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.

Does West syndrome go away?

West syndrome (also called infantile spasms) should go away by the time your child is 4 years old. But most people who had it will get another kind of epilepsy or seizure condition in childhood or as an adult.

What is the cause of West syndrome?

Any disorder that can lead to brain damage can be an underlying cause of West syndrome including trauma, brain malformations such as hemimegalencephaly or cortical dysplasia, infections, chromosomal abnormalities such as Down syndrome, neurocutaneous disorders such as tuberous sclerosis complex (TSC), Sturge Weber …

What is the prognosis of infantile spasms?

The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms. Children who have rapid initiation of treatment, normal development prior to infantile spasms, and no identifiable cause may do well.

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Can you live a normal life with infantile spasms?

An etiologic diagnosis is very important because it can lead to initiation of a specific therapy that may markedly improve the long-term developmental outcome. In fact, some children with infantile spasms may ultimately lead normal lives, but only if they are diagnosed and treated correctly.

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 rare?

West Syndrome Causes and Risk Factors

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.

What is the life expectancy for Lennox Gastaut Syndrome?

The progressive intellectual impairment associated with Lennox-Gastaut syndrome means that many patients require full-time care throughout life. The short-term mortality of Lennox-Gastaut syndrome is approximately 4-7%. This is largely due to seizure-related fatal accidents.

What is Doose Syndrome?

Myoclonic astatic epilepsy (MAE), also known as Doose syndrome, is an epilepsy syndrome of early childhood, most commonly appearing between ages 1 and 5 and featuring generalized seizures. Children will experience drop attacks and staring seizures, sometimes associated with falls.

How is West Syndrome diagnosed?

The diagnosis of West syndrome is made on a combination of the clinical features together with a typical EEG. The EEG shows a very disorganised pattern, called hypsarrhythmia. The EEG is always abnormal in children with West syndrome but sometimes the abnormality is seen only during sleep.

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What happens if infantile spasms are left untreated?

Left untreated, infantile spasms can lead to serious outcomes, including an estimated infant mortality rate of between 5% and 6%. The most significant concern, however, is that infantile spasms are associated with autism and intellectual deficits that permanently affect quality of life.

What is the best treatment for infantile spasms?

Treatment of infantile spasms has little class I data, but adrenocorticotropic hormone (ACTH), prednisolone and vigabatrin have the best evidence as first-line medications. Other therapies including the ketogenic diet and other anti-epileptics medications may also prove useful in the treatment of infantile spasms.

What does an infantile spasm look like?

Infantile spasms often look like a sudden, brief stiffening of a baby’s muscles. Symptoms may include: a cluster of spasms that may be associated with waking from sleep. jackknife seizures, where the body bends forward, the knees are pulled up, and the arms are thrown out to the side.

What age do infantile spasms start?

What are infantile spasms? 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.

How do infantile spasms damage the brain?

Most children with infantile spasms will have disorganized brain wave activity. This is known as modified hypsarrhythmia. Very chaotic brain wave activity to a milder response, known as hypsarrhythmia, can be seen in about two-thirds of children with the disorder.

Can infantile spasm be cured?

Many children with infantile spasms develop other kinds of epilepsy. (Watch examples of infantile spasms.) “Some of these children can be cured, but successful treatment often depends on prompt diagnosis,” said Hussain.

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