What Is an Epilepsy Syndrome?
An epilepsy syndrome is a type of epilepsy that is identified by a specific seizure type or types and by the findings on an EEG. Not everyone with epilepsy will have an epilepsy syndrome. They can be defined in over half of epilepsy starting before 2 years of age but are much less common in epilepsy starting in adulthood.
Identification of a specific epilepsy syndrome can give information about the best treatment, the likelihood of seizure control and remission, and the possibility of other conditions like intellectual disability. In some cases, discovery of a specific epilepsy syndrome can provide clues of what may be the cause.
In many cases, the syndromes begin at specific ages. Some start in infancy (before 2 years of age), others in childhood (between 2-17 years) but some can start in either childhood or adulthood.
Epilepsy syndromes are further divided by the type of seizures. Seizures can be generalized, focal, or both generalized and focal in onset, or where they start in the brain.
Some epilepsy syndromes have a very high likelihood of being outgrown in childhood. This means that the seizures will go away, and medication will not need to be taken any more. Other syndromes are characterized by developmental and epileptic encephalopathy (DEE). This means that the seizures are usually more frequent and severe, and less likely to respond to anti-seizure medication.
Learn More:What Is DEE?
Do Treatment Options Vary from One Syndrome to Another?
Treatment options for different epilepsy syndromes may vary based on many factors. This is why it is vital to see an epilepsy specialist or visit an epilepsy center.
Treatment options for difficult to treat epilepsy may include:
If you or your loved one is having a hard time controlling seizures, work closely with your healthcare team. They will help put you on the right path to the treatment plan that works best for you.
Learn More:Find an Epilepsy Specialist
Epilepsy Syndromes by Age
Below is a list of epilepsy syndromes grouped by the specific age they begin. However, diagnosis may take place at a later age.
Beginning in Infancy (prior to 2 years of age)
Self-limited epilepsy syndromes:
- Self-limited epilepsies in infancy
- Myoclonic epilepsy in infancy
- Genetic epilepsy with febrile seizures plus
Syndromes with DEE:
- Early infantile DEE (previously called Ohtahara syndrome, early myoclonic encephalopathy)
- Epilepsy of infancy with migrating focal seizures
- Infantile epileptic spasms syndrome (previously called Infantile Spasms or West syndrome)
- Dravet syndrome (previously called severe myoclonic epilepsy in infancy)
Beginning in Childhood (2-17 years of age)
Self-limited focal epilepsy syndromes:
- Self-limited epilepsies in childhood
- Childhood occipital visual epilepsy (previously called Gastaut syndrome or late-onset benign occipital epilepsy)
- Photosensitive occipital lobe epilepsy
Generalized epilepsy syndromes:
- Childhood absence epilepsy
- Epilepsy with myoclonic absences
- Epilepsy with eyelid myoclonia (previously called Jeavons syndrome)
Childhood-onset epilepsy syndromes with DEE:
- Epilepsy with myoclonic-atonic seizures (previously called myoclonic atonic epilepsy or Doose syndrome)
- Lennox-Gastaut syndrome
- Developmental/epileptic encephalopathy with spike wave activation in sleep (previously called continuous spike-wave in sleep, electrical status epilepticus in sleep or Landau-Kleffner syndrome)
- Febrile infection-related epilepsy syndrome (FIRES)
Beginning at a Variable Age (can begin in either child or adult ages)
Focal epilepsy syndromes:
- Mesial temporal lobe epilepsy with hippocampal sclerosis
- Familial temporal lobe epilepsy
- Sleep-related hypermotor epilepsy
- Familial focal epilepsy with variable foci
- Epilepsy with auditory features
Focal and generalized epilepsy syndromes:
- Epilepsy with reading induced seizures
Generalized epilepsy syndromes:
- Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
- Epilepsy with generalized tonic-clonic seizures alone
Epilepsy syndromes with DEE or with progressive neurological deterioration:
- Rasmussen syndrome
- Progressive myoclonus epilepsies
Learn More:Forms & Resources
Epilepsy centers provide you with a team of specialists to help you diagnose your epilepsy and explore treatment options.
Find in-depth information on anti-seizure medications so you know what to ask your doctor.
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Call our Epilepsy and Seizures 24/7 Helpline and talk with an epilepsy information specialist or submit a question online.
Tools & Resources
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