There is little information about this epilepsy syndrome. The International League Against Epilepsy includes self-limiting and pharmacoresponsive focal epilepsy in infancy within the syndromes called ‘self-limiting familial infantile seizures’ and ‘self-limiting infantile seizures (non-familial)’. No one really knows how common it is. Of every 100 children whose epilepsy starts at less than 2 years of age, between 5 and 10 will be found to have this epilepsy syndrome. It probably happens equally in boys and girls.
Previous names for self-limiting and pharmacoresponsive focal epilepsy in infancy
- Benign partial epilepsy in infancy
- Benign focal epilepsy in infancy
Seizures can start between 2 months and up to 18 months of age, but usually start between 4 and 9 months of age. The seizures are focal in type and often occur in clusters (groups), up to 5 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.
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 focal (affecting one part of the brain) rather than generalised (affecting both sides of the brain). Brain scans in children with this epilepsy syndrome are normal.
Self-limiting and pharmacoresponsive focal epilepsy in infancy 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 focal seizures to have a brain scan. The brain scan should always 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 self-limiting and pharmacoresponsive focal epilepsy in infancy do not need any other tests. A definite diagnosis of this epilepsy syndrome can often only be made when the child is between 3 and 5 years of age and when other epilepsy syndromes have been ruled out.
Other syndromes that may sometimes be confused with this syndrome include self-limited infantile convulsions, Dravet syndrome, epilepsy with migrating focal seizures in infancy, and Panayiotopoulos syndrome.
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 an 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 to 18 months after it has been started. The development and learning of children affected by this syndrome are usually thought to be normal, although this is not always the case. However, because not much is known about this syndrome it is difficult to be certain about the long-term outlook.
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 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 the Epilepsy Action Helpline.
Epilepsy Action would like to thank Dr Richard Appleton, consultant paediatric neurologist and honorary professor in paediatric neurology at Alder Hey Children’s Hospital, Liverpool, UK for preparing this information.