An Overview of West Syndrome

Understanding Infantile Spasms

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West syndrome is a seizure disorder (a form of epilepsy) that begins in infancy. It is often caused by brain damage due to trauma or illness. A specific type of seizure called infantile spasm is part of this syndrome, as are abnormal brain wave patterns called hypsarrhythmia and developmental delays. What the future holds for children with West syndrome largely depends on the underlying cause(s) behind it.

Symptoms of West Syndrome
Verywell / JR Bee

Symptoms

West syndrome most commonly begins in babies 4 to 8 months of age. About 90 percent of cases start in the first year of life.

Symptoms of West syndrome 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 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
  • Developmental problems: This is due to a 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.

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, the condition is called cryptogenic West syndrome.

West syndrome accounts for 2 percent of epilepsies in childhood, but 25 percent of cases 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 syndrome. The diagnostic process includes a thorough neurological examination to look for possible causes. This may include:

Other than a slight pain from a blood draw, 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
  • Pyridoxine

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

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

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

In a 2015 study of long-term outcomes, two-thirds of children with West syndrome went on to have what was considered an acceptable quality of life. 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.

Infantile spasms usually go away by mid-childhood. However, more than half of children with West syndrome will eventually develop other seizure types, with some of those patients developing a severe form of epilepsy known as Lennox-Gastaut syndrome. West syndrome may also raise the likelihood of developing autism.

Historically, though, about 5 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.

Research shows that the most important thing you can do for your child is to get a quick diagnosis, start treatment, and be dedicated to the treatment regimen.

Coping

Dealing with a serious illness in a child can be difficult on many levels. You will need to make adjustments to your daily life, manage financial implications of care, and navigate your own feelings about the situation. Remember that the best thing for your child is a caretaker who is at his or her best. These coping strategies can help.

Emotional Challenges

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.

This is all a normal. Educate yourself about your child's condition so you can make good decisions on their behalf, which can boost your confidence.

A support group, where you can learn from (and lean on) other parents, can be a huge help as well. Because West syndrome is rare, you may need to look online or join a local group that focuses on a broader topic, such as epilepsy or neurological illnesses.

Some organization may have resources to help you, including:

You may also benefit from seeing a mental health counselor who, at the very least, can provide you with a safe space to discuss how you're feeling and develop personal coping strategies.

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.

Social Challenges

It may become difficult to go on family outings because many settings aren't safe for your child, or because of strictly scheduled treatment regimens. Your child may be safest in a stroller, and that may limit the places it's easy for you to go. Some medications may have side effects such as sensitivity to heat, making a controlled environment much more desirable than being outside.

Instead of trying to make your normal routine fit your child's needs, you will likely have to make some adjustments to your lifestyle that put those needs front and center.

If you miss out on some gatherings because of the above issues, for example, plan some alternatives that work for you and your child. While this make take time to get used to, it can help you sidestep unnecessary challenges and stay connected to your loved ones.

Financial Issues

You may also have concerns about paying for your baby's medical care. 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, of course, want to give your child the best life possible. That can still happen—it just might not look like you expected. Learn about your child's needs, advocate for their care, and simply love them. While you will all face challenges and tough times, these three things will help ensure that your child is living as full of a life as possible with West syndrome.

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