West syndrome (infantile spasms)
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Last updated 03 Aug 2009, review date due 03 Feb 2010
Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.
Infantile spasms are a type of epilepsy with a characteristic age of onset (typical age when seizures start), pattern of seizures and electroencephalogram (EEG). This means that it is an ‘electroclinical epileptic syndrome’.
They can also be called ‘salaam spasms’, because the appearance of the seizures is like a bowing forwards or backwards movement, or ‘West syndrome’ after Dr West who first described the condition in his own four month old son in 1841.
This type of epilepsy occurs in about one in 3000 children. Every year in the UK about 350-400 children will develop West syndrome.
Symptoms
In nine out of 10 children with the condition, infantile spasms occur in the first year of life, typically between three and eight months old. Often, to begin with, the attacks are brief, infrequent and not typical of the syndrome, so it is quite common for the diagnosis to be made late. Frequently, because of the pattern of attacks and the cry that a child gives during or after an attack, they are initially thought to be colic.
The typical pattern is of a sudden flexion (bending forward) in a tonic (stiffening) fashion of the body, arms and legs. Sometimes, however, the episodes are extensor (arching). Usually, they are symmetrical (affecting both sides equally) but sometimes one side is affected more than the other.
Typically, each episode lasts just one or two seconds, there is then a pause for a few seconds followed by a further spasm. While single spasms may happen, infantile spasms usually happen in ‘runs’ or ‘clusters’ of several in a row.
It is common for babies who have infantile spasms to become irritable and for their development to slow up or even to go backwards until the spasms are controlled. These infants can also behave as if they cannot see, but this improves once the spasms are controlled and the EEG has improved.
Diagnosis
The diagnosis of infantile spasms is made by a combination of the typical features with a typical EEG. The EEG shows a very disorganised pattern termed ‘hypsarrhythmia’. The EEG is always abnormal in children with West syndrome but sometimes this abnormality is seen only during sleep.
Infantile spasms, like many other ‘electroclinical syndromes’, have lots of different causes. A particular cause will be found in seven or eight children out of every 10 children with West syndrome.
Most children with infantile spasms will need a number of tests (EEG, brain scans, blood tests, urine tests and, sometimes, spinal fluid and other tests) in order to try to identify the underlying cause.
Treatment
The main treatments used are steroids or vigabatrin (Sabril), Nitrazepam and sodium valproate (Epilim), may also be used. The type of steroids used are prednisolone or tetracosactide. Steroids must be used carefully as this treatment may cause some unpleasant side effects.
Prognosis (outlook)
In some children, infantile spasms respond easily to treatment, whereas in others they remain very resistant. Most children unfortunately go on to have other kinds of seizures in later childhood including an epilepsy syndrome called the Lennox-Gastaut syndrome. Most children also have learning difficulties. The prognosis for infantile spasms in both these respects is largely dependent on the underlying cause of the condition.
Support organisations
West Syndrome Support Group, c/o Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, www.cafamily.org.uk
Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, www.cafamily.org.uk
Epilepsy Helpline
- UK freephone 0808 800 5050
- International +44 113 210 8850
- Email: helpline@epilepsy.org.uk
- Txt msg: 07797 805 390 info
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Epilepsy Action blog
Comments
My daughter was diagnosed with West syndrome when she was 7 months old. She had blood on her brain from delivery and had seizure several hours after birth. She was treated with an anti seizure medicine. She was developing fine then she started with the spasm. The doctor's said that the injury from delivery caused her to develop West syndrome. They treated her with ACTH. My daughter is now 11 years old. She hasn't had a seizure since she had the ACTH treatment. She is in regular classes. She has to try a little harder but she is a B-C student. When they first diagnosed her they told me she would probably never walk or talk, this was in 1999. She is a true miracle. Please take advantage of any and all resources available to you. My daughter was in Infants and Toddlers from birth. There is so much information available but I know from experience early intervention is the key. I remember going to the er everyday for a month before they finally sent me to a neurologist who diagnosed her with West syndrome. Always follow your heart and be persistent. My prayers are with you and your child.
hello, my daughter was diagnosed with West syndrome five months, was late with sabrilex and Depakine, now she has five years and only takes Depakine, my uneasiness has always been that epsiodios in which his face is pale and when not sleeping comes to wake up at night but he noted that it tikcs nervous seconds on his shoulder, legs, ets.Electrostatic AHAN already made sleep but do not find anything and very tired lately and has dawns behavior changes frequently.
I've been with this concern and five years and I asked his neurologist and do not get any answer, who can help me write me to my mail thank you very much
I've just posted our own story. Andrew is 9m old (6m corrected as he was an extreme preemie) and he's just started treatment for IS. He's responding well to the steroids. We're only just home so have yet to see the Community Epilepsy Nurse, but the hospital were extremely good at diagnosing and treating the IS (unfortunately the Paed Ward nurses weren't so good, but that's another story!). Andrew had a cranial bleed before his birth, and they are presuming the IS was caused by that, they are not looking for any other cause.
My son Andrew started having IS 10 days ago, and after 3 days of thinking it might be colic or reflux, I took him to A&E where they admitted him immediately as they recognised his episodes. By this point he was having about five episodes a day, lasting 2-3 minutes each. He spent almost a week in hospital (you can't get an EEG over a weekend!), started receiving steroid treatment (Prednisolone) on the Tuesday morning, and came home on Friday afternoon as he responded well to the meds, down to one episode a day. We're now at 30 hours without an episode and keeping fingers crossed. He returns in 10 days for a follow-up EEG to see if the hypsarrythmia is still present. Side effects from the steroids noticeable so far are pudginess, grumpiness (but he was grumpy before!) and increased appetite, but as feeding has been an issue to date (he was small for gestational age), we're quite happy that he's eating & drinking more.
My son was diagnosed with IS when he was 13 months old. He'd been experiencing the spasms since he was 10 months old but it took us taking him to Vanderbilt Childrens Hospital in Nashville TN in order to find a team of Neurologist who actually listened to my concerns and tested him appropriately. My son has been on a number of treatment drugs including ACTH (which helped instantly) but not fully deleting the spasms. After a year on Keppra, Topamax, Clonapin, Lamictal, Depicote and so many others we decided to take a less toxic approach. Have you heard about the Ketogenic Diet? This is a high fat diet where the amount is spacifically calculated into meal for a ratio of 2-1, 3-1 or even 4-1 from sugar and carbs. It's used to help treat difficult to control epilespy. I thought at first this was a shot in the dark but soon realized the diet does make progress in congnitive areas. It slows down the brain process and allows children to consintrate on items which eventually they learn how to use. It's very time consuming and overwhelming at first but now after 2 1/2 years it's like anything else, a way of life. Now another drug that is rarely introduced b/c up until Aug of 08 it was not approved in the United States and had to be obtained only from Canada or Mexico is called Vigabatrin or Sabral is the name brand. The drug worked like a miracle for my son! He began doing things he'd never done before and he was 2 years old by this point. I would asked your childs neurologist about this drug FOR SURE. Good news is now it is approved in the US for Children only with IS so insurance companies will pay for it. We are still in the process of all the paperwork involved but it is well worth it. One thing you must understand is there is no one way to treat this problem. It's going to be up to your individual child to accept the drugs given then wait and see what happens. My son is now 4 and we still face challenges each day with his IS. Now we are almost certain his has developed into another syndrome called Angelmans. It's devistating as a parent to watch you child stuggle so much and still have no answers to why this happened or how we can make it stop but hang in there. Sometimes it will feel like a roller coaster but when you're up on that high point it's such a beautiful time. Good luck to you all. God Bless The Little Children!
My 14 year old son with Down Syndrome suffered from IS. I found that the neurologists tried too many "new" meds at that time and it was not until his Doctors stepped in and said enough that we started treatment with ACTH. The treatment itself is nasty. He puffed up and had resperitory challenges but after 16 weeks of treatment the spasms stopped. It took me a while to realize that the spasms did rob him developmentally.
Now at fourteen we are experienceing some activity. I am told that it is with the onset of puberty. I am just in the throws of appointments and chasing doctors so we have not began any treatment.
Ask your physician about ACTH. I think that i tis an expensive drug so this may be why they are hesitant to take this route first.
Hope this helps.
Take care
Dawna lee Toole
My son (9 months old) is living with infantile spasms at the moment. He is just out of hospital and is taking keppra and being weaned of epilim (as it did nothing for him). Its hard to watch as a parent, but we are there for him. Is anyone living through infant spasms? How has the medication worked? How have your health care professionals been during the process? Do you know the underlying cause of their start?
I would suggest that the page on Infantile Spasms is linked to the page on Pyridoxine Dependency, as the use of Vitamin B6 is discussed there. My son had Infantile Spasms as a baby (cause unknown) and the seizures did not lessen either with Vigabatrin or with injected/oral steroids. However the seizures stopped completely and his EEG became normal when given 10mg/kg of vitamin B6 daily by the NHS Consultant Paediatric Neurologist. He does not have Pyridoxine Dependency, which is a genetic condition, but he had pyridoxine responsive seizures.