Benign partial epilepsy in infancy

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.

There is limited knowledge about this epilepsy syndrome. However, it appears to be a quite common syndrome, particularly in young children.

Of every 100 children whose epilepsy starts at less than two years of age, between five and 10 will be found to have this epilepsy syndrome. It probably happens equally in boys and girls.

The important thing is to diagnose it from other conditions first before making a definite diagnosis of benign partial epilepsy in infancy. This means that it is sometimes called a 'diagnosis of exclusion'.

Symptoms

The seizures can start between two months and 18 months of age but usually start at between four and nine months of age.

The seizures are complex partial/focal and often occur in clusters (groups), up to five or even 10 seizures in each cluster. In the seizure, the child will stop what they are doing, their eyes stare straight ahead or to one side and their head may also turn to one side. Occasionally, there may also be twitching of one side of the face.

This seizure may then be followed by a secondarily generalised tonic-clonic seizure. The child's development is usually normal and they do not usually have any behavioural problems. This epilepsy syndrome may also run in families.

Diagnosis

A detailed history or description must be taken from someone who has actually seen the seizures. A video recording might be helpful, if it is difficult to describe the seizures.

The electroencephalogram (EEG) can be normal, but if the child has a seizure while the EEG is actually being done, it will always show abnormalities. These abnormalities are usually partial/focal rather than generalised. Brain scans are normal.

This epilepsy syndrome is usually only diagnosed after other possible epilepsy syndromes have been excluded - and particularly syndromes where there may be an underlying abnormality of the brain. This is why it is usually helpful or important for most children under two years of age who start having partial /focal seizures to have a brain scan.

A magnetic resonance imaging (MRI) brain scan can show more details than a computed tomography (CT) brain scan. The MRI scan should therefore be the scan of choice.

Sometimes a blood test will need to be done to make sure that the child does not have a low level of glucose or calcium that might have caused the seizures.

Children with benign partial epilepsy in infancy do not need any other tests. It is probably true to say that a firm diagnosis of benign partial epilepsy in infancy can only be made when the child is three, or even four or five years old.

Other syndromes that may sometimes be confused with this syndrome include benign familial neonatal infantile convulsions, migrating partial seizures (epilepsy) in infancy and Panayiotopoulos syndrome (also called 'early-onset benign childhood seizure susceptibility syndrome' or 'early onset benign occipital epilepsy').

Treatment

If the child has clusters of seizures and the seizures happen frequently (every few weeks), then an anti-epileptic drug (AED) may be prescribed. These could include carbamazepine (Tegretol), lamotrigine (Lamictal) or sodium valproate(Epilim). The newer AED, levetiracetam (Keppra) might also be helpful.

Prognosis (outlook)

From the limited knowledge about this epilepsy syndrome, it is usually controlled in most children by an anti-epileptic drug.

It is also thought that, in most children, the seizures stop happening after a few months or at most a year after they have started (this is called 'spontaneous remission'). This usually means that if the child is taking an AED it can be gradually withdrawn 12 or 18 months after it has been started.

Support Organisation

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

11 July 2007