Self Limited Late Onset Occipital Epilepsy Gastaut Syndrome

This syndrome is also known as:

  • Late-onset, childhood epilepsy with occipital paroxysms
  • Late onset benign occipital epilepsy of Gastaut
  • Self-limited late onset childhood occipital epilepsy of Gastaut

The EEG in self-limited, late-onset occipital epilepsy may be similar to Panayiatopoulos syndrome which is seen earlier in life. The EEG changes are found in the occipital lobes (back part of the brain) where the seizures typically begin. This area of the brain helps process visual information.

Seizures in this epilepsy initially involve simple visual symptoms.

  • A person may see colors or circular dots.
  • Loss of vision can occur.
  • Children can also have nystagmus (rapid back-and-forth movements of the eyes) and deviation or turning of the eyes in one direction.
  • As the seizure progresses, more of the visual field can become involved.
  • These seizures usually remain focal in the occipital lobes. Sometimes they may spread to both sides of the brain and a generalized tonic clonic seizure may happen.
  • Seizures typically last 1-3 minutes.
  • Children can have headaches after their seizures.

This is a rare type of epilepsy.

  • It accounts for less than 1% of epilepsy.
  • This epilepsy most commonly affects older school aged children, usually beginning around 8 years old.
  • A family history of epilepsy is seen in approximately 1 out of 3 families.

The diagnosis of late onset occipital epilepsy is based on the description of the seizures. Other tests needed include:

  • EEG (electroencephalogram): The EEG shows spikes in the occipital regions of the brain. Spikes will often increase or happen more often when children close their eyes.
  • MRI (magnetic resonance imaging): The MRI is typically normal, but is important to exclude other causes of epilepsy that can start in the occipital lobe.

Children typically require anti-seizure medication. Usually seizures can be controlled with one medication. Medications such as levetiracetam, oxcarbazepine, carbamazepine, and valproic acid have been used to good effect.

Most children stop having seizures 2 to 5 years after the epilepsy begins. Medicines can be withdrawn after discussion with the treating physician if seizure freedom persists. Some children do need lifelong anti-seizure medication therapy.

Authored By:

Michael Ciliberto MD

on Wednesday, July 29, 2020

Reviewed By:

Elaine Wirrell MD

on Wednesday, July 29, 2020

Resources

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