CHILD WITH MENTAL HEALTH PROBLEM Flashcards

1
Q

What is attachment behaviour?

A

Behaviour that results in an individual attaining proximity to a preferred other

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2
Q

Whats the secure base effect?

A

the phenomenon by which children are able to explore their environment without stress because they feel safe knowing that they can return to their caregiver,

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3
Q

Whats the characteristic effects of separation from an attachment figure?

A

Protest - the child may protest, crying and screaming,protesting angrily, clinging to the attachment figure and try to struggle to escape from those who pick the child up and try to stop the child from reaching the caregiver .

Despair - the child is upset and refuses peoples attempts of comfort.

Detachment - if the separation between the attachment figure and the child continues, the baby may start to play again with others but may also be wary. The child, upon the arrival of the caregiver who has gone, may also reject the caregiver and act angrily towards this attachment figure.

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4
Q

Whats a secure attachment?

A

Secure attachment is the healthiest form of attachment.
It describes an attachment where a child feels comforted by the presence of their caregiver. Securely attached children feel protected and that they have someone to rely on. Children with secure attachment prefer their caregiver over strangers, seek comfort in their caregiver, and are comfortable exploring their environment with their caregiver present.

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5
Q

Why is a secure attachment important?

A

By growing up with a sense of stability and care, securely attached children find it easier to investigate and interact with the world around them . Into adulthood, secure attachment translates into higher self-esteem, more long-term healthy relationships, and an increased ability to trust others for social support. Since they grow up with a positive caregiver relationship, securely attached children can replicate a healthy bond with others, in all types of relationships.

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6
Q

What are the 3 types of insecure attachment?

A

Anxious-ambivalent / anxious-preoccupied
Anxious-avoidant /anxious-dismissive
Fearful avoidant / disorganised

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7
Q

Whats the problem with insecure attachments?

A

If a child cannot rely on an adult to respond to their needs in times of stress, they are unable to learn how to soothe themselves, manage their emotions and engage in reciprocal relationships

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8
Q

What was the face recognition experiment?

A

newborn infants will follow a slowly moving schematic face stimulus with their head and eyes further than they will folow scrambled faces or blank stimuli
Despite immature visual systems, babies are still able to recognise primary care givers faces

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9
Q

What is the still face experiment?

A

where a mother faces her baby, and is asked to hold a ‘still face’, in which she does not react to the baby’s behaviours. The reactions of the baby are then observed. In general, the baby will become agitated by failed attempts to evoke a reaction in the mother.
This should raise awarensss about the importance of social interaction on a baby’s development especially in this world’s use of technology

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10
Q

Whats the most critical period for forming attachments?

A

First 2 years
An attachment style between child and caregiver will be formed by the end of the first 12 months.

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11
Q

Outline the developmental milestones for attachment?

A

Pre-Attachment Stage: Newborn to 3 months
Babies show no preference for their caregiver yet.
They are soothed by anyone who takes care of them.

Indiscriminate Attachment: 6 weeks to 7 months
Babies show some preference for their primary caregivers. They are still soothed by those who aren’t their caregiver but are better at differentiating between strangers and those they know.

Discriminate Attachment: 7 months to 11 months
Infants will show a clear preference for one caregiver. They will be upset if separated from their primary caregiver. Babies will now show anxiety around strangers.

Multiple Attachments: from approximately 9 months on
Babies can now bond with people other than their caregiver. They can be soothed by people other than their caregiver who they’ve bonded with, such as grandparents and older siblings.

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12
Q

Whats the internal working models concept?

A

Bowlby said… The relationship with our primary caregiver becomes internalised and will become a set of unconscious expectations. Once these are formed, the person’s social world will be organised based on these expectations.

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13
Q

What promotes attachment?

A

Maternal sensitivity
Warmth
Emotional responsiveness
Involvement
Reciprocity

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14
Q

What are insecure attachments usually associated with?

A

Poor parenting/abuse

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15
Q

What is ambivalent insecure attachment?

A

When you have negative views of self but positive views of others
When you are dependant on others for self worth and have a fear of losing relationships so are clingy on separation

Children with this insecure attachment are clingy to their caregiver, yet when their caregiver attempts to comfort them, the child remains distressed. This can be hard for the caregiver because they are consistently unable to soothe their child, which can lead to a negative cycle of interaction. The child wants to be close with their caregiver but doesn’t fully trust them for support.
As a child with ambivalent attachment grows, this bonding style will often lead to clinginess and distrust in other types of relationships that develop in their life. people with this attachment style are often anxious and uncertain, lacking in self-esteem. They crave emotional intimacy but worry that others don’t want to be with them

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16
Q

What is disorganised/fearful-avoidant insecure attachment?

A

Negative views of self and others
Seek and avoid closeness
Fearful of making connections

will show an inconsistent connection with their caregiver and often hold fear towards them. The child must rely on the caregiver for survival, but the caregiver is also a source of fear. This is because their caregiver is sometimes there for support and sometimes unavailable or emotionally damaging, so the child doesn’t know when they can count on their caregiver to meet their needs.
This type of attachment style often occurs in homes with abuse, leading to insecure attachment from trauma. It will often contribute to mental health issues in adulthood, like substance abuse and borderline personality disorder.
In this form of attachment, the child grows up believing that they are unworthy of love.

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17
Q

What is avoidant insecure attachment?

A

Positive views of self but negative views of others

characterized by a child who avoids their caregiver and does not seek comfort from them. These children will show little to no preference for their caregiver over a stranger. They will not seek out their parents when in times of distress.
Avoidant attachment is formed when a child feels they cannot consistently count on their caregiver to comfort and care for them. An avoidant child sees no preference for their caregiver over a stranger because it’s possible the stranger may be more attuned to their needs than their actual caregiver.
Those who grow up with an avoidant attachment are more likely to have challenges with intimacy later in life and be closed off from social relationships. By not growing up with a close connection to their caregiver, they have trouble finding a healthy connection to others in their life. Instead of craving intimacy, they’re so wary of closeness they try to avoid emotional connection with others. They’d rather not rely on others, or have others rely on them

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18
Q

What are risk factors for attachment diffiuclties?

A

Poverty
Parental mental health diffiuclties
Exposure to neglect, domestic violence or other forms of abuse
Alcohol/drug taking during pregnancy
Multiple home/school placements
Prem birth
Abandonement
Family bereavement

(Not insecure attachments can also occur in non-vulnerable children)

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19
Q

Whats the strange situation procedure?

A
  1. Caregiver (CG) sits with infant on floor for 3 minutes.
  2. Stranger enters room & engages infant for 3 minutes.
  3. Caregiver leaves for up to 3 minutes.
  4. CG returns and spends 3 minutes with infant.
  5. CG leaves and infant left alone for 3 minutes.
  6. Stranger returns for 3 minutes.
  7. CG returns.

As these stages occur the child and mother’;s behaviour is observed.

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20
Q

Outline the child and mother’s behaviour during the strange situation test in a secure attachment?

A

Mother is seen as available, dependable and warm. They respond to the child’s cues

Child explores room actively and will be distressed if the mother leaves. A positive reunion and accepts comfort easily

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21
Q

Outline the child and mother’s behaviour during the strange situation test in a anxious/avoidant attachment?

A

Mother is rejecting, angry and hostile if child makes demands in stressful situations

Child is not distressed by mother leaving and will ignore mother on reunion, focussing on the environment
The child had learnt to suppress behaviours normally used to alert mother

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22
Q

Outline the child and mother’s behaviour during the strange situation test in a ambivalent attachment?

A

Mother is inconsistent with her care and is unresponsive and insensitive to child’s needs and demands

Child is very distressed when mother leaves. Ambivalence on return both seeking comfort then rejecting it on reunion

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23
Q

Outline the child and mother’s behaviour during the strange situation test in a disorganised attachment?

A

Mother is frightened herself or frightening. Usually abusive or suffering abuse

Child has contradictory behaviours with strong proximity seeking then strong avoidance. Distress, anger, freezing and stereotypes

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24
Q

How do we manage attachment difficulties?

A

Psychoeducation and formulation of diffiuclties
Systemic work with whole family
Private therapy or via social care if eligible

(Within CAMHS no treatment is offered)

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25
Q

What are behavioural disorders?

A

involve a pattern of disruptive behaviors in children that last for at least 6 months and cause problems in school, at home and in social situations.

They are a common presentation that may or may not be associated with mental health difficulties
Behaviour should be seen as a form of communication

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26
Q

Whats the difference between conduct disorders and opposition defiant disorder?

A

ODD affects younger children and involved them being defiant, disobedient and disruptive BUT not aggressive or antisocial behaviour

Conduct disorders continue into adolescence, are more severe and often include aggressive or antisocial behaviour

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27
Q

How are behavioural and conduct disorders managed?

A

Prevention
Psychotherapy
Family therapy
Parenting support via social care

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28
Q

What are conduct disorders?

A

Disorders characterized by a repetitive and persistent pattern of dissocial, aggressive, or defiant conduct.
Such behaviour should amount to major violations of age-appropriate social expectations; it should therefore be more severe than ordinary childish mischief or adolescent rebelliousness and should imply an enduring pattern of behaviour (six months or longer).
E.g. cruelty to other people or animals, fire-setting, stealing, repeated lying

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29
Q

Whats the difference between socialised and Unsocialised conduct disorders?

A

Both have persistent dissociation or aggressive behaviour occurring but the individual either is well integrated into their peer group (socialised) or has significant pervasive abnormalities in their relationships with other children (Unsocialised)

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30
Q

What are mixed disorders of conduct and emotions?

A

A group of disorders characterized by the combination of persistently aggressive, dissocial or defiant behaviour with overt and marked symptoms of depression, anxiety or other emotional upsets.

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31
Q

What is reactive attachment disorder of childhood?

A

Starts in the first five years of life and is a rare but serious condition in which an infant or young child doesn’t establish healthy attachments with parents or caregivers.
The syndrome probably occurs as a direct result of severe parental neglect, abuse, or serious mishandling.

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32
Q

What is disinhibited social engagement disorder?

A

A particular pattern of abnormal social functioning that arises during the first five years of life
those with DSED appear to be extremely friendly and outgoing. They exhibit socially disinhibited behavior. This means they are impulsive and can easily talk to unknown people and random strangers. However, they may have trouble forming stable or meaningful bonds with others.
Causes include an absence of caregiver in first few years of life, lack of emotional support whilst growing up, neglect by caregiver, repeated changing of caregivers, negative experiences, or growing up in foster care or orphanages

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33
Q

What is elective mutism?

A

a refusal to speak in almost all social situations despite normal ability to do so. often attributed to defiance or the effect of trauma
Now also known as selective mutism

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34
Q

What is separation anxiety disorder of childhood?

A

differentiated from normal separation anxiety when it is of a severity that is statistically unusual and has an abnormal persistence beyond the usual age period, and when it is associated with significant problems in social functioning.

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35
Q

What is sibling rivalry disorder?

A

emotional disturbance usually following the birth of an immediately younger sibling is normal but this disorder should only be diagnosed if the degree/persistence of the disturbance is both statistically unusual and associated with abnormalities of social interaction.
The most common symptom of sibling rivalry disorder is frequent or continuous demands for attention

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36
Q

What is a tic?

A

an involuntary, rapid, recurrent, nonrhythmic motor movement (usually involving circumscribed muscle groups) or vocal production that is of sudden onset and that serves no apparent purpose. Tics tend to be experienced as irresistible but usually they can be suppressed for varying periods of time, are exacerbated by stress, and disappear during sleep.

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37
Q

What are common motor tics?

A

Simple - eye blinking, neck-jerking, shoulder-shrugging and facial grimacing
Complex - hitting oneself, jumping, hopping

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38
Q

What are common vocal tics?

A

Simple - throat-clearing, barking, sniffing and hissing
Complex - religion of particular words, often social unacceptable (coprolalia) and the repetition of one’s own sounds or words (palilalia)

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39
Q

What is transient tic disorder?

A

tics don’t persist for >12 months. Usually eye blinking, facial grimacing or head jerking

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40
Q

Whats a chronic motor or vocal tic disrder?

A

motor or vocal tics (not both) that last for more than a year

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41
Q

Whats Tourette’s syndrome/Gilles de la Tourette syndrome?

A

multiple motor tics and 1 or more vocal tics. Disorder worsens during adolescence and tends to persist into adult life.
The vocal tics are often multiple with explosive repetitive vocalizations, throat-clearing, and grunting, and there may be the use of obscene words or phrases.

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42
Q

What is coprolalia?

A

the involuntary and repetitive use of obscene language, as a symptom of mental illness or organic brain disease.

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43
Q

What is nonorganic enuresis?

A

A disorder characterized by involuntary voiding of urine, by day and by night, which is abnormal in relation to the individual’s mental age, and which is not a consequence of a lack of bladder control due to any neurological disorder, to epileptic attacks, or to any structural abnormality of the urinary tract. The enuresis may have been present from birth or it may have arisen following a period of acquired bladder control.

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44
Q

What is nonorganic encopresis?

A

Repeated, voluntary or involuntary passage of faeces, usually of normal or near-normal consistency, in places not appropriate for that purpose in the individual’s own sociocultural setting. The condition may represent an abnormal continuation of normal infantile incontinence, it may involve a loss of continence following the acquisition of bowel control, or it may involve the deliberate deposition of faeces in inappropriate places in spite of normal physiological bowel control

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45
Q

What is feeding disorder of childhood and infancy?

A

It generally involves food refusal and extreme faddiness in the presence of an adequate food supply, a reasonably competent caregiver, and the absence of organic disease. There may or may not be associated rumination (repeated regurgitation without nausea or gastrointestinal illness).

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46
Q

What is pica of infancy and childhood?

A

Persistent eating of non-nutritive substances (such as soil, paint chippings, etc.). It may occur as one of many symptoms that are part of a more widespread psychiatric disorder (such as autism), or as a relatively isolated psychopathological behaviour; only the latter is classified here. The phenomenon is most common in mentally retarded children

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47
Q

What are stereotyped movement disorders?

A

Voluntary, repetitive, stereotyped, nonfunctional movements that do not form part of any recognized psychiatric or neurological condition.
The movements that are of a non self-injurious variety include: body-rocking, head-rocking, hair-plucking, hair-twisting, finger-flicking mannerisms, and hand-flapping.
Stereotyped self-injurious behaviour includes repetitive head-banging, face-slapping, eye-poking, and biting of hands, lips or other body parts.
All the stereotyped movement disorders occur most frequently in association with mental retardation

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48
Q

What is stuttering?

A

Speech that is characterized by frequent repetition or prolongation of sounds or syllables or words, or by frequent hesitations or pauses that disrupt the rhythmic flow of speech. It should be classified as a disorder only if its severity is such as to markedly disturb the fluency of speech.

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49
Q

What is cluttering?

A

A rapid rate of speech with breakdown in fluency, but no repetitions or hesitations, of a severity to give rise to diminished speech intelligibility. Speech is erratic and dysrhythmic, with rapid jerky spurts that usually involve faulty phrasing patterns.

50
Q

Whats the prevalence of ADHD?

A

1%

51
Q

What are the core features of ADHD?

A

Hyperactivity - excessive motor activity when it is not appropriate (such as running around), or by excessive fidgeting, tapping, or talkativeness.
Impulsivity - hasty actions that occur in the moment without forethought and that have high potential for harm for the individual. Impulsive behaviour may manifest as social intrusiveness and/or making important decisions without considering the long-term consequences.
Inattention - manifested by wandering off task, lacking persistence, having difficulty in sustaining focus and being disorganized.

52
Q

Whats the criteria for ADHD?

A

Symptoms must start before 12 years of age.
Occur in two or more settings, such as at home and school.
Have been present for >6 months.
Interfere with, or reduce the quality of social, academic or occupational functioning.

53
Q

Whats the rating scale for ADHD?

A

Conners score

54
Q

How likely is it for there to be a comorbidity with ADHD?

A

ADHD is associated with increased risks of psychiatric disorders- ODD, conduct disorder, substance abuse, and possibly mood disorders. Autism spectrum disorder, dyslexia, dyscalculia, and dyspraxia are also over-represented.

55
Q

What are the long term effects of unrecognised ADHD?

A

conduct disorders, poor academic performance, poor peer relationships, low self-esteem and social skills.

56
Q

Whats the cause of ADHD?

A

The exact cause of ADHD is unknown but involves the interplay of multiple genetic and environmental factors that are thought to lead to altered brain neurochemistry and structure.
There is substantial evidence for a genetic contribution to ADHD, with a mean heritability of 76% demonstrated in twin studies. It has been hypothesised that several genes may interact to cause ADHD, or that ADHD may be the common phenotype for numerous variant alleles.
Environmental factors most strongly associated with ADHD are low birth weight and maternal smoking during pregnancy. Other risk factors include preterm delivery, epilepsy, acquired brain injury, lead exposure, iron deficiency, alcohol exposure during pregnancy, psychosocial adversity, and adverse maternal mental health.

57
Q

How does ADHD affect boys and girls differently?

A

B:g 2-5:1

58
Q

Whats the prognosis for ADHD?

A

At 25 years of age approximately 15% retained the full ADHD diagnosis, 65% were in ‘partial remission’
Over time, inattentive symptoms tend to persist and hyperactive-impulsive symptoms tend to recede.

59
Q

How do you manage ADHD in pre-school children?

A

ADHD-focused group parent-training programme is normally recommended first-line.
If ADHD symptoms across settings are still causing a significant impairment after environmental modifications have been implemented and reviewed, advice will be sought from a specialist ADHD service with expertise in managing ADHD in young children. Drug treatment may be considered with input from this service.

60
Q

How do you manage ADHD in school-aged children?

A
  1. Group-based support - providing education and information on causes and impact of ADHD as well as advice on parenting strategies
  2. Individual parent-training programmes for parents/carers when there are particular diffiuclties for families in attending group sessions
  3. Medication if symptoms are still causing a persistent significant impairment after environmental modifications have been implemented and reviewed.
  4. Course of CBT in those who have benefits from medication but whose symptoms are still causing significant impairment
61
Q

What medication can be offered to help manage ADHD sympotms?

A
  1. Methylphenidate (central nervous system stimulant - noradrenaline and dopamine reuptake inhibitor)
    Alternatives include lisdexamfetamine, dexamfetamine and atomoxetine
    Melatonin may be prescribed for those with insomnia and sleep hygiene measures have been insufficient
62
Q

How do you manage ADHD in adults?

A

Medication will usually be offered if ADHD symptoms are still causing a significant impairment after environmental modifications have been implemented and reviewed.

Non-pharmacological treatment in combination with medication may be considered for adults with ADHD who have benefited from medication but whose symptoms are still causing significant impairment. This can include a structured supportive psychological intervention focused on ADHD, regular follow-up either in person or by phone, and/or elements of or a full course of CBT.

63
Q

What are the main side effects of methylphenidate?

A

Loss of appetite
Sleep disturbances
Nausea
Headache
Raised bp
Increased anxiety

64
Q

What monitoring is done and how often, when on methylphenidate?

A

Monitor height, weight, pulse, bp at least 6 monthly

65
Q

What is CAMHS?

A

Child and mental health services

A service for all children up to 18 years where there is a mental health issue. They work with families and young people. Liaison with other agencies including social services, education, voluntary sector and health.

66
Q

Outline the tier system in CAMHS?

A

Tier 1 - early intervention and prevention, provided by schools, children centres, health visitors, school nurses
Tier 2 - early help and targeted services
Tier 3 - specialist teams e.g. ED services
Tier 4 - tertiary services/inpatient service

67
Q

What should you do if you suspect child maltreatment in primary care?

A

Refer immediately to either the local child social services, police or NSPCC. These all have statutory child protection powers to act immediately to secure the safety of the child.
Where there is no immediate danger, the local child social services os the preferred route.
Where there is immediate danger to a child the police have the authority under the Children’s Act to enter a house and remove a child for their safety for 72 hours. Examples of emergencies are recent sexual assault, unprotected child at risk of serious harm or any baby with signs of non-accidental injury.

68
Q

What is autism spectrum disorder characterised by?

A

Developmental impairments in social interaction
Impairments in social communication
Restricted repetitive patterns of behaviour, interests or activities

69
Q

What are examples of impairments in social interaction in ASD?

A

Difficulties in developing, maintaining and understanding relationships
Difficulties in engaging in normal conversations and sharing interests or emotions

70
Q

What are examples of impairments in social communication in ASD?

A

Using non-verbal communication in social interactions.
This may manifest as a lack of eye contact or a total lack of facial expression when interacting with others.

71
Q

What are examples of restricted, repetitive patterns of behaviours in ASD?

A

Stereotyped or repetitive motor movements (stimming), use of objects or speech, and idiosyncratic phrases
Inflexible adherence to daily routines or ritualised patterns of behaviour
Highly restricted, fixed interests that are abnormal in intensity or focus
Fascination with sensory aspects of the environment or over/under reactivity to sensations
Little imagination

72
Q

Whats the prevalence of ASD? Whats the ratio for M:F?

A

1 in 100
M:F 3-5:1

73
Q

What proportion of those with autism have an area in which they excel?

A

1 in 7

74
Q

When does autism have its onset?

A

Typically during the developmental period, in early childhood. But characteristic symptoms may not fully manifest until later when social demands exceed limited capacities

75
Q

What are some associated conditions with autism?

A

Mental retardation in 75%
Epilepsy in 30%
Mental health or behavioural disorders in 70%

76
Q

Whats the aetiology of autism?

A

Genetics - Strong underlying predisposition. Conditions such as Rett syndrome or fragile X syndrome are associated. 60x more common in twins
Environmental factors (e.g., toxin exposure, prenatal infections, gestational age <35/40, maternal use of sodium valproate etc) - May increase the risk of ASD. However, no specific causes have been identified.

77
Q

What are some difficulties in children associated with autism?

A

The impaired ability to learn in social situations - consequently, academic achievement is usually poor even for children and young people with an average or above average intelligence
Complicated routine care, such as for eating and sleeping.
Behaviour that challenges (defined as behaviour of such an intensity, frequency, or duration that the physical safety of the person, or others around them, is likely to be placed in serious jeopardy. It also includes behaviour that is likely to severely limit or delay access to and use of ordinary community facilities
An increased vulnerability to mental health problems, such as anxiety and depression.
High levels of stress in parents and/or carers and siblings.

78
Q

What are some difficulties in adults associated with autism?

A

Unemployment and the inability to live independently (only a minority of affected people with lower levels of impairment manage to work and live independently).
Poor general health (because people with ASD often do not seek help for medical problems).
An increased vulnerability to mental health problems, such as anxiety and depression.
Social isolation.

79
Q

Whats the prognosis of autism?

A

Unaffected language development and the absence of an associated intellectual disability are associated with a more favourable prognosis
The prognosis of ASD can be improved by early diagnosis and assessment because this aids understanding of why the child or young person is different from their peers.
Early diagnosis also ensures that the child or young person and the family and/or carers have prompt access to support and services in education, health services, and social care; a route into voluntary organizations; and contact with other children and families with similar experiences.

80
Q

What should you consider for the assessment environment when diagnosis autism?

A

Giving pt personal space
Avoiding patterns on walls/furnishings
Quiet noise levels (sound sensitivity)
Breaks
Visual cues and supports
Lighting (many experience hypersensitivity to lights)

81
Q

How is autism managed?

A

Family support
Behavioural methods
Social skills groups
Teach support
Special schooling
Anger management programme
Counselling
Practical help - e.g. housing, employment, educational services, finance
CBT for depression and anxiety

82
Q

What is Aspergers?

A

Impairment in social interaction couples with restricted, stereotyped interest and behaviours
However there are no significant abnormalities in language or cognitive development and symptoms are less severe than autism

People with Asperger’s typically have a normal or above-average intelligence without learning disabilities

83
Q

Whats the prevalence of Rett’s syndrome?

A

1 in 12,000

84
Q

What are examples of genetic conditions that commonly coexist with autism?

A

Fragile X syndrome
Tuberous sclerosis
Rett syndrome
Down syndrome
Neurofibromatosis

85
Q

What environmental factors are related to autism?

A

Prenatal infections
Perinatal complications
Exposure to toxins
Exposure to teratogens

86
Q

Whats Rett syndrome?

A

A rare neurological developmental disorder that impairs motor functioning
It’s X-linked autosomal dominant MeCP2 gene mutation (spontaneous)

87
Q

When does Rett syndrome typically begin to present?

A

6-18 months

88
Q

What are the stages of Rett syndrome?

A

Stage 1 is 6-18 months - decreased playfulness, eye contact, delayed development may begin (often this stage isn’t noticed until retrospectively looking at it)
Stage 2 is rapid deterioration and occurs at 1-4 years - massive regression in speech and motor skills, repetitive hand movements, breathing irregularities (hyperventilating or breath holding), and acquired microcephaly.
Stage 3 is plateaua stage and occurs at 2-10 - improvements in attention span, communication and eye contact but… seizures may occur and may develop apraxia (inability to perform purposeful movements)
Stage 4 is the late motor deterioration and occurs at ~10 years - progressive msucle weakness, rigidity, spasticity, scoliosis. Person remains cognitively stable

89
Q

Whats the prognosis for Retts syndrome?

A

After a decade most are bound to a wheelchair with incontinence, muscle wasting and rigidity and limited language ability
Life expectancy 40-50

90
Q

Whats the prevalence of childhood disintergrative disorder?

A

1.7 in 100,000

91
Q

How is Rett syndrome distinguished from childhood disintegrative disorder?

A

Rett syndrome exclusively affects girls and has distinct midline stereotyped hand movements and deceleration in head growth.

In Rett syndrome the period of normal development is usually short (ie, typically 6 months rather than a few years in CDD)

92
Q

How does childhood disintegrative disorder present?

A

about 2 years of normal development, followed by a loss of previously acquired skills e.g. language, social, adaptive skills, bladder and bowel control, motor skills. This happens before the age of 10 but commonly occurs in ages 3-4. It’s also associated with an autism-like impairment of social interaction as well as repetitive stereotyped interests and mannerisms. So, after deterioration, these children may resemble autistic children

93
Q

What is fragile X syndrome?

A

An autosomal dominant X-linked disease
Caused by a mutation in FMR1 gene
Noticeable by age 2 and lifelong

94
Q

What is Tuberous sclerosis?

A

a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain, kidneys, heart, eyes, lungs and skin.
Mutation in either TSC1 or TSC2 = unable to switch off mTOR so benign tumours called hamartoma form
Symptoms - hamartomas in brain can cause seizures, learning difficulties, cognitive deficits. Large angiomyolipoma can cause flank pain. Hamartomas in lungs can cause SOB and in retinas can cause vision changes

Normal life expectancy but lifetime cancer risk is increased

95
Q

What is Neurofibromatosis?

A

A group of conditions in which benign tumours grow in the nervous system
Autosomal dominant inheritance pattern
Type 1 is associated with neurodevelopmental disorders including autism spectrum disorder (ASD)

96
Q

Whats the prevalence of mental retardation?

A

1-2%
M:f 1.5:1

97
Q

What are the 3 core criteria for mental retardation?

A

• low intellectual ability (IQ <70)
• Significant impairment of social or adaptive functioning
• Onset in childhood

98
Q

Whats the IQ range and functioning in mild mental retardation?

A

50-69
Delayed but usually adequate use of language and self-care
Diffiuclties in academic work but can be greatly helped by educational programmes
Usually capable of unskilled or semi-skilled manual labour
May be able to live independantly

99
Q

Whats the IQ range and functioning in moderate mental retardation?

A

35-49
Language and comprehension are limited
Self-care and motor skills are retardation
May be able to do some simple practical work with supervision
Completely independant living is rarely achieved and they usually settle in supervised accommodation

100
Q

Whats the IQ range and functioning in severe mental retardation?

A

20-34
Marked degree of motor impairment
Little or no speech during early childhood; may learn to talk in school age
Capable of only elementary self-care skills
May be able to perform simple tasks under close supervision
Settle in group homes/family settings

101
Q

Whats the IQ range and functioning in profound mental retardation?

A

<20
Severely limited in ability to understand or comply with instructions
Severe motor impairment with restricted mobility and incontinence
Little or no self care
Usually require residential care

102
Q

What are some genetic causes of mental retardation?

A

Chormosmal - Down’s syndrome, fragile X syndrome, prader-William syndrome
Other - PKU, neurofibromatosis, tuberous sclerosis, lesch-nyhan syndrome, Tay-sachs disease, Rhett syndrome, Williams syndrome

103
Q

What are some prenatal causes of mental retardation?

A

Congenital infections - toxoplasmosis, rubella, cytomegalovirus, herpes simplex, zoster syphilis, AIDS
Substance use during pregnancy or prescribed drugs with teratogenic effects
Complications of pregnancy e,g, pre-eclampsia, IUGR, antepertum haemorrhage

104
Q

What are some perinatal causes of mental retardation?

A

Birth trauma and hypoxia
Prematurity
Kernitecterus
Intraventricular haemorrhage
Infections

105
Q

What are some environmental causes of mental retardation?

A

Poor, socioculturally deprived children
Neglect
Malnutrition
Poor linguistic and social stimulation
Abuse

106
Q

What are some psychiatric conditions that can cause mental retardation?

A

Pervasive developmental disorders e,g. Retts syndrome

107
Q

How can you prevent mental retardation?

A

Improved perinatal and child healthcare
Early detection of metabolic abnormalities
Genetic counselling, amniocentesis and chorionic villus sampling with the option of therapeutic abortion in pregnant women over 35 or with a Fhx of genetic disorders

108
Q

What are the reasons for premature deaths in LD?

A

Diagnostic overshadowing
Inability to identify signs of pain
Issues around consent and capacity
Excluding family and carers from best interest decisions
Opinions regarding QOL

109
Q

What are associated conditions with LDs?

A

25% visual impairment
25% hearing impairment
25% epilepsy
10-20% mental health problems
Dementia 4-5 times more likely
Cerebral palsy
H. Pylori infections
Coronary heart disease in 50% of those with Down’s syndrome
Hypothyroidism in 30% of those with downs
Autism

110
Q

What are some examples of barriers for those with LDs to access health?

A

Letters they don’t understand, hard to understand phone call options and touch screen systems, not able to read name badges, not able to understand speciality names e.g. orthopaedic, doctor speaking directly to carer and not person, using medical jargon, busy waiting rooms, 10 minute consultations aren’t long enough, struggling to understand what’s happening/what meds they take/why examinations need to be done etc…

111
Q

What is a community learning disability team?

A

an integrated team of social care and health staff. This team is made up of: community learning disability nurses, psychiatrists, psychologists, occupational therapists, speech and language therapists, physiotherapists and social workers.

112
Q

What is STOMP?

A

Stopping The Over Medication of People with a learning disability and/or autism

113
Q

What monitoring is done for those with learning disabilities?

A

It’s important that everyone over 14 who is on their doctor’s learning disability register has an annual health check. This will involve a physical check, checking urine/blood, ask about common problems e.g. changes in eyesight or epilepsy, talk about medications and vaccinations, ask if family and careers need extra help…

114
Q

What is the attachment theory?

A

This theory suggests the ability to form an emotional and physical attachment with another person is largely influenced by the early stages of attachment formed between a child and their primary caregiver.

115
Q

Why are there not many males with Rett syndrome?

A

Because they only have 1 X chromosome so when they have a mutated MeCP2 gene, they cannot make functional MeCP2 protein and therefore they die in utero or shortly after birth

116
Q

Which group of males may develop Rett syndrome and why?

A

Those with Klinefelter syndrome
They have XXY genotype so two X chromosomes means that even if they have a mutated MeCP2 gene, the other MeCP2 gene can make functional MeCP2 protein

117
Q

Why is the onset of Rett syndrome not from birth?

A

As the low levels of MeCP2 are sufficient at birth but as the brain grows and develops, the amount becomes insufficient and the brain fails to develop normally
MeCP2 is important for establishing neuronal connections ans therefore for brain development

118
Q

Other than neurological complications, what can Rett syndrome also cause?

A

Prolonged QT syndrome

119
Q

What is FMR1 gene?

A

Fragile X mental retardation 1
It expresses FMRP

120
Q

What are the symptoms of Fragile X?

A

Intellectual disability
Delayed speech
Delayed motor development
SOmtimes- autism, adhd, seizure disorders

Long narrow face, prominent jaw and forehead, large ears that stick out (more obvious when older)
Larger than usual testes after puberty

121
Q

Whats the difference between fragile X in females vs males?

A

Less severe symptoms or no symptoms in females due to their backup FMR1 gene on their other X chromosome

122
Q

Whats the function of the secure base?

A

To provide comfort
To allow the child to explore the environment