Paeds Neurology

Question 1

What are febrile seizures?

Answer 1

Simple self limiting tonic-clonic seizures associated with mild infections (particularly gastro)

1/30 kids will have one, 30-40% will have a second and 10% will have a 3rd

Typically occurs between the ages of 6 months to 6 years

1st febrile convulsaion <18months of age and FHx of epilepsy or febrile convulsions predicts recurrence

Question 2

What are features suggesting a febrile seizure is not a febrile seizure?

Answer 2

Lasts > 15mins
Recurrence within 24hrs
Any focal neurology or seizure activity
Toxic appearing

Question 3

What is status epilepticus in kids?

Answer 3

Seizure lasting >5mins
Or
recurrent seizures with no return to baseline in between

Question 4

Which anti-epileptic levels should routinely be check in patients taking them who have seizures?

Answer 4

Phenytoin
Carbamezapine
Phenobarbitone

These drugs can have variability in their pharmacokinetics, only test other anti-epileptic drug levels if adherence is thought to be an issue

Question 5

What is the difference in acute dosing of Keppra between adults and children?

Answer 5

Adults Keppra is 60mg/kg IV

Kids Keppra is 40mg/kg IV

Question 6

What ages should Phenytoin vs Phenobarbitone be used?

Answer 6

Phenytoin 20mg/kg is 2nd/3rd line in children >1yr of age

Phenobarbitone 20mg/kg is in liew of phenytoin in kids <1yrs old, or other contraindication to phenytoin (Dravet syndrome, allergy, already on pheyntoin)

Question 7

What is the maximum lifetime dose of Paraldehyde? What is its toxic breakdown product?

Answer 7

Maximum 30mls over a lifetime, increased used has progressive cancer risk

Toxic breakdown product is Crotonaldehyde

Question 8

Why is Valproate generally not use in children?

Answer 8

Valproate IV 30mg/kg over 5mins

Risk of hepatotoxicity in children <2yo and in those with metabolic disorders

Question 9

What are some other identifiable and potentially treatable causes of seizures?

Answer 9

Pyridoxine dependent epilepsy (give IV pyridoxine)
Thiamine deficiency
Hypomagnesaemia
Hyponatraemia
Hyperammonaemia
Local anaesthetic toxicity

Question 10

How does a ketogenic diet affect active seizure management?

Answer 10

Ketogenic diets take weeks to months to become effective
Giving glucose may negate this, so only give glucose to the patient if they are actively hypolycaemic

Question 11

What are episodic issues that mimic seizures in neonates?

Answer 11

Jitteriness/cold
Benign neonatal sleep myoclonus
Non-epileptic apnoea
Opisthotonus (back arching, spasticity, hyperextension)
Sometimes just normal movement

Question 12

What are episodic issues that mimic seizures in kids older than neonates?

Answer 12

Syncope
BRUE
Breath holding spells
Migraine with aura
Sydenhams Chorea (GAS and rheumatic fever)
Narcolepsy/Catoplexy
Tics
Panic attacks
PNES

Question 13

What is the most common cause of paediatric ataxia?

Answer 13

Acute cerebellar ataxia (40%)
Usually boys 2-4yo
An autimmune disorder causing cerebellar demyelination
Usually a recent acute infection, most common being VZV (26%)

Question 14

What are the differentials for paediatric ataxia?

Answer 14

Differentiate true ataxia from refusal to walk due to pain

Acute cerebellar ataxia
Drug ingestion
Illicit substance ingestion (ie alcohol in adolescents)
Seizure
Stroke/Bleed
Head trauma
Metabolic disorders
Meningoencephalitis
brain tumour
Genetic/congenital ataxias

Question 15

What is the most common cause of vertigo in childhood?

Answer 15

Migraine with vertiginous aura

Question 16

What are the most common causes of stroke in paediatrics?

Answer 16

Thrombus
- Sickle cell disease (the most common cause, also causes haemorrhage)
- Cardiac disease with embolisation
- Moyamoya disease

Haemorrhage
- Sickle cell disease
- AVM rupture
- coagulopathies (ITP, haemophilia etc)

Question 17

What is infantile botulism?

Answer 17

Occurs in infants <1yo, but more often <8months old

Infants stomach pH is less acidic (higher) than older children and cant breakdown the toxin

Presents with constipation, dry mouth, hypotonia/reflexia, poor head control, weak cry and poor suck

Question 18

What are the risk factors for infantile botulism?

Answer 18

Most commonly caused by honey ingestion

Exclusive breastfeeding is actually a risk factor

Treated with supportive management and BabyBIG, a monoclonal antibody against the Clostridium botulinum toxin

Question 19

What is the normal range of ICP in paediatrics? What is the normal CPP?

Answer 19

12 - 28cmH20
or
9 - 21mmHg

Normal CPP 40-60mmHg

Question 20

What are the clinical signs of raised ICP in infants <12months?

Answer 20

Bulging fontanelle
- Macrocephaly if chronic process
Unequal pupils
Cushings triad
- >BP, <Hr, Cheyne-Stokes breathing
Paradoxical tachycardia
N/V
Visual disturbances
Low GCS, altered gait/neurology

Question 21

What are the common and serious causes of Torticollis?

Answer 21

Serious
- Retropharyngeal abscess
- CNS tumour
- C-spines injury
- Suppurative Jugular thrombophlebitis (Lemierre syndrome)

Common
- Minor/Mod infection (URTI, upper lobe pneumonia, pharyngitis etc)
- Muscular sprain
- Dystonic reaction
- congenital torticollis
- Ocular torticollis
- Sandifer syndrome
- Benign paroxysmal torticollis

Question 22

Which children are at risk of atlantoaxial instability

Answer 22

Acute trauma to neck
Infection to upper C-spine
JRA and Ank Spond
Downs syndrome
Cerebral palsy
Morquio’s syndrome

Other important
- Klippel-Feil Syndrome
- Larsen Syndrome
- Osteogenesis imperfecta
- Marfans, Ehlers Danlos
- Steroid use
- Rickets
- Achondroplasia

Question 23

What are the causes of status epilepticus in children?

Answer 23

Febrile status epilepticus
- Most common cause
CNS infection
Head trauma
CNS malignancy
Congenital seizure disorders
- Withdrawal from meds
Hypoglycaemia
Metabolic abnormalities
- ie Hyperammonaemia, uraemia, hepatic encephalopathy
Electrolyte disturbance
Substance withdrawal (ie ETOH)
Toxicological
- TCA’s, theophylline, local anaesthetic, Lithium, metronidazole, cyclosporines etc

Question 24

What are the contraindications to lumbar puncture?

Answer 24

• GCS <8 or fluctuating
• Signs of raised ICP
• Bulging fontanelles
• New altered focal neurology
• septic shock or haemodynamic compromise
• Significant resp compromise
• Seizures <30mins ago
• INR >1.5 or platelts <50

Question 25

What are the differentials for paediatric ataxia?

Answer 25

Acute cerebellar ataxia 1

Question 26

What are the features suggestive of acute cerebellar ataxia?

Answer 26

• Viral prodrome
• Widebased/staggering gait
• Cerebellar signs
• -ve Rhomberg test
• Preservation of vibration and joint sense position
• Absence of red flags

Acute cerebellar ataxia treated with reassurance, no further Ix

Question 27

What are the typical Ix for paediatric ataxia?

Answer 27

• MRI/CT
• Electrolytes
• BSL
• FBE/LFT’s
• Specific levels (tox, anticonvulsants, ethylene glycol, ETOH) depending on the clinical scenario
• LP after neuroimaging
• EEG