It is not known how common this syndrome is. It may affect between 1 in 10 or 1 in 20 of all children with epilepsy. The most common age it happens is 3 to 5 years, but it may affect children as young as 1, and as old as 10 years. Boys and girls appear to be equally affected. It does not usually run in families. More research needs to be undertaken in this epilepsy syndrome.
Panayiotopoulos syndrome is different to the epilepsy syndrome called ‘benign epilepsy of childhood with occipital paroxysms (BECOP)’. BECOP is sometimes called Gastaut syndrome. Gastaut syndrome is less common than Panayiotopoulos syndrome. In BECOP, or Gastaut syndrome, the children are usually older, between 6 and 13 years. The seizures include problems with vision and flashing lights and are usually accompanied by headaches. The seizures are briefer in duration than in Panayiotopoulos syndrome.
Other names for Panayiotopoulos syndrome
• Early-onset benign partial epilepsy with occipital paroxysms
• Idiopathic susceptibility to early-onset benign childhood seizures with EEG occipital spikes
The main seizure type is known as ‘autonomic’. The child may have a sudden change in behaviour and then they become pale, complain of feeling sick and will usually vomit. The pupils of the eyes often become very large (dilated), the face becomes flushed and there may be a lot of sweating and drooling. The eyes may fill with tears, even though the child is not crying.
All of these symptoms are the ‘autonomic’ features. The child may become unresponsive and their head may turn and become fixed to one side. It may remain turned to the side for many minutes, even up to 20 or 30 minutes. Very often the seizure will then end with jerking movements (called clonic movements) down one, or occasionally both, sides of the body. After the seizure the child will be upset and may sleep.
Over two-thirds of the seizures happen in sleep. The sleep may be during the night or during a day-time nap. The seizures are often long-lasting, and may last 20, 30 or even 60 minutes. Even following the long seizures, once the child has woken up, they will be back to normal. The seizures do not usually happen that often, only once or twice every few months. However, some children may have much more frequent seizures.
The diagnosis of Panayiotopoulos syndrome is made on a detailed account of the child’s symptoms. Important features in making the diagnosis are that the episodes usually happen during sleep, and are nearly always accompanied by autonomic features, and vomiting. The child’s age is also important as most seizures happen in young children.
The EEG (electroencephalogram) often shows abnormal activity (called spikes) on both sides of the brain. This is usually towards the back of the head (in the part of the brain called the occipital lobes). Sometimes the EEG may only show the abnormality if it is carried out when the child is sleeping. However, the EEG may also be normal in up to a quarter of children with this epilepsy syndrome.
Brain scans such as CT (computerised tomography) and MRI (magnetic resonance imaging) are usually normal. Rarely, some children with Panayiotopoulos syndrome have been reported to have had abnormal brain scans. This is why more research is needed in this particular epilepsy syndrome.
Not every child needs to be given epilepsy medicine. This is because the seizures happen infrequently and because most children will stop having seizures within a few months after the seizures start. Epilepsy medicine is usually prescribed if the child has had more than 3 or 4 seizures and particularly if the parents are worried. The medicines most commonly used are sodium valproate (Epilim) or carbamazepine (Tegretol). Lamotrigine (Lamictal) or the newer medicines such as levetiracetam (Keppra), may also be effective, but this is not known for sure.
Information about treatments for children can be found on the Medicines for Children website.
There may be research studies going on in this epilepsy syndrome. Your child’s hospital doctor will be able to discuss this with you.
It will be very unlikely that a child with this epilepsy syndrome will have prolonged or repeated seizures. However, if this does happen, your child’s hospital doctor (paediatrician or paediatric neurologist) will discuss a ‘rescue’ or emergency care plan with you to treat any prolonged or repeated seizures.
Most, if not all children with Panayiotopoulos syndrome, will show normal development in the first few years of life. Most will also have no learning or behaviour problems. Even the longer seizures do not cause any problems, and it is not thought that the seizures are dangerous.
Nearly all children will stop having seizures within 2 or 3 years after the seizures first start, sometimes earlier. This is called a ‘remission’ of the epilepsy. The remission of the epilepsy happens of its own accord and is not affected by the use of epilepsy medicines. Finally, it seems to be very rare for a child with this epilepsy syndrome to have seizures either continuing into, or re-starting, in adult life.
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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 the child has 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.