PAEDIATRIC NEUROLOGY
NEURO-EMBRYOLOGY
EMBRYOLOGICAL STEPS EVENT
1. Gastrulation forming the trilaminar layer of embryo
2. Neurulation Ectoderm cells form brain, spinal cord and PNS
3. Somite development Mesoderm cells form myotomes, dermatomes and sclerotomes
4. Cardiovascular system development Heart tube develops from splanchnic mesoderm
5. Head and neck development From 5 pairs of pharyngeal arches
6. Organogenetic period From weeks 4-8
Brain
(developed from 3 primary vesicles
ROSTRAL (forebrain, midbrain, hindbrain) ® expand to
neural tube form each section of brain)
Cavity = forms ventricles of brain
Medial cells of
neural plate SPINAL cord
CAUDAL neural [Lateral walls of neural tube thicken to reduce size
tube of neural canal ® only small central canal after 10
Notochord secretes Shh (sonic weeks]
hedgehog) to thicken ectoderm [caudal to 4th
and form neural plate pair of somites] Canal = forms central canal of spinal
cord
Lateral cells of Pinch Off To Form Spinal Ganglion To
neural plate Neural Crest Form PNS
Neuroembryological defects:
CONDITION Cause Clinical observation Symptoms
Spina bifida When one or more neural arches fail to close during the 4th Tuft of hair at back of • Bowel obstruction
week of development = incomplete SC spine • Faecal / urinary incontinence
• Muscle weakness
Meroencephaly Failure of rostral neuropore to close during the 4th week Smaller head • Forebrain, midbrain and hindbrain absent
• Cognitive impairment
• FTT – short stature
SQUINT (STRABISMUS)
PP Types Sx Ix Mx
• Strabismus / squint = • Esotropia (affected eye • Double vision Eye exam Treatment BEFORE age of 8 as
misalignment of eyes towards nose) • Amblyopia (lazy eye Ø Acuity visual fields are still developing
• Young age allows for • Exotropia (affected eye becomes Ø Fields Ø Pead opthal referral
adaptation leading to one towards ear) increasingly Ø Reflexes Ø Occlusive patch to cover
dominant eye vs one lazy • Hypertropia = affected eye disconnected from Ø Fundoscopy (rule out Rb, good eye
eye towards ear (upward brain) cataracts + other retinal issues) Ø Atropine drops on good
Causes moving affected eye) eye
Ø Hydrocephalus • Hypotropia (downward Hirschberg’s test = shine pen-torch Ø Rx: cataracts and
Ø CP moving affected eye) 1m away – check if reflection is NOT refractive errors
Ø SoL (e.g. Rb) central or symmetrical
Ø Trauma Cover test – see below
, SYNCOPE WORK-UP – MAY CAUSES
VASOVAGAL BREATH-HOLDING OTHER SYNCOPAL
(Syncopal episodes) SPELL CAUSES
ANS dysfn where strong stimulation of vagus nerve Involuntary episodes when child holds Indications for Paediatric CT (PECARN score)
causes activation of PSNS breath usually after upsetting or scaring
PP them
Ø Causing systemic vasodilatation, reduced
cerebral circulation and hypoperfusion of brain Ø Usu. between 6 and 18/12 old
tissue
Primary syncope (simple fainting) Idiopathic
Ø dehydration Ø child upset, frightened or pain
Ø hypoglycemia (missed meals)
Ø extended standing in warm environ.
Ø ++ emotions = pain, sudden surprise, sight of DDx:
blood Ø Fe def. (↑ risk of BHS)
Secondary causes Ø Seizure
Cause
Ø Cardiac?
Ø dehydration
Ø hypoglycaemia
Ø anaemia
Ø infection
Ø anaphylaxis
Ø arrhythmias
Ø valvular heart disease or HOCM
Prodrome 2 types
• Hot/clammy 1) Cyanotic breath holding spells
• Dizzy / lightheaded upset or worked up child letting out long
cry causing them to stop breathing AND
• Blurry or dark vision LOC
• Headache - recover within min but lethargy
Sx Event
• Sudden LOC – fall to ground 2) Reflex anoxic seizures (pallid
• Twitching/shaking/convulsion? breath holding spells)
Post-ictal startled child causes vagus nerve to send HEAD INJURIES
strong signals to heart to stop beating Ø Raccoon eyes (peri-orbital) or battle sign (mastoid)
• No WILD
- turns pale, LOC +/- twitching Ø CSF otorrhea, rhinorrhoea
• Rapid recovery + memory of events - resolves within 30s Ø FND = RAPD, abnormal posture, GCS < 13
None None Ø Examine C-spine
Comp.
- Falls risk? – bleeding disorder - Most outgrow by 4 or 5 yo
Ø Check for physical injuries Exclude other pathologies REVERSIBLE CAUSES
Ø Identify possible concurrent illnesses Ø FBC 1) HYPOglycaemia (< 3.5mM)
o ECG (arrythmia, long QT) Ø EUC Ø Jitteriness in babies (seen if BSL < 2mM)
Ø LFT
Ix o 24 hr ECG ( if paroxysmal Ø Causes = sepsis (for preceding illness), T1DM, congenital
Ø BSL
arrhythmias suspected) diseases, alcohol OD
Ø Fe studies
o Bloods (FBC, EUC, BSL) Ø ECG Ø Rx: IV 10% dextroses (2mL/kg) or glucogel
Ø UA Ø AIM TO AVOID BRAIN DAMAGE
o ECHO
2) HypoNa (< 125mM)
Ø Reassure – most resolve by adulthood Breath holding spells linked with Fe def. Ø Replace with 1-2mM 0.9% NS
(more common in teenager girls) anaemia
Ø Replace 3mL / 3% NS (if severe)
Lifestyle advice Ø Self-limiting and not harmful Ø *Avoid risk of ODS (pontine demyelination)
Ø Avoid dehydration Ø Rx for Fe deficiency
3) Raised ICP
Ø First aid – protect head and limbs
Ø Avoid Skipping meals from injury Ø Cushing’s triad (irregular RR, widened PP, bradycardia)
Mx Ø Avoid standing for long periods of time Ø Rx: IV mannitol and HoB elevation
If seizures or underlying pathology present refer to Ø Ix: CT brain + fundoscopy
appropriate specialist 4) Infection
Ø Locate source – FBC, EUC, LFT, CRP, ABG, Blood culture,
CXR, Urine MSU (M/C/S), swabs
Ø Rx: IV ceftriaxone or acyclovir
HEADACHES IN PAEDIATRICS
Assoc. Treatment for migraines
• Unilateral severe throbbing headache Acute Mx:
Classical • Visual aura AND/OR photophobia/phonophobia Ø Rest, fluids and low stimulus environment
MIGRAINES • N/V +/- abdo pain Ø paracetamol
• May have had recurrent central abdo pain as child Ø Triptans ® 50mg sumi
Ø NSAIDs
• CENTRAL ABDOMINAL PAIN > 1 hr
Ø Anti-emetics (maxolon)
• Nausea/ vomiting
Abdominal Long-term Mx:
• Anorexia + Pallor Ø Avoid trigger (stress, lights, smells, dehydration, choc, critic
Migraines
• Headache acid, poor sleep)
• May develop into classical migraines during adulthood Ø CBT
Identify cause Ø Headache diary
Infections Ø Relaxation (massage)
• Viral URTI, otitis media, sinusitis, tonsillitis
Ø Vitamin B2 (riboflavin) – reduce freq. + severity
• Facial pain – behind nose, forehead and eyes (over respective sinuses Ø Amitriptyline (TCA) but AE = fatigue, dizzy, depression,
– ethmoid, sphenoid, maxillary and frontal) insomnia
Sinusitis
• Coryza - nasal congestion, rhinorrhea, lacrimation Ø Prophylaxis (with Panadol + propranolol]
• Rx: supportive and resolves within 2-3 weeks
*Consider SoL – if suspicious findings (e.g. UWL, persistent headache, FND)
