are creative and intuitive, but their full potential may not be achieved
because of poor concentration. If untreated, ADHD interferes with educational and
social development and predisposes to mental illness.
8.1
Epidemiology
The prevalence of ADHD varies in different countries due to different diagnostic
practices. For example, in the UK it is about 1%, but as high as 5% in the US.3 More
males are diagnosed with ADHD than females in a ratio of 9:1.3
It occurs in all cultures and all social classes.25
8.2
Aetiology
The aetiology of ADHD is a mixture of genetic (prevalence 5 times higher in
relatives), medical (as a result of encephalitis) and emotional (child abuse)
causes.3,4,26 Lead poisoning and food additives are also thought to play a role.4
8.3
Core Clinical Features
Unless it is very severe, ADHD is not usually recognised until the child has started
school.3
Inattention
Easily distractible, forgetful, difficulty sustaining tasks such as play, learning and
work.
Overactivity
Fidgety, reckless, socially disinhibited, inappropriately active, talking excessively.
Impulsivity
Interrupts and intrudes, unable to “wait their turn.”
People with ADHD tend to be clumsy, accident-prone and get into trouble with
parents and teachers. Others learn to avoid them, so they become socially isolated.9
8.4
Complications
20% of children diagnosed with ADHD have learning difficulties, including speech,
language, social and relationship problems.25
A significant number of adults labelled as suffering from personality disorder are
actually suffering from ADHD, and as such are likely to respond to medication.26
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8.5
Treatment
Ritalin (methylphenidate) is an amphetamine-like stimulant. It has the paradoxical
effects of decreasing activity level and improving attention. It helps to improve
academic performance and relationships.27
Medication produces a short-lived improvement after each dose, but it is not a
permanent cure.24,28
8.6
Prognosis
By the second decade, the problems of impulsivity and inattention tend to improve,
even without medication. However, the learning difficulties caused by ADHD in
childhood have long-term consequences.26 About 60% of adults continue to
experience problems.25,28 There are high levels of psychiatric co-morbidity:3,25
Psychiatric co-morbidity in adults:
ADHD + mood disorders – 18 - 59%
ADHD + anxiety – 10 - 50%
ADHD + antisocial personality disorder – 12%
ADHD + substance abuse – 20 - 30%
Adults with ADHD are most likely to succeed in employment where it provides a
stimulating, yet structured, environment.
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9.
Management of Learning Disability
In a specialist child development clinic, the assessment of learning disability begins
with interviews with the patient, their parents and other carers. The family history,
obstetric history, developmental milestones and schooling history are particularly
important. A physical examination includes assessments of vision and hearing.
Standardised measures of intelligence, language, motor and social skills complete
the assessment.
When children with learning disabilities suffer psychiatric symptoms, drug treatments
are used less often than in adult psychiatry. The emphasis is on psychological
treatments and working with the whole family to solve problems. In adults with
learning disabilities, the treatment of medical and psychiatric problems is similar to
that in other patients, but some forms of psychological treatment may not be
appropriate, depending on the patient’s intellectual abilities.
9.1
Education
Children with learning disability are usually educated within mainstream schools. An
educational psychologist will assess their educational needs, and in the most severe
cases, (1% of children), may recommend that they attend a school specialising in
the education of children with learning disabilities: Special Schooling. This
assessment of special educational needs is called a “Statement of Need.” The
Statement is very significant because the local education authority is obliged to
provide the services that it recommends.29
A typical UK school might include 3% of pupils with a Statement of Need and a
further 17% within the less severe category: Special Educational Needs.29 Children
with mild learning disability spend most of their time as part of the main class, but
receive additional individual and/or small group teaching.
Since 1992, there has been provision for college education for those with learning
disabilities up to the age of 25. Courses include literacy skills, development of
personal relationships and leisure activities.12
(The classification in this protocol uses medical definitions of mild, moderate, severe
and profound learning disability. In other contexts, the terms may have different
meaning, and this should be remembered when interpreting medical evidence from
an educational professional.)
9.2
Family Support
The birth of a child with disabilities puts great strain on most families. The parents
may grieve for the loss of their anticipated healthy child. The additional physical and
financial burdens of caring for their child can lead to marital disharmony. However,
the majority of families eventually adjust, with support from healthcare professionals,
social workers, teachers, family, friends and self-help groups.
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9.3
Creative Therapies
Activities such as art, music and drama can help a person with learning disabilities
to express themselves.
9.4
Employment Opportunities
Sheltered workplaces allow those with practical skills to develop a routine and a role
in society. Opportunities for supported employment can be accessed via the
Disability Employment Adviser at Jobcentre Plus. Other employment agencies
include: The Shaw Trust, Remploy and the Mencap Pathway Employment Scheme.
The 1995 Disability Discrimination Act protects employees with learning disability.
9.5
Accommodation and Supervision
A large majority of people with learning disability are able to live independently or
with their families.5 Institutional care is only required for a minority of adults.
Typically, it is now provided in small community units. Periods of respite care can
provide an essential break for carers.
40% of parents caring for a child with learning disability are over the age of 60.
Projects are exploring how to support this group of people with learning disability in
their transition to new living arrangements.30
9.6
Key Worker
The primary care team, psychiatrists with a special interest in learning disability and
social workers aim to coordinate their efforts to provide for the health and social
needs of adults with learning disability. Many agencies can be involved, and it has
been found that the appointment of a key worker can help a person with learning
disability to gain assistance when it is needed.14
9.7
Psychological Therapies
Suitably modified behavioural and cognitive techniques can be successfully applied
to patients with learning disabilities. For example, problems such as wetting and
soiling, impulsive behaviour and phobias can be treated by behavioural therapy.
This approach works by offering praise and rewards for practising the desired
behaviours.9,14
9.8
Drug Treatments
Sedative antipsychotics are occasionally used as an adjunct to behavioural
strategies in managing severe behavioural disorders, although evidence of benefit is
lacking.3,31
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9.9
Self-Help Groups
People with learning disabilities and their carers can share information and gain
support from others with similar experiences. These groups also aim to influence the
provision of services and facilities for disabled people.
9.10 Prevention
The availability of genetic counselling and antenatal diagnosis of conditions such as
Down’s syndrome has led to a reduction in the incidence of some learning
disabilities. Improved perinatal care reduces the risk of brain damage. The early
detection of hormonal or metabolic problems such as hypothyroidism or
phenylketonuria allows treatment before learning disability sets in.5
There is some evidence that educational intervention in children of mothers with mild
learning disability may improve their educational performance, (though not IQ), and
reduce the risk of conduct disorders.5
9.11 Outcome
Overall, people with learning disability are living longer and enjoying a better quality
of life because of improvements in health and social provision.32
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10. Main Disabling Effects
People with learning disability all have impaired performance of intellectual tasks
such as learning, short-term memory, use of concepts and problem solving. Some
may have problems with spatial awareness, which may cause difficulty with
dressing, for example. Poor language skills cause problems with social interaction,
and are strongly associated with behavioural disorders. Additional disabilities such
as epilepsy, impaired vision and hearing and physical problems often compound the
disabling effects of learning disability. Learning disability runs a chronic life-long
course.
10.1 Assessing the Claimant
The assessment should be made using all the information available. This includes
information from the claimant’s file, informal observations, medical history, typical
day, appropriate physical examination, and assessment of their mental state.
Some causes of learning disability are associated with particular facial appearances
or physical features. When present, these may indicate the likely range of disability.
When it is available, information from family or carers accompanying the claimant
will be valuable. However, sometimes carers can be over-protective, and it is
essential to develop a rapport with the claimant so that their language and social
skills can be observed.
10.1.1 Mild Learning Disability
Claimants who are suffering from mild learning disability will have attended
mainstream schooling. They may be living in their own home, with their family, or in
supported accommodation. They will be able to do most things for themselves,
although they are likely to need help with managing their finances. Their typical day
history will reveal little or no restriction in their activities of daily living: they will be
able to travel independently on public transport, do their shopping, enjoy contact
with friends and family, and develop interests and hobbies.
10.1.2 Moderate, Severe and Profound Learning Disability
Claimants within these categories of learning disability will not be able to live
independently. .
Certain syndromes always cause severe learning disability. Many of these are
described in the table in
Section 12. Common conditions such as autism and
Down’s syndrome encompass a spectrum of severity, and these cases should be
assessed individually.
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10.2 Helpful Questions for Assessing the Disabling Effects of
Learning Disability
Who is accompanying the claimant at the assessment? Was their presence
necessary? Those with mild learning disability may be able to attend an
examination centre alone or cope with an assessment at home without a
companion.
Does the claimant also have a physical disability, epilepsy, or a mental illness?
The combined effects of multiple disabilities are likely to be severe.
What sort of education has the claimant had? Receipt of a “Statement of Need”
is significant, while attendance at a “Special School” indicates a very high level
of learning difficulties.
Where is the claimant living? Do they have a home of their own, are they living
with their family, in supported accommodation, or in long-term residential care?
Is the claimant currently attending support groups or college for further education
courses in life skills and independent living? What is the planned outcome: are
they aiming to live independently, or to gain work in a sheltered or open
environment?
Is the claimant able to initiate and complete household tasks? Can they plan and
prepare a meal? Can they go shopping independently? What is the change from
£1 if a bag of sweets costs 75p?
How did the claimant travel to the examination centre? Some claimants will be
able to travel alone on familiar routes, but would not be able to cope with a
journey to an unfamiliar destination.
10.3 How to Assess the Disabling Effects of ADHD
ADHD is a treatable condition, which tends to improve in adult life. Each case should
be assessed individually, with special emphasis on the typical day and assessment
of the mental state.
10.4 IB-PCA Considerations
The IB-PCA assesses the ability to work in the open jobs market, not sheltered
placements.
The criterion for exemption due to severe learning disability is: “
a condition which
results from the arrested or incomplete development of the brain, or severe damage
to the brain and which involves severe impairment of intelligence and social
functioning”.33
This can be interpreted as a person who is incapable of living independently. If
sufficient medical evidence had been available, these claimants would have been
exempted or accepted at the scrutiny stage. Note that the definition of moderate
learning disability in this protocol may cause the individual to fall within the category
of “severe learning disability” for exemption purposes.
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Severe learning disability may include one or more of:
An inability to learn more than the most basic skills such as feeding, dressing
and using the toilet.
The need for help with some or all bodily functions.
A failure to be aware of dangers, thus requiring supervision.
Severe behaviour problems that require supervision, such as self-harm or
violence.
It is very rare that a claimant with ADHD fulfils the criteria for exemption on the
grounds of severe learning disability.
For the purposes of the IB-PCA, dyslexia on its own does not cause significant
disability.
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11. Reference Table of Selected Rare Syndromes
Disabling
Syndrome Aetiology
Description34-38
Effects
Characteristic happy smile, inappropriate laughter,
jerky movements, and wide-based gait. There is
Angelman
Chromosome severe learning disability without speech but with
Severe
Syndrome
Abnormality
some ability to sign. Epilepsy occurs in over 80%.
The condition is stable. It has an incidence of about
1 in 20,000.
There is a typical appearance and growth failure,
learning disability (mean IQ 60-70), hyperkinesis
Fetal Alcohol
Toxic
and microcephaly. The severity of disability depends
Moderate
Syndrome
on the level of alcohol intake. Incidence is about 1 in
1000 births.
(XXY) Males with an extra X chromosome. Features
Klinefelter
Chromosome include gynaecomastia and sparse facial hair. Mild
Mild
Syndrome
Abnormality
learning disability is associated with a small
proportion of cases.
This condition affects males. Treatment with
Disorder of
allopurinol can control the associated gout, but it
Lesch-Nyan
uric acid
cannot prevent the neurological syndrome of
Severe
Syndrome
metabolism
choreoathetosis, spasticity, learning disability (IQ
40-65), and self-mutilation.
Occurs in A, B & C types. Until school age,
Niemann-Pick Disorder of lipid development is typically normal, then there is
Fatal
Disease
metabolism
severe motor and intellectual deterioration. There is
no effective treatment. Affects 1 in 10,000 births.
An autosomal recessive disease, which occurs in 1
Error of
in 10,000 births. It is routinely screened for in the
Phenylketonuria phenylalanine
None
UK, and can be controlled by restricting the intake of
metabolism
protein.
Features include: short stature, small hands and
Prader-Willi
Chromosome feet, severe obesity and IQ 50-80. The incidence is Moderate
Syndrome
Abnormality
about 1 in 20,000 births.
This condition begins to cause severe learning
disability in the first 2 years of life, and eventually
X chromosome
Rett’s Syndrome
results in severe global disability. “Hand wringing”
Severe
abnormality
movements are a typical feature. It affects 1 in
10,000 girls.
Facial port wine stain indicates haemangiomas on
Sturge-Weber
the ipsilateral cerebral hemisphere. These cause
Sporadic
Moderate
Syndrome
contralateral seizures, often with hemiparesis and
hemianopia. Learning disability is common.
Progressive motor weakness from 6 months of age,
Tay-Sachs
Ganglioside
and seizures, blindness, and deafness. The child
Fatal
Disease
storage disease dies before it is 5 years old. The incidence is 1 in
2000 in Ashkenazi Jews. Autosomal recessive.
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This condition is named after tuber-like growths on
the brain and other tissues. It is associated with 50% have
Tuberous
Chromosome learning disability, epilepsy, and characteristic skin learning
Sclerosis
Abnormality
lesions, including facial angiofibromas. Affects 1 in
disability
8000 births. Normal life expectancy for all sufferers.
(45X) These girls lack an X chromosome. They
Turner’s
Chromosome have short stature and a webbed neck. IQ is usually
Mild
Syndrome
Abnormality
average, but they have impaired verbal and
numerical skills and right-left disorientation.
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12. Reference List
1.
Mencap. Understanding Learning Disability. http://www.mencap.org.uk 2002;
2.
WHO. World Health Report. http://www.who.int/whr/2001 2002;
3.
D. Bloye. Crash Course Psychiatry. Mosby, 1999.
4.
Michael Gelder, Richard Mayou, Philip Cowen. Shorter Oxford Textbook of
Psychiatry. Oxford University Press, 2001.
5.
Cornelius Katona, Mary Robertson. Psychiatry at a Glance. 2000.
6.
Hassiotis A. Behavioural and cognitive-behavioural interventions for outwardly-
directed aggressive behaviour in people with learning disabilities.
Cochrane
Database of Systematic Reviews 2002;
Issue 1, 2002.
7.
White Paper - New Strategy for Learning Disability in the 21st Century. 2002.
8.
Foundation for People with Learning Disabilities. How many people have
learning disabilities? http://www.learningdisabilities.org.uk 2002;
9.
Lesley Stevens, Ian Rodin. Psychiatry: an illustrated colour text. Churchill
Livingstone, 2001.
10. Emerson E, Azmi S, Hatton C, Caine A, Parrott R, Wolstenholme J. Is there an
increased prevalence of severe learning disabilities among British Asians?
Ethnicity & Health 1997;
2:317-21.
11. Department of Health. Learning Disabilities.
http://www.doh.gov.uk/learningdisabilities/facts.htm 2002;
12. Foundation for People with Learning Disabilities. Employment for people with
learning disabilities. http://
www.learningdisabilities.org.uk 2002;
13. ABC of Mental Health. BMJ Books, 1998.
14. B. K. Puri, P. J. Laking, I. H. Treasaden. Textbook of Psychiatry. Churchill
LIvingstone, 1996.
15. National Autistic Society. Autism and Asperger Syndrome. http://
www.nas.org.uk
2002;
16. Fombonne E. The epidemiology of autism: a review.
Psychological Medicine
1999;
29:769-86.
17. Jacobson JL, Jacobson AM. Psychiatric Secrets. Hanley & Belfus, 2001.
18. Korkmaz B. Infantile autism: adult outcome.
Seminars in Clinical
Neuropsychiatry 2000;
5:164-70.
19. The Down's Syndrome Association. Down's Syndrome. http://www.dsa-uk.com
2002;
20. National Down's Syndrome Society. http://www.ndss.org/main.html 2002.
21. The National Institute for Child Health and Development. Fragile X Syndrome.
http://www.nichd.nih.gov 2002;
22. National Institute of Child Health and Human Development. Facts About Fragile
X Syndrome. http://www.nichd.nih.gov/publications/pubs/sub2.htm 2002.
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23. Victoria State Government, Australia. Fragile X Syndrome Fact Sheets for
Health Professionals. http://hnb.ffh.vic.gov.au/commcare/yafs_yf.nsf 2002.
24. The Royal College of Psychiatrists. Mental Health and Growing Up Second
Edition. Attention Deficit Disorder and Hyperactivity. 2002.
25. McCann BS, Roy-Byrne P. Attention-deficit/hyperactivity disorder and learning
disabilities in adults.
Seminars in Clinical Neuropsychiatry 2000;
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26. Cosgrove P.V.F. Attention Deficit Hyperactivity Disorder A UK Review.
Primary
Care Psychiatry 1997;
3:101-13.
27. Anonymous. British National Formulary. British Medical Association and Royal
Pharmaceutical Society of Great Britain, 2001.
28. Kewley GD. Personal paper: attention deficit hyperactivity disorder is
underdiagnosed and undertreated in Britain.
BMJ 1998;
316:1594-6.
29. Department for Education and Skills. Special Educational Needs in England
2001. http://www.dfes.gov.uk/statistics/DB/SBU/b0301/sb12-2001.pdf
30. Foundation for People with Learning Disabilities. Older people with learning
disabilities. http://www.learningdisabilities.org.uk 2002;
31. Brylewski J. Antipsychotic medication for challenging behaviour in people with
learning disability.
Cochrane Database of Systematic Reviews 2002;
Issue 1,
2002.
32. Holland AJ. Ageing and learning disability.
British Journal of Psychiatry
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176:26-31.
33. Medical Services. Incapacity Benefit Handbook for Approved Doctors. 2000.
34. Hope RA, Longmore JM, McManus SK, Wood-Allum CA. Oxford Handbook of
Clinical Medicine. Oxford University Press, 1998.
35. The Oxford Textbook of Medicine on CD ROM. Oxford University Press, 1996.
36. Rett’s Disord
er http://www.psychnet-uk.com/dsm_iv/retts_disorder.htm 2002.
37. Niemann-Pick Disease
http://www.niemann-pick.freeserve.co.uk/description.htm 2002.
38. Tuberous Sclerosis Association. http://www.tuberous-sclerosis.org2002.
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