Paeds development and neurology (ILA 5) Flashcards

(90 cards)

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
Which syndromes are associated with sensorineural hearing loss?
``` Alports syndrome waardenburgs ushers pondered jervell-Lange Nielsen branchio-oto-renal syndrome stickler down syndrome ```
26
What are the clinical features of sensorineural hearing loss?
1. irreversible 2. profound hearing loss 3. gets worse over time
27
How is sensorineural hearing loss managed?
1. early amplification with hearing aids 2. cochlear implants + sit at front of class, gestures, lip movements
28
Outline the causes of conductive hearing loss
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
29
What are the clinical features of conductive hearing loss?
1. intermittent or resolves 2. maximum of 60 dB hearing loss 3. affects low frequency sounds more
30
How is conductive hearing loss managed?
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
31
Which hearing tests are done in newborns?
Newborn Hearing Screening Programme = "evoked otoacoustic emission test" soft echo = healthy cochlea if that comes back abnormal, do the "auditory brainstem response audiometry"
32
how is hearing tested as children get older?
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
33
at what age do we develop 6/6 visual acuity?
at 3-4 years old 6/6 - if the patient can read the last line on a Snellen chart from 6m away
34
What can cause a squint?
family history congenial cataracts retinoblastoma
35
What is a squint and what are the two types of squint?
= strabismus = misalignment of the visual axes 1. concomitant = common 2. paralytic = rare
36
Describe concomitant squints and how they occur
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)
37
Describe paralytic squints and how they occur
due to paralysis of the motor nerves and extra ocular muscles (could be due to tumour) O/E- limited movement of the eye
38
How is a squint detected?
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
Which test is used to identify the nature of the squint?
COVER TEST | when squint present and fixing eye covered, squinting eye moves to take up fixation
40
How is a squint managed?
1. eye patches! 2. referral to secondary care (MRI if paralytic squint to rule out space occupying lesion)
41
What is hypermetropia?
= LONG SIGHTED correct early to avoid irreversible damage to vision, very common
42
What is myopia?
= SHORT SIGHTED uncommon in children
43
What is astigmatism?
= ABNORAML CORNEAL CURVATURW
44
What is amblyopia?
= PERMANENT LOSS OF VISUAL ACUITY affects 1 eye, correction with glasses, need quick treatment
45
Define cerebral palsy
permanent disorder of movement and posture and motor function due to a non progressive abnormality in the developing brain
46
What are the common causes of cerebral palsy?
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
Outline the features of the Gross Motor Function Classification system for cerebral palsy
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
What are the classical early features of cerebral palsy?
1. abnormal tone, spasticity 2. delayed motor milestones 3. feeding problems 4. abnormal gait 5. preferred use of one hand before 12 months
49
What are the complications of cerebral palsy?
learning difficulties epilepsy squints hearing impairment
50
How is cerebral palsy classified?
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 2. ataxic cerebral palsy (4%) = mostly genetically determined due to brain injury
51
Describe the symptoms of spastic cerebral palsy
limb tone increased/ spasticity brisk deep tends reflexes extensor plantar responses
52
What are the differences between the 3 types of spastic cerebral palsy?
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
Define seizure
paroxysmal abnormality of motor, sensory, autonomic +/- cognitive function, due to transient brain dysfunction
54
What is a febrile seizure?
an epileptic seizure accompanied by a fever, in the absence of an intracranial infection (6 months - 6 years old)
55
How is cerebral palsy managed?
``` clinical diagnosis (can do MRI) MDT approach botox injection for hypotonia, oral diazepam, oral baclofen for spasms analgesia anti convulsants ```
56
Describe the presentation of a febrile seizure
initial viral infection with a fever brief (<5m) generalised tonic clonic seizure early on in infection
57
What is the difference between a simple or complex febrile seizure?
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
How is a febrile seizure managed?
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
What is the difference between focal and generalised seizures?
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
How do focal seizures in the frontal lobe present?
clonic movements (Jacksonian march) tonic seizures with both upper limbs high for several seconds
61
How do focal seizures present in the temporal lobe?
strange warning feeling / aura lip smacking plucking at clothes deja vu feeling
62
How do parietal focal seizures present?
contralateral dysasthesias or distorted body image
63
How do occipital focal seizures present?
stereotyped visual hallucinations
64
Describe tonic clonic seizures
``` limbs stiffen (tonic) and jerk (clonic) Loss of consciousness - cyanosed, breathing irregular biting of tongue incontinence deep sleep after ```
65
Describe absence "petit mal" seizures
``` lasts only a few seconds- 30 secs transient loss of consciousness flickering of eyes unaware episodes induced by hyperventilating 10% progress to JME ```
66
Describe tonic seizures
generalised increase in muscle tone (stiffen)
67
Describe juvenile myoclonic epilepsy
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
Describe atonic seizures
myoclonic jerk followed by transient loss of muscle tone | sudden fall to the floor or drop of head
69
which diagnostic tests are used to diagnose epilepsy?
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
What is first line therapy for generalised epilepsy?
sodium valproate 2nd line = carbamazepine, lamotrigine
71
When is AED therapy discontinued?
after 2 years free of seizures
72
List the adverse effects of valproate
weight gain hair loss liver failure (LFT yearly) teratogenic
73
List the adverse effects of carbamazepine
rash hyponatraemia ataxia interfere with oral contraceptives
74
What is status epilepticus and how is it managed?
>30mins tonic clonic seizure but treat after 5 mins buccal midazolam** or IV lorazepam
75
How and when do infantile spasms/West syndrome present?
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
Describe the typical EEG of west syndrome?
hypsarrhymia
77
How is west syndrome managed?
vigabatrin (GABA transaminase inhibition) + corticosteroids
78
How and when does Lennox Gastraut syndrome present?
1-3 years old extension of infanile spasms multiple seizure types e.g. atonic, absence, tonic
79
Describe the typical EEG of Lennox Gastraut syndrome
slow spike and wave
80
What is the prognosis of Lennox Gastraut syndrome?
poor | ketogenic diet may help
81
What is the typical EEG finding of childhood absence epilepsy?
symmetrical 3 Hz spike and wave
82
How does benign rolandic epilepsy present?
tonic clonic seizures in sleep paraethesia (unilateral facial) on waking up 4-10 years old, more common in boys
83
describe the features of Panyioto-poulos syndrome
``` autonomic features vomiting unresponsive starting in sleep head and eye deviation can progress to convulsive seizure ```
84
List some of the triggers of seizures
``` missed medication missed meal flashing lights noise hyperventilation ```
85
List some of the triggers of syncope
``` hot low blood sugar trauma exertion upright cardiac problem ```
86
Define syncope
sudden reduction in cerebral perfusion with oxygenated blood either secondary to reduced cerebral blood flow or reduced oxygen content
87
What are the differences between a seizure and a syncope?
``` 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
List the differentials for a "funny turn"
``` blue breath holding spells reflex systolic syncope syncope migraine benign paroxysmal vertigo cardiac arrhythmias ```
89
What is spina bifida?
failure of neural tube to close in first trimester due to insufficient folic acids or drugs (valproate, carbamazepine)
90
What are the 3 types of spina bifida?
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