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 difficulties, or both. The results of an electroencephalogram (EEG) are also used to classify epilepsy syndromes.
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
This is a relatively common type of epilepsy affecting about one in seven or one in 10 of all children with epilepsy. The most common age it begins is three to five years, but it may affect children as young as one, and as old as 15 years. Boys and girls appear to be equally affected. It does not usually run in families. However, 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)’. In BECOP, the children are usually older and the seizures consist of problems with vision and flashing lights and are usually accompanied by headaches.
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) and there may be a lot of sweating and drooling (these are the ‘autonomic’ features). The child may become unresponsive and their head my turn and become fixed to one side. It may remain turned to the side for many 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 will occur 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 are very infrequent. Many children will have just one or two seizures while others may have four or more.
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 occur during sleep, and are nearly always accompanied by autonomic features, and vomiting. The child’s age is also important as most seizures occur in young children.
The EEG 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 (CT and MRI) are usually normal.
Epilepsy Action has more information on diagnosing epilepsy.
Not every child needs to be given epilepsy medicine. This is because the seizures happen infrequently and because most children will stop having seizures soon after the seizures start. An epilepsy medicine is usually prescribed if the child has had more than three or four seizures and particularly if the parents are worried. The medicines most commonly used are sodium valproate (Epilim) or carbamazepine (Tegretol). The newer epilepsy medicines may also be effective, but this is not known for sure.
Epilepsy Action has more information about treatment.
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 two or three years after the seizures first start. 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 type of epilepsy to have seizures either continuing into, or re-starting, in adult life.
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Epilepsy Action would like to thank
- Dr Richard Appleton, and Dr Rachel Kneen, consultant paediatric neurologists at Alder Hey Children’s Hospital, Liverpool, UK and
- Dr Stewart Macleod, consultant paediatric neurologist at Royal Hospital for Sick Children, Glasgow, UK.
They have kindly prepared the information on this page. It is based on their own research, experience and expertise.