Paediatric seizures Flashcards
Describe the seizures in Dravet syndrome.
Children <12 months First seizure febrile Last >10 minutes Unilateral Triggered by warm water
How is Dravet syndrome managed?
Ketogenic diet
Sodium valproate, clobazam and topiramate
What is the prognosis of Dravet syndrome?
Poor prognosis, life-long
20% mortality
Developmental delay after 2 years
Describe the seizures in infantile spasms (West syndrome)
Absence seizures, generalised
Recurring tonic seizures
Contractions of the head, neck and trunk, usually in flexion
Salaam / jack-knife spasms - sudden drawing up of legs, hunching forward, flinging arms out
Child may cry before or after
What is the EEG finding in infantile spasms?
Hypsarrhythmia when seizure is not occurring and
High-voltage spike and wave pattern
How are infantile spasms managed?
Steroids
Tetracosactrin, vigabatrin, clonazepam, nitrazepam
What is the prognosis for infantile spasms?
Poor prognosis , developmental delay
Develop Lennox Gastraut syndrome
What is the aetiology of infantile spasms?
Brain injury or hypoxia
Genetic and metabolic syndromes
Describe the seizures in childhood absence seizures
6 year olds
Short period of blanking out and staring into space, loss of awareness ~10s
Automatisms, like lip smacking
What is the EEG finding in absence seizures?
Generalised 3Hz spie wave
Which age group gets absence seizures?
6 year olds
What is the management for absence seizures?
Ethosuximide
Sodium valproate
Describe the seizures in juvenile myoclonic epilepsy
Generalised myoclonic after waking and tonic-clonic
Brief bilateral limb jerking, awareness may be maintained
2-3s duration
Which age group gets juvenile myoclonic epilepsy?
Adolescence
What is the prognosis for juvenile myoclonic epilepsy?
Life-long
May improve
What is the management for juvenile myoclonic epilepsy?
Lifestyle changes, regular sleep, reduced EtOH and stress (common triggers)
Sodium valproate
Describe the seizures in juvenile absence epilepsy.
Absence seizures last 45 seconds
Less frequent than childhood absence seizures, less than daily
What are the EEG changes in juvenile absence epilepsy?
Generalized polyspike or spike and wave discharges at 3-4 Hz
What is the management for juvenile absence epilepsy?
Ethosuximide
Sodium valproate
Which age group gets juvenile absence epilepsy?
13 years old
What is the prognosis for juvenile absence epilepsy?
Life-long
Develop tonic-clonic seizures
Attention problems
Describe the seizures in Lennox-Gastaut Syndrome
5 year olds
Epileptic encepalopahty with multiple types of seizures, particularly tonic and aclonic.
What are the EEG changes in Lennox-Gastaut Syndrome ?
Awake - diffuse or widespread background slowing and slow spike-wave bursts.
Aleep - generalized paroxysmal fast activity.
Which age group gets Lennox-Gastaut Syndrome?
5 year old
What is the prognosis for Lennox-Gastaut syndrome?
Severe, life-long
Developmental delay behavioural problems
What is the management for Lennox-Gastaut syndrome?
Ketogenic diet
Sodium valproate
Describe the seizures in Rolandic epilepsy/benign childhood epilepsy with centrotemporal spikes
Seizures occur mostly when asleep but can occur when awake
Facial symptoms - gurgling, tongue/face movements, sensation changes
Tonic-clonic seizures can occur
What are the EEG changes in Rolandic epilepsy?
Contralateral spikes
Which age group gets Rolandic epilepsy?
6 year olds
What is the management for Rolandinc epilepsy?
No management
Carbamazepine, sodium valproate, sulthiame
What is the mechanism of action of sodium valproate?
Blocks voltage and used dependent sodium channels
Other actions - enhancement of GABA, inhibition of glutamate and blockade of T-type calcium channels.
What is the mechanism of action of vigabatrin?
Increases brain concentrations of GABA and GABA-mediated inhibitionr by reversibly inhibits GABA aminotransferase
What is the mechanism of action of topiramate?
Blocks voltage-dependent sodium channels. Enhances activity of GABA on postsynaptic chloride channels.
What is the mechanism of action of carbamazepine?
Blocks voltage-dependent and use-dependent sodium channels.
What is the mechanism of action of ethosuximide?
Acts of voltage-dependent calcium channels. Thereby, reduces conductance in thalamic neurones.
Which seizures is ethosuximide used for?
Childhood absence seizures and juvenile absence epilepsy
