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West syndrome is a rare disorder that causes epilepsy in infants. The syndrome is also known as generalized flexion epilepsy, infantile encephalopathy, and infantile spasms. Epilepsy symptoms manifest between three and twelve months after birth in ninety percent of children who develop West syndrome. Approximately two percent of children with epilepsy have this form of the condition. Overall prevalence rate is two per 10,000 children under ten.
The root cause of the syndrome is thought to be abnormal interaction between the brainstem and the cortex. These abnormal interactions usually result from brain defect or injury that may occur prenatally, during birth, or after birth. An additional factor is that in newborns and infants the central nervous system has not yet matured; this is thought to be important in the development of the syndrome.
The most common symptoms of the disorder are epilepsy and spasms. West syndrome does not have a specific spectrum of symptoms that appear, but physical and mental developmental delay may also accompany the syndrome. This is not due to the syndrome itself, however, but is usually a reflection of the brain injury that causes the epilepsy symptoms.
West syndrome treatment is carried out to improve quality of life, as it is usually not possible to provide a complete cure. Treatment comprises a combination of several different medications, which may include prednisone, benzodiazepines, and vitamin B-6. The goal of treatment is to reduce the frequency of seizures, while keeping number of medications as low as possible to minimize adverse side effects.
In addition to medication there are other possible treatments for the syndrome. A ketogenic diet, in which carbohydrate intake is reduced in favor of increased intake of fats and protein, is effective for some people with epileptic disorders. The last resort treatment is a surgery called focal cortical resection, in which the area of the brain where seizures originate is removed. Focal cortical resection is only carried out when other treatments have failed, and only when it is possible to remove the brain tissue without damaging other important areas of the brain.
West syndrome prognosis is only considered on an individual basis. With a variety of different causes and possible outcomes, there is no general prognosis. Statistically, around fifty percent of children who develop the syndrome can become seizure-free with medication. Approximately five percent of children will die before they reach the age of five, due to the brain injury causing the syndrome, or side effects from treatment.