About Infantile Spasms
What are Infantile Spasms?
An infantile spasm (IS) is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood known as West Syndrome. West Syndrome is characterized by infantile spasms, developmental regression, and a specific pattern on electroencephalography (EEG) testing called hypsarrhythmia (chaotic brain waves). The onset of infantile spasms is usually in the first year of life, typically between 4-8 months. Infantile spasms usually stop by age five, but may be replaced by other seizure types.
Symptoms and Frequency
The seizures primarily consist of a sudden bending forward of the body with stiffening of the arms and legs; some children arch their backs as they extend their arms and legs. Spasms tend to occur upon awakening or after feeding, and often occur in clusters of up to 100 spasms at a time. Infants may have dozens of clusters and several hundred spasms per day. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to spasms, making it important to identify the underlying cause. In some children, no cause can be found.
How is it Usually Treated?
The primary approved treatments for children with infantile spasms include:
- Steroid Therapy (adrenocorticotropic hormone [ACTH by injection] or prednisone)
- A seizure medication called Sabril (vigabatrin)
Some pediatric seizure doctors prefer therapy with Sabril (vigabatrin) or other seizure medications that may appear helpful for IS, for example Depakote (valproate) or Topamax (topimate) before steroid therapy.
What is a hemispherectomy?
Hemispherectomy is a very rare surgical procedure where one cerebral hemisphere (half of the brain) is removed or disabled.
When is a hemispherectomy done to treat seizures?
A hemispherectomy is performed in children who have severe and intractable seizure disorders. Many of these children do not respond to seizure medications and/or the Ketogenic Diet. They often have severe damage to only one side of the brain (although not always), and may already have paralysis on one side of the body (hemiparesis). Hemispherectomy is typically performed on children with Rasmussen’s syndrome and on children who were born with or who have had strokes in early childhood who have seizures that are difficult to control and, on other children who experience the early onset of uncontrollable seizures that are limited to one side of the brain. However, hemispherectomy is not necessarily appropriate for everyone with intractable seizure disorders.
You can find more information on Infantile Spasms and treatments on the links below:
www.ninds.nih.gov/disorders/infantilespasms/infantilespasms.htm