Epilepsy is the most common neurological condition in childhood, affecting approximately 50 000, or 1 in 240, children and young people under the age of 16 years in the UK (Joint Epilepsy Council of the UK, 2011). Epilepsy describes a tendency to have seizures, where a sudden surge in brain activity manifests as abnormal behaviour patterns (Fisher et al, 2005). There are many different seizure types and so appropriate recognition and response to seizures can be difficult (Kramer et al, 1998). Furthermore, a prolonged convulsive seizure, termed status epilepticus, can cause serious brain damage, or in rare cases, death (Appleton et al, 2000). As such, the capability to recognise and appropriately respond to childhood seizures is critical for healthcare professionals caring for children and young people with epilepsy.
‘Epilepsy describes a tendency to have seizures, where a sudden surge in brain activity manifests as abnormal behaviour patterns….There are many different seizure types and so appropriate recognition and response to seizures can be difficult… the capability to recognise and appropriately respond to childhood seizures is critical for healthcare professionals’
Seizures: what are they and what causes them?
A seizure describes abnormal behaviour patterns resulting from a sudden surge in electrical activity in one or more parts of the brain (Fisher et al, 2017). Any process that interrupts normal brain signalling can cause seizures. Febrile seizures, where high body temperature triggers abnormal brain activity, are the most common cause of childhood seizure, affecting 2–5% of children aged 6 months–5 years (American Academy of Pediatrics, 2011). Other potential seizure causes include traumatic brain injury, hypoglycaemia and metabolic disturbances, central nervous system infection, and, very rarely, brain tumours (Box 1) (Mwipopo et al, 2016; Wilne et al, 2006). Conversely, seizures can have no clear cause, termed ‘unprovoked seizures’, which may lead to a diagnosis of epilepsy.
Box 1.COMMON CAUSES OF SEIZURE AND EPILEPSY IN CHILDRENReversible or temporary causes of seizure
- Infection (febrile seizure)
- Central nervous system infection (meningitis or encephalitis)
- Metabolic derangement (hypoglycaemia, hyponatraemia, hypocalcaemia)
- Traumatic brain injury
Epilepsy causes (causes of increased seizure tendency)
- Prior brain injury (meningitis, stroke, trauma)
- Perinatal event (hypoxic-ischaemic event, haemorrhage, infarction)
- Structural changes (tumours, neurocutaneous disorders)
- Malformations of cortical development
- Inborn errors of metabolism
- Idiopathic (most epilepsy has no confirmed cause)
(SINGH AND GAILLARD, 2009)
Epilepsy: definition
Epilepsy is a condition that describes a tendency for someone to have seizures. The International League Against Epilepsy defines epilepsy as a disease of the brain clinically defined by (Fisher et al, 2014):
- At least two unprovoked seizures occurring more than 24 hours apart; or
- One unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years; or
- Diagnosis of an epilepsy syndrome.
The term ‘unprovoked’ refers to the absence of a temporary or reversible brain disturbance, such as fever or concussion. However, identification of causative factors, such as persistent metabolic or structural problems, may determine epilepsy causation rather than necessarily negate its diagnosis. Nevertheless, the majority of epilepsy in children and young people has no known cause (Camfield and Camfield, 2015; Aaberg et al, 2017).
Seizures: what are the different types of childhood seizures?
Seizures are classified as being either generalised or focal in onset (Box 2) (Fisher et al, 2017). Generalised seizures affect both cerebral hemispheres at once without warning and usually cause impaired awareness or loss of consciousness (Major and Thiele, 2007). The principle generalised seizure types are tonic-clonic, absence, myoclonic, and atonic seizures. In contrast, focal seizures start in one area of the brain and awareness may be maintained or diminished, termed focal aware or focal impaired awareness seizures, respectively (Arzimanoglou et al, 2003). A focal seizure may subsequently spread to both cerebral hemispheres, termed focal to bilateral tonic-clonic seizure. In such cases, the focal seizure is a warning, sometimes referred to as an ‘aura’, that a tonic-clonic seizure is about to occur (Major and Thiele, 2007). Children and young people may enter a ‘postictal’ period after seizure termination, which describes the time immediately following a seizure where a child may be drowsy and have altered cognition (Fisher and Schachter, 2000).
Box 2.SIMPLIFIED CLASSIFICATION OF COMMON CHILDHOOD SEIZURES
- Focal onset Affects one brain area. Area affected determines signs and symptoms. Types of focal seizures:
- Aware
- Impaired awareness
- Focal to bilateral tonic-clonic
- Generalised onset Affects both cerebral hemispheres at once without warning. Associated with loss of consciousness. Types of generalised seizures:
- Tonic-clonic
- Absence
- Myoclonic
- Atonic
(AABERG ET AL, 2017)
Generalised seizures
Generalised tonic-clonic seizures, where a child's muscles become rigid (tonic), before rhythmical jerking movements (clonic), are the most common and recognisable childhood seizure. Tonic-clonic seizures account for 36–64% of childhood seizures, an estimate that combines generalised and focal to bilateral tonic-clonic seizures (Eriksson and Koivikko, 1997; Beilmann et al, 1999; Waaler et al, 2000; Ling Kwong et al, 2001; Ünver et al, 2015). Absence seizures account for 3–7% of childhood seizures and describe episodes where a child becomes blank and unresponsive for a few seconds, sometimes mistaken for daydreaming (Kramer et al, 1998; Beilmann et al, 1999; Ünver et al, 2015). Myoclonic seizures account for 0.3–9% of childhood seizures and describe sudden muscle twitching, which typically lasts only one or two seconds (Kramer et al, 1998; Ling Kwong et al, 2001; Ünver et al, 2015). Myoclonic seizures do not usually impair consciousness, in contrast to all other common forms of generalised seizure.
Focal seizures
Focal impaired awareness and focal aware seizures account for 18–23% and 3–10% of childhood seizures, respectively (Kramer et al, 1998; Beilmann et al, 1999; Ünver et al, 2015). The behaviour pattern of focal seizures, and whether awareness is impaired, depends on the brain region affected (Arzimanoglou et al, 2003). Seizures originating in the temporal lobe typically impair awareness and are associated with oroalimentary automatisms, such as lip smacking, chewing, or swallowing, and sensory disturbances, such as unusual tastes or smells and a rising sensation in the stomach. Seizures affecting the frontal lobe are usually sleep-related and cause motor symptoms such as kicking, head movements and unusual body posturing. Parietal lobe involvement may cause unusual sensations such as numbness and paraesthesia, and occipital lobe involvement can cause visual phenomena, such as seeing flashing lights, patterns, or images (Guerrini, 2006).
Individualised treatment plans
All children and young people with epilepsy should have an individualised treatment plan, or ‘epilepsy passport’, which should detail all required steps for the emergency management of a child's seizures (National Institute of Health and Care Excellence (NICE), 2013). However, this may not always be available and so healthcare professionals caring for children and young people with epilepsy should know, and be able to deliver, essential seizure management.
‘All children with epilepsy should have an individualised treatment plan, or “epilepsy passport”, which should detail all require steps for the emergency management of a child's seizures’
First aid management of seizures
Different seizure types require different levels of support, although the principles of airway, breathing and circulation (ABC) optimisation, ensuring patient safety, timing the seizure length, and staying with the child until they are fully recovered are consistent throughout (Epilepsy Society, 2018).
During focal aware seizures, normal consciousness is maintained and so a child should not require any specific first aid measures, other than reassurance (Epilepsy Australia, 2020). During focal impaired awareness seizures, a child has variable control over their actions, and so first aid measures should include guiding the child towards a safe area and ensuring no attempts are made to restrain the child (Epilepsy Society, 2018; Epilepsy Australia, 2020). After the seizure, the child may remain drowsy and so attempts should be made to gently rouse the child, and, if unable to do so, the child should be placed in the recovery position. Both myoclonic and absence seizures typically last only a few seconds and so first aid is usually limited to ensuring that a child is fully recovered and has not sustained an injury from the seizure (Epilepsy Society, 2018).
Generalised tonic-clonic and focal to bilateral tonic-clonic seizures have the greatest potential to be prolonged and cause harm (Scott, 2014). A timer should be started with the seizure to determine treatment steps and signal when emergency services should be requested. With regards to safety, the child should be kept away from hazards, their head should be cushioned, and their movements should not be restrained. In terms of basic airway and breathing management, nothing should be put in their mouth, and there should be nothing that hinders their breathing. These first aid measures are usually all that is required during the seizure, and once it is finished, the child should be placed in the recovery position, airway and breathing checked, and the child should be observed until they are fully recovered. An ambulance should be called if (Epilepsy Society, 2018):
- The child is not breathing normally after the seizure stops
- The seizure lasts more than five minutes
- The seizure lasts two minutes longer than is usual for them
- The child has never had a seizure before
- A second seizure occurs without complete recovery in between seizures
- The child has significantly injured themselves.
Clinician management
Where personnel are trained and confident to do so, further ABC management should be performed (Box 3) (McKenzie et al, 2021). Airway maintenance is critical and may be supported by temporarily positioning the child on their side to facilitate secretion drainage, chin lift or jaw thrust manoeuvres as required, and use of a nasopharyngeal airway (NICE, 2011; Brown et al, 2019). Oxygen, if available, should be given, and the child's vital signs should be monitored. Child respirations may be supported with a Bag-Valve-Mask apparatus if required. Intravenous access should be obtained, and blood glucose levels checked and, if low, treated according to local hypoglycaemic protocols (NICE, 2011; Brown et al, 2019).
Box 3.CONVULSIVE SEIZURE TREATMENT ALGORITHM0–5 minutes
- Safety: Do not restrain, remove hazards, cushion head
- Time seizure from its onset, monitor vital signs
- Maintain adequate ABCs, consider: a) postural drainage, airway manoeuvres, nasopharyngeal airway; b) oxygen, Bag-Valve-Mask ventilation c) intravenous access
- Check and correct blood glucose
If individualised treatment plan available, follow plan, otherwise follow NICE guidelines for medication administration.≥5 minutes from seizure onsetAdminister first dose benzodiazepine, one of:
- Buccal midazolam
- Rectal diazepam
- Intravenous or intraosseous lorazepam
≥15 minutes from seizure onsetAdminister second dose of benzodiazepine:
- Intravenous or intraosseous lorazepam
- If intravenous or intraosseous access unobtainable, give repeat dose of buccal or rectal benzodiazepine
Further hospital treatment
- Intravenous phenytoin or phenobarbitone infusion (≥25 minutes from seizure onset)
- Rapid sequence induction and intubation (≥45 minutes from seizure onset)
(NICE, 2011)
Syncope and non-epileptic att acks can mimic a seizure in older children, as can reflex asystolic syncope and breath-holding att acks in younger children. Accurate description of an episode, or bett er, videorecording of it, helps specialists to determine the nature of an episode.
‘A detailed history can usually delineate seizures from seizure mimcs; however, video recordings, neuroimaging and electroencephalograms may be required before specialists can determine the nature of an episode’
Medication for seizures
Although most seizures self terminate, benzodiazepine medication should be given either when directed by a child's individualised care plan, or when a seizure meets the definition of convulsive status epilepticus (Glauser et al, 2016). Convulsive status epilepticus is clinically defined as more than 5 minutes of either continuous convulsive seizure or two or more discrete convulsive seizures between which there is incomplete recovery of consciousness (Trinka et al, 2015; Lowensteine et al, 1999). When the child does not have available intravenous or intraosseous access, the benzodiazepine administered should be either buccal midazolam (5mg for 1–4 years old, 7.5mg for 5–9 years old, 10mg for >10 years old), or rectal diazepam (5mg for 1 month–1 year old, 5–10mg for 2–11 years old, 10–20mg for >12 years old) (NICE, 2011; Paediatric Formulary Committee, 2021). If the seizure continues for 10 minutes after administration of a first benzodiazepine dose, a second benzodiazepine dose should be given. Ideally, this should be given intravenously or intraosseously, but if access cannot be obtained, a further full dose of buccal or rectal benzodiazepine should be given (Brown et al, 2019). Timely benzodiazepine administration is critical for reducing adverse outcomes in convulsive status epilepticus (Gaínza-Lein et al, 2018).
Benzodiazepine adverse effects
Benzodiazepines are sedatives and their adverse effects include respiratory depression, hypotension, and sedation (Maier et al, 2021). Healthcare professionals should consider and be able to manage these issues, primarily through supporting respiration by bag-mask-valve ventilation, as required. However, these adverse effects are also associated with prolonged status epilepticus and are more likely to occur if benzodiazepines are not administered, and so the risk of adverse effects should not restrict appropriate use of benzodiazepines (Glauser et al, 2016).
Further hospital management of convulsive status epilepticus
If the second dose of benzodiazepine fails to terminate the seizure, further anticonvulsant medication should be given in hospital. A phenytoin or phenobarbitone infusion, the choice of which is dependent on whether the child already takes oral phenytoin, should be started 10 minutes aft er administration of the second benzodiazepine dose. If this fails to terminate the seizure, rapid sequence induction and intubation, with subsequent paediatric Intensive Care Unit admission, is required (NICE, 2011).
Management of nonconvulsive status epilepticus
A minority of child status epilepticus is nonconvulsive, the two most recognised forms being prolonged absence and prolonged complex partial seizures (Gurcharran and Grinspan, 2019). Nonconvulsive status epilepticus requires electroencephalogram testing before a definitive diagnosis can be made and there is a lack of clear, evidence-based guidance for its treatment (Baker et al, 2019). As such, prehospital management should focus on ABC support and hospital transfer, where further investigative workup can occur before using anticonvulsant medication.
Secondary prophylaxis of seizures
Antiepileptic drugs (AEDs) are the mainstay of epilepsy treatment and should only be initiated by a specialist (NICE, 2012). They are usually started after a second unprovoked seizure in a child (Lee, 2019). The principal goal of AEDs is to prevent further seizures, although this must be balanced against the tolerability of medication side effects (Egunsola et al, 2018). AED monotherapy will effectively prevent seizures in 60–70% of children and young people (Camfield et al, 1997; Dudley et al, 2009), and the remainder will be considered for AED polytherapy, surgery, vagal nerve stimulation, and ketogenic diets (Laxer et al, 2014). The high efficacy of AEDs underlines the importance of medication adherence for seizure prevention. In addition, accurate documentation of a seizure event, with transfer of this information to a child's specialist team or GP, will better inform specialist decisions regarding AED treatment.
‘All first seizures should be reviewed in hospital, and so parental advice is best delivered by healthcare professionals with relevant paediatric expertise, supported by additional investigations.’
Avoidance of seizure triggers also helps to reduce the likelihood of seizures, the most common childhood triggers being illness and fever, sleep deprivation, emotional stress, and menstruation in females (Fang et al, 2008). A minority of childhood seizures are photosensitive, so avoidance of intense light stimuli is important for this cohort (Covanis, 2005).
Parental advice
All first seizures should be reviewed in hospital, and so parental advice is best delivered by healthcare professionals with relevant paediatric expertise, supported by additional investigations. If a seizure is confirmed, healthcare professionals are likely to recommend some activity modifications (Camfield and Camfield, 2008). For bathing, showers are recommended over baths, and an adult should be aware of when a child is in the shower and the door should not be locked. Swimming should be appropriately supervised, and a responsible swim buddy may initially be recommended. Restrictions on tree and other high climbing may also initially be recommended.
Seizure mimics
There are many conditions that can mimic a seizure. Syncope and non-epileptic attacks are common mimics in older children and young people (Babiker and Prasad, 2015), whereas reflex asystolic syncope and breath-holding attacks are common mimics in preschool-aged children and young people (Prasad and Babiker, 2015). A detailed history can usually delineate seizures from seizure mimics; however, video recordings, neuroimaging and electroencephalograms may be required before specialists can determine the nature of an episode (Stainman and Kossoff, 2020; Luat et al, 2015).
Seizure mimics should also be initially managed with supportive ABC measures, and emergency assistance should be requested for prolonged episodes or where breathing is impaired. As such, immediate determination of whether an episode constitutes a seizure is not necessarily required, nor is it always possible. Prolonged non-epileptic attacks can be difficult to differentiate from status epilepticus and are frequently mistreated with anticonvulsants (Viarasilpa et al, 2020). Typical features of non-epileptic attacks include eye closure, self-protective manoeuvres, and side-to-side head or body movements (Babiker and Prasad, 2015). When a non-epileptic attack is suspected, the clinical decision not to use anticonvulsants must balance the likelihood of an episode being a non-epileptic attack against the potential serious harm of untreated status epilepticus.
Conclusions
The heterogenous nature of different seizure types, combined with potential seizure mimics, can confuse the acute management of seizures. Prehospital clinicians should focus on adequate maintenance of airway and breathing, blood glucose check where possible, prompt request for ambulance services where an episode is prolonged, and benzodiazepine administration for prolonged convulsive seizures. Individualised care plans and epilepsy passports can simplify management further and should be located where possible.
‘Prehospital clinicians should focus on adequate maintenance of airway and breathing, blood glucose check where possible, prompt request for ambulance services where an episode is prolonged, and benzodiazepine administration for prolonged convulsive seizures.’
KEY POINTS
- A seizure describes the signs and symptoms that arise from a sudden abnormal surge in electrical activity in one or more parts of the brain.
- Epilepsy is a disease of the brain that describes a tendency to have seizures.
- Although there are many different seizure types, emergency seizure management follows the same principles: child safety, ABC support, and urgent hospital transfer for all seizures that last longer than 5 minutes.
- Convulsive seizures lasting more than 5 minutes require treatment with benzodiazepines. Buccal midazolam or rectal diazepam are first line medications where there is no intravenous or intraosseous access.
- Close observation should continue after seizure termination until the child is fully recovered.
- Children with epilepsy should have individualised treatment plans which simplify emergency seizure management.
REFLECTIVE QUESTIONS
- What is the difference between a seizure and epilepsy?
- What are the signs of different seizure types?
- What are the principles of managing a child with a suspected seizure?
- When and how do you give a benzodiazepine to a child with seizure?