Developmental

Question 1

Gross motor milestones in a 6 week old

Answer 1

Head lag still present on pulling from supine to sitting position.

Head can be held in same plane as body when held in ventral suspension.

Question 2

Fine motor and vision at 6 weeks

Answer 2

Fixes and follows through 90 degrees in the horizontal plane

Question 3

Language at 6 weeks

Answer 3

Makes throaty noises

Question 4

Social milestones at 6 weeks

Answer 4

Smiles in response to appropriate stimuli

Question 5

Gross motor milestones at 3 months

Answer 5

• Able to raise head and chest on forearms in prone position
• Head steady when pulled to sit

Question 6

Fine motor and vision milestones at 3 months

Answer 6

• Fix and follow through 180 degrees in horizontal plane.
• Hands beginning to be brought to midline
• Attempts to make contact with offered object.

Question 7

What language and communication skills would you expect from an 18 month old child?

Answer 7

• Single words - up to 50 Jargon communication
• Word strings…mum…car…keys
• Pseudo 2 word utterances….allgone
• Dances or bounces to music
• Follows simple one-step instructions
• Looks at person talking
• Identifies objects in a book
• Looks for objects out of sight

Question 8

What language and communication skills would you expect from a 24 month old child?

Answer 8

• 2 words together ….daddygone
• Uses words to request objects rather than just to name
• Can ask questions….whats this?
• Can answer questions…whats this?
• Names objects and pictures spontaneously
• Responds to simple to part requests
• Uses negatives….no
• Points to some body parts when asked
• Use of jargon decreases

Question 9

What language and communication skills would you expect from a 3 yr old child?

Answer 9

• 3 or more word sentences
• Vocab of several hundred words
• Talks about things not present eg. happened in the past
• Uses some adjectives and adverbs
• Talks about actions of others
• Adds information to that of others
• Asks increasing number of questions
• Answers Qs
• Refers to self with full name
• Enjoys repeating words and sounds
• Initiates simple actions
• Can listen attentively to short stories and books

Question 10

Autism is a neurobehavioural disorder involving a triad of impairments/restrictions. What is the triad?

Answer 10

1. Impairments in social skills
2. Impairments in communication
3. Repetitive and stereotype patterns of behaviour

Question 11

At 9, 12 and 18 months what do you expect in joint attention?

Answer 11

• 9 mo follow pointing
• 12mo proto imperative pointing (object is the goal)
• 18mo proto declarative pointing (sharing is the goal), bring objects to show caregiver

Question 12

For a couple who already have a child with ASD, what is the chance of the next child also having ASD?

Answer 12

20%

Question 13

In ASD, the antipsychotic risperidone can help with what type of behaviour?

Answer 13

Self-injurious behaviour

Question 14

The PURPLE period describes a time in infancy which is stressful for parents due to the infants crying. What does the PURPLE stand for?

Answer 14

P - Peak of Crying. Baby cries more each week. Cries most at 2 months and then decreases at 3-4 months

U - Unexpected. Crying can come and go and you don’t know why.

R - Resists soothing. Your baby may not stop crying no matter what you try.

P - Pain-like face. Baby may look like they are in pain, even when they are not.

L - Long lasting. Can last up to 5 hours per day, sometimes more.

E - Evening. Your baby may cry more in the late afternoon and evening.

Question 15

What is the normal developmental task of the “terrible two’s”? (Toddler period)

Answer 15

Develop autonomy and independence.

Question 16

What are the main developmental tasks in the preschool period (3-5 years)?

Answer 16

Language, cognitive ability and social-emotional skills.

Question 17

What is narcolepsy?

Answer 17

Clinical syndrome of excessive daytime sleepiness with cataplexy, hypnagogic hallucinations, and sleep paralysis.

Question 18

What is insomnia of childhood?

Answer 18

Repeated difficulty initiating and/or maintaining sleep that occurs despite age-appropriate time and opportunity for sleep.

Question 19

One of the most common sleep disorders found in infants and toddlers is behavioural insomnia of childhood, sleep onset association type. Why do children develop this disorder?

Answer 19

The child learns to fall asleep only under certain conditions or associations which typically require parental presence, ie. being rocked or fed, and does not develop the ability to self soothe.

During the night, the pt then wakes as part of normal sleep cycle (brief arousal at end of 60-90 min cycle) and is not able to go back to sleep without the same conditions being present.

Question 20

Behavioural insomnia of childhood, limit setting type is a type of sleep disturbance more common in preschool aged and older children. How does this disorder develop and what type of behaviours do the children display?

Answer 20

Often the result of parental difficulties in limit setting, and managing behaviour. Includes the unwillingness to set consistent bedtime rules and enforce a regular bedtime routine. May be exacerbated by the childs oppositional behaviour.

Typical behaviours include stalling and refusing to go to bed.

Question 21

What is psychophysiologic or primary insomnia?

Answer 21

AKA “learned insomnia”. Insomnia that is not primarily a result of parent behaviour, another sleep disturbance or a psychiatric illness.

Usually occurs in adolescents and is characterised by learned sleep preventing associtions and heightened physiologic arousal resulting in sleeplessness and decreased daytime functioning.

A hallmark is excssive worry about sleep and an exaggerated concern of the potential daytime consequences.

Question 22

What is the BEARS screening tool?

Answer 22

A screening tool assessing the five major sleep disorders affecting children aged 2-18 years.

B - Bedtime problems

E - Excessive daytime sleepiness

A - Awakenings during the night

R - Regularity and duration of sleep

S - Snoring

Question 23

Sleepwalking, sleep terrors and confusional arousals are all partial arousal parasomnias which are more common in preschool and school aged children because of the relatively higher percentage of slow wave sleep in younger children.

What are the clinical manifestations of the partial arousal parasomnias?

Answer 23

• Usually amnesia for the events.
• Occurs at transition out of “deep” or SWS so has clinical features of awake (ambulation, vocalizations) and sleeping (high arousal threshold, unresponsiveness to the environment) states.
• Typically during first few hours of sleep
• Few minutes to an hour
• Sleep terrors are sudden in onset with high degree of autonomic arousal (tachycardia, dilated pupils).
• Attempts at comforting or waking results in increased agitation. Pt also avoids comforting and waking.

Question 24

According to Erikson’s, what psychosocial crises occur at ages:

1. 0-2 years
2. 2-4 years
3. 4-5 years
4. 5-12 years
5. 13-19 years

Answer 24

1. Basic trust vs mistrust
2. Autonomy vs shame and doubt
3. Initiative vs guilt
4. Industry vs inferiority
5. Identity vs role confusion

Question 25

What are some impaired social skills demonstrated by a child with ASD?

Answer 25

1. Non-verbal behaviours eg eye contact, body posture 2. Failure to develop peer relationships 3. Lack of social and emotional sharing

Question 26

What are some impairments in verbal and non verbal communication demonstrated by a child wtih ASD?

Answer 26

1. Delay in development of spoken language 2. No attempt to communicate by other means 3. Inability to initate conversation 4. Stereotyped and repetitive language, lack of imaginative play

Question 27

What are some of the repetitive and stereotype patterns of behaviour demonstrated by a child with ASD?

Answer 27

1. Adherence to routines 2. Lack of imaginative play and behaviour 3. Restrictive patterns of interest 4. Preoccupation with parts of objects 5. Repetitive motor mannerisms eg. hand flapping, door closing

Question 28

Cataplexy is pathognomonic for narcolepsy. The prevalenceo of narcolepsy with cataplexy is 0.2-0.5/10,000 (cf 3-16 per 10,000 of narcolepsy without cataplexy). What is cataplexy?

Answer 28

* An abrupt, bilateral, partial or complete loss of muscle tone, classically triggered by an intense positive emotion (eg. laughter, surprise). * Typically brief - seconds to minutes * Fully reversible with complete recovery to normal tone when episode ends. * Rarely the first symptom of narcolepsy but often presents within 12 months of EDS (Excessive Daytime Sleepiness).

Question 29

What is the treatment for narcolepsy?

Answer 29

**_Non Pharmacologic_** * Avoid drugs that can produce daytime sleepiness or insomnia. * Napping/sleep hygeine - one to two 20 minute naps will often improve sleepiness for 1-3 hours. Sleep deprivation may worsen symptoms so pts should maintain regular and adequate sleep schedule. * Psychosocial support **_Pharmacologic_** * **Modafinil -** non-amphetamine "wakefullness promoting agent". MOA not well understood but may increase dopaminergic signalling. * **Methylphenidate -** CNS stimulant and potent wakefullness promoting drug. Second line agent due to sympathomimetic side effects. * **Amphetamines** - CNS stimulants and most potent wakefulness promoting drugs. Also second line agent due to sympathomimetic side effects. Can also improve cataplexy, hypnagogic hallucinations and sleep paralysis.

Question 30

What is enuresis?

Answer 30

Repeated voiding of urine into clothes or bed at least twice a week for at least 3 consecutive months in a child who is at least 5 yr of age, not caused by medications or a medical condition. Diurnal enuresis defines wetting while awake and nocturnal enuresis refers to voiding during sleep.

Question 31

What is the difference between primary and secondary enuresis?

Answer 31

**Primary enuresis** occurs in children who have never been consistently dry through the night **Secondary enuresis** refers to the resumption of wetting after at least 6 months of dryness.

Question 32

What is the prevalence of enuresis at ages 5 and 10 years?

Answer 32

5 years: boys 7% and girls 3% 10 years: boys 3% and girls 2%

Question 33

What language and communication skills would you expect to see in a 3 month old?

Answer 33

* Regards speaker * Chuckles * Vocalizes when talked to

Question 34

Social milestones at 3 months?

Answer 34

* Expression of disgust (sour taste, loud sound) * Visually follows person who is moving across a room

Question 35

Fine motor and vision milestones in a 6 month old?

Answer 35

* Transfers hand to hand * Reaches with one hand

Question 36

Gross motor milestones in a 6 month old?

Answer 36

* Sits momentarily propped on hands * Pivots in prone position * Prone--bears weight on one hand * Feeds self crackers * Places hands on bottle

Question 37

Language milestones at 6 months?

Answer 37

* Stops momentarily to "no" * Gestures for "up" * Listens then vocalizes when adult stops * Smiles/vocalizes to mirror

Question 38

Social milestones at 6 months?

Answer 38

* Stranger anxiety: recognizes familiar versus unfamiliar people

Question 39

Gross motor milestones at 12 months?

Answer 39

* Stands well with arms high and legs splayed (posterior protection) * Independent steps

Question 40

Fine motor and vision milestones at 12 months?

Answer 40

* Scribbles after demo * Fine pincer grasp of pellet * Holds crayon * Attempts tower of two cubes

Question 41

Language milestones at 12 months?

Answer 41

* Follows one-step command with gesture * Recognizes names of two objects - looks when named * Points in order to get desired object (proto-imperative pointing * Uses several gestures with vocalizing (waving, reaching, etc)

Question 42

Social milestones at 12 months?

Answer 42

* Shows objects to parent to share interest * Points in order to get desired object (proto-imperative pointing)

Question 43

Social milestones at 18 months?

Answer 43

* Engages in pretend play with other people * Begins to show possessiveness and shame (when does wrong)

Question 44

Social milestones at 2 years?

Answer 44

* Parallel play * Begins to mask emotions for social etiquette

Question 45

Social milestones at 3 years?

Answer 45

* Sharing * Fear of imaginary things * Imaginative play * Uses words to describe what someone else is thinking

Question 46

Like children with Autistic Disorder, children with Aspergers Disorder have **impairments in social interactions** and show **restrictive, repetitive** patterns of behavior, interests, or achievements with other people which cause significant impairments in social or occupational functioning. Unlike ASD however, children with Aspergers disorder meet their normal language milestone with single words and 2 years and communicative phrases at 3 years. What communication deficits does a child with Aspergers disorder have?

Answer 46

Deficits in nonverbal and pragmatic aspects of communication (facial expressions, gestures) but do not have the severe language delays and impairments that characterize AD.

Question 47

What are the characteristics of ADHD?

Answer 47

* Inattention, including increased distractibility and difficulty sustaining attention * Poor impulse control and decreased self-inhibitory capacity * Motor overactivity and motor restlessness

Question 48

Ritalin, Ritalin LA and Concerta (all methylphenidate) are a psychostimulant medication used in the management of ADHD. Ritalin is immediate release and has a duration of action is 3-4 hours. Ritalin LA and concerta are extended release and have a duration of action of 8-10 and 10-12 hours respectively. What are the significant side effects of this medication?

Answer 48

* Moderate appetite suppression * Mild sleep disturbances * Transient weight loss * Irritability * Emergence of tics

Question 49

Strattera (atomoxetine) is an extended release psychostimulant with a 12 hour duration of action used in management of ADHD. What are the significant side effects of this medication.

Answer 49

* Nervousness, sleep problems, fatigue, stomach upset, dizziness, dry mouth * Can lead in rare cases to severe liver injury or to suicidal ideation

Question 50

What is neuroleptic malignant syndrome?

Answer 50

* A rare and potentially fatal reaction that can occur during treatment with **antipsychotic agents**. * The syndrome generally manifests with **fever, muscle rigidity, autonomic instability, and delirium**. * Associated with elevated serum creatine phosphokinase levels, a metabolic acidosis, and high end-tidal CO2 excretion.

Question 51

In ADHD neuroimaging studies indicate smaller brain volumes in some brain structures. What are these?

Answer 51

Pre frontal cortex and basal ganglia. A 5-10% reduction has been found in children with ADHD. These structures are rich in dopamine receptors and medications used in the treatment of ADHD have a dopaminergic action. This has lead to the **dopamine hypothesis** which postulates that disturbances in the dopamine system may be related to the onset of ADHD.

Question 52

What is the most common underlying neurocognitive deficit in children with reading disability (developmental dyslexia)?

Answer 52

Phonemic awareness Phonemese are the smallest units of sound. ie. Cat can broken into three sounds - c/a/t

Question 53

Name the cranial sutures.

Answer 53

1. Metopic 2. Coronal 3. Sagital 4. Lambdoid

Question 54

What are the important known co-morbid conditions to be excluded in Down Syndrome?

Answer 54

* Cardiac disease * Gut anomolies: duodenal atresia, hirschprung * Ophthalmology * Audiology - middle ear disease

Question 55

What conditions should be screened in a patient with Down Syndrome?

Answer 55

* Hypothyroidism - screen T4 and TSH every 2 years * Coeliac disease - occurs in 5% DS population. * OSA * Atlanto-axial instability

Question 56

Looking at the FMR-1 gene, a triplet repeat of \>200 leads to what?

Answer 56

Fragile X full mutation. 55-200 is a premutaton * Primary ovarian insufficiency * Fragile X associated tremor ataxia syndrome (FXTAS) * Social anxiety, depression * ADHD, ASD

Question 57

What are clinical features of Fragile X?

Answer 57

* Intellectual disabilities * Autism * Attention defecits * Facial features * Macro-orchidism (usually post puberty)

Question 58

What is FXTAS?

Answer 58

Fragile X Tremor Ataxia Syndrome Male premutation carriers (and to a lesser extent females) are at risk after their 50s of FXTAS with ataxia, parkinsonian features and cognitive defects.

Question 59

What is Rett Syndrome?

Answer 59

Mutation in MECP2 gene. Affects 1in 10 000 females * Normal early development * Withdrawn autistic behaviour, loss of language * Loss of hand skilss, stereotypic hand movements * Deceleration of head growth * Seizure disorder * Breathing irregularities * Progressive course with well described stages

Question 60

What is cerebral palsy?

Answer 60

A group of developmental disorders of movement and posture, causing activity restriction or disability, that are attributed to disturbances occurring in the fetal or infant brain. The motor impairment may be accompanied by a seizure disorder and by impairent of sensation, cognition, communication and/or behaviour. Defined by topography * Quadraplegia (all 4 limbs) * Hemiplegia (upper limbs) * Diplegia (lower limbs with minimal upper limbs) And pathophysiology * Pyramidal - predominantly spastic * Extrapyramidal - dyskinetic types including hypotonic, choreoathetoid, and ataxic

Question 61

What are the causes of poor nutrition in Cerebral Palsy?

Answer 61

Oro-motor/oro-pharyngeal inco ordination Slow rate of feeding Spillage \>50% Poor dentition Early satiety Parent/caregiver ratio Behaviour disturbance

Question 62

What are the pharmocological options for the management of drooling in a patient with cerebral palsy?

Answer 62

* Hyoscine (scopolamine) * Glycopyrolate if unsucessful * benztropine * atropine

Question 63

2008A Q16 At 24-months of age, what percentage of children will spontaneously feed a doll with a pretend bottle? A. 10% B. 25% C. 50% D. 75% E. 90%

Answer 63

E. 90%

Question 64

2008A Q26 What is the key developmental task of early infancy? A. Autonomy. B. Motor control. C. Secure attachment. D. Self-regulation. E. Separation.

Answer 64

C. Secure attachment.

Question 65

What is the classic history of night terrors?

Answer 65

Night terrors classically occur a few hours after onset of sleep in stage 3 or 4 sleep, they occur typically between the ages of 2 and 7 years and most often in boys. The child appears terrified and may scream with associated dilated pupils, tachycardia, tachypnea, and agitation. He/she remains unresponsive and difficult to rouse – attempts to do so may worsen the symptoms. The child has no memory of the event the following morning (unlike nightmares which occur later in the night and are associated with memory). Diagnosis is based on history therefore a sleep diary is the most useful next step in management. Rarely video EEG monitoring may be useful.