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.

What is West baby syndrome?

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

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.

Can adults have West syndrome?

Complications of West syndrome Clinical spasms very rarely persist in adulthood. In fact, these spasms, as well as the typical electroencephalogram (EEG) pattern of West syndrome, spontaneously disappear by 3 to 4 years of age.

Is West syndrome genetic?

Genetics of West syndrome Mutations in least 100 individual genes have been identified so far that cause West syndrome. Some of these genes include CDKL5, SCN2A, SCN8A and STXBP1. Tuberous sclerosis, which is caused by pathogenic variants in the genes TSC1 and TSC2, can also lead to West syndrome.

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.

Do babies outgrow infantile spasms?

Who gets it? Infantile spasms are considered an age-specific epilepsy. They typically begin in an infant between 3 and 8 months of age. In most children, IS starts by 1 year of age and usually stop by 2 to 4 years of age.