West Syndrome – Seizure Disorder of Infants

West Syndrome is an epileptic condition that typically presents in the first year of life, mostly between 4-8 months of age. The formal definition involves infantile spasms, developmental delay and hypsarrythmia, an abnormal brain electrical pattern, on brain EEG. Infantile spasms are sometimes subtle to parents, and other times very debilitating to infants. They manifest as clusters of rapid movements with the appearance of head jerking, body stiffening and bending, or extending the body out with an arched back . Clusters of infantile spasms are often followed by crying and can be associated with lack of responsiveness, decreased eye contact or decreased alertness.

West Syndrome is rare, and frequently seen in children who already have some sort of pre-existing brain condition like Down Syndrome or Tuberous Sclerosis. However, about 40% of children who present with infantile spasms are previously healthy, and it is important to identify them early as failure to control this seizure activity can lead to a loss of developmental skills.

If you suspect that your child is having these repetitive, jerking behaviors, it is best to see your pediatrician as soon as possible. If you can, try to capture the behaviors on a video so you can share them with the doctor.

If a neurologist believes your child may have West Syndrome, they will likely perform an EEG to evaluate the electrical activity of the brain as well as blood work and possibly an imaging study of the brain. In the case that seizures are suspected, the risks and benefits of treatment will likely be discussed with your pediatric neurologist. The primary treatment for West Syndrome is pituitary hormone ACTH or the drug vigabatrin. However the field of research is growing and other options include prednisone, anti-seizure medications, dietary changes or even surgery. It also may be important to evaluate your child for an underlying abnormality which may have lead to the onset of West Syndrome. This is important, because if a source of seizure can be identified, specific treatments can be applied.

West Syndrome unfortunately can be devastating neurologically to children, and it can have poor impacts on children’s development in the future, and can even lead to mortality. That is why it is so important to speak to a doctor immediately if you believe you recognize these symptoms in your child.

If your child has West Syndrome, you are not alone. There are a variety of resources available to families with children with epilepsy that can help provide you with good information and support while navigating the challenging and murky waters of initial diagnosis. One excellent resource is the Epilepsy Foundation.