All types of childhood epilepsy: a complete guide

Epilepsy is one of the most common chronic neurological conditions in children, so much so that in 1965 the World Health Organisation (WHO) recognised it as a social disease. It is characterised by the presence of recurrent epileptic seizures, which can occur at any time during a person’s life.

In this article, we will seek to provide comprehensive guidance on the condition, identifying the different types of childhood epilepsy and jointly examining causes, symptoms and the treatment options currently available.

How many types of childhood epilepsy are there?

Epilepsy is a neurological condition that manifests itself in many forms, so it would be more appropriate to speak of “epilepsies” in the plural, rather than of a single entity. This wide range of clinical manifestations also implies different effects on daily life: some forms of epilepsy allow an almost normal quality of life; others, more rare, are particularly severe and can be accompanied by neurological, cognitive, behavioural and mental symptoms.

Among the main symptoms of epilepsy in children are epileptic seizures, which are highly variable clinical phenomena characterised by sudden and transient episodes. The cause to be investigated is the abnormal functioning of neurons located within the grey substance, in the cerebral cortex.

An initial classification of epileptic attacks depends on the areas of the brain that are involved:

  • focal seizures are defined as seizures that originate in a specific area of the brain and that, in some cases, may subsequently spread to other brain regions;
  • generalised seizures is the term for seizures that affect both hemispheres from the very beginning.

Focal seizures are further divided based on subject awareness during the seizure:

  • focal seizures with preserved awareness, when the person remains aware of what is going on and can interact;
  • focal crisis with altered awareness, in which the patient loses their ability to perceive the surrounding environment and respond.

On the other hand, generalised seizures are divided into:

  • motor seizures: for example, tonic-clonic, clonic, myoclonic and atonic seizures;
  • non-motor seizures: such as typical or atypical absence seizures.

Epilepsy can therefore be classified according to the type of seizure that occurs. We will then distinguish between:

  • focal epilepsy
  • generalised epilepsy
  • mixed epilepsy (focal and generalised epilepsy)
  • unknown onset epilepsy, when the origin of the seizures cannot be determined.

Paediatric epilepsy also comes in many different forms, each with its own characteristics and symptoms. Understanding which type we are dealing with is essential to determining the most appropriate treatment.

Which ones are the most common?

Childhood benign epilepsy with central temporal or rolandal tips (also known as BECRS) is the most common epilepsy syndrome in children, with an estimated incidence of about 21 cases per 100,000 children under the age of 15.

However, it is not the only possible form. Let’s have a look at some of the ways in which epilepsy can occur in children.

Absence or “petit mal” seizures

Absence seizures, also known as “petit mal seizures”, are characterised by a brief loss of consciousness. The child seems to be staring into space for a few seconds and then resumes the activity as if nothing had happened. These seizures are common in idiopathic epilepsy and usually last for about 5 to 15 seconds. Generalised tonic-clonic seizures may also occur in association. It should be stressed, however, that the evolution of the disorder is favourable in most cases.

Atonic seizures

Atonic seizures result in sudden loss of muscle tone and state of consciousness, which can lead to sudden falls and trauma.

Generalised tonic-clonic seizures

Generalised tonic-clonic seizures are more marked and dramatic. They begin with a tonic phase, in which the muscles stiffen, and then continue with a clonic phase of convulsive movements. These seizures are often associated with Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome

This syndrome is a severe form of epilepsy that manifests itself in different types of seizures, including tonic-clonic, atonic, and absence seizures. It affects between 1 and 4% of childhood epilepsy cases and, unfortunately, is often resistant to conventional treatments. The onset typically occurs between 3 and 5 years, however – almost always – before the age of 8. The syndrome often causes the development of certain cognitive disorders, especially related to memory and reasoning.

Juvenile myoclonic epilepsy

Juvenile myoclonic epilepsy is an inherited syndrome that occurs during adolescence with myoclonic seizures (short, involuntary muscle contractions) upon awakening, often followed by generalised tonic-clonic seizures.

Dravet syndrome

Dravet syndrome is a rare and severe form of epilepsy that begins in the first year of the child’s life. It is characterised by prolonged and febrile convulsions, which lead to progressively impaired neurological development. In fact, most patients have a state of disability (both motor and cognitive) that continues into adulthood.

Idiopathic epilepsy

Idiopathic epilepsy refers to forms of epilepsy with no apparent cause or apparent brain injury. in fact, it often originates from a genetic component.

Rolandic epilepsy

Rolandic epilepsy is a benign form that occurs in children between the ages of 3 and 13. Seizures, often at night, involve the face and mouth, and tend to resolve spontaneously with age.

Focal epilepsy

As we mentioned earlier, focal epilepsy originates in a specific area of the brain. Symptoms vary depending on the affected area and, as we have seen, can include involuntary movements, strange sensations and changes in consciousness.

The Jacksonian March

This is a focal epilepsy manifestation where seizures are located in a specific area. Usually, the attack starts on one side of the face and then involves the arm. This is followed by temporary speech loss and the presence of clonic movements of the eyelids. During the seizure, however, contact with the surrounding environment is maintained. In some cases, the attack can evolve into a generalised seizure or it can remain exclusively sensory in nature, manifesting in the form of pain and paraesthesia located in one part of the body. This is what is termed a classic example of partial motor seizure.

Nocturnal epilepsy

Nocturnal epilepsy occurs primarily during sleep. Seizures can range from simple night movements to complex seizures.

Partial epilepsy

Partial epilepsy affects only one part of the brain. They can be simple, without loss of consciousness, or complex, with altered awareness.

Convulsions or “grand mal” seizures

Convulsions, or “grand mal” seizures, are generalised tonic-clonic seizures involving the entire body, often accompanied by loss of consciousness and intense seizure movements.

Morphic epilepsy

Morphic epilepsy is a rare form of sleep seizures and may be associated with sleep disorders and behavioural changes.

What may the causes be?

But what are the causes of childhood epilepsy? Creating a complete and exhaustive list of the reasons behind this condition is very difficult. We can say, however, that from anaetiological point of view, epilepsy is categorised as:

  • structural, when caused by visible head injuries, damage or malformations;
  • genetic, when derived from specific genetic mutations, such as most idiopathic epilepsies or Dravet syndrome;
  • infectious, resulting from infectious diseases such as meningitis, encephalitis or other infections of the central nervous system;
  • metabolism, when it is associated with some rare disorders of metabolism;
  • dysimmune, i.e. caused by diseases of the immune system;
  • idiopathic, when the cause is unknown.

Diagnosis and therapies

The diagnosis of epilepsy in children requires a thorough assessment, including:

  • detailed medical history, in order to collect as much detailed information as possible about symptoms and triggers;
  • neurological examinations, to assess the neurological functions of the child;
  • electroencephalogram (EEG), in which the electrical activity of the brain is monitored, to understand what type of crisis occurs and in what area of the brain;
  • brain imaging, through MRI or CT scan to detect or rule out any brain injury.

Treatment options for childhood epilepsy are different and include different strategies, depending on the case of course. Anti-epileptic drugs are the first line of treatment and most children respond well to this type of therapy. Another option is the ketogenic diet, a diet high in fat and low in carbohydrates which may be effective in some selected cases, but it is necessary to undertake it under strict medical control and without improvising it. In cases where the drugs are not sufficient, surgery may be considered: specifically, resection, disconnection or neuromodulation surgery can be performed, so the epilogenic area is removed or isolated from the rest of the brain. Finally, vagus nerve stimulation (SCV) is a technique that stimulates the vagus nerve and reduces the frequency of seizures by implanting a device.

In conclusion, we have seen that epilepsy is a complex disease and therefore a multidisciplinary and personalised approach is needed. There are many critical issues, from co-morbidities in epilepsy to the social impact of the disease: with the right information and resources, however, it is possible to manage the condition effectively, improving the lives of children and their families.