Status epilepticus is the most common childhood medical neurological emergency.
The Neurocritical Care Society guidelines define status epilepticus as:
“A seizure with 5 minutes or more of continuous clinical and/or electrographic seizure or recurrent seizure activity without recovery between seizures.”
The majority (approximately 90%) of seizures will spontaneously terminate within four minutes, with no intervention required. Convulsions lasting more than five minutes, however, are unlikely to cease spontaneously and will usually require treatment to aid termination. Convulsions lasting longer than 30 minutes are associated with an increased risk of death and long-term neurological consequences.
Aetiology
The causes of status epilepticus in children include the following:
Structural:
- Intracranial tumour
- Cerebrovascular disease
- Head injury
- Cortical dysplasia
Infectious:
- CNS infection (meningitis, encephalitis)
- Tuberculosis
- Cerebral malaria
Metabolic:
- Metabolic disturbance (e.g. electrolyte imbalance, glucose imbalance, organ failure)
- Metabolic disorders
- Anoxic injury
- Mitochondrial disorders
Toxicity or drug-related:
- Low or high level of anti- seizure medication
- Withdrawal of anti-seizure medication
- Other drug/alcohol overdose
- Neurotoxins and poisons
Inflammatory:
- Autoimmune disorders
- Neurocutaneous disorders
Genetic:
- Dravet syndrome
- Ring chromosome 20
- Angelman syndrome
- Fragile X syndrome
- Rett syndrome
- Trisomy 21
Primary survey and resuscitation
Any child presenting with status epilepticus should be managed using the standard ABCDE approach:
Airway:
- Maintain the airway and manage secretions
- Red flag – airway obstruction not responding to jaw thrust or airway adjunct
Breathing:
- Administer high-flow oxygen
- Consider intubation and ventilation
- Red flag – respiratory failure
Circulation:
- Establish IV/IO access
- Check vital signs
- Red flag – signs of shock
Disability:
- Assess conscious level and check pupils
- Don’t forget the glucose!
- Red flags – symptoms or signs of raised ICP, encephalopathy or focal neurology
Exposure:
- Expose and examine thoroughly
- Red flags – purpuric rash, puncture marks or signs of head trauma
APLS red flag algorithm
If any red flags are detected in the primary survey, then it is likely that the child will need to be intubated and ventilated urgently and anaesthetic or intensive care support should be obtained urgently.
Reversible causes should be identified and treated during this period:
- Correct hypoglycaemia with 3ml/kg 10% glucose
- Treat hyponatraemia less than 125 mmol/l with 3-5 ml/kg of 3% sodium chloride
- Aim for normothermia
- Treat meningoencephalitis with IV ceftriaxone and acyclovir
- Check ammonia
- Consider specific antidotes
- A LUMBAR SHOULD NOT BE PERFORMED
Indications for a CT head scan include the following:
- New prolonged seizure
- New focal seizure
- Refractory seizures
- New neurological deficit
- Suspected raised intracranial pressure
- Suspected space-occupying lesion
- Ventriculoperitoneal shunt in situ
- Trauma
- Possible non-accidental injury
APLS status epilepticus algorithm
The current APLS algorithm for the treatment of the status epilepticus in children is as follows:
Step 1 (5 minutes after start of convulsion):
In a child that has been convulsing for 5 minutes or more an initial dose of benzodiazepine should be given:
- Lorazepam 0.1 mg/kg should be given IV or IO if vascular access is available
- Buccal midazolam 0.5 mg/kg or rectal diazepam 0.5 mg/kg can be given as alternatives if no vascular access is available or in the pre-hospital setting
Step 2 (5 minutes after 1st benzodiazepine):
If the convulsion continues for a further 5 minutes a second dose of benzodiazepine should be given, and senior help should be summoned.
Step 3 (5 minutes after 2nd benzodiazepine):
At this stage senior help is needed to reassess the child and advise on management.
Anaesthetic or intensive care support should be obtained urgently, as the child may need anaesthetising and intubating if this step is unsuccessful.
Give 40 mg/kg of levetiracetam IV (maximum 3 g) over 5 minutes. This should be given even if the child usually takes oral levetiracetam.
If seizures continue despite loading with levetiracetam then termination with induction of anaesthesia and intubation is expected. However, if the advanced airway team is not yet prepared and the ABCDE assessment is stable, then:
- If not already on phenytoin, then a phenytoin infusion should be set up (20 mg/kg IV infusion over 20 minutes)
- If already taking phenytoin, then phenobarbitone can be used in its place (20 mg/kg IV infusion over 20 minutes)
Rectal paraldehyde at a dose of 0.8 ml/kg of the 50:50 (maximum dose 20 ml) may still appear on individual seizure management plans and can be given in some circumstances.
Step 4 (10 minutes after finish of infusion):
If the child is still convulsing at this stage, then a full paediatric emergency team, including an anaesthetist, paediatric intensivist, or other practitioner with advanced paediatric and airway skills must be present.
Rapid sequence induction is recommended. Ketamine is considered the safest choice of induction agent. Thiopentone and propofol are sometimes used by experienced practitioners but can cause profound hypotension and rarely cardiac arrest. All induction agents should be used with a rapid-onset muscle relaxant, e.g. rocuronium.
Investigations
The following investigations should be considered to aid the diagnostic work-up of a child presenting with status epilepticus:
- Blood tests – full blood count, urea and electrolytes, bone profile, magnesium, glucose, liver function tests, arterial blood gas, c-reactive protein
- Blood cultures if febrile
- Brain imaging – CT head or MRI
- Lumbar puncture for CSF analysis – BUT ONLY WHEN STABLE
- Blood and urine for toxicology
- Metabolic screen – serum ammonia, lactate, plasma amino acids, urine organic acids
- Anti-seizure medication levels if on treatment
- Electroencephalogram (EEG)
Key updates
Notable updates in 7th edition APLS include:
- Pre-hospital treatment with buccal midazolam or rectal diazepam is endorsed at step 1
- There is now a shorter 5-minute interval between benzodiazepine doses in steps 1 and 2
- The second line drug has now changed to levetiracetam
- In step 4, if the team are ready, they should proceed to RSI with either ketamine, thiopental or propofol. If the team are not ready either phenytoin or phenobarbital can be given and if immediately after completing this, if the child is still convulsing, the team should proceed to RSI.
Header image used on licence from Shutterstock
Thank you to the joint editorial team of www.mrcemexamprep.net for this article.