Transcript Slide 1

Psychiatry of learning disability
RT Alexander
Consultant Psychiatrist, St John’s House Hospital, PiC LD
Services, Diss, Norfolk
& Hon Visiting Clinical Fellow, University of Leicester
Prevalence of LD
• In general population, overall prevalence of LD
is around 3% (Mild: 2.5%, Moderate: 0.4%,
Severe/Profound: 0.1%).
• In those with LD, those with Mild LD make up
around 80%. Those with moderate, severe and
profound together make up less than 20%.
•
•
•
•
Mental disorder in adults with intellectual disability. 2: The rate of behaviour disorders among a
community-based population aged between 16 and 64 years.
Article
Journal of Intellectual Disability Research. 45(6):506-514, December 2001.
Deb, S.; Thomas, M.; Bright, C.
Abstract:
Despite the difficulty of defining behaviour disorder, most previous studies have reported a high
rate of behaviour disorders in people with intellectual disability (ID). The aim of the present study
was to establish the overall rate and types of behaviour disorders in a population-based sample of
adults with ID. The other aim was to explore the possible risk factors that are associated with the
overall rate as well as different types of behaviour disorders. One hundred and one adults with ID
aged between 16 and 64 years were randomly selected from a sample of 246 such adults, i.e. those
who were known to the Vale of Glamorgan Social Services Department in South Wales, UK.
Thirteen behaviour disorders were rated according to the Disability Assessment Schedule.
Background data on subjects were also collected, and were subsequently analysed to assess the
relationship between different risk factors and behaviour disorders. Sixty-one subjects (60.4%) had
at least one behaviour disorder of any severity or frequency. Twenty-three per cent of subjects
showed aggression, 24% self-injurious behaviour, 36% temper tantrum, 26% overactivity, 29%
screaming, 38% attention-seeking behaviour, 20% objectionable habits, 18% night-time disturbance
and 12% of subjects showed destructiveness. Statistically significant associations were seen
between the rate of overall behaviour disorder and the use of psychotropic medication, and
between family and group home residence. The rate of aggression was significantly associated with
the use of psychotropic medication. The rate of self-injurious behaviour was significantly associated
with the severity of ID, female gender and poor communication abilities. The rate of temper
tantrum was significantly associated with the use of psychotropic medication. Twenty-four subjects
showed severe or frequent aggression, destructiveness, self-injury or temper tantrum, and 11
individuals showed real challenging behaviours. Severe behaviour problems were significantly
associated with female gender, severity of ID, the presence of a history of epilepsy and attendance
at day activities.
•
•
•
Mental disorder in adults with intellectual disability. 1: Prevalence of functional psychiatric illness
among a community-based population aged between 16 and 64 years.
Journal of Intellectual Disability Research. 45(6):495-505, December 2001.
Deb, S.; Thomas, M.; Bright, C.
Abstract:
The reported prevalence of psychiatric illness among adults with intellectual disability (ID) varies widely
between 10 and 39%; however, many methodological problems exist. The aims of the present study were
to establish the prevalence of functional psychiatric illness among adults with ID who live in the
community, in order to compare the overall rate and types of psychiatric illness between the population
with ID and the general population without ID, and to establish the risk factors associated with psychiatric
illness in adults with ID. The study was done in two stages. In the first part, a trained psychiatrist
interviewed 101 randomly selected adults with ID and their carers using the Mini Psychiatric Assessment
Schedule for adults with Developmental Disability (Mini PAS-ADD) to screen for psychiatric caseness. Out
of these 101 adults, 90 had sufficient communicative abilities that made the administration of Mini PASADD possible. A second trained psychiatrist interviewed 19 out of the 20 adults who were diagnosed as
psychiatric cases according to the initial Mini PAS-ADD interview. This psychiatrist interviewed patients and
their carers in line with the full PAS-ADD interview. The second psychiatrist was blind to the initial
diagnoses made according to the Mini PAS-ADD questionnaire. A final psychiatric diagnosis was made
according to International Classification of Diseases - 10th Revision (ICD-10) criteria. Some 14.4% (95%
confidence interval = 7.4-21.4%) of the cohort had a psychiatric diagnosis according to ICD-10 criteria:
4.4% had schizophrenia, 2.2% depressive disorder, 2.2% generalized anxiety disorder, 4.4% phobic
disorder and 1% delusional disorder. The overall rate of functional psychiatric illness (point prevalence)
was similar to that found in the general population (16%). However, the rates of schizophrenic illness
and phobic disorder were significantly higher in the study cohort compared with those in the general
population (0.4% and 1.1%, respectively). Increasing age and the presence of physical disability were
significantly associated with the occurrence of psychiatric illness. Out of the 11 remaining adults with
severe ID, two (18%) had a diagnosis of a psychiatric illness (one mania and one anxiety disorder)
according to the Diagnostic Assessment for the Severely Handicapped (DASH) questionnaire.
MCQ 1. Epidemiology
Which of the following statements is most accurate?
a.
b.
c.
d.
e.
There is no difference in the prevalence of mental retardation
between the sexes.
The administrative prevalence of learning disability is three times
its true prevalence.
About a third of those with learning disability would come under
the category of mild learning disability.
The prevalence of moderate and severe learning difficulty (IQ<50)
is 3-4 per 1000
The prevalence of mental retardation is strongly related to social
class.
MCQ 1. Epidemiology
Which of the following statements is most accurate?
a.
b.
c.
d.
e.
There is no difference in the prevalence of mental retardation between the
sexes.
The administrative prevalence of learning disability is three times its true
prevalence.
About a third of those with learning disability would come under the category of
mild learning disability.
The prevalence of moderate and severe learning difficulty (IQ<50) is 3-4 per
1000
The prevalence of mental retardation is strongly related to social class.
Answer: d
MCQ 2. Learning disability- Psychiatric morbidity
All of the following are true of depression in people with
learning disability except
a. They are less likely to complain of low mood.
b. The depression is less likely to respond to tricyclic
antidepressants.
c. Irritability is a depressive equivalent.
d. It is more difficult to establish depressive cognitions.
e. Temper tantrums are depressive equivalents.
MCQ 2. Learning disability- Psychiatric morbidity
All of the following are true of depression in people with learning disability
except
a. They are less likely to complain of low mood.
b. The depression is less likely to respond to tricyclic antidepressants.
c. Irritability is a depressive equivalent.
d. It is more difficult to establish depressive cognitions.
e. Temper tantrums are depressive equivalents.
Answer: b. (People with LD have limited communication skills and limited
ability to express their subjective feelings. They are less likely to complain
of low mood. Depression in people with LD may manifest with irritability,
unexplained temper tantrums or aggression which are considered
‘depressive equivalents’).
MCQ 3. Learning disability- Psychiatric morbidity
All of the following are true of schizophrenia in people with
learning disability except
a.
b.
c.
d.
The point prevalence is 3% .
The highest rates are in those with mild and moderate LD.
Has an earlier onset than in those of normal intelligence.
Poverty of thinking is more common than in those of
normal intelligence.
e. Delusions are more well systematised and elaborate than
in those of normal intelligence.
MCQ 3. Learning disability- Psychiatric morbidity
All of the following are true of schizophrenia in people with learning disability except
a.
b.
c.
d.
e.
•
The point prevalence is 3% .
The highest rates are in those with mild and moderate LD.
Has an earlier onset than in those of normal intelligence.
Poverty of thinking is more common than in those of normal intelligence.
Delusions are more well systematised and elaborate than in those of normal intelligence.
Answer: e. (The point prevalence of schizophrenia in people with LD is 3% compared to 0.4% in
general population. The highest rates are in those with mild and moderate LD. Schizophrenia has an
earlier onset in people with LD than in general population. Poverty of thinking is more common,
delusions are less elaborate and hallucinations may have a simple and repetitive content. Kraepelin
named dementia praecox arising in a setting of pre-existing intellectual impairment as
propfschizophrenie).
MCQ 4. Learning disability- Psychiatric morbidity
All of the following are true about people with mild learning disability
except
a. They have a higher prevalence of somatisation than the general
population.
b. They have a higher prevalence of brooding than the general
population.
c. They have a lower prevalence of sleep disturbances than the
general population.
d. They have a lower prevalence of complaints of low mood when
depressed.
e. They tend to have temper tantrums when depressed.
MCQ 4. Learning disability- Psychiatric morbidity
All of the following are true about people with mild learning disability except
a. They have a higher prevalence of somatisation than the general
population.
b. They have a higher prevalence of brooding than the general population.
c. They have a lower prevalence of sleep disturbances than the general
population.
d. They have a lower prevalence of complaints of low mood when
depressed.
e. They tend to have temper tantrums when depressed.
Answer: c. (People with mild LD have a higher prevalence of brooding,
somatisation and sleep disturbances than the general population).
MCQ 5. Learning disability- Epidemiology
Which of the following is most accurate
a.
b.
c.
d.
e.
The prevalence of learning disability (LD) is 5%.
The prevalence of learning disability is higher among males.
Severe learning disability is much more common in social class IV
and V.
Children from families with low socio-economic status are at
higher risk of severe LD.
Learning disability has no relationship with maternal alcohol
consumption.
MCQ 5. Learning disability- Epidemiology
Which of the following is most accurate
a.
The prevalence of learning disability (LD) is 5%.
b.
The prevalence of learning disability is higher among males.
c.
Severe learning disability is much more common in social class IV and V.
d.
Children from families with low socio-economic status are at higher risk of severe LD.
e.
Learning disability has no relationship with maternal alcohol consumption.
Answer: b. (The approximate prevalence of LD in the general population is: Mild =1.5 to 2%, Moderate
and severe = 0.5% (range = 0.16-0.73%) and profound = 0.05% . Mild LD is more common in lower
than higher social classes. However, findings of association between social class and severe LD has
been inconsistent. Learning disability is marginally more common in males. Mental retardation can
be linked to deprived environment due to the fact that these environments are linked to other risks
such as malnutrition, poor medical care, child abuse, usage of alcohol and substances and teenage
pregnancies and may place children from lower socio-economic status at higher risk.).
MCQ 6. Genetic Syndromes-DD
A 5 year child presented with autistic symptoms and
hyperphagia & hypotonia. Select one diagnosis.
a. Angelman syndrome
b. Down syndrome
c. Fragile X syndrome
d. Lesch Nyhan syndrome
e. Prader Willi syndrome
MCQ 6. Genetic Syndromes-DD
A 5 year child presented with autistic symptoms and hyperphagia &
hypotonia. Select one diagnosis.
a. Angelman syndrome
b. Down syndrome
c. Fragile X syndrome
d. Lesch Nyhan syndrome
e. Prader Willi syndrome
Answer: e. Characterised by hypotonia, hyperphagia, obesity,
genital hypoplasia. In the pre-obesity phase can be limp, sleepy,
unresponsive. Later develops hyperphagia.
MCQ 7. Genetic Syndromes-DD
A 4 year old boy with compulsive overeating and self injury by
skin picking. Select one diagnosis.
a. Angelman syndrome
b. Down syndrome
c. Fragile X syndrome
d. Lesch Nyhan syndrome
e. Prader Willi syndrome
MCQ 7. Genetic Syndromes-DD
A 4 year old boy with compulsive overeating and self injury by skin picking.
Select one diagnosis.
a. Angelman syndrome
b. Down syndrome
c. Fragile X syndrome
d. Lesch Nyhan syndrome
e. Prader Willi syndrome
Answer: e. Characterised by hypotonia, hyperphagia, obesity, genital
hypoplasia. In the pre-obesity phase can be limp, sleepy, unresponsive.
Later develops hyperphagia. Self injury/ skin picking can be associated.
MCQ 8. Genetic Syndromes- DD
A 4 year old girl presented with autistic symptoms, loss of
skills and hand wringing. Select one most likely diagnosis.
a.
b.
c.
d.
e.
Angelman syndrome
Down syndrome
Fragile X syndrome
Rett syndrome
Prader Willi syndrome
MCQ 8. Genetic Syndromes- DD
A 4 year old girl presented with autistic symptoms, loss of skills and hand
wringing. Select one most likely diagnosis.
a.
b.
c.
d.
e.
Angelman syndrome
Down syndrome
Fragile X syndrome
Rett syndrome
Prader Willi syndrome
Answer: d. Handwringing stereotypies typical of the syndrome. Almost all
sufferers are female. Initial ?normal development till 18 to 24 months
followed by hand wringing/ hand flapping stereotypies and mental
deterioration. Autistic features may be present.
MCQ 9. Genetic Syndromes-DD
A 5 year old boy presented with autistic symptoms. His
maternal grandfather & maternal uncle had the
condition. Select ONE diagnosis
a.
b.
c.
d.
e.
Fragile X syndrome
Rett syndrome
Prader Willi syndrome
Down syndrome
Acute myeloid leukaemia
MCQ 9. Genetic Syndromes-DD
A 5 year old boy presented with autistic symptoms. His maternal
grandfather & maternal uncle had the condition. Select ONE
diagnosis
a.
b.
c.
d.
e.
Fragile X syndrome
Rett syndrome
Prader Willi syndrome
Down syndrome
Acute myeloid leukaemia
Answer: a. The most common cause of inherited mental retardation
MCQ 10. Fragile X syndrome
Which of the following statements is true?
a. Speech is normal in Fragile X syndrome.
b. Fragile X syndrome is an autosomal dominant
disorder.
c. Incessant staring is a feature of Fragile X syndrome.
d. Cryptorchidism is common in Fragile X syndrome.
e. Fragile X syndrome is the most common inherited
cause of LD.
MCQ 10. Fragile X syndrome
Which of the following statements is true?
a.
b.
c.
d.
e.
Speech is normal in Fragile X syndrome.
Fragile X syndrome is an autosomal dominant disorder.
Incessant staring is a feature of Fragile X syndrome.
Cryptorchidism is common in Fragile X syndrome.
Fragile X syndrome is the most common inherited cause of LD.
Answer: e (The abnormalities of speech include perseveration of words and phrases, echolalia, palilalia,
‘cluttering’, narrative speech, comprehension difficulties with high association compounds,
productive semantic errors and speech sound substitution difficulties. Syntactic competency and
semantic concepts remain intact in Fragile X males. Fragile X syndrome is an X-linked disorder. It
shows an X-linked dominant inheritance with incomplete penetrance. It is the most common LD
syndrome caused by mutation of a single gene. It is the most common inherited cause of LD. It
accounts for 50% of all cases of X-linked LD. Gaze avoidance is characteristic of Fragile X syndrome.
Cryptorchidism, i.e., undescended testes is rare. Macro-orchidism, i.e., enlarged testes is present in
90% of adults with Fragile X syndrome. It is usually obvious after puberty but not always before).
MCQ 11. Fragile X syndrome- genetics
Which of the following statements is true?
a. Fragile X syndrome is caused by a CAG repeat on the X
chromosome, long arm Xq27.3
b. Fragile X syndrome is caused by a CGG repeat on the X
chromosome, long arm Xq27.3
c. Female carriers of Fragile X syndrome have more cognitive
impairment than males.
d. In cases of phenotypically manifested Fragile X syndrome, CGG
repeats are less than 30.
e. Fragile X syndrome is caused by a CGG repeat on the X
chromosome, short arm.
MCQ 11. Fragile X syndrome- genetics
Which of the following statements is true?
a.
Fragile X syndrome is caused by a CAG repeat on the X chromosome, long arm Xq27.3
b.
Fragile X syndrome is caused by a CGG repeat on the X chromosome, long arm Xq27.3
c.
Female carriers of Fragile X syndrome have more cognitive impairment than males.
d.
In cases of phenotypically manifested Fragile X syndrome, CGG repeats are less than 30.
e.
Fragile X syndrome is caused by a CGG repeat on the X chromosome, short arm.
Answer: b (Fragile X syndrome is caused by abnormal nucleotide CGG repeat at a fragile site on the long
arm of the X chromosome (Xq27.3). Between 6 and 54 CGG repeats are expected in normal
individuals with an average of 30 repeats. In cases of phenotypically manifested Fragile X
syndrome, CGG repeats range from 230 to over 1000. Most female carriers have normal
intelligence. However, 30-50% have mild LD.)
EMQ 1
Options
Angelman syndrome
Down syndrome
Fragile X
Lesch Nyhan
Phenlyketoneurea
ADHD
Prader Willi
Rett syndrome
Hurlers syndrome
Hunter syndrome
Lead in
Three different 5 year children presented with autistic symptoms. Choose one diagnosis each
1: The child has hyperphagia & hypotonia
2: She has hand wringing
3: His maternal grandfather & maternal uncle had the condition
EMQ 1
Options
Angelman syndrome
Down syndrome
Fragile X
Lesch Nyhan
Phenlyketoneurea
ADHD
Prader Willi
Rett syndrome
Hurlers syndrome
Hunter syndrome
Lead in
Three different 5 year children presented with autistic symptoms. Choose one diagnosis each
1. The child has hyperphagia & hypotonia
2: She has hand wringing
3: His maternal grandfather & maternal uncle had the condition
Answers
1= Prader-Willi syndrome: Hypotonia, hyperphagia, obesity, genital hypoplasia, delayed motor milestones. In
the pre obesity phase these children are limp, sleepy and unresponsive and they go on to develop
hyperphagia.
2= Rett syndrome: Hand wringing, a hallmark of the disease. Virtually all patients are female because the
disease is X-linked. Males with RS are believed to die in utero. The girls develop normally up to age 18m to
2y and then start showing evidence of mental deterioration preceded by the development of abnormal
involuntary stereotyped motor movements in the form of hand-flapping and hand wringing movements.
3= Fragile X syndrome: is the most common cause of inherited mental retardation and, after trisomy 21, is the
second most common cause of genetically associated mental deficiencies.
EMIQ 2
Options
Hand biting, pulling of fingernails and toenails (onychotillomania)
Disorder of cholesterol synthesis
Loss of acquired milestones
Cafe au lait spots
47XXY
45X0
47XYY
Trisomy 13
Trisomy 18
Lead in
Select one option for each of the following syndromes
1.
2.
3.
4.
5.
Klinefelter’s syndrome
Smith Magenis Syndrome
Retts syndrome
Tuberous sclerosis
Smith Lemli Opitz Syndrome (SLOS)
EMIQ 2
Options
Hand biting, pulling of fingernails and toenails (onychotillomania)
Disorder of cholesterol synthesis
Loss of acquired milestones
Cafe au lait spots
47XXY
45X0
47XYY
Trisomy 13
Trisomy 18
Lead in
Select one option for each of the following syndromes
1.
2.
3.
4.
5.
Klinefelter’s syndrome
Smith Magenis Syndrome
Retts syndrome
Tuberous sclerosis
Smith Lemli Opitz Syndrome (SLOS)
ANSWERS
1. 47XXY
2. Hand biting, pulling of fingernails and toenails (onychotillomania). (These are features of
Smith-Magenis Syndrome, a rare condition caused by a deletion in chromosome 17p11.2)
3. Loss of acquired milestones
4. Cafe au lait spots
5. Disorder of cholesterol synthesis (Smith-Lemli-Optiz syndrome (SLOS) is a multiple congenital
anomalies/mental retardation syndrome caused by a defect in cholesterol synthesis, caused
by a deficiency of enzyme 3-beta-hydroxysterol-delta-7-reductase. Affected individuals have
low plasma cholesterol and severely afeected ones have multiple congenital malformations
and are often miscarried. Dysmorphic facial features and microcephaly are typical. They can
also have learning and behavioural disabilities.)
EMQ 3
Options:
Paternal deletion on 15q11-13
Maternal deletion on 15q11-13
Deletion of 17p 11.2
The allele APO E2 2 in the gene for apolipoprotein
Tau Mutation
Neuro Regulin
Variations of Acetyl Choline Transferase
Lead in:
Match the above genetic markers with these clinical scenarios
1.
2.
3.
4.
A young man who eats excessively and has mild learning disability.
A 25 Year Old with a 2 year history of decreased personal Care, with bizarre behaviour and
persecutory delusions.
Angelman Syndrome
Child with moderate LD, severe self-injurious behaviour and ‘self-hugging’ behaviours.
EMQ 3
Options:
Paternal deletion on 15q11-13
Maternal deletion on 15q11-13
Deletion of 17p 11.2
The allele APO E2 2 in the gene for apolipoprotein
Tau Mutation
Neuro Regulin
Variations of Acetyl Choline Transferase
Lead in:
Match the above genetic markers with these clinical scenarios
1.
2.
3.
4.
A young man who eats excessively and has mild learning disability.
A 25 Year Old with a 2 year history of decreased personal Care, with bizarre behaviour and persecutory delusions.
Angelman Syndrome
Child with moderate LD, severe self-injurious behaviour and ‘self-hugging’ behaviours.
Answers
1= Prader Willi (Hyperphagia, mild to moderate retardation and obesity) . Paternal deletion on
15q11-15
2= Neuro Regulin – a gene implicated in schizophrenia (as is dysbindin)
3= Maternal deletion on 15q11-15
4= Deletion of 17p 11.2 (Smith Magenis Syndrome)
MCQ 12. Asperger syndrome
Which of the following is true?
a. People with Asperger’s syndrome usually have an IQ less than 70.
b. Impaired prosody of speech is a feature of Asperger syndrome.
c. Patients with Asperger syndrome have a wider repertoire of emotional
facial expression.
d. Asperger syndrome is more common in females than in males.
e. Asperger syndrome is a risk factor for schizophrenia.
MCQ 12. Asperger syndrome
Which of the following is true?
a. People with Asperger’s syndrome usually have an IQ less than 70.
b. Impaired prosody of speech is a feature of Asperger syndrome.
c. Patients with Asperger syndrome have a wider repertoire of emotional facial expression.
d. Asperger syndrome is more common in females than in males.
e. Asperger syndrome is a risk factor for schizophrenia.
Answer: b (IQ and language in people with Asperger’s syndrome are normal or in some
cases superior. Prosody is the stress, intonation and tone of voice. This is impaired
in Asperger syndrome. Patients with autistic spectrum disorders may have a limited
range of facial expression. Both epidemiological and clinical studies report a male:
female ratio of 3.5-4: 1 for children with autism. However this ratio varies with the
IQ level. Females with autism who have IQs in the normal range are 50 times less
common than males. The male female ratio for Asperger syndrome appears to be
closer to 9:1).
MCQ 13. Asperger syndrome
A mother brings her 8-year-old son to see you in
clinic. She is concerned that he may have
Asperger’s syndrome. Which of these would lead
you to doubt that diagnosis?
a. He stands throughout the interview.
b. He has a restricted range of interests.
c. His mother reports that he did not develop
language until the age of 4years.
d. He speaks in a monotonous voice.
e. His mother says he has an above average IQ.
MCQ 13. Asperger syndrome
A mother brings her 8-year-old son to see you in clinic. She is
concerned that he may have Asperger’s syndrome. Which of these
would lead you to doubt that diagnosis?
a.
b.
c.
d.
e.
He stands throughout the interview.
He has a restricted range of interests.
His mother reports that he did not develop language until the age
of 4years.
He speaks in a monotonous voice.
His mother says he has an above average IQ.
• Answer: c
MCQ 14. Down’s syndrome- genetics
Which of the following is true?
a. Down’s syndrome affects approximately 1% of live
births.
b. Most people with Down’s have moderate to severe
learning disability.
c. Translocation Down’s syndromes often have
borderline intellectual functioning.
d. Translocation accounts for 95% of Down’s syndrome.
e. If a couple has one child with Down’s syndrome due
to translocation, the risk of a subsequent child with
the syndrome is about 1% .
MCQ 14. Down’s syndrome- genetics
Which of the following is true?
a.
Down’s syndrome affects approximately 1% of live births.
b.
Most people with Down’s have moderate to severe learning disability.
c.
Translocation Down’s syndromes often have borderline intellectual functioning.
d.
Translocation accounts for 95% of Down’s syndrome.
e.
If a couple has one child with Down’s syndrome due to translocation, the risk of a subsequent child with the syndrome is about 1% .
Answer: b (Down’s syndrome is the most common cause of mental retardation. It affects
approximately 1 in 1000 live births. The incidence increases with maternal age as follows: 1529 years = 1 in 1500 live births, 30-34 years = 1 in 800, 35-39 years = 1 in 270, 40-44 years = 1
in 100, > 45 years = 1 in 50.
Most people with Down’s have an IQ between 20 and 55 (moderate to severe learning disability).
Only 15% have an IQ above 50. In general, the Down’s syndrome population has an IQ range
of 20-55, i.e., severe to moderate LD. Those with a Translocation and Trisomy 21 tend to have
moderate to severe intellectual deficits. 1-3% show Mosaicism and may have normal
intelligence and achievement.
Trisomy 21 accounts for 94% cases. The cause is unknown. The risk of recurrence is 1%.
Translocation accounts for 3-5%. Translocation is familial. It can be identified at birth and
used for genetic counselling. The risk of recurrence is 10%. Mosaicism is found in 1-3%. They
may have normal intelligence.)
MCQ 15. Down’s syndrome- morbidity
Which of the following is true?
a. Hypomania is common in Down’s syndrome
adults.
b. The narrowed hypopharynx increases the risk of
sleep apnoea in Down’s syndrome.
c. Sensorineural deafness is very rare in Down’s
syndrome.
d. Language development is normal in Down’s
syndrome.
e. Depression is rare in Down’s syndrome adults.
MCQ 15. Down’s syndrome- morbidity
Which of the following is true?
a.
Hypomania is common in Down’s syndrome adults.
b.
The narrowed hypopharynx increases the risk of sleep apnoea in Down’s syndrome.
c.
Sensorineural deafness is very rare in Down’s syndrome.
d.
Language development is normal in Down’s syndrome.
e.
Depression is rare in Down’s syndrome adults.
Answer: b
Bipolar affective disorder (BPAD) is not common in Down’s syndrome. However, previous reports that
BPAD does not occur at all in Down’s syndrome have been proved wrong. The frequency and clinical
features of BPAD in Down’s syndrome are the same as in non-Down’s syndrome learning disability.
The narrowed hypopharynx increases the risk of sleep apnoea. Immune system defects increase the
vulnerability to infections, particularly, recurrent respiratory infections.
Most children with Down’s syndrome have unilateral or bilateral hearing impairment. The high
prevalence of conduction deafness especially in children may be caused by glue ear. Otitis media
affects many children with Down’s syndrome and may be exacerbated by the structural anomalies
of the ear. The hearing impairment in children is compounded by the later onset of sensorineural
deafness. Thus, there is a high prevalence of both conduction and sensorineural hearing loss in
adults with Down’s syndrome.
All developments slow down after the first 6 months to 1 year. Language is not an exception to this.
Depression is a well recognised psychiatric problem in Down’s syndrome.)
MCQ 16. Down’s syndrome- dementia
Which of the following is true about dementia in
Down’s syndrome?
a. Females have an earlier age of onset than males.
b. The duration of dementia is approximately 16 years.
c. Multi-infarct dementia is as common as Alzheimer
type dementia.
d. By 40 years, 50% of those with Down’s syndrome
have developed a dementia.
e. There is no relationship between age and dementia
in Down’s syndrome.
MCQ 16. Down’s syndrome- dementia
Which of the following is true about dementia in Down’s syndrome?
a.
b.
c.
d.
e.
Females have an earlier age of onset than males.
The duration of dementia is approximately 16 years.
Multi-infarct dementia is as common as Alzheimer type dementia.
By 40 years, 50% of those with Down’s syndrome have developed a dementia.
There is no relationship between age and dementia in Down’s syndrome.
Answer: a (Prevalence of Alzheimer’s increases with age 30-39 years = 2-3%, 40-49
years = 9-10%, 50-59 years = 36-40%, 60-69 years = 55%. Females have an earlier
age of onset than males. The duration of dementia is approximately 6 years.
Down’s dementia is of the Alzheimer type. Down’s is reputedly an atheroma free
model).
MCQ 17. Down’s syndrome- thyroid
dysfunction
The life time prevalence of thyroid dysfunction
in Down’s syndrome is about
a.
b.
c.
d.
e.
Less than 1%
5 to 10%
25%
50%
Over 75%
MCQ 17. Down’s syndrome- thyroid dysfunction
The life time prevalence of thyroid dysfunction in Down’s
syndrome is about
a.
b.
c.
d.
e.
Less than 1%
5 to 10%
25%
50%
Over 75%
• Answer: c
MCQ 18. Down’s syndrome- psychiatric morbidity
Apart from Alzheimer’s type dementia, which of the
following disorders is most likely to show an
increase in people with Down’s syndrome.
a. Pervasive developmental disorders
b. Depressive disorder
c. OCD
d. Anxiety disorder
e. Bipolar disorder
MCQ 18. Down’s syndrome- psychiatric morbidity
Apart from Alzheimer’s type dementia, which of the following
disorders is most likely to show an increase in people with
Down’s syndrome.
a. Pervasive developmental disorders
b. Depressive disorder
c. OCD
d. Anxiety disorder
e. Bipolar disorder
Answer: b
MCQ 19. Forensic Learning Disability
Regarding learning disability and offending behaviour, select the most
appropriate statement.
a.
b.
c.
d.
e.
Most offences are committed by those with mild learning
disability.
Fire setting is the most common offence category committed by
people with learning disability.
Sexual offending is the most common offence category committed
by people with learning disability.
Those with moderate LD are most likely to be convicted of murder.
Offenders with learning disability tend to have higher psychopathy
scores.
MCQ 19. Forensic Learning Disability
Regarding learning disability and offending behaviour, select the most appropriate
statement.
a.
b.
c.
d.
e.
Most offences are committed by those with mild learning disability.
Fire setting is the most common offence category committed by people with
learning disability.
Sex ual offending is the most common offence category committed by people
with learning disability.
Those with moderate LD are most likely to be convicted of murder.
Offenders with learning disability tend to have higher psychopathy scores.
Answer: a. Among people with LD who come to the attention of the criminal justice
system, the vast majority have mild LD.
MCQ 20. Which of the following conditions have a
higher point prevalence in people with learning
disability than in the general population?
a.
b.
c.
d.
e.
Schizophrenia
Bipolar disorders
Alcohol dependence syndrome
Harmful use of alcohol
Completed suicides
MCQ 20. Which of the following conditions have a higher
point prevalence in people with learning disability than in
the general population?
a.
b.
c.
d.
e.
Schizophrenia
Bipolar disorders
Alcohol dependence syndrome
Harmful use of alcohol
Completed suicides
Answer: a (Refer to studies 1 and 2)