The effective treatment of West syndrome still poses a great challenge in pediatric neurology. In the overall long-term view, the majority of patients with this syndrome suffer a poor outcome due to chronic epilepsy, mental retardation, and other neurodevelopmental shortcomings.
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However, the prognosis for each West syndrome case is greatly dependent on the underlying etiology of the seizures. Therefore, each case of symptomatic, cryptogenic, and idiopathic West syndrome must be considered on an individual basis. For example, while 28-50% of infants with idiopathic West syndrome will have normal or near-normal cognitive development, this value is significantly reduced to 14% in those suffering from symptomatic 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. However, up to 50-70% of children diagnosed with infantile spasms will develop other types of seizures.
In addition, mental retardation, which is usually of a profound nature, also occurs in 70-90% of patients in the future, accompanied by psychiatric problems. Even though epileptic spasms usually reduce in number by mid-childhood, 18-50% of children will develop Lennox-Gastaut syndrome or some other form of symptomatic generalized epilepsy.
Little Lulu’s 'West Syndrome' Battle | Studio 10
For an unknown reason, West syndrome occurs in 1-5% of children with Down's syndrome who may also have a more positive prognosis. In fact, these individuals are less likely to develop Lennox-Gastaut syndrome or other forms of epilepsy. On the other hand, if their seizures are difficult to control, the child is at risk of developing an autism spectrum disorder.
However, the early detection and prompt administration of effective treatment have been shown to improve the resultant neurodevelopmental outcomes, such as severe-to-profound retardation. Currently, approximately half of all children with West syndrome become entirely free of the attacks with the aid of medication. Notably, the number of children who would benefit from these treatments would be an even higher value if the optimal regimen for treatment was known.
Early pharmacological action would be particularly beneficial for idiopathic cases where, in general, children have the most promising prognosis. In fact, 28-50% of children with idiopathic West syndrome have normal or borderline-normal cognitive development following diagnosis, which is comparable to only 14% of children with symptomatic West syndrome.
In addition to underlying etiology, treatment is also known to influence the outcome a patient faces later in life. For example, a good prognosis might be expected if the age of symptom onset is older than 4 months, there is an absence of EEG abnormalities, spasms are of short duration, or if there is a rapid response to treatment (less than a month) and without recurrence. Therefore, regardless of what the underlying cause is, the aim of treatment is to achieve control of the seizures as soon as possible, particularly if there is a hope for normal intellectual development.
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.