Answers to questions families often have about caring for their child with 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 5, but may be replaced by other seizure types. 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. Infantile Spasms (NINDS)

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.

Spasms are usually diagnosed by evaluating the clinical circumstances, the presence of developmental delay or regression, and an abnormal EEG—one that shows a particular pattern called "hypsarrhthymia." Additional testing may be performed to look for the underlying cause of the infantile spasms.

The prognosis for children with IS is dependent on the underlying causes of the seizures. The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms. Spasms usually resolve by mid-childhood, but more than half of the children with IS will develop other types of seizures. IS will sometimes gradually turn into Lennox-Gastaut syndrome, an epileptic disorder of later childhood.

The risk to your family and future babies will depend on the underlying cause for the spasms. Some spasms may have a genetic component and may be possible or likely in future pregnancies. Other spasms may be caused by brain damage; e.g., prenatal stroke.

Treatment with corticosteroids such as prednisone is standard, although serious side effects can occur. Several newer antiepileptic medications, such as topiramate, may ease some symptoms. Some children have spasms as the result of brain lesions and surgical removal of these lesions may result in improvement.

The choice of one therapy over another should be made only after a full discussion of benefits and risks with your child's physician. Although both of these treatments have potentially dangerous side effects, the consequences of untreated infantile spasms are often devastating. You might consider joining a support group of parents who have made a similar choice to get a parent perspective. See Epilepsy Foundation Utah Chapter.