QuestionSBA
QuestionChoice
Levetiracetam is an antiepileptic medication that can be used in the treatment of childhood epilepsy. However, this child has only had one self-limiting episode and does not yet have a confirmed diagnosis of epilepsy; therefore, starting an antiepileptic medication is not indicated here
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Start levetiracetam
QuestionChoice
Paracetamol is one of the mainstays of treatment in febrile convulsions. This child is well, and no fever is reported; therefore, paracetamol is not currently indicated
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Administer paracetamol
QuestionChoice
This child may have had a first presentation of epilepsy and therefore needs to be followed up in a specialist clinic such as a first fit clinic
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Reassure and discharge
QuestionChoice
This child may well be developing epilepsy and, therefore, should be followed up in a first fit clinic. The parents should be educated on what do to if it happens again and advised on when to present to the hospital (if seizures ongoing for an extended period of time or if having multiple seizures in quick succession). They should also be advised to try to record any further episodes
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Refer to first fit clinic
QuestionChoice
Buccal midazolam is useful in the management of acute seizure and status epilepticus. This child is currently well and has recovered from the episode, so the use of benzodiazepines is not indicated here
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Administer buccal midazolam
QuestionComment
Including that the child has been recently well without preceding viral illness would make the history a touch more complete for us
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QuestionComment
I agree, but here's what I found online: Most febrile seizures occur between 6 months and 3 years of age with the peak incidence at 18 months. Approximately 6–15% occur after 4 years, and onset after 6 years is unusual.
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duodeno duodenos tommy
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Pleomorphic Adenoma
QuestionComment
if he is still drowsy wouldnt this be status epilepticus 

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Hypertension Kinase
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# Summary
 

Epilepsy is a group of neurological disorders typified by chronic and recurrent unprovoked seizures, which result from abnormal electrical brain activity. Both focal and generalised seizures may occur, with symptoms corresponding to the affected brain areas. Diagnosis is primarily through EEG, and management involves a comprehensive approach, including optimising anti-epileptic medications, preparing emergency seizure plans, and providing support.
 
 
# Definition
 

Epilepsy refers to a spectrum of neurological disorders characterised by chronic and recurrent unprovoked seizures. This stems from abnormal brain electrical activity, which can be either synchronous or excessive, leading to focal (restricted to a single lobe) or generalised (involving both hemispheres) symptoms and signs.
 
Status epilepticus is defined as a seizure lasting 5 minutes or more, or two or more seizures without complete recovery between episodes. 
 

# Epidemiology
 

 -  Epilepsy affects roughly 1% of the population in the UK.
 -  One in every 220 children in the UK has epilepsy, with over 60,000 children and young people in the UK affected. 
 -  It occurs more commonly in children than adults. 
 

# Aetiology
 

Epilepsy can be due to an idiopathic generalised epilepsy syndrome (see classification below), or can be caused by various factors including:

- Genetic predispositions
    - Children of parents with epilepsy are more likely to have seizures, however, the overall risk is still low. 
- Head trauma
- Tumours
    - More common in supratentorial tumours 
    - Can be seen due to hamartomas in Tuberous Sclerosis 
- Infectious diseases
    - Meningitis and encephalitis 
- Prenatal injuries
- Electrolyte disturbances 
    - Hyper and hyponatraemia 
    - Hypoglycaemia
    - Hypocalcaemia
    - Hypomagnesaemia
- Developmental disorders
- Metabolic disorders
    - Maple Syrup Urine Disease
    - MCAD deficiency 

In children with established epilepsy, seizures can be triggered by:
 
 - Sleep deprivation 
 - Playing video games or watching television 


## Febrile Convulsions 

An important cause of seizures in children is fever. Febrile convulsions, or febrile seizures, are common in children between 6 months to 6 years, presenting as seizures that occur during a fever. The seizures are typically generalised tonic-clonic in nature and usually last less than 5 minutes. Approximately 1 in 20 children will have a febrile seizure. 
 
Diagnosis is generally made from history alone, and management involves treating the underlying fever cause and ensuring the child is safe whilst they are having the seizure. 


# Classification

There are many idiopathic epilepsy syndromes affecting children. These include: 


## Childhood Absence Epilepsy (CAE)

Absence seizures can occur up to 100 times per day, and many episodes may not be noticed. It is common in children aged 4-10 years and often does not persist into adulthood. Diagnosis is often based on clinical history and EEG showing spike and wave activity, stimulated by hyperventilation. 

CAE is typically responsive to anti-epileptics such as ethosuximide. CAE is associated with a good prognosis, as children often do not develop other types of seizures.


## West Syndrome
 

West Syndrome, or infantile spasms, starts between 4-8 months of age. The primary symptom is myoclonic jerking, termed 'jack-knife' spasms, which usually occur in clusters. This syndrome is often associated with developmental regression and high morbidity. Diagnosis is typically made based on the distinctive form of seizure activity and characteristic EEG findings of hypsarrhythmia. Most children may have genetic abnormalities, metabolic conditions or brain injuries/malformations, however, 1 in 3 have no known cause for the seizures. 

Management usually includes prednisolone, ACTH, and/or vigabatrin. Early treatment optimises the child's development, as most children will have intellectual disability later in life. Many children with West syndrome develop Lennox-Gastaut later in childhood.
 
##  Dravet's Syndrome
 
Severe myoclonic epilepsy typically onsets in infancy in an otherwise healthy infant. Typically will present with convulsive status epilepticus during intercurrent illness or following a vaccination. It is typically refractory to anti-epileptic treatment. 

The syndrome is also associated with loss of developmental milestones and Autism Spectrum Disorder. 

Diagnosis is achieved by genetic testing, and management includes multidisciplinary team engagement. Unfortunately, there is an estimated 15% mortality by age 20.

## Lennox Gastaut Syndrome

A syndrome of tonic, atonic and atypical absence seizures occurring in children aged 3-5 years. Absence seizures are considered atypical as they last longer and have a gradual onset when compared to typical absence seizures. EEG will show classic findings of generalised spike-and-wave discharges.

It is subdivided into idiopathic or symptomatic. In idiopathic Lennox Gastaut syndrome, the child has normal psychomotor development. In symptomatic Lennox Gastaut syndrome, there are associated neurological or neuroradiological abnormalities (i.e. tuberous sclerosis, brain malformations, birth injuries). 

In addition to seizures, children often have motor, behavioural and language dysfunction. 

Management is difficult, as seizures are commonly refractory to anti-epileptics. Sodium valproate is used first line, but other options include lamotrigine and clobazam. Surgical intervention including corpus callostomy and vagus nerve stimulation may be tried for refractory cases. 

## Juvenile Myoclonic Epilepsy 

Most commonly onset in adolescence and early adulthood, patients will have early morning myoclonic jerks of the upper limbs - with arms "flinging" in the air. After a few months, the myoclonic jerks commonly progress to generalised tonic-clonic seizures. Patients may also suffer from absence seizures, and children may have a history of childhood absence seizures. 

While many have a family history of JME in distant relatives, there is no genetic test specific for JME. Seizures are often triggered by lack of sleep, stress, flashing lights or alcohol intake. Management focuses on avoiding these triggers. It is typically well controlled using anti-epileptic medications, with sodium valproate and lamotrigine being the medications of choice. Most patients require lifelong treatment with anti-epileptic drugs. 

## Panayiotopoulos Syndrome

Features autonomic seizures with ictal vomiting, pupil dilation and syncope lasting up to 30 minutes. Some children will have vision changes including flashing lights, blurring, and loss of vision. It onsets in childhood, around age 3-6 years. EEG will show characteristic multiple shifting foci predominantly in the occipital region. 

Children have otherwise normal development; typically, seizures stop after 2-3 years. There is no associated increased risk of epilepsy later in life. 

## Benign Epilepsy with Centrotermporal Spikes (BECTS) 

This condition, formerly known as Benign Rolandic Epilepsy, typically affects children aged 3-10 years and mostly occurs during sleep. Parents might notice the child experiencing a tonic seizure overnight or find the child on the floor or with disrupted bedding in the morning. The diagnosis is based on history and characteristic EEG findings during sleep of centro-temporal spikes.

It is the most common form of childhood epilepsy and is associated with an excellent prognosis, with resolution in most children by adolescence. 
 

# Signs and Symptoms
 
Epilepsy may present with focal seizures, generalised seizures, or both. 

Focal seizures may produce symptoms specific to the involved lobe: 

- Occipital lobe seizures may cause visual phenomena 
- Temporal lobe seizures may cause the child to experience unusual smells 


Generalised seizures may include:

- Typical absence seizures:
    - Brief, sudden onset, episodes of unresponsiveness where the child may stop what they are doing and stare 
    - The child may also flutter their eyelids or movements of their arms 
    - They will not respond even to vigorous stimulation
- Atypical Absence seizures:
    - Children will be unresponsive and commonly stare blankly, but these episodes occur more gradually and last longer than typical absence seizures. 
- Tonic: muscle stiffening
- Tonic-clonic: muscle stiffening followed by rhythmic jerking
- Myoclonic: brief jerking movements
    - Such as seen in infantile spasms or juvenile myoclonic epilepsy
- Atonic: sudden falls, the child suddenly becomes limp 
    - Can be seen in Lenox-Gastaut or Dravet syndrome 

 

# Differential Diagnosis
 

 -  **Syncope**: A brief loss of consciousness, with rapid recovery. Often associated with prodromal symptoms of dizziness, nausea and changes to the vision. Associated with a history of prolonged standing, dehydration or heat. 
 -  **Non-epileptic attack**: Also referred to as dissociative seizures, the child or young person may have convulsive movements of their body/limbs, become incontinent or become unresponsive. These seizures tend to last longer than epileptic seizures and are not managed with benzodiazepines. 
 -  **Night terrors**: Episodes occur at night, when the child awakens inconsolably, crying and screaming. The child does not remember the episodes in the morning, and children outgrow these episodes by age 10. 
 -  **Day Dreaming**: A common differential for absence seizures. This typically onsets more gradually. It is able to be ended by others attempting to gain the child's attention. 

# Investigations
 

Refer children urgently (less than 2 weeks) for assessment by a paediatrician with expertise in assessing first seizures. 

 -  Epilepsy is diagnosed based on history and examination
 -  An EEG may be used to provide information about seizure type, but an EEG is not used to exclude a diagnosis of epilepsy. 
       -  Seizures can be provoked by hyperventilation, photic stimulation, or sleep deprivation.
 -  Imaging modalities such as MRI or CT are usually performed to rule out structural brain abnormalities.
 -  An ECG may be done to identify cardiac conditions which may mimic epilepsy. 

If epilepsy onset is below age 2 or there are other features (i.e. learning disability, structural abnormality, autism spectrum disorder), then whole genome sequencing may be done. 
 

# Management

When there is a known reversible cause for the seizure (i.e. electrolyte or metabolic disturbance), this should be managed appropriately (i.e. glucose for hypoglycaemia). 

For idiopathic seizures or those associated with epilepsy syndromes, management necessitates a multidisciplinary approach to optimise the child's care. This may include: 

- Anti-epileptic medications
- Some children will be recommended a ketogenic diet or low glycaemic diet 
- Surgery may be used in refractory cases or if the seizures are caused by tumours
        - This may include removal of a tumour, vagus nerve stimulation or corpus callosotomy 
- MDT support for comorbidities such as educational and psychological support 
- Emergency seizure plans for both home and school 
        - Parents should be advised to ensure their child is safe whilst having a seizure, to call 999 and start a timer. Once the seizure has finished, they should place the child in the recovery position. 

# Complications

The rates of complications depend on the underlying cause of epilepsy, but may include:

- Mood disorders
- Status Epilepticus 
        - Prolonged seizure activity lasting more than 5 minutes. 
- Sudden Unexpected Death in Epilepsy (SUDEP)
        - A rare complication of epilepsy in which an otherwise healthy individual with epilepsy dies unexpectedly. 
- Developmental delay or regression 

# Prognosis 

The prognosis of epilepsy in childhood is dependent on the underlying cause. Childhood Absence seizures and Benign Rolandic Epilepsy both are associated with a good prognosis. 

# NICE Guidelines
 
[NICE Guidelines on Epilepsies in Children, Young People and Adults](https://www.nice.org.uk/guidance/ng217) 

# References 

 [NHS page on Febrile Seizures](https://www.nhs.uk/conditions/febrile-seizures/)
 
 [Resuscitation Council UK Paediatric Status Epilepticus Algorithm](https://www.resus.org.uk/sites/default/files/2022-03/RCUK%20Paediatric%20emergency%20algorithms%20and%20resources%20Mar%2022%20V1.pdf) 
 
 [Non Epileptic Attack Disorder](https://www.mind.org.uk/information-support/types-of-mental-health-problems/dissociation-and-dissociative-disorders/nead/)
 
 [Patient Info Epilepsy in Children and Young People](https://patient.info/doctor/epilepsy-in-children-and-young-people) 
 
 [Patient Info Lennox-Gastaut Syndrome](https://patient.info/doctor/lennox-gastaut-syndrome) 
 
 [Epilepsy Society Children with Epilepsy](https://epilepsysociety.org.uk/children)
 
 [Epilepsy Action Childhood Absence Epilepsy](https://www.epilepsy.org.uk/info/syndromes/childhood-absence-epilepsy-cae)
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Seizures in paediatrics
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Paediatrics
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In a child experiencing their first seizure, referral to a first fit clinic is essential for further evaluation and management.
A 5-year-old boy is brought to the Emergency Department after his parents witness a seizure at home. This has never happened before, and they are understandably very concerned. On review in the department, his observations are normal; he is well though slightly drowsy.

What is your next step in management?
a