Health & Medical Neurological Conditions

9 Generalized Epilepsy Syndromes of Childhood

Updated May 16, 2014.

Written or reviewed by a board-certified physician. See About.com's Medical Review Board.

A seizure is caused by a combination of three things: insufficient inhibition of abnormal electrical activity, too much electrical excitation of the brain, and an abnormal part of the brain where the seizure can begin. The result is abnormal electrical activity that can often be detected on an electroencephalogram (EEG).

While a seizure may develop due to something like a stroke or trauma, some people are essentially born with an abnormal predisposition towards seizures.

In this case, seizures may develop during childhood. In children, these seizures sometimes first appear during infancy. In other cases, seizures first appear when the child is a little older (over the age of two).

There are two main divisions in seizures: "generalized" and "partial." A generalized seizure appears to affect every part of the body and brain all at once. A partial syndrome only impacts part of the body, and may or may not spread to involve the rest, in which case the partial seizure is said to have generalized.

Epilepsy syndromes are sometimes but not always associated with changes in development or learning ability. To learn more about those severe forms of childhood epilepsy,read here. Here we'll review the generalized epilepsy syndromes of childhood, as opposed to epileptic syndromes of infancy.

Generalized Epilepsy Syndromes

Generalized seizures probably start deep in the brain where they cannot be detected by the electrodes of an electroencephalogram. By the time the abnormal electrical activity reaches the surface (cortex) of the brain, it appears as if the entire brain starts seizing at once, in what is called a "grand mal" or "generalized tonic-clonic seizure."

  • Myoclonic Astatic Epilepsy

    Myoclonic astatic epilepsy, also known as Doose syndrome, usually occurs between the age of 2 to 6 years, though it may occur when younger as well. In addition to myoclonus, children with Doose syndrome have a variety of other generalized seizures as well. When the syndrome begins, the EEG may appear normal, though patterns become abnormal later in the disease course. A variety of antiepileptic drugs can help control the seizures, and the outcome is generally good, though just over 40 percent of children with Doose syndrome may have a borderline IQ or mental handicap.
  • Generalized Epilepsy with Febrile Seizures Plus (GEFS+)

    Children with GEFS+ usually first have a seizure precipitated by a fever. Most of the time a febrile seizure is relatively benign, but in this case such seizures persist beyond the age of 6, and other seizures develop as well. The disorder is inherited in an autosomal dominant fashion, and is best treated with valproate. Most children do well, but up to 30 percent may develop a more severe form of epilepsy.
  • Childhood Absence Epilepsy

    Childhood absence epilepsy is a relatively common form of childhood epilepsy. It's not clear how the disease develops, but it's possible there are some genetics involved. Children with CAE have multiple small seizures every day, during which they may appear to just stare off into space for a small period. They have no memory what happens during those times. The EEG pattern of childhood absence epilepsy is well-known even to basic medical students, with brief periods of spikes appearing at a frequency of 3 Hertz. Children with CAE usually respond well to the drug ethosuximide, and their symptoms resolve a few years after the first seizure appears.
  • Eyelid Myoclonia With and Without Absences

    These are actually two disorders associated with rapid eyelid twitching (eyelid myoclonia) and upward deviation of the eyes. In eyelid myoclonia with absences, consciousness is lost for a brief period. In eyelid myoclonia without absences, children remember everything. Generalized seizures with loss of consciousness may also develop. Even if these symptoms improve, the eyelid myoclonia will likely persist for a lifetime.
  • Perioral Myoclonia with Absences

    This seizure syndrome develops between the ages of 2 to 13. The mouth twitches during brief periods of lost consciousness. Generalized tonic-clonic seizures, where the entire body stiffens and spasms, also develop. Unfortunately, this form of epilepsy is often resistant to medication.
  • Epilepsy with Myoclonic Absences

    The average age of developing this syndrome is seven years. In additions to myoclonic absence seizures, in which the child may stare off and have fast muscle twitches, children may have generalized tonic-clonic seizures as well. Unlike CAE, in this form of epilepsy seizures only resolve in about one-third of children, though cognitive development between seizures is normal.
  • Juvenile Myoclonic Epilepsy

    Juvenile myoclonic epilepsy (JME) is a very well-studied epilepsy syndrome. The syndrome affects between 4 to 10 percent of all patients with epilepsy. The first seizure usually occurs in late childhood. Seizures include absences (brief spells of lost consciousness in which the patient just seems to stare off), and generalized tonic-clonic seizures as well. Seizures tend to be worst in the morning just after awakening. The EEG has a characteristic pattern that can be helpful in making the diagnosis. Additional tests during the EEG, such as a strobe light, may elicit the abnormal pattern. The seizures usually respond well to appropriate medical therapy.
  • Juvenile Absence Epilepsy

    in many ways, juvenile absence epilepsy is identical to childhood epilepsy. The main difference is the age at which children have their first seizures. Fewer absence seizures are experienced each day, but unlike CAE, lifelong treatment is frequently required to control seizures.
  • Epilepsy with Grand Mal on Awakening

    As the name suggests, seizures in this syndrome typically are associated with waking up The first seizure can develop any time between the age of 6 to 35. It's thought to be genetic, and the response to antiepileptic medication is good. Relapses occur with sleep deprivation or cessation of medications.

Childhood epilepsies can be frightening, but much of the time seizures can be controlled and a child can have a normal life. It is important for parents to consider what bothers their child most about epilepsy, and ensure that those concerns are addressed.

Sources:

Elaine Wirrell, Katherine C. Nickels "Pediatric Epilepsy Syndromes". Continuum: Epilepsy, Volume 16, Number 3, June 2010.

Gerald M Finichel. Clinical Pediatric Neurology. 6th Edition. Sanders-Elsevier, 2009.
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