Paeds development and neurology (ILA 5) Flashcards

1
Q

what are the 4 functional areas of child development?

A
  1. gross motor
  2. fine motor and vision
  3. hearing, speech and language
  4. social, emotional and behaviour
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2
Q

A newborn should have which skills?

A

GM - limbs flexed, symmetrical, marked head lag on pulling up, fixed posture

FMV- fixes and follows face

HSL- starts to loud noises

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3
Q

At 6-8 weeks old, which skills should they have developed?

A

GM- raises head to 45 degrees in prone position

FMV- follows moving object and follows face by moving head

SEB- smiles responsively

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4
Q

At 6 months old, which skills are developed?

A

GM- sit without support with rounded back, lying on abdomen with arms extended

FMV- palmar grasp

HSL- double syllables “adah”

SEB- puts food in mouths, not shy

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5
Q

At 8-9 months old, which skills are developed?

A

GM- crawling, pulls to stand

FMV- points with finger, early pincer

HSL- says mama and dada

SEB- shy

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6
Q

At 12 months old, which skills are developed?

A

GM- stands independently, walk unsteadily

FMV- points, mature pincer grip

HSL- 2-3 words, understands name

SEB- drinks from a cup with 2 hands, uses spoon

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7
Q

At 15 months, which skills are developed?

A

GM- walks steadily

FMV- immature grip of pencil, tower of 2

HSL- 2-6 words, understands simple commands

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8
Q

At 18 months old, which skills are developed?

A

GM- squats to pick up toys

FMV- building a tower of 3, makes marks with crayons, circular scribble

HSL- 6-10 words, shows 4 parts of the body

SEB- use spoon to get food in mouth, helps with dressing

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9
Q

at 2 years old, which skills are developed?

A

GM- run, walk up stairs

FMV- draw and can copy a line, build a tower of 6

HSL- uses simple phrases

SEB- symbolic play, dry by day, pulls off some clothing

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10
Q

at 3 years old, which skills are developed?

A

GM- jumps, ride a bike

FMV- draws, tower of 9, copies a circle

HSL- talks constantly in 3-4 word sentences, understands 2 joined commands

SEB- interactive play, parallel play, takes turn

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11
Q

At what age do children play together?

A

4 years old

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12
Q

List the primitive reflexes evident at birth

A
  1. moro reflex= sudden extension of the head causes symmetrical extension then flexion of the arms
  2. grasp = flexion of fingers when object put in their hand
  3. rooting = head turns to stimulus when touched near the mouth
  4. stepping response= stepping movements when held vertically and feet touch a surface
  5. asymmetrical tonic neck reflect
  6. sucking reflex = child sucks when nipple / teat placed in mouth
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13
Q

List the postural reflexes that children develop

A

postural reflexes essential for independent sitting and walking

  1. labyrinthine righting = head moves in opposite direction to which body is tilted
  2. postural support= when held upright, legs take weight and baby may push up
  3. lateral propping = in sitting, arms extend on side which the child falls as a saving mechanism
  4. paracute = when suspended face down, baby arm extend to save themselves
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14
Q

List some prenatal causes of developmental delay

A

genetic e.g. downs syndrome, fragile X syndrome, neurofibromatosis

congenital infection e.g. rubella, CMV, HIV

Teratogenic e.g. alcohol, drug abuse

Metabolic e.g. hypothyroidism, phenylketonuria

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15
Q

List some perinatal causes of developmental delay

A

extreme prematurity

hypoxic ischaemic encephalopathy

Metabolic e.g. hypoglycaemia, hyperbilirubinaemia

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16
Q

List some postnatal causes of developmental delay

A

infections e.g. rubella, CMV, toxicoplasmosis

Trauma e.g. head injury

Metabolic e.g. hypoglycaemia

Vascular e.g. stroke

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17
Q

Which features of developmental delay would concern you and would therefore make a referral?

A

not smiling at 10 weeks

cannot sit unsupported at 12 months

cannot walk at 18 months

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18
Q

What is the most common cause of development problems?

A

cerebral palsy

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19
Q

What is global developmental delay?

A

slow/impaired acquisition of all developmental skills

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20
Q

Outline the multi-disciplinary team involved in a childs development

A
  1. paediatricians
  2. physiotherapists
  3. occupational therapists
  4. speech and language therapists
  5. clinical psychologists
  6. dietician
  7. specialist health visitor
  8. social worker
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21
Q

Outline “normal hearing” milestones from birth to 12 months old

A

after birth - startles and blinks at sudden noise

by 1 month - notice sudden prolonged sounds, pauses

by 4 months - quietens or smiles to sound of voice, turn head towards you

by 7 months- turns immediately to your voice

by 9 months - listens attentively to familiar everyday sounds, pleasure in babbling

by 12 months - shows response to own name, understands bye and no

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22
Q

List some possible behavioural changes that could indicate hearing loss

A
appears to daydream
watches speakers face for clues
wants to sit close to TV and volume louder
misunderstands
inappropriate answers
speech fuzzy 
irritable or aggressive
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23
Q

what are the 2 types of hearing loss?

A
  1. sensorineural = lesion in the cochlea or auditory nerve

2. conductive = from abnormalities in the ear canal or middle ear

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24
Q

List the causes of sensorineural hearing loss

A
  1. genetic - syndromes!
  2. congenital infections e.g. CMV, rubella
  3. postnatal infections e.g. meningitis
  4. cerebral palsy
  5. preterm
  6. head injury
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25
Q

Which syndromes are associated with sensorineural hearing loss?

A
Alports syndrome 
waardenburgs 
ushers
pondered 
jervell-Lange Nielsen
branchio-oto-renal syndrome
stickler
down syndrome
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26
Q

What are the clinical features of sensorineural hearing loss?

A
  1. irreversible
  2. profound hearing loss
  3. gets worse over time
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27
Q

How is sensorineural hearing loss managed?

A
  1. early amplification with hearing aids
  2. cochlear implants

+ sit at front of class, gestures, lip movements

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28
Q

Outline the causes of conductive hearing loss

A
  1. otitis media with effusion = glue ear
  2. eustachian tube dysfunction e.g. downs syndrome, cleft palate, pierre robin sequence
  3. middle ear infection
  4. perforated ear drum
  5. wax
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29
Q

What are the clinical features of conductive hearing loss?

A
  1. intermittent or resolves
  2. maximum of 60 dB hearing loss
  3. affects low frequency sounds more
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30
Q

How is conductive hearing loss managed?

A

test bone conduction and if doesn’t resolve in 3 months…

  1. decongestant or long course antibiotics
  2. insert tympanovstomy tubes (grommets)
  3. hearing aid
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31
Q

Which hearing tests are done in newborns?

A

Newborn Hearing Screening Programme
= “evoked otoacoustic emission test”
soft echo = healthy cochlea

if that comes back abnormal, do the “auditory brainstem response audiometry”

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32
Q

how is hearing tested as children get older?

A

distraction testing = performed at 6-9 months of age if not had newborn screening

visual reinforcement audiometry = useful to assess impairment in 10-18 months olds

> 2.5 years = performance testing and speech discrimination testing e.g. kendall toy test, McCormia toy test

3 years = pure tone audiometry

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33
Q

at what age do we develop 6/6 visual acuity?

A

at 3-4 years old

6/6 - if the patient can read the last line on a Snellen chart from 6m away

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34
Q

What can cause a squint?

A

family history
congenial cataracts
retinoblastoma

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35
Q

What is a squint and what are the two types of squint?

A

= strabismus = misalignment of the visual axes

  1. concomitant = common
  2. paralytic = rare
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36
Q

Describe concomitant squints and how they occur

A

usually due to refractive error in 1 or both eyes causing imbalance of extra ocular muscles

O/E - normal muscles so full movement of eye, squinting eye often turns inwards (convergent)

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37
Q

Describe paralytic squints and how they occur

A

due to paralysis of the motor nerves and extra ocular muscles (could be due to tumour)

O/E- limited movement of the eye

38
Q

How is a squint detected?

A

CORNEAL LIGTH REFLEX TEST
use pen torch held at 30cm distance to produce reflections of both corneas simultaneously
if light reflection not equal = squint!!

39
Q

Which test is used to identify the nature of the squint?

A

COVER TEST

when squint present and fixing eye covered, squinting eye moves to take up fixation

40
Q

How is a squint managed?

A
  1. eye patches!
  2. referral to secondary care
    (MRI if paralytic squint to rule out space occupying lesion)
41
Q

What is hypermetropia?

A

= LONG SIGHTED

correct early to avoid irreversible damage to vision, very common

42
Q

What is myopia?

A

= SHORT SIGHTED

uncommon in children

43
Q

What is astigmatism?

A

= ABNORAML CORNEAL CURVATURW

44
Q

What is amblyopia?

A

= PERMANENT LOSS OF VISUAL ACUITY

affects 1 eye, correction with glasses, need quick treatment

45
Q

Define cerebral palsy

A

permanent disorder of movement and posture and motor function due to a non progressive abnormality in the developing brain

46
Q

What are the common causes of cerebral palsy?

A

PRENATAL (80%)
cerebral malformations/haemorrhage *
congenital infections e.g. rubella, CMV, toxoplasmosis
genetic syndromes

DURING DELIVERY
hypoxic-ischaemic injury
trauma

POSTNATAL 
infection - meningitis*, encephalitis
encephalopathy
head trauma - NAI, AI
intraventricular haemorrhage 
kernicterus
47
Q

Outline the features of the Gross Motor Function Classification system for cerebral palsy

A

Level 1 = walk without limitation

Level 2= walk with limitation

Level 3= walk using handheld mobility device

Level 4= self mobility with limitations

Level 5= transported in a manual wheelchair

48
Q

What are the classical early features of cerebral palsy?

A
  1. abnormal tone, spasticity
  2. delayed motor milestones
  3. feeding problems
  4. abnormal gait
  5. preferred use of one hand before 12 months
49
Q

What are the complications of cerebral palsy?

A

learning difficulties
epilepsy
squints
hearing impairment

50
Q

How is cerebral palsy classified?

A
  1. spastic cerebral palsy (90%) = damage to the upper motor neurone pathway (pyramidal or corticospinal)
    - hemiplegia, quadriplegia, diplegia
  2. dyskinetic cerebral palsy (6%) = due to damage in the basal ganglia and extra pyramidal pathways
  3. ataxic cerebral palsy (4%) = mostly genetically determined due to brain injury
51
Q

Describe the symptoms of spastic cerebral palsy

A

limb tone increased/ spasticity
brisk deep tends reflexes
extensor plantar responses

52
Q

What are the differences between the 3 types of spastic cerebral palsy?

A
  1. unilateral (hemiplegia)
    often due to perinatal middle cerebral artery infarct
  2. bilateral (quadriplegia)
    damage to periventricular areas of developing brain, all 4 limbs affected, poor head control, low central tone
  3. bilateral (diplegia)
    all 4 limbs affected, walking abnormal
53
Q

Define seizure

A

paroxysmal abnormality of motor, sensory, autonomic +/- cognitive function, due to transient brain dysfunction

54
Q

What is a febrile seizure?

A

an epileptic seizure accompanied by a fever, in the absence of an intracranial infection (6 months - 6 years old)

55
Q

How is cerebral palsy managed?

A
clinical diagnosis (can do MRI)
MDT approach 
botox injection for hypotonia, oral diazepam, oral baclofen for spasms 
analgesia
anti convulsants
56
Q

Describe the presentation of a febrile seizure

A

initial viral infection with a fever

brief (<5m) generalised tonic clonic seizure early on in infection

57
Q

What is the difference between a simple or complex febrile seizure?

A

SIMPLE= <15 min
generalised seizure, no reoccurrence within 24 hours, recover within an hour

COMPLEX= 15-30 mins
focal seizure, may have repeat within 24 hr

58
Q

How is a febrile seizure managed?

A
  1. infection screen (blood cultures, lumbar puncture, urine culture) to check for no bacterial infection and exclude meningitis
  2. no treatment needed unless >5 mins
  3. family taught first aid management of seizures
59
Q

What is the difference between focal and generalised seizures?

A

FOCAL
arises from one part/ one hemisphere of the brain
e.g. frontal, temporal, parietal, occipital

GENERALISED
seizure arise from both hemispheres, no warning
e.g. tonic clonic, absence, myotonic, atonic, tonic

60
Q

How do focal seizures in the frontal lobe present?

A

clonic movements
(Jacksonian march)

tonic seizures with both upper limbs high for several seconds

61
Q

How do focal seizures present in the temporal lobe?

A

strange warning feeling / aura
lip smacking
plucking at clothes
deja vu feeling

62
Q

How do parietal focal seizures present?

A

contralateral dysasthesias or distorted body image

63
Q

How do occipital focal seizures present?

A

stereotyped visual hallucinations

64
Q

Describe tonic clonic seizures

A
limbs stiffen (tonic) and jerk (clonic)
Loss of consciousness - cyanosed, breathing irregular 
biting of tongue 
incontinence 
deep sleep after
65
Q

Describe absence “petit mal” seizures

A
lasts only a few seconds- 30 secs
transient loss of consciousness
flickering of eyes
unaware 
episodes induced by hyperventilating
10% progress to JME
66
Q

Describe tonic seizures

A

generalised increase in muscle tone (stiffen)

67
Q

Describe juvenile myoclonic epilepsy

A

sudden brief jerking movements of the limbs, neck of trunk
often in the morning , clumsy, throw breakfast
daytime absences
provoked by sleep deprivation or photo stimulation

68
Q

Describe atonic seizures

A

myoclonic jerk followed by transient loss of muscle tone

sudden fall to the floor or drop of head

69
Q

which diagnostic tests are used to diagnose epilepsy?

A
  1. history - detailed history from child and eyewitness
  2. ECG - rule out arrhythmias
  3. EEG e.g. sleep deprived EEG, 24 hr ambulatory EEG, 5 day video telemetry
  4. MRI/CT*
70
Q

What is first line therapy for generalised epilepsy?

A

sodium valproate

2nd line = carbamazepine, lamotrigine

71
Q

When is AED therapy discontinued?

A

after 2 years free of seizures

72
Q

List the adverse effects of valproate

A

weight gain
hair loss
liver failure (LFT yearly)
teratogenic

73
Q

List the adverse effects of carbamazepine

A

rash
hyponatraemia
ataxia
interfere with oral contraceptives

74
Q

What is status epilepticus and how is it managed?

A

> 30mins tonic clonic seizure but treat after 5 mins

buccal midazolam** or IV lorazepam

75
Q

How and when do infantile spasms/West syndrome present?

A

3-12 months old

violent flexor spasms of head, trunk and limbs followed by extension of arms

1-2 seconds long , several times a day (up to 50 x)

head nodding

76
Q

Describe the typical EEG of west syndrome?

A

hypsarrhymia

77
Q

How is west syndrome managed?

A

vigabatrin (GABA transaminase inhibition) + corticosteroids

78
Q

How and when does Lennox Gastraut syndrome present?

A

1-3 years old

extension of infanile spasms
multiple seizure types e.g. atonic, absence, tonic

79
Q

Describe the typical EEG of Lennox Gastraut syndrome

A

slow spike and wave

80
Q

What is the prognosis of Lennox Gastraut syndrome?

A

poor

ketogenic diet may help

81
Q

What is the typical EEG finding of childhood absence epilepsy?

A

symmetrical 3 Hz spike and wave

82
Q

How does benign rolandic epilepsy present?

A

tonic clonic seizures in sleep
paraethesia (unilateral facial) on waking up
4-10 years old, more common in boys

83
Q

describe the features of Panyioto-poulos syndrome

A
autonomic features
vomiting
unresponsive starting in sleep
head and eye deviation
can progress to convulsive seizure
84
Q

List some of the triggers of seizures

A
missed medication
missed meal
flashing lights 
noise
hyperventilation
85
Q

List some of the triggers of syncope

A
hot
low blood sugar 
trauma
exertion
upright 
cardiac problem
86
Q

Define syncope

A

sudden reduction in cerebral perfusion with oxygenated blood either secondary to reduced cerebral blood flow or reduced oxygen content

87
Q

What are the differences between a seizure and a syncope?

A
SEIZURE
longer duration
incontinence and tongue biting 
various colour changes 
confused, sleepy, 30-60 mins to come round
SYNCOPE
shorter duration
maybe? incontinence or tongue biting
pale
quicker to come round
88
Q

List the differentials for a “funny turn”

A
blue breath holding spells
reflex systolic syncope
syncope
migraine
benign paroxysmal vertigo
cardiac arrhythmias
89
Q

What is spina bifida?

A

failure of neural tube to close in first trimester due to insufficient folic acids or drugs (valproate, carbamazepine)

90
Q

What are the 3 types of spina bifida?

A
  1. occulta - hidden defect, hair lower back, asymptomatic
  2. meningocele- meninges protrude but not exposed, spinal cord intact
  3. myelomeningocele - opne lesion, severe weakness and disability