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West’s syndrome is an epileptic disorder that results from damage to the brain. This disease occurs in children and infants and is a serious disease that is difficult to treat. What are the causes of West’s syndrome and how do you recognize the symptoms of the disease in a child? What is the prognosis and treatment for West syndrome?
What is West’s syndrome?
West syndrome is defined as one of the more severe cases of epileptic encephalopathy and is a type of childhood epilepsy. This disease entity is named after William James West, the physician who first described the disease in 1841 in the Lancet journal. The case he described concerned the person closest to him, because it was his own son. West’s syndrome is a disease that is diagnosed in an average of 1 in 3500 children, and boys are much more often diagnosed with the disease. West syndrome usually appears between three months and one year of age. The younger the child is, the worse the prognosis of the disease is. A characteristic feature of West’s syndrome are flexion seizures occurring at different frequencies and progressive psychomotor retardation of the child, which is the result of damage to the nervous structures.
West’s syndrome – causes of the disease
Determining the causes of West syndrome is not the easiest thing to do, as it is caused by all conditions that are associated with organic changes in the brain. However, specialists point to the most common causes of West syndrome and among them:
- causes caused by prenatal factors – these are any genetic mutations, metabolic diseases, infections, Down syndrome, brain developmental disorders, hypoxia and cerebral ischemia;
- causes caused by perinatal factors – hypoglycemia or perinatal hypoxia;
- causes caused by postnatal factors – brain injuries, phenylketonuria, tumors of the central nervous system, neuroinfections, cerebral ischemia;
- causes caused by idiopathic factors.
West’s syndrome – symptoms
The most characteristic symptom of West syndrome are flexion seizures. When such a seizure occurs, there is a sudden spasm of the flexor muscles. It is visible in the child by the bending of the head with the torso towards the front. Limbs are also bent. The baby’s legs usually bend towards the torso, while the hands are thrown forward. The seizures in West syndrome occur in series and often occur in times of awakening or falling asleep. Additional symptoms that can be observed in the case of West syndrome are: disturbance of consciousness, drooling, vomiting, sudden redness of the face, increased sweating, and psychomotor restlessness. The effect of the lack of proper treatment of West’s syndrome is another symptom, i.e. inhibition of psychomotor development.
Treatment of West’s syndrome
Treatment of West’s syndrome is a long-term process, the effect of which is based on the reduction of epileptic seizures and the improvement of the psychomotor functioning of the child. The treatment of West’s syndrome is extended to include a ketogenic diet, i.e. a diet rich in fat and low in carbohydrates. Unsaturated fats improve the functioning of the nervous system and improve the processes of creating new nerve connections. In the treatment of West syndrome, hormonal treatment and stimulation of the vagus nerve are also used. In some cases, the doctor decides to perform a neurosurgical operation that aims to destroy epileptic foci in the patient’s brain.
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