This epilepsy syndrome was described for the first time in 1995. This makes it one of the newer epilepsy syndromes. It is also very rare and occurs in between one in 200,000 and one in 400,000 people.
Most children with this syndrome start having seizures in the first few weeks of life; all will start having seizures by 6 months of age.
Recent research has found some abnormal genes in a number of children with this syndrome. These genetic abnormalities are called ‘mutations’. The genes are involved with the flow of certain ions (particularly potassium) through ‘channels’ in the nerve cells. The abnormal passage of potassium through these channels causes the epilepsy. It is very likely other genetic mutations will be found in the future. Although results of tests to look for a cause are normal, with time the MRI scan may show the brain shrinks in size (brain atrophy).
Although this epilepsy syndrome is thought to be a genetic condition, it does not seem to run in families. This means that it is very rare for two children, with the same biological parents, to have this epilepsy syndrome.
Other names for epilepsy of infancy with migrating focal seizures
- Migrating partial epilepsy in infancy
- Migrating partial seizures of infancy
- Malignant migrating partial seizures in infancy
Different things happen in the seizures:
- There may be some stiffness of the body, arms or legs (either affecting one side of the body or both sides of the body). The head may turn forcibly to one side or another
- There may be chewing movements or some very brief cycling or pedalling movements of the arms or legs
- There may be some unusual features during the seizures, including flushing of the face, drooling of saliva and tears. These are called ‘autonomic’ features
All of these symptoms are types of focal (previously called partial) seizures, meaning the seizures start in one area of the brain. Sometimes these seizures may then spread to affect both sides of the brain, called focal to bilateral tonic-clonic seizures. The tonic-clonic seizures more commonly affect older children (aged 2 years and above).
The seizures may only happen every few days in the first few weeks after the epilepsy starts. However, very rapidly, the seizures increase in frequency to the point where they may happen many times every day, even up to 100 times a day. Seizures are usually brief in duration in younger children, lasting 30 seconds to one minute but in older children the seizures may last many minutes.
Most babies make very little developmental progress or can even lose what little development they may have made before the epilepsy starts.
Doctors diagnose this syndrome by taking a very detailed account or ‘history’ of the seizures and especially at what age they start. It may also be very helpful for parents to video the seizures and show the video to the hospital doctor and particularly the paediatric neurologist.
The electroencephalogram (EEG) may be very helpful. It will usually confirm the diagnosis if the baby has at least 2 or 3 of their seizures during the EEG recording. This is because it will show that different focal seizures start in different parts of the brain. This is why it is called ‘migrating’ focal or partial epilepsy of infancy (as the seizures ‘migrate’ or move from one part of the brain to another). Sometimes the child will need to be admitted to the specialist epilepsy centre for a prolonged video-EEG telemetry recording to make sure the seizures can be ‘captured’.
Babies and infants with this type of epilepsy must always be referred to a paediatric neurologist for specialist treatment. It is very unlikely that any epilepsy medicines will ever fully control the seizures. Sometimes medicines will have an effect for a short time, but this will then wear off. Frequent changes to epilepsy medicines are made. Sometimes more uncommon epilepsy medicines are tried, including stiripentol or triple bromides; bromides are the oldest epilepsy medicines ever used. Other treatments such as the ketogenic diet, steroids (prednisolone) or the vagus nerve stimulator (VNS) may also be used.
Information about treatments for children can be found on the Medicines for Children website.
There may be research studies taking place that are relevant to this epilepsy syndrome. Your child’s hospital doctor will be able to discuss this with you.
If your child has this syndrome they will usually have prolonged or repeated seizures. Your child’s hospital doctor will discuss a ‘rescue’ or emergency care plan with you to treat any prolonged or repeated seizures.
The outlook is usually very poor. This is because the seizures never come under control for more than a few days or a week at a time and sometimes they are never controlled. Sadly, many children with this syndrome die in childhood due to the complications of the disorder, including repeated chest infections. Infants and children will be dependent for all their care and will have problems with feeding. Most children will need to have a gastrostomy feeding (PEG) tube inserted to ensure they can receive all the fluids and nutrition they need. Those who do survive will usually have severe neurological and developmental problems, even if the seizures are partly controlled.
Together for Short Lives
Freephone helpline: 0808 8088 100
What is a syndrome?
A syndrome is a group of signs and symptoms that, added together, suggest a particular medical condition. In epilepsy, examples of these signs and symptoms would be things like the age at which seizures begin, the type of seizures, whether the child is male or female and whether they have physical or learning disabilities, or both. The results of an EEG are also used to help identify epilepsy syndromes.
If you would like to know more about an epilepsy syndrome, please speak to your doctor. If you would like to know more about epilepsy in general, please contact Epilepsy Action.
Epilepsy Action would like to thank Dr Richard Appleton, consultant paediatric neurologist at Alder Hey Children’s Hospital, Liverpool, UK for preparing this information.