What is the life expectancy of a child with West syndrome?

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. A study of 214 Finnish children over 25 years of age showed about 61% dying at or before age 10 years.

Do babies 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.

Is West syndrome fatal?

It's rare, but some children who had West syndrome as babies will die before adulthood. It usually happens before the age of 10.

How long do babies with infantile spasms live?

Most noticeable in newborns and slowly disappears by four to six months of age. Often start occurring between the ages of three and 12 months and continue.

Is West syndrome a disability?

West syndrome is a constellation of symptoms characterized by epileptic/infantile spasms, abnormal brain wave patterns called hypsarrhythmia and intellectual disability.

Life with West Syndrome | Fighting for Families Series | The Baby Cubby

Is West syndrome terminal?

About five in every 100 infants and children with West syndrome do not survive beyond five years of age. A study of 214 Finnish children over 25 years of age showed about 61% dying at or before age 10 years.

Do infantile spasms cause brain damage?

Infantile spasms are a complex and rare disorder that can have very serious consequences. It can lead to death in some children and cause intellectual disabilities and developmental problems in others. Even if the seizures are managed, the damaging brain effects can remain.

Do infantile spasms cause mental retardation?

A new study found that nearly half of babies who suffer from infantile spasms are not accurately diagnosed for more than a month and this delay can increase the risk of intellectual disability, autism, lifelong epilepsy, and death.

What is the difference between West syndrome and infantile spasms?

(See Presentation and Workup.) West syndrome is an age-dependent expression of a damaged brain, and most patients with infantile spasms have some degree of developmental delay. The term infantile spasm has been used to describe the seizure type, the epilepsy syndrome, or both.

Can you grow out of infantile spasms?

Infantile spasms usually stop by age five, but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause.

Can infantile spasms come back after treatment?

The spasms will have disappeared by the age of 14 months in about three quarters of infants with this disorder. After this age the spasms can still come back but this is not common. infantile spasms can continue through to school age if they do not respond to treatment but eventually they do stop on their own.

How do you prevent infantile spasms?

Infantile spasms usually are treated with seizure medicines or steroids. If medicines don't control the spasms, a special diet, such as the ketogenic diet, might help. Sometimes, doctors may recommend surgery.

What is the best treatment for infantile spasms?

How often do infantile spasms occur in a day?

Babies may have as many as 100 spasms a day. The seizures may be more likely to happen just as the baby is waking up. 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.

How is West syndrome treated?

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 infections cause infantile spasms?

Infections were considered to be etiological factors in 29 patients (10%) with infantile spasms; congenital CMV (n = 5), congenital or acquired CMV (n = 1), acquired CMV (n = 5), congenital rubella (n = 2), herpes simplex virus (n = 5), enterovirus (n = 1), adenovirus (n = 1), viral encephalitis of unknown agent (n = 3 ...

Is West syndrome caused by vaccines?

In addition, decades of epidemiological research into the issue presented in this case—whether pertussis vaccination causes West Syndrome—has not yielded reliable evidence of a causal link.

What causes sudden death in epilepsy?

A seizure may cause a person to have pauses in breathing (apnea). If these pauses last too long, they can reduce the oxygen in the blood to a life-threatening level. In addition, during a convulsive seizure a person's airway sometimes may get covered or obstructed, leading to suffocation.

Are infantile spasms genetic?

Infantile Spasm Causes and Risk Factors

Some infants may have had a brain injury, such as a stroke or meningitis. Others may have been born with a genetic mutation or have problems with brain development. Certain medical and genetic conditions, like Down syndrome, increase the risk of having infantile spasms.

Can you interrupt an infantile spasm?

There is no treatment to stop a cluster of infantile spasms after it has started. During a cluster, you can hold your baby or place them on a soft surface (Picture 1). Also, if possible, you should: Take a video of the cluster for the doctor to see.

Do infantile spasms make babies cry?

Infantile spasms only last a second or two but often come in a cluster (multiple spasms in a row). Infantile spasms commonly occur around times of sleep, especially upon awakening. After a spasm, babies will often be irritable or cry.

At what age do infantile spasms start?

Infantile spasms, first described with a group of symptoms known as West syndrome, is a form of epilepsy ​that occurs in 1 in 2,000 children. It typically begins between 2-12 months of age and peaks between 4-8 months of age.

Do infantile spasms hurt?

They may have many series of spasms per day. This type of seizure is usually only seen in young children. After a spasm, the baby may cry or laugh. The spasms do not cause pain, but the baby may cry because the quick jerking movement surprises them.

Can infantile spasms cause autism?

The research also hints that treatments for infantile spasms could be effective in treating ASD and should be studied as a potential therapy for autism in general. Children who suffer from infantile spasms have a 30% to 40% risk of developing ASD, according to studies.

Will infantile spasms always show on EEG?

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). This pattern is called 'hypsarrhythmia'.