What does West syndrome look like?

During the seizures, which last for one to two seconds each, the child's body may suddenly bend forward and the arms and legs stiffen. Some children arch their backs while extending their arms and legs. The spasms most commonly occur during transitions between waking and sleeping and after feeding.

Can you live a normal life with West syndrome?

Unfortunately, up to about 90% of all children with West syndrome still suffer severe cognitive and physical impairments. Life expectancy for West syndrome is variable. About five in every 100 infants and children with West syndrome do not survive beyond five years of age.

What age does West syndrome start?

Symptoms associated with West syndrome usually begin during the first year of life. The average age of onset for epileptic spasms is at 6 months. Epileptic spasms are characterized by involuntary muscle spasms that occur due to episodes of uncontrolled electrical disturbances in the brain (seizures).

What are the features of West syndrome?

West syndrome is characterized by a specific type of seizure (infantile spasms) seen in infancy and childhood. This syndrome leads to developmental regression and causes a specific pattern, known as hypsarrhythmia (chaotic brain waves), on electroencephalography (EEG) testing.

How common is West syndrome?

West syndrome happens in about one in 2,500-3,000 children.

Signs of Infantile Spasms

How long do people with West syndrome live?

In a population-based study from Finland, children with West syndrome were followed into adulthood for 20–35 y or till death. A quarter had favorable cognitive outcome with professional settlement; however, psychiatric comorbidities were also seen in a quarter. Two-thirds had epilepsy of which LGS was seen in 18%.

Can you grow out of West syndrome?

Children who have typical development before the onset of spasms, receive effective treatment to control the spasms soon after their first appearance, and have no underlying brain abnormalities may do well. The infantile spasms of West syndrome usually stop by age 5, but they may be replaced by other types of seizures.

Are you born with West syndrome?

Most infants get it before they're a year old, usually between months 4 and 8. A little over half of babies who have West syndrome are boys. Things that can cause West syndrome include: Changes in your child's genes.

Is West syndrome a disability?

The most common spasms cause a child to bend forward with their arms or legs being raised. Spasms can cause changes in behaviour and speech. West syndrome, like epilepsy, is a disability and may result in special educational needs (SEN).

Is West syndrome progressive?

An animal model of West syndrome exhibits a progressive increase in epileptic spasms and learning and memory deficits. West syndrome, the most prevalent type of syndromic epileptic encephalopathy affecting infants, is a devastating and often fatal condition.

What is the treatment for West syndrome?

Steroids and anti-seizure medication are the most common treatments. The steroid may have adrenocorticotropic hormone (ACTH) and be injected into the muscles. Steroids might also be given in pill form. For some children a specific diet may help their condition.

Is West syndrome a form of Down syndrome?

Infantile spasms or West syndrome is the most frequent epilepsy syndrome in children with Down syndrome. West syndrome occurs in 0.6–13% of children with Down syndrome, representing 12.8–32% of seizures in these children [2], [7].

What can trigger infantile spasms?

Infantile spasms can be caused by problems with the way the brain developed in the womb, infections, brain injury, or abnormal blood vessels in the brain (such as an arteriovenous malformations). Infantile spasms also can happen in babies with some types of metabolic and genetic disorders.

What are the long term effects of West syndrome?

West syndrome (WS) is commonly associated with a poor long-term outcome including a small but significant mortality, infantile spasms that are resistant to treatment, the development of other seizure types and impaired cognitive and psychosocial functioning.

Can a baby with infantile spasms live a normal life?

In fact, some children with infantile spasms may ultimately lead normal lives, but only if they are diagnosed and treated correctly.

At what age do infantile spasms start?

Infantile spasms most often begin between 4 and 7 months, but can start any time in the first few years of life. Later onset spasms may also occur but are rare.

Can babies outgrow infantile spasms?

Infantile spasms usually stop by age 4 or 5. But more than half of children who have IS will develop other types of seizures, like Lennox-Gastaut syndrome, an epileptic disorder of later childhood. Children with IS have a higher risk of autism.

What is the death rate of West syndrome?

West syndrome is generally not life-threatening but it occurs more often in children with life-threatening conditions. Furthermore, the treatments used for West syndrome can (rarely) cause death. As a result, only 5 out of every 100 children diagnosed with West syndrome will not survive beyond the age of five years.

How many times a day do infantile spasms occur?

To that end, here's what pediatricians should look for:

Infantile spasms often occur in clusters, with each spasm occurring every five to 10 seconds over a period of minutes ranging from three to 10 minutes or longer.

Do infantile spasms happen while sleeping?

Infantile spasms often occur when a child is falling asleep or waking up. The child's body may suddenly jerk, flex or extend. Sometimes, the arms are flung out, the knees are pulled up and the body bends forward. Less often, the head can be thrown back while the body and legs stiffen to a straight position.

What happens if you don't treat infantile spasms?

If not treated, the disorder can permanently harm development. The main treatments include hormonal therapy and vigabatrin, an anti-seizure medicine. Infantile spasms may occur in children without prior medical problems. However, most children get infantile spasms after the diagnosis of another brain disorder.

What ethnicity is more likely to have Down syndrome?

Down Syndrome rates increased over time among individuals who identify as Black, Hispanic, or AIAN, but not among white or Asian individuals. Whether accessing and navigating changes in Down syndrome testing is contributing to these disparities in outcomes needs further study.

Do babies cry after infantile spasms?

Infantile spasms often happen one after another in a cluster with five- to 10-second pauses in between spasms. After a spasm or series of spasms, your baby may appear upset or cry — but not always. In some cases, infantile spasms are very subtle and difficult to notice.

Does West syndrome cause mental retardation?

Only 14% of infants with symptomatic West syndrome have normal or borderline-normal cognitive development, compared with 28-50% of infants with idiopathic infantile spasms. Mental retardation is severe in 70% of patients, often with psychiatric problems such as autistic features or hyperactivity.

What is the difference between West syndrome and infantile spasms?

Infantile spasms (also called IS) are also known as West syndrome because it was first described by Dr. William James West in the 1840s. These seizures may be subtle and be confused with other normal baby behaviors or colic.