It is very unfortunate that during childhood there may be a disease, which if not detected in time can cause serious neurological damage and even death to children under 5 years old This pathology that I want to talk to you about today is known as Syndrome from West and I want to tell you Signs that alert parents to West syndrome in children.
He West syndrome is an epileptic encephalopathy characterized by a triad: seizures or infantile spasms, psychomotor retardation and electroencephalogram abnormalities, although any of the three may be missing. I say that it is a disease that saddens our children, because it manages to erase the smile from their faces, due to the presence of said muscle spasms.
The name of this syndrome is due to the English doctor, William Jame West (1793 - 1848), who discovered it for the first time, in his own daughter. Its incidence is 1 in every 4 to 6 thousand children and it is more frequent in males. Regarding the mortality of this syndrome, it covers 5% of patients.
The seizures or spasms start from 3 to 8 months (in some children from birth), up to 5 years of age, but with the risk that they can develop into another type of seizure pathology (epilepsy).
10% could evolve to a completely normal life and about half of West syndrome patients could develop epileptic syndromes, such as Lennox Gastart syndrome, with severe sequelae of mental retardation and severe epilepsy.
The causes are due to pathologies that occur around pregnancy and after birth, such as prenatal (cerebral dysplasia, chromosomal abnormalities, infectious or metabolic diseases or ischemia), perinatal (hypoglycemia or hypoxic-ischemic encephalopathy) and / or the less frequent, postnatal (brain tumor, hemorrhage, viral or bacterial infections).
As I said at the beginning, the symptoms of this syndrome are based on a triad (although at the time of making the diagnosis, one of the three symptoms may be missing):
They are sudden contractions, generally symmetrical at the level of the neck, trunk and extremities. And they are usually accompanied by loss of consciousness. The spasms or seizures can be of: flexion, extension or mixed.
When the spasms begin, there is a sudden bending forward with stiffness of the neck, trunk and extremities and there may be a deviation of the gaze. Seizures generally occur on awakening and after meals (rarely before sleep or during sleep) and can occur in series or be separated by 5 to 30 second intervals and last more than 10 minutes.
There are cases where psychomotor retardation can occur even before epileptic spasms. The acquired motor skills are lost and there are neurological alterations (they do not turn around, they do not sit, the reflexes are altered).
The baby's mood changes: they lose their smile, become irritable, cry for no reason, do not follow objects with their eyes and present sensory disturbances. There are also changes in sleep patterns, where the patient does not sleep the appropriate hours, his sleep is restless and with sudden awakenings.
Known as hypsaarrhythmias, translated into a slowing down and disorganization of brain electrical activity.
The goal of treatment is to reduce or eliminate seizures and give these little ones a better quality of life. If detected at an early age, with corticosteroid therapy, vigabatrin, and anticonvulsant drugs, 10% of patients can regain a normal life. When the cause is due to localized brain lesions, removing the lesion can eliminate seizures and give the patient quality of life.
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