Tracey - posted on 11/11/2009 ( 3 moms have responded )




Infantile spasms may begin up to age 2, but most commonly begin between 4 and 6 months, with about 90 percent beginning in the first year. The causes are widely variable and in some cases are unknown. However, the most common causes are tuberous sclerosis and perinatal asphyxia (lack of oxygen).
Parents sometimes attribute the cause to pertussis vaccination, a connection that has yet to be proven. Since the peak in onset of spasms occurs at the same age range that the vaccination is given, it is likely this concurrent timing is the only link.


The characteristic symptoms of infantile spasms are usually rapid, though generally not violent, with muscular contractions or extensions of limbs or other parts of the body. The spasms last one to two seconds and occur in clusters ranging from a few to more than 100 at a time. Infants may experience up to 60 clusters a day. The episodes rarely occur during sleep but are common in the early morning and just after naps.

The following are behaviors or symptoms that might occur in what is classified as infantile spasms or West syndrome:

• Repetitive forward head nodding or bobbing
• Bowing from the waist when sitting
• Drawing up of knees when lying down
• Extending/stiffening the neck, trunk, arms and legs
• Crossing arms across body as if self-hugging
• Thrusting arms to the side, elbows bent

Infants may cry and show irritability during or after a flurry of spasms.

Spasms are easily missed, especially if they occur singly or in small or infrequent clusters. Other potential symptoms that parents might notice are a loss of muscle tone, loss of head control or reaching ability, loss of eye contact, inattention to sounds, lack of responsiveness, poor smiling, or decrease in alertness.

Evaluation - Infantile spasms / West syndrome

Almost any condition that can cause brain damage is a potential cause of infantile spasms. Brain abnormalities are seen in about three-fourths of infants with this syndrome. In tracking down the specific cause, the doctor will likely order a variety of laboratory tests and EEGs, plus a CT Scan or MRI brain imaging scan.
The initial step is a detailed medical history and physical examination. Among the signs the doctor will look for are neurological abnormalities that might have been present before the onset of seizures. Since tuberous sclerosis is often associated with infantile spasms, an examination of the skin for possible lesions (hypopigmentation) typical of tuberous sclerosis may be conducted.
Brain-wave patterns are especially informative in diagnosing infantile spasms. EEG tracings taken during and between seizures, and during both sleep and wakefulness are useful in confirming the diagnosis. A chaotic pattern (hypsarrythmia) between seizures is characteristic of those with infantile spasms. The pattern during sleep is markedly different and, in some infants, the disordered waves may be seen to lessen or disappear.
Neuroimaging by CT or MRI is conducted for possible signs of tuberous sclerosis, brain infection, or structural abnormality. If the cause still remains unknown, the initial battery of laboratory tests ordered by the doctor will likely be expanded to rule out metabolic and other potential causes.

Treatment - Infantile spasms / West syndrome

Spasms in some infants may stop spontaneously without any treatment. But most will require some form of therapy, the primary drugs for which are adrenocorticotropic hormone (ACTH) or synthetic corticosteroid drug such as prednisone. These therapies can have a dramatic effect in arresting or slowing seizure activity and the treatment period may be relatively short. The side effects can be significant, however, and long-term use of these drugs requires careful monitoring.
Some infants may respond to conventional anticonvulsant drugs such as felbamate, lamotrigine, topiramate and valproic acid. Infantile spasms associated with tuberous sclerosis responds especially well to vigabatrin. However, vigabatrin is not available in the United States since it is toxic to the eye and can cause loss of peripheral vision.
A medically controlled ketogenic diet may control or reduce seizures in some infants when medications fail. Also brain surgery may be another option when the seizures are identified with a lesion or structural abnormality that can be removed without damage to vital tissue. For example, new innovations in surgical procedures have begun to increase the success rate for surgery in infants with tuberous sclerosis.

Outcome - Infantile spasms / West syndrome

The prognosis for children with infantile spasms is directly related to the cause. Infants with a known cause for their spasms have a better prognosis than those with an unknown cause for their spasms. Infants with no signs of neurological abnormality or developmental delay before the onset of spasms also have better outcomes. Those whose seizures come under control quickly or cease early tend to do better as well.
Infantile spasms rarely continue beyond age 5, but are sometimes replaced by other seizure types. A significant number of infants with this syndrome have long-term cognitive and learning impairment. Some may have a behavior disorder accompanied by autistic symptoms. These impairments are likely due to the same brain injury that causes the seizures.
The presence of other seizures types with the spasms may also suggest a poor outcome. In the most severe cases, seizures will continue and the condition may evolve into the Lennox-Gastaut syndrome.

**Presented by Tracey Alderson, Administrator, Epilepsy Awareness 2009
**Information provided through approved Epilepsy Information Websites


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Tracey - posted on 01/24/2012




Hi Sarah,

I'm so sorry to hear about your present findings regarding your daughter's condition. I apologize if my notes sounded unclear. "The prognosis for children with infantile spasms is directly related to the cause" - simply means that success or lack of success with infantile spasms can be directly related to either; a treatable cause, an untreatable cause, or if there is no cause found, an unknown cause..."Infants with a known cause for their spasms have a better prognosis than those with an unknown cause for their spasms." - refers to when they find a treatable cause - knowing causes such as lesions, benign tumors, etc. can be helpful in giving specialists needed knowledge on how to best treat someone and in some cases correct the cause and in turn stop the seizures..."Infants with no signs of neurological abnormality or developmental delay before the onset of spasms also have better outcomes" - refers to better outcomes for those who are neurologically in tact and have good cognitive response; they may or may not know the cause for these individuals...."Those whose seizures come under control quickly or cease early tend to do better as well" - refers to many individuals, with both idiopathic epilepsies and those diagnosed with a cause.

All seizure disorder can have a less favourable outcome when certain causes are found. In your daughter's case with infantile spasms, they found a "neurological abnormality" called "lissencephaly", which refers to a brain formation disorder. Their neurological findings or reason, in other words, the cause for the seizures is untreatable. So even though the seizures can now be explained and they can understand the pattern for the seizures, fixing this particular cause isn't possible, therefore the prognosis does not look good.

Normally speaking once a cause is found the cause becomes the first medical condition to focus on and the seizures become the secondary medical condition. However, if they find an untreatable cause, they can only continue to treat the epilepsy by trying to control the seizures. Which brings me to Vigabatrin - I said it's not available, because it's not an easy drug to obtain and the side effects can be very serious. However, if this is the drug that your specialist thinks is the best for your daughter than that is up to you to decide. Just keep in mind that anything is possible and although a prognosis is an educated guess at what your daughter's outcome will be, nothing is for sure yet.

Certainly, remaining realistic makes sense. But I also know from my own experience a prognosis can sometimes be wrong. My team of specialists never expected my uncontrolled seizures would ever get under control. They expected because of the location of an un-operable brain lesion deep inside my brain, it would be just about impossible for me to gain control of my seizures. Then after a few years of being sick of it I said I was lowering my medication, and everyone looked at me as if I was nuts...It took me over 15 years of having uncontrolled grand mals, but I finally took my last one just over 2 years ago - and so far, I've been 2 years seizure free. All my point here is, who really knows?

I know you and your family are going through some tough times and every decision you make is a difficult one. I truly hope the very best for your daughter. And best of luck to you and your family.

Wishing you all the best,

Take care!

Tracey Alderson



Sarah - posted on 01/18/2012




ummm, yes, yes, yes, and really no! Much of this is spot on but not all. My daughter started having infantile spasms at 5 and a half months old. From what I have read, those for whom no cause can be found have a BETTER chance of a good outcome than for those like my daughter where they find a defect. She has lissencephaly and her prognosis is heartbreaking. So we have no chance for a "good" outcome because there is nothing that can be done to correct her brain malformation. Second, vigabatrin IS available in the US, I have a package of it on my counter. You must, however, get it from a epileptologist that is part of "SHARE" (Support, Help And Resources for Epilepsy) . Only doctors that are a part of this can prescribe it and you must order it through their special arrives by mail directly at your door.

Tracey - posted on 11/20/2009




Here is a reply from a mom to this same article I posted on "Moms of Epileptic/Seizure Disorder Kids"...please read what she has to say about her situation:

Oh my goodness! This was so interesting for me to read! My daughter is now 2yrs old and has been having infantile spasms since she was about 4 months old. Before that, she was developing and functioning normally. She was diagnosed with 'partial lissencephaly' or 'pachygyria' and we were told that that was what was causing the seizures.

What was so interesting to me about your post though are the behaviors/symptoms that can occur with it... I thought my daughter's head bobbing was more because she was trying to gain control over her head and that the way she folds her legs up into her tummy or throws her arms, elbows bent, out to her sides was due to her being uncomfortable. Very interesting!

We've been able to get her down to about 4-7 seizures per week and once in a while she'll go for several days without one but it's still a constant guessing game. We just never know what kind of day she's going to have and if she'll have a seizure or not.

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