INFANTILE ONSET SEIZURES (also known as WEST SYNDROME)

Tracey - posted on 11/11/2009 ( 7 moms have responded )

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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.

Symptoms:

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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Danielle - posted on 11/19/2009

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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.

Tracey - posted on 11/25/2009

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Hello to everyone,



Thank you to all of you for your personal input. I know watching your child go through these seizures is not easy. And finding a good specialist who can assess your child's needs is of the utmost importance. Understanding that it is not always the seizures themselves doing damage to the brain and causing developmental delays is also important; a lot depends on individual circumstances (cause of the seizures, types of seizures, etc). And sometimes the medication or combination of medications can cause some of these problems as well. But taking medication to control the different types of seizures still far outweighs the risks involved of not taking medication for a seizure disorder. And parents are great monitors and extremely important when it comes to communicating information to the specialist. Therefore if your child appears too drugged or overmedicated, or if the pattern of your child's seizures change suddenly, or if the medication appears to be causing a change in your child's personality, do not just accept this - make sure you voice it. Taking too much medication can cause more seizures and not taking enough or not taking the appropriate medication will get little or no control. And if a child is taking a medication that's obviously altering his/her personality this is a good indication that this medication is not appropriate for your child. And if they continue to take mood/personality altering medications there could be other issues to deal with as well; anger, depression, mood disorder. Chances are this will affect your child's ability to concentrate and follow rules/instructions and could add to the difficulties learning, building friendships/relationships, and generally being happy. Working with your neurologist and support group to correct these issues as soon as possible is advisable. Sometimes finding the right combination of medications to obtain or gain back both quality of life and have better seizure control takes a lot of time and patience. And sometime *less is more* works better - but not in all cases!



The ketogenic diet may be another option available to you if your child is experiencing uncontrolled seizures. This is a very strict diet, extremely high in fats; everything has to be measured/weighed, but it often works surprisingly well in really young children. The scientists still aren't completely sure why this diet works so well but my own theory is; fat is extremely important for normal brain development in babies and some babies brains may demand more fat than others and possibly wasn't getting enough through the usual sources. Therefore on this diet the brain can absorb more fats. And for some, this diet has proven to be very successful; only requiring a couple of years of dedication and then the child has become completely seizure free.



Good luck to all and take care!



Sincerely,

Tracey Alderson, Administrator, Epilepsy Awareness 2009

Tracey - posted on 11/16/2009

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Quoting vicki:

My 16 year old daughter was diagnosed with infantile spasms at 14 weeks old after 2 weeks of thorough testing. There was no known reason or cause. She is severely mentally and physically disabled and has a developmental age of around 5 months old. Her seizures are still to this day impossible to control, she has grand mal seizures now. This can be a very debilitating condition and we know our daughter suffers the most extreme form.



Hi Vicki,



I'm very sorry to hear what your daughter and your family has had to learn to cope with.  My seizures started when I was 29 and I too have uncontrolled complex partial and grand mal seizures.  Though I haven't endured the loss of motor or acedemic skills my point is Epilepsy can hit anyone anywhere at any age.  And sometimes it seems there is no logic or reason.  Unfortunately aprroximately 1/3 of people with seizure disorders don't gain control of their seizures.  Since I can relate to having an uncontrolled seizure disorder and losing a family member who had Epilepsy to suicide I now speak publicly about Epilepsy whenever I am asked.  And I hope by bringing the amount of awareness to the public as I am doing that eventually the majority of people will truly begin to understand that Epilepsy can be a lot more devastating for some than for others.  I hope you and your family are working with the best specialists available to you and that you have a good support group available as well.  Sometimes dealing with a disability like this is not easy.  It is just something we learn to live with and do our best to see any positive from it. 



Take care and I wish you all the best!



Sincerely,



Tracey Alderson, Administrator, Epilepsy Awareness 2009

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Daughter, Wife, Mom, Sometimes - posted on 03/17/2014

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My daughter was diagnosed with IS when she was 5months old. She tried many medication and what finally worked for her was Topomax. She has since been seizure free for 16months.
Recently, I have noticed head nod/ shoulder shrug. It happens singly and looks involuntary. But at the same time she does not seem bothered by it.
The doctors can't give me an answer and because it happens infrequently, it is difficult for it to be filmed.
Anyone has experience in this or seen/ heard something similar that can shed some light to this puzzle?
Many thanks.

Neda - posted on 02/02/2014

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Just want to say hi to all you brave moms out there dealing with seizures control in your love ones. I myself am mother of 9 months old son with profound lissencephaly and infantile spasms. We have tried Sabril, Prednisolone and Keppra and none of them have worked. My son has a gtube now and we are going to try the Ketogenic diet for him.
Wish you all good luck and pray for all of you in such a difficult journey

Amy - posted on 11/25/2009

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my daughter was diagnosed with infantile spasms at 3monthes and also went through all the vary of test they do it was found that she has a genetic disorder called tubular sclorosis witch means tumers grow on any part of her body she is now nearly 2 years old and in this time she has had 3 diffrent types of seizuar at the worst she was doind around 80 a day she is at the moment having absent seizers witch she takes epillium and sodium valpurate with no results as yet i have been told of all the problems she may have as she gets older but as yet she is developing fine fingers crossed this will stay the same

Vicki - posted on 11/13/2009

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My 16 year old daughter was diagnosed with infantile spasms at 14 weeks old after 2 weeks of thorough testing. There was no known reason or cause. She is severely mentally and physically disabled and has a developmental age of around 5 months old. Her seizures are still to this day impossible to control, she has grand mal seizures now. This can be a very debilitating condition and we know our daughter suffers the most extreme form.

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