Paeds development and neurology (ILA 5) Flashcards
what are the 4 functional areas of child development?
- gross motor
- fine motor and vision
- hearing, speech and language
- social, emotional and behaviour
A newborn should have which skills?
GM - limbs flexed, symmetrical, marked head lag on pulling up, fixed posture
FMV- fixes and follows face
HSL- starts to loud noises
At 6-8 weeks old, which skills should they have developed?
GM- raises head to 45 degrees in prone position
FMV- follows moving object and follows face by moving head
SEB- smiles responsively
At 6 months old, which skills are developed?
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
At 8-9 months old, which skills are developed?
GM- crawling, pulls to stand
FMV- points with finger, early pincer
HSL- says mama and dada
SEB- shy
At 12 months old, which skills are developed?
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
At 15 months, which skills are developed?
GM- walks steadily
FMV- immature grip of pencil, tower of 2
HSL- 2-6 words, understands simple commands
At 18 months old, which skills are developed?
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
at 2 years old, which skills are developed?
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
at 3 years old, which skills are developed?
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
At what age do children play together?
4 years old
List the primitive reflexes evident at birth
- moro reflex= sudden extension of the head causes symmetrical extension then flexion of the arms
- grasp = flexion of fingers when object put in their hand
- rooting = head turns to stimulus when touched near the mouth
- stepping response= stepping movements when held vertically and feet touch a surface
- asymmetrical tonic neck reflect
- sucking reflex = child sucks when nipple / teat placed in mouth
List the postural reflexes that children develop
postural reflexes essential for independent sitting and walking
- labyrinthine righting = head moves in opposite direction to which body is tilted
- postural support= when held upright, legs take weight and baby may push up
- lateral propping = in sitting, arms extend on side which the child falls as a saving mechanism
- paracute = when suspended face down, baby arm extend to save themselves
List some prenatal causes of developmental delay
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
List some perinatal causes of developmental delay
extreme prematurity
hypoxic ischaemic encephalopathy
Metabolic e.g. hypoglycaemia, hyperbilirubinaemia
List some postnatal causes of developmental delay
infections e.g. rubella, CMV, toxicoplasmosis
Trauma e.g. head injury
Metabolic e.g. hypoglycaemia
Vascular e.g. stroke
Which features of developmental delay would concern you and would therefore make a referral?
not smiling at 10 weeks
cannot sit unsupported at 12 months
cannot walk at 18 months
What is the most common cause of development problems?
cerebral palsy
What is global developmental delay?
slow/impaired acquisition of all developmental skills
Outline the multi-disciplinary team involved in a childs development
- paediatricians
- physiotherapists
- occupational therapists
- speech and language therapists
- clinical psychologists
- dietician
- specialist health visitor
- social worker
Outline “normal hearing” milestones from birth to 12 months old
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
List some possible behavioural changes that could indicate hearing loss
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
what are the 2 types of hearing loss?
- sensorineural = lesion in the cochlea or auditory nerve
2. conductive = from abnormalities in the ear canal or middle ear
List the causes of sensorineural hearing loss
- genetic - syndromes!
- congenital infections e.g. CMV, rubella
- postnatal infections e.g. meningitis
- cerebral palsy
- preterm
- head injury
Which syndromes are associated with sensorineural hearing loss?
Alports syndrome waardenburgs ushers pondered jervell-Lange Nielsen branchio-oto-renal syndrome stickler down syndrome
What are the clinical features of sensorineural hearing loss?
- irreversible
- profound hearing loss
- gets worse over time
How is sensorineural hearing loss managed?
- early amplification with hearing aids
- cochlear implants
+ sit at front of class, gestures, lip movements
Outline the causes of conductive hearing loss
- otitis media with effusion = glue ear
- eustachian tube dysfunction e.g. downs syndrome, cleft palate, pierre robin sequence
- middle ear infection
- perforated ear drum
- wax
What are the clinical features of conductive hearing loss?
- intermittent or resolves
- maximum of 60 dB hearing loss
- affects low frequency sounds more
How is conductive hearing loss managed?
test bone conduction and if doesn’t resolve in 3 months…
- decongestant or long course antibiotics
- insert tympanovstomy tubes (grommets)
- hearing aid
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”
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
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
What can cause a squint?
family history
congenial cataracts
retinoblastoma
What is a squint and what are the two types of squint?
= strabismus = misalignment of the visual axes
- concomitant = common
- paralytic = rare
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)
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
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!!
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
How is a squint managed?
- eye patches!
- referral to secondary care
(MRI if paralytic squint to rule out space occupying lesion)
What is hypermetropia?
= LONG SIGHTED
correct early to avoid irreversible damage to vision, very common
What is myopia?
= SHORT SIGHTED
uncommon in children
What is astigmatism?
= ABNORAML CORNEAL CURVATURW
What is amblyopia?
= PERMANENT LOSS OF VISUAL ACUITY
affects 1 eye, correction with glasses, need quick treatment
Define cerebral palsy
permanent disorder of movement and posture and motor function due to a non progressive abnormality in the developing brain
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
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
What are the classical early features of cerebral palsy?
- abnormal tone, spasticity
- delayed motor milestones
- feeding problems
- abnormal gait
- preferred use of one hand before 12 months
What are the complications of cerebral palsy?
learning difficulties
epilepsy
squints
hearing impairment
How is cerebral palsy classified?
- spastic cerebral palsy (90%) = damage to the upper motor neurone pathway (pyramidal or corticospinal)
- hemiplegia, quadriplegia, diplegia - dyskinetic cerebral palsy (6%) = due to damage in the basal ganglia and extra pyramidal pathways
- ataxic cerebral palsy (4%) = mostly genetically determined due to brain injury
Describe the symptoms of spastic cerebral palsy
limb tone increased/ spasticity
brisk deep tends reflexes
extensor plantar responses
What are the differences between the 3 types of spastic cerebral palsy?
- unilateral (hemiplegia)
often due to perinatal middle cerebral artery infarct - bilateral (quadriplegia)
damage to periventricular areas of developing brain, all 4 limbs affected, poor head control, low central tone - bilateral (diplegia)
all 4 limbs affected, walking abnormal
Define seizure
paroxysmal abnormality of motor, sensory, autonomic +/- cognitive function, due to transient brain dysfunction
What is a febrile seizure?
an epileptic seizure accompanied by a fever, in the absence of an intracranial infection (6 months - 6 years old)
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
Describe the presentation of a febrile seizure
initial viral infection with a fever
brief (<5m) generalised tonic clonic seizure early on in infection
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
How is a febrile seizure managed?
- infection screen (blood cultures, lumbar puncture, urine culture) to check for no bacterial infection and exclude meningitis
- no treatment needed unless >5 mins
- family taught first aid management of seizures
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
How do focal seizures in the frontal lobe present?
clonic movements
(Jacksonian march)
tonic seizures with both upper limbs high for several seconds
How do focal seizures present in the temporal lobe?
strange warning feeling / aura
lip smacking
plucking at clothes
deja vu feeling
How do parietal focal seizures present?
contralateral dysasthesias or distorted body image
How do occipital focal seizures present?
stereotyped visual hallucinations
Describe tonic clonic seizures
limbs stiffen (tonic) and jerk (clonic) Loss of consciousness - cyanosed, breathing irregular biting of tongue incontinence deep sleep after
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
Describe tonic seizures
generalised increase in muscle tone (stiffen)
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
Describe atonic seizures
myoclonic jerk followed by transient loss of muscle tone
sudden fall to the floor or drop of head
which diagnostic tests are used to diagnose epilepsy?
- history - detailed history from child and eyewitness
- ECG - rule out arrhythmias
- EEG e.g. sleep deprived EEG, 24 hr ambulatory EEG, 5 day video telemetry
- MRI/CT*
What is first line therapy for generalised epilepsy?
sodium valproate
2nd line = carbamazepine, lamotrigine
When is AED therapy discontinued?
after 2 years free of seizures
List the adverse effects of valproate
weight gain
hair loss
liver failure (LFT yearly)
teratogenic
List the adverse effects of carbamazepine
rash
hyponatraemia
ataxia
interfere with oral contraceptives
What is status epilepticus and how is it managed?
> 30mins tonic clonic seizure but treat after 5 mins
buccal midazolam** or IV lorazepam
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
Describe the typical EEG of west syndrome?
hypsarrhymia
How is west syndrome managed?
vigabatrin (GABA transaminase inhibition) + corticosteroids
How and when does Lennox Gastraut syndrome present?
1-3 years old
extension of infanile spasms
multiple seizure types e.g. atonic, absence, tonic
Describe the typical EEG of Lennox Gastraut syndrome
slow spike and wave
What is the prognosis of Lennox Gastraut syndrome?
poor
ketogenic diet may help
What is the typical EEG finding of childhood absence epilepsy?
symmetrical 3 Hz spike and wave
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
describe the features of Panyioto-poulos syndrome
autonomic features vomiting unresponsive starting in sleep head and eye deviation can progress to convulsive seizure
List some of the triggers of seizures
missed medication missed meal flashing lights noise hyperventilation
List some of the triggers of syncope
hot low blood sugar trauma exertion upright cardiac problem
Define syncope
sudden reduction in cerebral perfusion with oxygenated blood either secondary to reduced cerebral blood flow or reduced oxygen content
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
List the differentials for a “funny turn”
blue breath holding spells reflex systolic syncope syncope migraine benign paroxysmal vertigo cardiac arrhythmias
What is spina bifida?
failure of neural tube to close in first trimester due to insufficient folic acids or drugs (valproate, carbamazepine)
What are the 3 types of spina bifida?
- occulta - hidden defect, hair lower back, asymptomatic
- meningocele- meninges protrude but not exposed, spinal cord intact
- myelomeningocele - opne lesion, severe weakness and disability
