Self-limiting late-onset occipital epilepsy is a rare epilepsy syndrome. It can start at any age, from 18 months to 17 years, but it usually begins in middle childhood between 7 and 11 years of age. This syndrome is different to early-onset occipital epilepsy, also known as Panayiotopoulos syndrome.
About one third of children with this syndrome may have a family history of epilepsy. Some children have had seizures with illnesses with high temperatures (febrile convulsions or febrile seizures) before starting to have self-limiting late-onset occipital epilepsy. Most children with this condition have a normal neurological examination and no learning difficulties. The results of magnetic resonance imaging (MRI) brain scans on children with this syndrome are normal.
Other names for self-limiting late-onset occipital epilepsy
- Benign epilepsy of childhood with occipital paroxysms (BECOP)
- Late-onset occipital epilepsy
- Gastaut-type childhood occipital epilepsy
- Gastaut syndrome
The seizures usually affect vision. Complete or partial visual loss during seizures happens in about half the children who have this type of epilepsy. Many may experience a sensation of flashing lights, often multi-coloured spots or other shapes. The flashing lights usually last only seconds to at most a few minutes. Visual hallucinations (seeing things) are rare, but happen in some children. About half have jerking of one side of the body. Occasionally children may have semi-purposeful movements and strange behaviour. About half of the children with this syndrome have jerking of one side of the body; these are often called 'hemiconvulsions'. The jerking down one side of the body may spread to involve the other side and cause a generalised tonic-clonic seizure. Headaches, during or after the seizure, are a very common feature of this epilepsy syndrome.
This epilepsy syndrome may sometimes be misdiagnosed as migraine. In migraine, the flashing lights are different. They are very sharp and angular or zig-zagged-shaped lines. They are also very bright and coloured white, silver or gold. In migraine he flashing lights also last much longer, up to 15 or 30 minutes, or occasionally longer.
In the electroencephalogram (EEG), which records the electrical activity in the brain, abnormalities are seen in the occipital region (the very back part of the brain) in one or both sides of the brain. Some children may have seizures as they move between dark and brightly-lit areas. Because this syndrome is rare, most children who are thought to have it will need an MRI brain scan before the syndrome can be confidently diagnosed. This is to make sure there is no abnormality in the occipital lobes. However, the MRI scan will usually be normal.
The seizures respond readily to epilepsy medicines such as sodium valproate (Epilim), or carbamazepine (Tegretol). There is no information as to whether the newer epilepsy medicines such as lamotrigine (Lamictal), levetiracetam (Keppra) or topiramate (Topamax) are more or less effective than sodium valproate or carbamazepine in the treatment of this syndrome.
There may be research studies taking place that are relevant to this epilepsy syndrome. Your child’s hospital doctor will be able to discuss this with you.
Information about treatments for children can be found on the Medicines for Children website.
The seizures usually get better by age 15-16 years and any epilepsy medicines can be gradually withdrawn. This is why the syndrome is called self-limiting. However, seizures may continue into adulthood in about 5 to 10 out of 100 young people with this syndrome and they will need to stay on an epilepsy medicine.
Most children and young people have normal educational progress. A few may have some mild learning difficulties.
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 are: 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 important in the diagnosis of 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.
Epilepsy Action would like to thank Dr Richard Appleton, consultant paediatric neurologist at Alder Hey Children’s Hospital, Liverpool, UK for preparing this information.