What is West syndrome?

West syndrome, also known as infantile spasm, is a very uncommon epileptic seizure disease that was first described by Dr. W. West (in 1841) in his own son.

Who gets West syndrome?

West Syndrome is an age related disease in infants. Symptoms of the syndrome appear between 3 and 12 months of age, usually occurring at the fifth month.

What are the signs and symptoms of West syndrome?

The following conditions are symptoms of West syndrome:

What causes West syndrome?

Any type of brain damage can cause West syndrome, for example:

  • Severe brain injury at birth
  • Brain malformations
  • Metabolic disease
  • Chromosome abnormalities
  • Tuberous sclerosis (a genetic condition)

Cryptogenic West syndrome is the term used when the cause of the syndrome cannot be identified.

Is there a test to diagnose West syndrome?

The diagnosis is made if the infant or child has at least two of the three symptoms of hypsarrhythmia, mental retardation, or infantile seizures.

Girl with epilepsy

What Types of Seizures Start in Infancy?

Several types of seizures affect infants and children. West syndrome or infantile spasms is the most common type of seizure in infants and children. Infantile seizures are clusters of seizures that usually begin before the age of 6 months. During these seizures the infant may bend and cry out.

What is the treatment for West syndrome? Is there a cure?

Since there are so many potential causes of West syndrome, treatment is based on the cause. There is no cure for West syndrome, so unfortunately, the only treatment is to reduce symptoms.

West syndrome treatments usually include a course of prednisolone and/or an anti-epileptic medication. In a few individuals, surgery in the brain may help reduce symptoms.

What is the prognosis and life expectancy for West syndrome?

Due to the various causes of West syndrome, each individual will have a somewhat different prognosis. However, children that can become free of attacks with appropriate medication (about 50% of all individuals with West syndrome) have a fair prognosis.

Individuals that do not have EEG abnormalities and show early response to treatment have a better prognosis. 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. A study of 214 Finnish children over 25 years of age showed about 61% dying at or before age 10 years.

What are other names for West syndrome?

West syndrome has had many different names, including:

  • Infantile epileptic encephalopathy
  • Infantile myoclonic encephalopathy
  • Generalized flexion epilepsy
  • Massive myoclonia
  • Salaam spasms
  • Jackknife convulsions
  • Infantile spasms

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References


Glauser, Tracy A., MD. Infantile Spasm (West Syndrome). Medscape. Updated: Oct 16, 2014.
<https://emedicine.medscape.com/article/1176431-overview>

NORD. West Syndrome. Updated 2015.
<https://rarediseases.org/rare-diseases/west-syndrome/>

Syndrome. West Syndrome.
<http://syndrome.org/west-syndrome/>
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