Generalised epilepsy with febrile seizure plus (GEFS+)

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.
Epilepsy Action has more information about seizure types, learning disabilities, and the EEG.
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.
Generalised epilepsy with febrile seizures plus
Generalised epilepsy with febrile seizures plus (GEFS+) is an unusual epilepsy syndrome. It describes families who have several members
from different generations with epileptic seizures. The epileptic seizures nearly always start after a family member has had febrile
convulsions.
Febrile convulsions are seizures associated with a high temperature. Usually febrile convulsions stop after the age of six years. In GEFS+
families, children may go on to have febrile seizures well beyond this age. They may also develop other seizure types not associated with a
high temperature. Very rarely, there may be a family member with a very severe type of epilepsy called Dravet syndrome. Dravet syndrome
is also called severe myoclonic epilepsy in infancy (SMEI).
GEFS+ has only recently been described as an epilepsy syndrome in its own right. Some people with GEFS+ are found to have a specific
fault in their genes. This may provide some clues to the genetic basis of some forms of epilepsy. It has caused great interest among epilepsy
researchers.
Epilepsy Action has information about Dravet syndrome.

Symptoms
The seizure types in this syndrome vary from person to person. They may include febrile seizures (febrile convulsions); febrile seizures plus;
tonic-clonic seizures; absence seizures; myoclonic seizures; atonic seizures (drop attacks); and focal (partial) seizures.
Febrile seizures (febrile convulsions)
These seizures always happen during a period of illness, such as a viral infection. They are associated with a high temperature. The seizures
are usually tonic-clonic seizures lasting less than five minutes. Sometimes the seizures can last longer and need emergency medical
treatment. The normal age range for this type of seizure is six months to six years. Although most children only have a small number of
seizures (fewer than five), some children have frequent febrile seizures throughout early childhood.
Febrile seizures plus
This is similar to febrile seizures, but the child has seizures beyond the normal age range. Again, the seizures are always associated with a
high temperature. The seizures usually stop by the time the child reaches the age of 10 or 12.
Some people within GEFS+ families may also develop other epilepsy syndromes, specifically myoclonic astatic epilepsy (Doose syndrome)
or Dravet syndrome.
The majority of people with GEFS+ have normal intelligence and learning abilities. However those with myoclonic astatic epilepsy, and
particularly Dravet syndrome, may have varying degrees of learning difficulties and behavioural problems.
Epilepsy Action has further information on myoclonic astatic epilepsy.

Diagnosis
GEFS+ is usually identified when the doctor takes a careful family history from someone who has febrile seizures outside the normal age
range. This may involve talking to immediate family members, particularly to grandparents, who may remember their children or nieces and
nephews having febrile convulsions or other seizure types as a child.
There have been a number of small genetic abnormalities found in some families with GEFS+. The abnormalities can be found in a simple
blood test. However these tests are not routinely available at the moment and are usually only requested as part of a research study.
Treatment
Treatment very much depends on the seizure type or types each person has. For febrile convulsions, regular treatment with epilepsy
medicines is not usually required. However, families may be provided with rescue medicine if their child has prolonged febrile convulsions.
People who have other seizures not associated with a high temperature may be prescribed epilepsy medicines such as sodium valproate
(Epilim), lamotrigine (Lamictal), ethosuximide (Zarontin) and clobazam (Frisium).
In the rare cases where children have more severe forms of epilepsy, such as myoclonic astatic epilepsy or Dravet syndrome, children may
need combinations of epilepsy medicines to treat seizures. A medicine called stiripentol (Diacomit) may be particularly effective in Dravet
syndrome, when used in combination with sodium valproate and clobazam.
Prognosis (outlook)
The outlook depends on the childs seizure types. Febrile convulsions usually stop after the age of six years. Some children may have febrile
seizures past the age of six years, though they usually become less frequent. Most stop before the child reaches puberty. With other children,
the outlook is more varied. The majority will have their seizures well controlled with epilepsy medicines, and their seizures may stop in late
childhood/early teenage years. However in some cases of myoclonic astatic epilepsy, and in most cases of Dravets syndrome, the seizures
are difficult to treat. They are usually lifelong (although becoming less frequent through adolescence) and are associated with learning and
behaviour difficulties.
https://www.epilepsy.org.uk/info/syndromes/gefs-generalised-epilepsy-with-febrile-seizure-plus