Saturday, February 7, 2009

Some info about IS. Brocs story

What is it like?

Here's a typical story: "At first I thought Chris was just having the little body jerks when he was moved or startled, like my other children had when they were infants. But then I knew something was wrong. The jerks became more violent, and his tiny body was thrust forward and his arms flew apart. They only lasted a few seconds but started to occur in groups lasting a few minutes. It was so hard to see such a young baby having these things."

Infantile spasms (also called West syndrome because it was first described by Dr. William James West, in the 1840s) consist of a sudden jerk followed by stiffening. Often the arms are flung out as the knees are pulled up and the body bends forward ("jackknife seizures"). Less often, the head can be thrown back as the body and legs stiffen in a straight-out position, or movements can be more subtle and limited to the neck or other body parts. Each seizure lasts only a second or two but they usually occur close together in a series. Sometimes the spasms are mistaken for colic, but the cramps of colic do not occur in a series.

Infantile spasms are most common just after waking up and rarely occur during sleep.

Who gets it?

Infantile spasms begin between 3 and 12 months of age and usually stop by the age of 2 to 4 years. They are uncommon, affecting only one baby out of a few thousand. About 60% of the affected infants have some brain disorder or brain injury before the seizures begin, but the others have had no apparent injury and have been developing normally. There is no evidence that family history, the baby's sex, or factors such as immunizations are related to infantile spasms.

Tell me more

When a baby with infantile spasms has an EEG, the doctor usually will see an unusual pattern called hypsarrhythmia (HIP-sa-RITH-me-ah) when the seizures are not occurring. This chaotic, high-voltage pattern is often helpful in confirming the diagnosis.

Babies with infantile spasms seem to stop developing and may lose skills that they had already mastered, such as sitting, rolling over, or babbling.

How is it treated?

Steroid therapy (adrenocorticotropic hormone [ACTH] or prednisone) is the primary treatment for infantile spasms. Some experts recommend trying a seizure medicine such as Sabril (vigabatrin, not available in the United States), Depakote (valproate), or Topamax (topiramate) before steroid therapy. In countries where it is available, Sabril is often used as the initial therapy because it is relatively safe (especially for short-term use) and effective. It is especially effective for children with tuberous sclerosis (a disorder associated with abnormalities involving the brain, skin, heart, and other parts of the body). Sabril is associated with damage to the retina of the eye and should be used with caution in children.

What's the outlook?

Most children with infantile spasms are mentally retarded later in life. Those whose spasms are related to an underlying developmental brain disorder or injury have a higher likelihood of moderate to severe retardation. The outlook is brighter for those who were developing normally before the spasms started: 10 to 20% will have normal mental function and some others may be only mildly impaired. Some children with infantile spasms develop autism. Many doctors believe that the quicker the seizures are controlled, the better the results will be.

When the spasms stop, many children later develop other kinds of epilepsy. About one-fifth of children who have had infantile spasms will have the Lennox-Gastaut syndrome.


Ellen said...

Hi. I have not visited for a while, so I am just reading about everything's that gone on. I am so, so sorry. It is good that they caught Charli's infantile spasms now, so they can be treated. And, I hope your husband is doing OK. That is also mind-boggling, but I guess he is very lucky, it could have been worse.

I hope you have friends and family around to help you out. I wish there was something I could do.

JSmith5780 said...

I'll comment again since you mention Vigabatrin in this post and incase someone else comes looking.

My son was on Vigabatrin for about 3.5 years. He has had NO VISUAL effects from the Vigabatrin. Also, in early January a special committee to the FDA recommended approval of Vigabatrin for use in children with Infantile Spasms and for adults suffering from partial seizures. The FDA has not formally approved it but they are expected to in the coming weeks.

If anyone has questions, feel free to contact me through my blog. I am a supporter of Vigabatrin and attended the FDA hearings.