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Epilepsy Syndromes

Dr. with young boy
There are many epilepsy syndromes, and more are still being identified. A syndrome is literally a set of related symptoms that occur together. Epilepsy syndromes are defined on the basis of seizure type, seizure location (for partial seizures), EEG features, age of onset, related symptoms, and sometimes cause.

It is important for your child’s doctor to diagnose a particular epilepsy syndrome, if possible. Defining the epilepsy syndrome gives information about:

  • the prospects for seizure control and long-term outcome
  • the best medication to try first
  • any other problems that may arise as a result of the epilepsy or in combination with the epilepsy, including problems with learning and behaviour

In some cases, it is very easy to tell that a child has a particular epilepsy syndrome. In other cases, however, diagnosis is more complex. Some syndromes have features that are very similar to other syndromes. Some can be considered as points on a continuum; for instance, juvenile absence epilepsy has some overlap with juvenile myoclonic epilepsy, which in turn overlaps with generalized tonic-clonic seizures on awakening. Sometimes one syndrome can turn into another; for instance, some children with infantile spasms go on to develop Lennox-Gastaut syndrome. 

The brain develops and changes rapidly in childhood, especially during the first year of life. Different epilepsy syndromes are related to different stages of brain development; therefore, different syndromes appear at different ages.  It can be difficult, especially in very young children whose symptoms are not clear-cut or those with complex EEG features, to tell whether the child is showing symptoms of partial or generalized epilepsy and to define a particular syndrome.

This section discusses several different epilepsy syndromes, including:

  • Infantile spasms  (may be referred to as West Syndrome)
  • Focal epilepsy syndromes: benign epilepsy of childhood with centrotemporal spikes (BECTS), temporal lobe epilepsies, and Rasmussen syndrome
  • Generalized epilepsy syndromes: childhood and juvenile absence epilepsy and juvenile myoclonic epilepsy
  • Other epilepsy syndromes, including Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and reflex epilepsy

Some less common epilepsy syndromes are reviewed at the end of the section. For detailed information about each syndrome, click the links on the left.

Although they are fairly common, febrile seizures and most  neonatal seizures are not considered to be epilepsy syndromes.

They are discussed in the section on “Types of Seizures.”

Elizabeth J. Donner, MD, FRCPC