About West Syndrome

What is West Syndrome?

According to Wikipedia, West Syndrome is “an uncommon to rare epileptic disorder in infants.”  Many sites will use words like “detrimental” or “catastrophic” because it can cause such severe consequences to the infant brain.  In order to be diagnosed as West Syndrome, the child must exhibit two of the following:  infantile spasms although West Syndrome can be called “Infantile Spasms” as well, an abnormal EEG and mental retardation.  The spasms or seizures can affect only parts of the body such as the arms and the legs or you can see it take over the entire body itself.  They generally appear in clusters that can contain one to over 40 seizures in a single cluster.  The seizures can be very non-descript which is generally why the infant is not treated in a timely manner.  Many parents or physicians think the child is having gas, colic or the Moro reflex (startle). West Syndrome has three different classifications.  There is symptomatic meaning a known cause has been found which in turn triggered the seizures.  Causes vary from cortical dysplasia, bacterial meningitis, tuberous sclerosis and many more.  Cryptogenic is when there is suspicion of an underlying cause, but no evidence can be found to support this.  Infants with cryptogenic West Syndrome generally have another neurological disorder.  Finally, idiopathic is when a cause simply can’t be determined.

How is it diagnosed?

West Syndrome is diagnosed by the onset of seizures as well as an abnormal EEG.  The EEG must show evidence of a brain pattern called a hypsarrhythmia.  A CAT scan, MRI, blood tests or genetic testing may be performed in order to determine the underlying cause for the condition.

How is it treated?

There are many different types of treatment although the most popular are adrenocorticotrophic hormone (ACTH), Vigabatrin or another form of steroid treatment.  Most often these drugs are prescribed and if proven ineffective then alternative drugs and options are used.

What is the prognosis?

The prognosis is varied by many different factors.  How soon was the child diagnosed?  Did the treatment work?  What type of West Syndrome does the child have?  Are there other illnesses?  Research shows a grim outlook for children with West Syndrome.  Many go on to have cognitive, learning and behavioral disabilities.  Other types of neurological issues such as cerebral palsy and autism can arise even after the seizures are treated   Even though the seizures associated with West Syndrome often disappear at around age two, many children (50%) will go on to have another form of epilepsy. This is the information provided on the internet.  I have personally read stories of children that have gone on to have normal, full functioning lives.  I was hesitant to write about the prognosis, but these are the statistics – take them for what you will.  I know we have tried to stay distant from the statistics as Lola is creating her own path with this condition.  Even with all of the data, this does not mean there aren’t miracle cases and this does not mean one shouldn’t have hope.  In fact, I found one study though a bit older (2001) conducted by a group of Finnish researchers. They thought, “Factors associated with a good prognosis were cryptogenic etiology, normal development before the onset of the spasms, a short treatment lag, and a good response to adrenocorticotropic hormone; this was seen in both the symptomatic and the cryptogenic group, and there were no relapses.”  Their conclusion based on the study was, “The outcome in children with West syndrome seems to be better than is generally believed.” One aspect that can contribute to a good prognosis is with the help of physical, occupational and behavioral therapy.  The seizures or spasms can be considered catastrophic to an infant’s brain because they can interfere in crucial stages of development.  While the seizures themselves don’t cause brain damage, the severity of the underlying cause of the seizures is what inhibits the infant from developing normally.  This is where therapy is crucial.  With the help of therapists, the child can learn how to meet milestones such as sitting up, crawling, walking, feeding themselves and more.

What should I do if I think my child is having a seizure?

Call your pediatrician immediately and if you can’t get through then go to the emergency room.  I cannot stress the importance of diagnosing and treating this condition early.  Be sure to take a video of your child having a seizure to be sure your doctor sees the seizure firsthand.  Do not take no for an answer if you believe there is something wrong.  Every day the condition goes untreated could potentially be many milestones your child will have to work extra hard to reach.

Links we found helpful:

Little seizures, BIG consequences

Understanding Infantile Spasms

Finnish Study (2001)

Infantile Spasms Forum

Infantile Spasms Group on Facebook

Epilepsy Forum

Info about Prelone (prednisone)

Info about ACTH

Cortical Visual Impairment

Thinking Outside the Light-Box: Vision Therapy Support Group on Facebook

The links we provided are ones that have benefited us the most.  The information on West Syndrome is incredibly sad and overwhelming especially when your child is first diagnosed.  All you want is to find information that can offer a bit of hope and there is little to none on the internet.  In the beginning, I drove myself (and my husband) crazy with all of the negative information I found.  I had to convince myself to stop googling every thought that came to mind.  I was scared for Lola.  I was afraid of what her future would hold.   But I had to realize that Lola is on her own journey with West Syndrome.  We don’t know what the future holds for her, but we know she is on the right path.  If your child is diagnosed with West Syndrome, I would recommend you do the same.  It is a hard reality to face, but every case is different.  Every child has their own story.  I am much more sane now because I have trust in Lola, although I most certainly have an occasional relapse.  I am trusting in myself and my husband more as well as Lola’s team of physicians and therapists.  Most importantly, though, I’m having faith.