What is West syndrome?

West syndrome is considered an epileptic encephalopathy, a condition in which the epileptic seizures can directly contribute to cognitive and behavioral impairments. Early and effective control of the seizures can improve the child's long-term outlook.

What is West syndrome life expectancy?

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.

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.

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.

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 is West syndrome

Do children 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.

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).

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 another name for West syndrome?

Infantile spasms are a very specific type of seizure with a characteristic age of onset (a typical age when seizures start). They are nearly always accompanied by a very characteristic pattern on the electroencephalogram (EEG).

How common is West syndrome?

This condition is rare. It affects fewer than 6 babies out 10,000. 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.

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.

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 life expectancy of infantile spasms?

About 5–30% of children with infantile spasms die within 12 months of treatment; 50% of the deaths are related to the medical treatment, and mortality is greater in infants with symptomatic causes.

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 child with Williams syndrome live a normal life?

Most people with Williams syndrome have a normal life expectancy, but some may have a reduced life expectancy due to complications of the disease (such as cardiovascular problems).

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.

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.

Is infantile spasms related to autism?

BALTIMORE – A severe form of epilepsy known as infantile spasms may increase the risk of children developing autism spectrum disorder (ASD) due to the condition's unique seizures and resulting chaotic brainwaves, suggests research being presented at the American Epilepsy Society Annual Meeting.

What can be mistaken for infantile spasms?

Infantile spasms are caused by a condition in a baby's brain and include repetitive but often subtle movements—such as jerking of the mid-section, dropping of the head, raising of the arms, or wide-eyed blinks. IS can be misdiagnosed as colic, reflux, or a startle reflex.

Can stress cause infantile spasms?

Acknowledgment of the diverse and multiple etiologies led to the hypothesis that stress in the developing brain is a common factor in the development of infantile spasms (10–12). Stress increases release of an endogenous hormone, corticotropin-releasing hormone (CRH).

What happens if you don't treat infantile spasms?

Left untreated, infantile spasms may result in developmental delays, intellectual disability and lifelong epilepsy. “Infantile spasms may be due to underlying neurological problems. However, for some, they are an isolated problem in an otherwise healthy child.

Do infantile spasms happen when baby is falling asleep?

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.

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.