childhood epilepsy syndromes
How childhood epilepsy syndromes are diagnosed, details of some specific syndromes and sources of further support.
What is a syndrome?
A syndrome is a group of signs or symptoms that happen together and help to identify a unique medical condition.
What is a ‘childhood epilepsy syndrome’?
If your child is diagnosed with an epilepsy syndrome, it means that
their epilepsy has some specific signs and symptoms. These include:
An EEG test is painless and it records patterns of electrical
activity in the brain. Some epilepsy syndromes have a particular pattern
so the test can be helpful in finding the correct
diagnosis. An epilepsy syndrome can only be diagnosed by looking at all the signs and symptoms together.
If your child is diagnosed with an epilepsy syndrome it may help the
paediatrician (doctor who specialises in treating children) to plan
their care. For example, choosing treatment options or deciding whether
further tests are needed.
Different types of syndrome
Syndromes can vary greatly. Some are called ‘benign’ which means
children become seizure-free (have no seizures) once they reach a
certain age. Other syndromes are ‘severe’ and children have seizures
which are difficult to control.
Anti-epileptic drugs (AEDs) may be tried alone or in combination with each other and some non-drug treatments may also be tried, for example the
ketogenic diet.
Many children with severe epilepsy syndromes have additional
difficulties with learning and behaviour and may need extra support.
Examples of childhood epilepsy syndromes
Benign rolandic epilepsy
This syndrome affects 15% of children with epilepsy and can start at any time between the ages of 3 and 10.
Children may have very few seizures and most become seizure-free by
the age of 16. They may have simple focal seizures, (sometimes called
simple partial seizures) often at night, which begin with a tingling
feeling in the mouth, gurgling or grunting noises and dribbling. Speech
can be temporarily affected and symptoms may develop into a generalised
tonic clonic (convulsive) seizure. AEDs may not be necessary but they
can be helpful to control seizures.
Childhood absence epilepsy
This syndrome starts between the ages of 4 and 10 and can affect up
to 12% of children with epilepsy under 16. Absence seizures happen
frequently and are very brief, lasting only a few seconds. Because of
this they often go unnoticed.
During a seizure a child will become unconscious. They may look blank
or stare and their eyelids may flutter. They may not respond to what is
happening around them or be aware of what they are doing. Seizures
respond well to medication. If a child is seizure-free for two years
medication is sometimes reduced gradually. Up to 90% of children with
CAE will grow out of seizures by the age of 12. Occasionally a child may
also have other types of seizure.
Juvenile myoclonic epilepsy
This syndrome starts between the ages of 12 and 18. Many children have three different types of seizure:
- myoclonic seizures (brief muscle jerks) in the upper body
- tonic clonic seizures
- absence seizures.
These often happen shortly as, or shortly after, the child or
young person wakes up. Medication can be successful in controlling
seizures, which often continue into adulthood and may become less
severe.
Tiredness, stress and alcohol can trigger seizures. Up to 40% of
children or young people with JME have seizures that are triggered by
flashing or flickering lights .
Infantile spasms (or West syndrome)
This syndrome often begins in the first year of life and can affect
children who have had a previous brain injury before the age of 6
months. It is identified by brief spasms or jerks which happen in
‘clusters’. Spasms can affect the whole body or just the arms and legs.
Each cluster can include between 10 – 100 individual spasms, which often
happen when the child is waking up. Ongoing studies are looking at
using AEDs and steroids to treat this syndrome, although around 25% of
children have spasms that do not respond well to medication. Many
children develop problems with learning or behaviour. Some may go on to
develop Lennox-Gastaut syndrome.
Lennox-Gastaut syndrome (LGS)
This syndrome usually begins between the ages of 3 and 5, but can
start as late as adolescence. Children may have several different types
of seizure with this syndrome. These include tonic (where the muscles
suddenly become stiff), atonic (where the muscles suddenly relax),
myoclonic, tonic clonic and atypical absences. Atypical absences often
last longer than normal absences and are different as a child may be
responsive and aware of their surroundings.
Many children also develop learning difficulties as well as behaviour problems.
This syndrome can be very difficult to treat with AEDs, and most
children need a combination of different drugs. Some non-drug treatments
such as the
ketogenic diet and
vagus nerve stimulation therapy (VNS) can also be helpful. Seizures often continue into adult life.
Who can I talk to?
If your child has been diagnosed with a childhood epilepsy syndrome
you may have concerns or questions. You can get information and advice
from a paediatrician with an interest in epilepsy or a paediatric
neurologist. Support may also be available through an epilepsy
specialist nurse, counsellor, support group or helpline.
Some people find it helpful to talk to friends or family about their
child’s epilepsy. You might find it helpful to speak to other parents.
Support groups and online forums can be a useful way to share your
experiences.
