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 they have physical or learning disabilities, or both. The results of an electroencephalogram (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.
Benign partial epilepsy in infancy
There is little information about this epilepsy syndrome. No one really knows how common it is. 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. It is important to rule out other causes of partial (focal) seizures in infants before making a diagnosis of benign partial epilepsy in infancy. This is usually called a 'diagnosis of exclusion'.
Seizures can start between two months and up to 18 months of age, but usually start 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 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.
Epilepsy Action has more information about focal seizures.
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 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 generally partial/focal rather than generalised. Brain scans are normal. This epilepsy syndrome is usually only diagnosed after other possible types of epilepsy or epilepsy syndromes have been ruled out. This particularly refers to syndromes where there may be an underlying abnormality of the brain (called symptomatic focal epilepsy). This is why it is important for most children under two years of age who have partial/focal seizures to have a brain scan. The brain scan should be a magnetic resonance imaging (MRI) rather than a computed tomography (CT) brain scan. 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. A definite diagnosis of benign partial epilepsy in infancy can often only be made when the child is three or even five years of age and when other epilepsy syndromes have been ruled out.
Other syndromes that may sometimes be confused with this syndrome include benign infantile convulsions, Dravet syndrome, migrating partial seizures (epilepsy) in infancy, and Panayiotopoulos syndrome. Epilepsy Action has more information about these syndromes.
If the child has clusters of seizures and the seizures happen frequently (every week or few weeks), then an epilepsy medicine may be prescribed. This could include carbamazepine (Tegretol), levetiracetam (Keppra), lamotrigine (Lamictal) or sodium valproate (Epilim).
There may be research studies open that are relevant to this epilepsy syndrome. Your child’s doctor will be able to discuss this with you.
Information about treatments for children can be found on the Medicines for Children website.
From the limited knowledge about this epilepsy syndrome, it is possible to say that the seizures are usually controlled in most children by epilepsy medicine. 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 epilepsy medicine it can be gradually withdrawn 12 or 18 months after it has been started. The development and learning of children affected by this syndrome are usually thought to be normal. However, because not much is known about this syndrome it is difficult to be very clear about the long-term outlook.
Epilepsy Action would like to thank Drs Richard Appleton and Rachel Kneen (at Alder Hey Children’s Hospital, Liverpool) and Stewart Macleod (at Yorkhill Children’s Hospital, Glasgow), consultants who specialise in children’s epilepsy, who have updated this fact sheet.