Black man holds a Black baby in a nursery with clouds on the wall.

A Typical Story

Noah started having spells when he was 5 months old. He would “crunch” both arms and legs into his body and his head would pull forward. Each spell lasted only 2 seconds but these would occur in clusters lasting several minutes. He often became irritable during the cluster and would cry.

Within days of their onset, Noah became less visually alert. 

Infantile Spasms Are An Emergency, by Brain Recovery Project

What is an epileptic spasm?

  • An epileptic spasm consists of brief (1-3 second) events of arm, leg and head flexion (arms and legs pull into the body) or extension. Spasms typically occur in clusters with events every 5-10 seconds over a 5-10 minute period.
  • Spasms often are seen shortly after waking, and clusters typically are seen several times per day.
  • Infants often become irritable and may cry during the cluster – sometimes this leads to a misdiagnosis of colic.

Where does an epileptic spasms start in the brain?

  • An epileptic spasm may occur due to an abnormality in a small portion of the brain, or may reflect a more generalized brain problem. 
  • Spasms that are due to a small abnormality often may affect one side of the body more than the other or may have pulling of the head or eyes to one side.

Who is at risk for epileptic spasms?

  • Spasms most commonly occur in infancy. The most common epilepsy type is West syndrome (also known as infantile spasms syndrome).
  • They can also be seen in other early-onset epilepsies such as Ohtahara syndrome. Less frequently they are seen in persons with Lennox-Gastaut syndrome.
  • In most cases, spasms ultimately will go away by the early preschool years, but often other seizure types can emerge.
  • Persons with epileptic spasms have higher rates of developing drug-resistant epilepsy and many also will have significant developmental concerns long-term.
  • The earlier spasms are recognized and appropriately treated, the better the developmental outcome.

How can I tell if someone is having epileptic spasms?

  • If you think that someone may be having epileptic spasms, a video recording of the events can be invaluable in making an early diagnosis. One or more videos can be easily obtained on your cellular phone.

What happens after a cluster of spasms?

  • Epileptic spasms occur in clusters and typically do not require any rescue medication during the actual event.

How are epileptic spasms diagnosed?

  • If you think that someone is having epileptic or infantile spasms, it can be very helpful to capture a video of these on your cellular phone to share with her healthcare provider.
  • It is essential to recognize and treat spasms as quickly as possible as delayed treatment can correlate with poorer developmental outcome.  
  • Many children with epileptic or infantile spasms will have a characteristic pattern on their EEG called hypsarrhythmia.
  • In other cases, this pattern may be absent but the EEG still abnormal. In such cases it is very helpful to admit the person for video EEG monitoring to confirm that the events are epileptic or infantile spasms.
  • Once spasms have been diagnosed, patients will generally undergo further testing to determine the cause. This often will involve an MRI, blood work, and possibly genetic studies.

How are epileptic or infantile spasms treated?

  • Infantile spasms should be rapidly identified and treated with specific medications that target spasms. 
  • In most cases, the usual antiseizure medications are not effective. Specific medications that target spasms and should be used as first-line treatment include high-dose oral steroids, ACTH or vigabatrin.
  • If spasms do not come under control with a trial of steroids and vigabatrin, it is important to be seen at a specialized Epilepsy Center to evaluate the underlying cause and see whether epilepsy surgery might be an option.

Learn More

 

Authored By: 
Elaine Wirrell MD
Authored Date: 
10/2020