IS 101

Infantile Spasms (IS) is a rare form of epilepsy that affects about 1 in 3500 live births, more commonly in boys. It is considered to be a “catastrophic” epilepsy, as it is usually very difficult to control the seizures and most cases result in mental retardation. One study of 214 IS children reported that 31% died, 45% resulted in retardation, and 24% had near normal development [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1464162/].

IS is also known as West Syndrome but experts debate between whether only 2 or all 3 of the following characteristics are required to call it that: spasm-like seizures, developmental delays/regression, and hypsarrythmic EEG. Onset is usually around 4-8 months, peaking at around 5 months of age. The seizures occur in clusters/episodes, either as head drops, myoclonic convulsions or jackknife jerks. They can happen many times throughout the day.

There are three types of IS:

  • Symptomatic: brain damage seen on MRI/CT/PET, and hypsarrythmic EEG (Kaleb is in this category)
  • Cryptogenic: other neurological symptoms but cannot find actual cause, ie. hypsarrythmia but normal MRI/etc
  • Idiopathic: no other signs or symptoms, otherwise normal, has hypsarrythmia but normal MRI/etc

Statistically, idiopathic cases have the best prognosis, followed by cryptogenic cases. Early treatment also offers better prognosis.

Unfortunately, there is no perfect treatment for IS, and you may have to try all the various ones out there until one works best for your child.

ACTH = The steroid drug ACTH, along with VGB, is usually used as a first line of treatment against IS. Statistically, it has about a ~70% chance of ceasing spasms, with a relapse rate of ~25%.

VGB = Vigabatrin aka Sabril is another forerunner in the lines of treatment. Also about a 70% success rate, it’s most effective on Tubular Sclerosis patients.

Prednisone (or Prednisolone) is oral steroids and is sometimes also used first since it may have similar efficacy as ACTH when used at higher doses with slightly less side effects.

Other treatments: pyridoxine therapy (for pyridoxine deficiency), topiramate (Topamax), lamotrigine (Lamictal), levetiracetam (Keppra), zonisamide (Zonegran). An alternative treatment is the Ketogenic diet. It looks like a modified Atkins/diabetic diet but with stricter proportions, is difficult to manage, and requires doctor approval. Some patients could also potentially be surgical candidates. That only applies when there is something that can be physically removed from the brain. (Kaleb is not currently considered a surgical candidate.)

Epilepsy.com provides a good overview on IS: http://www.epilepsy.com/EPILEPSY/EPILEPSY_INFANTILESPASMS

Note: CDC recommends delaying the DTAP vaccination when suffering from an epileptic disorder, including IS: http://www.cdc.gov/vaccines/recs/vac-admin/contraindications-vacc.htm

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3 thoughts on “IS 101

  1. Thank you for putting together the information for your friends and family on this page. It is so helpful to be able to understand the rest of your blog. We are thinking about your family right now and sending thoughts, prayers, and love your way.

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