Epilepsy with eyelid myoclonia

Other names for epilepsy with eyelid myoclonia (EEM)

• Jeavons syndrome
• Eyelid myoclonia with absences (EMA)
• Sunflower syndrome or EEM with prominent photic induction

Who gets EEM?

Epilepsy with eyelid myoclonia (EEM) most often starts in children aged between 6 and 8 years. But it can start any time between the ages of 2 and 14 years. It’s more common in girls than boys. It’s not linked to any other health problems or problems at birth.

Less than half of children with EEM have a family history of epilepsy.

Symptoms

If your child has EEM, they may have multiple seizures every day. The seizures usually only last for 1 to 3 seconds. Children with EEM are photosensitive. This means that the seizures are triggered by flickering light. They can also come on when your child closes their eyes.

Stress and lack of sleep may also be a trigger for seizures.

During a seizure, your child may have the following symptoms:

• Their eyelids may jerk rhythmically and repeatedly (eyelid myoclonia)
• Their eyeballs may roll upwards, so you can see the white part of their eyes
• Their head may jerk backwards

Some children may have absence seizures at the same time as eyelid myoclonia. This means they also lose some awareness and may not respond.

Children with EEM may also get tonic-clonic seizures. A child having this type of seizure may fall unconscious and make jerking movements with their arms and legs. This type of seizure doesn’t happen often, and it’s usually over within 2 to 3 minutes. They’re more likely when your child is tired, or they’re exposed to bright lights.

Some children have a particular type of EEM, where they actively trigger seizures themselves. They do this by turning towards a light and waving their hand in front of their eyes, or using other methods to create a flickering effect. This used to be called Sunflower syndrome, but is now called EEM with prominent photic induction.

Diagnosis

Your child should see a specialist doctor called a paediatrician for an assessment. The paediatrician will examine your child and take a record of exactly what happens during their seizures. If you’re able to take a video recording of the seizures, this can be very helpful. This may be difficult though, because the seizures only last a few seconds.

Your child’s paediatrician will suggest some tests to confirm a diagnosis. This may include an EEG (electroencephalogram) to look at electrical activity in the brain. This test can be particularly helpful if your child has a seizure while the EEG is being done. The EEG should show if your child has photosensitivity.

The paediatrician may also refer your child to a doctor who specialises in epilepsy in children, called a paediatric neurologist. This is because this epilepsy syndrome is rare and the seizures may be difficult to control.

Sometimes the paediatrician or paediatric neurologist may suggest genetic testing. If your child has a family history of epilepsy, has learning disabilities or their seizures are not able to be controlled by medication this is more likely.

Other tests, such as brain scans, aren’t usually needed to diagnose EEM. But your doctor may suggest them if they need to rule out other conditions or other epilepsy syndromes. Because EEM can look similar to other syndromes, sometimes it can be mistaken for more common epilepsy syndromes such as childhood absence epilepsy or juvenile myoclonic epilepsy.

Treatment

Your child’s doctor may prescribe epilepsy medicines to help control your child’s seizures. These may include:

• Sodium valproate
• Lamotrigine
• Ethosuximide
• Levetiracetam
• Clobazam

Epilepsy medicines can often help to stop tonic-clonic seizures. But the eyelid myoclonia symptoms can be more difficult to get under control with medicines.

Sodium valproate can harm an unborn baby if taken during pregnancy. For this reason, if your child is able to get pregnant, or may do when they’re older, your child’s paediatrician will usually suggest an alternative medicine. If they do recommend treatment with sodium valproate, they will discuss the risks and benefits with you and your child first.

Some people will need a combination of two epilepsy medicines taken together to control the seizures. The most common combinations are sodium valproate with ethosuximide or sodium valproate with clobazam.

Information about treatments for children can be found on the Medicines for Children website.

If your child has EEM with prominent photic induction, and is actively triggering seizures, their paediatrician or paediatric neurologist may suggest ways to manage their behaviour. This can be difficult, particularly as many children also have learning disabilities. Other things you can do to help include using:

• Wide-brimmed hats
• Wraparound sunglasses to reduce exposure to the light
• Glasses with special dark blue lenses (called Z1 lenses)

Outlook

EEM can be difficult to treat and the outlook is very varied. It’s usually something that you have for life. Over a quarter of children will have drug resistant epilepsy – this means their seizures cannot be controlled by one or a combination of two medicines taken together. This is more likely if the seizures began at a very young age. But over a quarter will be able to completely control their seizures using medicine. As your child gets older, their seizures may become less frequent. Their sensitivity to light usually reduces too, but rarely goes away completely.

Children and young people are more likely to become seizure free if:

• They began having seizures when they were an older child
• If they have not had tonic clonic seizures
• If they never had febrile seizures
• If they do not have any psychiatric conditions

Children with EEM may sometimes have learning difficulties, although this isn’t always the case. It’s more likely in those children with prominent photic induction. With this form of EEM, around half of children have learning disabilities or problems with attention. If your child’s seizures started when they were very young, this makes it more likely that they will have a learning disability or a psychiatric condition.

Your child’s paediatrician or paediatric neurologist will be able advise you on getting support in these areas if needed. We also have more information on how epilepsy can affect learning and behaviour.

Support

Contact – for families with disabled children

Freephone helpline: 0808 808 3555
Email: helpline@contact.org.uk