Panayiotopoulos syndrome

Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.

This is also known as:

• early-onset benign partial epilepsy with occipital paroxysms
• idiopathic susceptibility to early-onset benign childhood seizures with electroencephalographic (EEG) occipital spikes.

This is a recently described epilepsy syndrome. It is said to be 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 aged as young as one, and as old as 15 years of age.

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.

Early-onset benign partial epilepsy with occipital paroxysms (Panayiotopoulos syndrome) is different from the epilepsy syndrome called ‘benign epilepsy of childhood with occipital paroxysms (BECOP)’. In this other syndrome (BECOP), the children are usually older and the seizures consist of problems with vision and flashing lights and are usually accompanied by headaches.

Symptoms

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 may turn and become fixed to one side. It may remain turned to the side for many minutes. Very often the seizure will then end by jerking movements (called clonic movements) down one, or occasionally both, sides of the body. After the seizures 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, and after 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.

Diagnosis

The diagnosis of early-onset benign partial epilepsy with occipital paroxysms or Panayiotopoulos syndrome is made on a detailed account of the child’s symptoms.

Important features in making the diagnosis are: 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 electroencephalogram (EEG) often shows abnormal activity (called spikes) on both sides of the brain and 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.

Treatment

Not every child needs to be given anti-epileptic drugs. This is because the seizures occur infrequently and because most children will stop having seizures soon after the seizures start.

An anti-epileptic drug is usually prescribed if the child has had more than three or four seizures and particularly if the parents are worried.

The anti-epileptic drugs most commonly used are sodium valproate (Epilim) or carbamazepine (Tegretol); the newer drugs may also be effective, but this is not known for sure.

Prognosis (outlook)

Most, if not all children with early-onset benign partial epilepsy with occipital paroxysms or 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 started. This is called a ‘remission’ of the epilepsy. The remission of the epilepsy is spontaneous (it happens of its own accord) and is not affected by the use of anti-epileptic drugs.

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.

Support organisation

Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, www.cafamily.org.uk

27 April 2007