West syndrome is a rare disease characterized by frequent epileptic seizures, most common in children and beginning to appear during the baby’s first year of life; The first seizures usually occur between 3 and 5 months of life, although diagnosis can be made up to 12 months.
There are 3 types of this syndrome, symptomatic, idiopathic and cryptogenic, and in the symptomatic the baby has such a cause that the baby has been out of breath for a long time; Cryptogenic is when it is caused by another disease or abnormality of the brain, and idiopathic is when the cause cannot be discovered and the baby may have a normal motor development, such as sitting and crawling.
- The hallmarks of this syndrome are a delay in psychomotor development.
- Daily epileptic seizures (sometimes more than 100).
- As well as tests such as the electroencephalogram confirming suspicion.
- About 90% of children with this syndrome often have mental retardation.
- Autism and oral disturbances.
- Very common Bruxism.
- Oral breathing.
- Denity malocclusion and gingivitis are the most common changes in these children.
Most commonly, the carrier of this syndrome is also affected by other brain disorders, which can interfere with treatment, have worse development, be difficult to control; However, there are babies if they heal completely.
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 brain oxygenation at the time of delivery or shortly after birth, and hypoglycemia.
Some situations that seem to promote this syndrome are brain malformations, prematurity, sepsis, Angelman syndrome, stroke or infections such as rubella or cytomegalovirus during pregnancy, in addition to drug use or excessive alcohol use during pregnancy; another cause is the Mutation of the Aristaless (ARX) homeobox gene on the X chromosome.
Treatment for West syndrome should be initiated as soon as possible, as during epileptic seizures, the brain can suffer irreversible damage, severely committing the health and development of the baby.
The use of drugs such as the hormone adrenorticotropic (ACTH) is an alternative treatment, in addition to physiotherapy and hydrotherapy.Your doctor may prescribe medications such as sodium valproate, vigabathrin, pyridoxine, and benzodiazepines.
In simpler cases, when West syndrome is not related to other diseases, when it does not generate symptoms, that is, when its cause is unknown, it is considered an idiopathic West syndrome and when the child is initially treated, then when the first seizures appear, the disease can be controlled, with chances of recovery., without the need for physical therapy, and the child may have normal development.
However, when the baby has other associated diseases and his or her health is severe, the disease cannot be cured, although treatments can bring more comfort.The person best positioned to indicate the health of the baby is the neuropediatra who, after evaluating all the tests, will be able to indicate the most appropriate medications and the need for psychomotor stimulation and physiotherapy sessions.