West syndrome is a rare form of epilepsy that affects newborns, with symptoms appearing three to twelve months after birth. It is caused by abnormal interactions between the brainstem and cortex, and treatment involves a combination of medications, a ketogenic diet, and surgery. The prognosis varies on an individual basis, with about 50% of children becoming seizure-free with medication and 5% dying before the age of five.
West syndrome is a rare disease that causes epilepsy in newborns. The syndrome is also known as generalized flexion epilepsy, infantile encephalopathy, and infantile spasms. Symptoms of epilepsy appear three to twelve months after birth in ninety percent of babies who develop West syndrome. About two percent of children with epilepsy have this form of the condition. The global prevalence rate is two in 10,000 children under ten.
The main cause of the syndrome is thought to be the abnormal interaction between the brainstem and cortex. These abnormal interactions usually result from brain defects or lesions that can occur before birth, during birth, or after birth. An additional factor is that in infants and children 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 can also accompany the syndrome. This is not due to the syndrome itself, however, but is usually a reflection of the brain injury causing the symptoms of epilepsy.
Treatment of West syndrome is done to improve the quality of life, as a complete cure cannot usually be provided. Treatment involves a combination of several medications, which may include prednisone, benzodiazepines, and vitamin B-6. The goal of treatment is to reduce the frequency of seizures, while keeping the number of medications as low as possible to minimize adverse side effects.
In addition to medications, there are other possible treatments for the syndrome. A ketogenic diet, in which carbohydrate intake is reduced in favor of increased fat and protein intake, is effective for some people with epileptic disorders. Treatment of last resort is surgery called a focal cortical resection, in which the area of the brain where the seizures originate is removed. Focal cortical resection is done only when other treatments have failed and only when brain tissue can be removed without damaging other important areas of the brain.
The prognosis of West syndrome is considered only on an individual basis. With a variety of different causes and possible outcomes, there is no overall prognosis. Statistically, about fifty percent of children who develop the syndrome can be seizure free with medication. About five percent of children will die before they reach the age of five from the brain injury causing the syndrome or from side effects of treatment.
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