West syndrome is a rare disease characterized by frequent epileptic seizures, being more common among boys and beginning to manifest during the first year of the baby's life. Usually the first crises happen between the 3 and 5 months of life, although the diagnosis can be made until the 12 months.
There are 3 types of this syndrome, the symptomatic, idiopathic and cryptogenic, and in the symptomatic the baby presents a cause as the baby has been a long time without breathing at birth; cryptogenic is when it is caused by some other disease or brain abnormality, and the idiopathic is when the cause can not be discovered and the baby may exhibit normal, motor-like crawling and crawling.
Main features
The hallmark characteristics of this syndrome are delayed psychomotor development, daily epileptic seizures (sometimes more than 100), and tests such as the electroencephalogram that confirm the suspicion. About 90% of children with this syndrome usually have mental retardation, autism and oral disorders are very common. Bruxism, mouth breathing, dental malocclusion and gingivitis are the most common changes in these children.
The most frequent is that the carrier of this syndrome is also affected by other cerebral disorders, that can make difficult the treatment, having a worse development, being of difficult 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, but the most common are problems at birth, such as lack of cerebral oxygenation at the time of delivery or soon after birth, and hypoglycaemia.
Some situations that seem to favor this syndrome are poor brain training, prematurity, sepsis, Angelman syndrome, stroke, or infections such as rubella or cytomegalovirus during pregnancy, as well as drug use or excessive alcohol consumption during pregnancy. Another cause is the mutation in the Aristaless-related gene homeobox (ARX) on the X chromosome.
Treatment for West Syndrome
Treatment for West Syndrome should be started as early as possible because during epileptic seizures, the brain may suffer irreversible damage, severely compromising the baby's health and development.
The use of medications such as adrenocorticotrophic hormone (ACTH) is an alternative treatment, as well as physiotherapy and hydrotherapy. Remedies such as sodium valproate, vigabatrin, pyridoxine and benzodiazepines may be prescribed by your doctor.
West's syndrome has a cure?
In the simplest cases, when the West syndrome is not related to other diseases, when it does not generate symptoms, that is, when its cause is unknown, being considered West syndrome idiopathic and when the child receives the treatment initially, soon when the first crises arise, the disease can be controlled, there is a chance of healing, and physical therapy is not necessary, and the child can develop normally.
However, when the baby has other associated illnesses and when his health condition is severe, the illness can not be cured, although the treatments may bring more comfort. The best person to indicate that the state of health of the baby is the neuropediatra who after evaluating all the exams, may indicate the most indicated remedies and the need for psychomotor stimulation and physiotherapy sessions.
