West syndrome is a rare disease characterized by frequent epileptic seizures, which is more common among boys and begins to manifest during the first year of the baby's life. Generally, the first crises occur between 3 and 5 months of life, although the diagnosis can be made until 12 months.
There are 3 types of this syndrome, the symptomatic, idiopathic and the cryptogenic, and in the symptomatic the baby has a cause such as the baby had not been breathing for a long time at birth; cryptogenic is when it is caused by some other brain disease or abnormality, and the idiopathic is when the cause cannot be discovered and the baby may have normal motor development, such as sitting and crawling.
Main features
The hallmarks of this syndrome are delayed psychomotor development, daily epileptic seizures (sometimes more than 100), in addition to tests such as the electroencephalogram that confirm the suspicion. About 90% of children with this syndrome usually have mental retardation, autism and oral changes are very common. Bruxism, mouth breathing, dental malocclusion and gingivitis are the most common changes in these children.
The most frequent is that the bearer of this syndrome is also affected by other brain disorders, which can hinder treatment, having a worse development, being difficult to control. However, there are babies if they recover completely.
Causes of West syndrome
The causes of this disease, which can be caused by several factors, are not known for certain, but the most common are problems at birth, such as lack of cerebral oxygenation at the time of delivery or shortly after birth, and hypoglycemia.
Some situations that seem to favor this syndrome are brain malformation, prematurity, sepsis, Angelman syndrome, stroke, or infections such as rubella or cytomegalovirus during pregnancy, in addition to the use of drugs or excessive alcohol consumption during pregnancy. Another cause is the mutation in the gene Aristaless-related homeobox (ARX) on the X chromosome.
How the treatment is done
Treatment for West Syndrome should be started as soon as possible, because during epileptic seizures the brain can suffer irreversible damage, seriously compromising the baby's health and development.
The use of medications such as adrenocorticotrophic hormone (ACTH) is an alternative treatment, in addition to physiotherapy and hydrotherapy. Medications such as sodium valproate, vigabatrin, pyridoxine and benzodiazepines can be prescribed by the doctor.
Is West syndrome curable?
In the simplest cases, when West syndrome is not related to other diseases, when it does not generate symptoms, that is, when its cause is not known, being considered idiopathic West syndrome and when the child receives treatment initially, soon when the first crises appear, the disease can be controlled, with a chance of cure, without the need for physical therapy, and the child may have a normal development.
However, when the baby has other associated diseases and when his health is serious, the disease cannot be cured, although treatments can bring more comfort. The best person to indicate that the baby's health status is the neuropediatrician who, after evaluating all the exams, will be able to indicate the most suitable medicines and the need for psychomotor stimulation and physiotherapy sessions.
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