About Infantile Spasms (IS)  
 

What is Infantile Spasms (IS)?

Infantile spasms is an uncommon epilepsy syndrome that typically begins in infancy. Also known as West syndrome, it was first characterized by Dr. William James West in the mid-19th century as he sought help for his own affected child. Infantile spasms is considered to be a “catastrophic childhood epilepsy” due to the difficulty in controlling its symptoms and the developmental problems that can occur as a result of IS.

 
“Infantile spasms is classified as a catastrophic childhood seizure disorder, and it’s not catastrophic because of the way the spasms look. If a child is having infantile spasms, it doesn’t look like such a big thing...just little jerks.Dr Don Shields, MD Infantile Spasms It does not look frightening, yet the impact could not be greater. That child with infantile spasms is now threatened with a very poor developmental outcome. Unfortunately, the majority of children are going to eventually be found to be cognitively impaired, and most will continue to have other seizure disorders later on.”
Don Shields, M.D.
Professor Emeritus of Neurology
and Pediatrics
David Geffen School of Medicine
at UCLA

Infantile Spasms typically begins in the first 4 to 8 months of life and is characterized by flexion (bending and jerking) of the trunk (torso) or neck and extremities (arms and legs). An episode can range from a subtle head jerk to a flexion that lasts for a few seconds. Most often, the spasms occur in clusters. Click here to see what infantile spasms look like.


Infantile having infantile spasms video

Infantile spasms are characterized by hypsarrhythmia (hips-A-’rith-mE-uh), a highly disorganized and chaotic pattern of electroencephalogram (EEG) abnormality. The presence of hypsarrhythmia, which does not typically occur with other forms of epilepsy, can help to confirm a diagnosis of IS. Furthermore, there is a strong correlation between hypsarrhythmia and the cognitive impairment (damage) and developmental delays that are often associated with infantile spasms. That’s why an EEG is so important; it helps determine what the underlying disorder is that needs to be treated.