Infantile Spasms Program

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The UCLA Infantile Spasms Program offers comprehensive services for children with infantile spasms, including rigorous diagnostics, on-site genetic testing, an array of medications, surgery, dietary therapies, neurostimulation devices, alternative approaches, and neurodevelopmental assessment. We focus on expedited access to expert physicians and rapid protocol-driven and evidence-based approaches to treatment. UCLA Mattel Children's Hospital is a major referral center for infantile spasms care and is active in research, both at UCLA and on a multi-center basis with partners across the US and abroad. Research endeavors include multiple ongoing clinical trials, and numerous studies exploring the nature of infantile spasms, including the potential use of quantitative EEG, functional neuroimaging, whole exome genetic sequencing, and broad efforts to identify factors that predict the best treatment for individual patients.

The Basics

Infantile spasms, also called West Syndrome, is a type of epilepsy that tends to affect infants under the age of 2 years, and is characterized by clusters of peculiar seizures. These seizures-termed spasms are involuntary and usually consist of a brief interruption of behavior, often with lifting and extension of the arms and bending forward at the waist. In many cases, this behavior is accompanied by a rapid and forceful drop of the head. Each cluster typically lasts a few minutes and there are many variations of this pattern. After individual spasms, children often cry. Clusters of spasms most often occur upon awakening, but can happen at any time of the day. In comparison to epilepsy, which is very common, infantile spasms are fairly rare. There are approximately 2,000 to 4,000 new cases in the United States each year.

Causes

There are many potential causes of infantile spasms. Almost any brain abnormality or injury to the brain has the potential to cause infantile spasms. Nevertheless, despite thorough medical evaluation, approximately 10% of children with infantile spasms have no identified cause. It is unclear why some infants with risk factors for infantile spasms ultimately develop it, while others do not.

Diagnosis & Evaluation

Children with suspected infantile spasms should be promptly evaluated by a pediatric neurologist or pediatric epileptologist. The cornerstone in the diagnosis of infantile spasms is a test called video-electroencephalography, or "video-EEG" for short. This test consists of simultaneous video and electroencephalography (EEG). EEG testing allows visualization of brain waves and is accomplished by the temporary attachment of electrodes to the scalp with a variety of adhesives. To confirm the diagnosis of infantile spasms, video-EEG is needed to verify specific brain-wave patterns during the spasms and document specific patterns in between the spasms. Neurologists will specifically look for a pattern called hypsarrhythmia, which is characterized by chaotic, high-amplitude brain waves.

Outcomes

The outcome of infantile spasms is highly varied, but infantile spasms is notorious for causing poor cognitive development, such as mental retardation. The underlying cause of infantile spasms, if identified, is the key factor that can help predict developmental success or failure. Of equal importance, it is essential that infantile spasms is promptly and successfully treated. As such, when infantile spasms are suspected, the diagnosis should be rapidly confirmed, a search for the cause of infantile spasms should begin and treatment should be started immediately. Normal development is possible in those cases in which an underlying cause of infantile spasms is mild or unknown (often called "cryptogenic") and treatment is prompt and successful.