An Overview of West Syndrome

Understanding Infantile Spasms

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Hands applying electrodes to baby for electroencephalography
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West syndrome is a seizure disorder (a form of epilepsy) that begins in infancy. A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays.

This condition is often caused by brain damage due to trauma or illness.

Symptoms

West syndrome most commonly begins in babies between 4 and 8 months. About 90 percent of cases start in the first year of life. Symptoms include:

  • Infantile spasms: Most often in this type of seizure, the baby suddenly bends forward at the waist and the body, arms, and legs stiffen. This is sometimes called a “jackknife” seizure. Some babies may instead arch their backs, bob their heads, or cross their arms across the body like they are hugging themselves. These spasms last a few seconds and occur in clusters of anywhere from two to 100 spasms at a time. Some babies have dozens of these clusters of spasms in a day. The spasms most commonly occur after a baby wakes up in the morning or after a nap.
  • Hypsarrhythmia: A baby with West syndrome will have abnormal, chaotic brain wave patterns called hypsarrhythmia. An electroencephalogram (EEG) detects these by recording electrical activity from the brain in the form of brain waves.
  • Developmental problems: This is due to the brain injury that has caused West syndrome.

Other symptoms may be present because of the underlying disorder causing West syndrome. Other neurological disorders, such as cerebral palsy and autism may also be present.

Infantile spasms usually resolve by about age 5, but more than half of children with West syndrome will develop other types of seizures, such as Lennox-Gastaut syndrome.

Many children with West syndrome have long-term cognitive and learning impairments, most likely due to the underlying condition that caused the infantile spasms.

Causes

Almost any condition that can cause brain damage can cause West syndrome. The two most common causes are tuberous sclerosis and lack of oxygen during birth. Sometimes a cause is suspected but can’t be confirmed; in this case, it is called “cryptogenic West syndrome.”

West syndrome accounts for two percent of epilepsies in childhood, but 25 percent of epilepsy that begins in the first year of life. The rate of infantile spasm is estimated at between 2.5 and 6 per 10,000 live births.

Diagnosis

If your baby is having infantile spasms, your doctor will likely test for West The diagnostic process includes a thorough neurological examination to look for possible causes. This may include:

  • Laboratory tests
  • A brain scan using computed tomography (CT) or magnetic resonance imaging (MRI)
  • An EEG to look for hypsarrhythmia

These tests can be frightening. Other than a slight pain from a blood draw, though, your child shouldn't be in pain or distress due to these tests.

Early diagnosis is important. The sooner your baby can start treatment, the more likely it is that they'll have a good outcome.

Treatment

The most common drugs used to treat West syndrome are:

  • Adrenocorticotropic hormone (ACTH)
  • Prednisone
  • Vigabatrin
  • Pyroxidine

These treatments may be very effective in stopping or slowing the infantile spasms. Other drugs that are sometimes used include:

  • Felbatol (felbamate)
  • Lamictal (lamotrigine)
  • Topamax (topiramate)
  • Depakote (valproic acid)

If medications aren't effective enough, and especially in cases involving malformation or tuberous sclerosis complex, doctors may recommend surgical removal of damaged brain tissue. This type of surgery is performed commonly for several types of epilepsy and generally has good outcomes.

Prognosis

What the future holds for your child depends largely on the underlying cause(s) of West syndrome.

Infantile spasms usually go away by mid-childhood. However, more than half of children with West syndrome eventually develop other seizure disorders, such as Lennox-Gastaut syndrome. West syndrome may also raise the likelihood of developing autism. Historically, about five percent of children with West syndrome haven't survived past the age of five, often due to serious underlying conditions that caused seizures to begin with.

In a 2015 study of long-term outcomes, two-thirds of children with West syndrome went on to have an acceptable quality of life. Research shows that the most important thing you can do for your child is to get a quick diagnosis, start treatment, and be religious about the treatment regimen.

Children who developed normally before having infantile spasms and don't have an obvious underlying cause (such as brain damage or neurological illness) have the best outcomes.

Coping

Dealing with a serious illness in a child is difficult on many levels. Your child will need special care, which can be daunting and physically exhausting. You'll likely deal with a complicated mix of emotions including fear, anger, grief, and guilt—even if you couldn't have done anything to prevent your child's condition.

You may also have concerns about paying for your baby's medical care and whether you'll be able to continue working outside the home.

First, allow yourself to go through a grief process. That's a normal part of adjusting to the changes forced upon our lives by illness. Educate yourself about your child's condition so you're empowered to make good decisions for them. Gather whatever resources you can—from family and friends, your community, healthcare providers, and, if necessary, government programs.

Check with your insurance company to see what is and isn't covered. If you need help paying for medications, check the drug company's website. Many of them have programs that can help. Check with social services agencies about Medicaid or other state insurance options.

A Word From Verywell

You, as a caregiver, need to ensure that you have breaks and can practice self-care. Enlist the help of family and friends, and look into options such as having a healthcare worker come to your home.

Join a support group so you can lean on and learn from people in a similar situation. Don't hesitate to see a mental health professional, as well.

You want to give your child the best life possible. While you may have to adjust your expectations of what that will look like, by learning about your child's needs, advocating for their care, and loving them, you can still give your child a good life.

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