paediatric neurology Flashcards
clinical evaluation of childhood headache disorders
isolated acute
recurrent acute
chronic progressive
chronic non-progressive
pointers to childhood migraine
- assoc abdo pain, nausea, vomiting
- focal symptoms/signs before, during, after attack: visual disturbance, paresthesia, weakness
- pallor
- aggravated by light/noise
- relation to fatigue/stress
- helped by sleep, rest, dark, quiet
- FH often positive
features of tension type headahce
diffuse, symmetrical
band-like distribution
present most of time
contant ache
features of migraine
hemicranial pain throb/pulsatile abdo pain, nausea, vomit relieved by rest photo/phono-phobia visual, sensory, motor aura FH
pointers to raised intracranial pressure
aggravated by activities that raise ICP e.g. coughing, bending
woken from sleep with headache +/- vomiting
pointers to analgesic overuse headache
headache is back before allowed to use another dose
paracetamol/NSAIDs
partic problem with compound analgesics e.g. cocodamol
indications for neuroimaging
- features of cerebellar dysfunction
- features of raised ICP
- new focal neurological deficit e.g. new squint
- seizures, esp focal
- personality change
- unexplained deterioration of school work
migraine management
acute attack: effective pain relief, triptans
preventative (at least 1wk): pizotifen, propranolol, amitryptyline, topiramate, valproate
tension type headache treatment
- reassurance: no sinister cause
- attention to underlying physical, psychological, emotional problems
acute attacks: simple analgesia
prevention: amitryptiline
seizure/fit
any sudden attack from whatever cause
syncope
faint - a neuro-cardiogenic mechanism
convulsion
seizure where there is prominent motor activity
epileptic seizure
an electrical phenomenon
an abnormal excessive hyper-synchronous discharge from a group of cortical neurons
paroxysmal change in motor, sensory or cognitive function
what does seizure manifestation depend on
seizure location
degree of anatomical spread over cortex
duration - of abnormal electrical discharge
epilepsy
a tendency to recurrent, unprovoked (spontaneous) epileptic seizures
EEG for supportive evidence - single epileptic seizure doesn’t mean they have epilepsy
non-epileptic seizures and other mimics in children
- acute symptomatic seizures
- reflex anoxic seizure
- syncope
- parasomnias e.g. night terror
- behavioural stereotypies
- psychogenic non-epileptic seizure
acute symptomatic seizures
due to acute insults to brain e.g. hypoxia-ischaemia, hypoglycaemia, trauma, infection
reflex anoxic seizures
vagal overstimulation, always provoked/triggered by certain stimuli
common in toddlers
febrile convulsion
seizure occurring usually between 3mo and 5yrs, assoc with fever but without evidence of intracranial infection or defined cause of seizure
commonest cause of acute symptomatic seizure in childhood
what is an epileptic fit chemically triggered by
decreased inhibition (gama-amino-butyric acid, GABA)
excessive excitation (glutamate and aspartate)
excessive influx of Na and Ca ions
mechanism of epileptic fit
triggered by imbalance between excitatory and inhibitory neurones
chemical stimulation produces an electrical current
summation of a multitude of electrical potentials results in depolarisation of many neurones which can lead to seizures, can be recorded from surface electrodes (electroencephalogram)
types of epileptic seizures
partial/focal seizures
generalised seizures
partial/focal seizures
seizure focus restricted to 1 hemisphere/part of one hemisphere
generalised seizure
neurones recruited from both halves of brain - both hemispheres involved
childhood vs adult onset epilepsies
majority generalised
majority idiopathic
seizures subtle in childhood - can be missed by parents/carers
diagnosis challenging - large no paroxysmal evens in kids, difficulty getting good history
stepwise approach to diagnosing epilepsy
- is the paroxysmal event epileptic in nature?
- is it epilepy?
- what type of seizures are occuring?
- what is the epilepsy syndrome?
- what is the aetiology?
- what are the social and educational effects on the child?
role of the EEG
-limited value in deciding when the pt has epilepsy
role in assessing and managing diagnosed epilepsy. useful in identifying seizure types, seizure syndrome and aetiology
diagnosing epilepsy
history
video of event
ECG in convulsive events (rule out long QT syndrome)
EEG
MRI brain - aetiology
genetics
metabolic tests - esp if assoc with developmental delay/regression
management of epilepsies in children
anti-epileptic drugs is diagnosis clear, they control seizures - not a cure
start with 1 AED and slow upward titration until side-effects manifest or drug considered inefficient
drug treatment of epilepsy
generalised epilepsies: sodium valproate (not for girls) or levetiracetam
focal epilepsies: carbamazepine
other: steroids, immunoglobulines, ketogenic diet (for drug-resistant epilepsies)
epilepsy management: surgery
vagal nerve stimulator
resection
head size problems
macrocephaly
microcephaly
in infant skull: what suture holds the frontal bones together
metopic suture
in infant skull: what suture holds parietal bones together
sagittal suture
in infant skull: why are sutures open
to allow brain to grow
order in which fontanelles close
posterior fontanelle usually closes 2-3mo after birth
gradual fusion of sutures
anterior fontanelle usually closed between 1-3yoa (avg 18mo)
when is OFC measured
routine between birth-3yrs
should be interpreted of rest of family - measure parents to see if kid is inkeeping with rest of family
microcephaly definition
mild: OFC <2 standard deviations below mean
moderate/severe: OFC < 3 SD
what does microcephaly indicate
usually indicates small brain
‘micranencephaly’
causes of microcephaly
antenatal
postnatal
genetic
environment
macrocephaly definition
OFC > 2SD
macrocephaly: what is indicated if they’re crossing centiles upwards
underlying brain or CSF is expanding in a way it shouldn’t
plagiocephaly
flat head - usually one one side
brachycephaly
short head which is also flat at back
scaphocephaly
head with unusual boat shape
craniosynostosis
premature fusion of cranial sutures
if they fuse too quickly brain has no room to grow and may end up with very odd head shape
what direction to skull bones normally grow
perpendicular to the sutures
deformational plagiocephaly
most common reason for abnormal head shape
to do with the position child is in e..g how they sleep, lie
when to suspect a neuromuscular disorder
baby floppy from birth slips from your hands paucity of limb movements alert but less motor activity delayed motor milestones able to walk but frequent falls
duchenne muscular dystrophy: genetics
Xp21, dystrophin gene
male infants
x-linked
duchenne muscular dystrophy: presentation
delayed gross motor skills symmetrical proximal weakness Gower's sign positive waddling gait cardiomyopathy respiratory involvement in teens
elevated creatinine kinase levels
anatomical approach to neuromuscular conditions: muscle
muscular dystrophies
myopathies - congenital and inflammatory
myotonic syndromes
anatomical approach to neuromuscular conditions: neuromuscular junction
myasthenic syndrome
anatomical approach to neuromuscular conditions: nerve
hereditary or acquired neuropathies
anatomical approach to neuromuscular conditions: anterior horn cell
spinal muscular atrophy
myotonia
inability for muscle to relax after contraction - sustained muscle contraction
simple vs complex febrile seizure
simple <10mins, generalised, doesn’t reccur in 24hrs
complex >10/15mins, focal, recurs within 24hrs
treatment complex febrile seizure
rectal diazepam
buccal midazolam
features febrile seizure
6mo-5yrs assoc fever generalised tonic clonic 3-6mins recover within an hour
