West Syndrome - What It Is, Symptoms and Treatmentsof this disease that many are unaware of. In additionWest Syndromeis a severe form of epilepsy that develops in people when still children. The name was given in honor of the English doctor William James West, the first scholar to write about the disorder. Related to the baby's age, this syndrome usually manifests between the third and twelfth month. THEWest SyndromeIt has several causes, and the most common is the one related to organic changes of the brain caused in prenatal, perinatal or postnatal.
Statistically it has been shown that boys are more affected withWest Syndromethan the girls. Clinical signs involve spasms, developmental delay and electroencephalographic tracing with hypersarrhythmia pattern. The crises cause flexion of the head, abduction of the upper limbs and folding of the legs. Small screams are also common during spasms. Each crisis takes about a few seconds, enough to drive the parents crazy.
Causes of West Syndrome:
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Tuberous sclerosis, an inherited autosomal dominant condition, is the most common prenatal cause of childhood spasms. In those with cryptogenic spasms no specific cause is identified, although it can be said to exist and epilepsy is symptomatic.
Children with idiopathic spasms have normal psychomotor development before the onset of symptoms and no cause is found or presumed. A family history of childhood spasms is uncommon, but about 17% of patients may have a history of other forms of epilepsy. A detention or regression of psychomotor development accompanies spasms in 70 to 95% of patients..
Most of the time the causes of the disease are unknown, although in some circumstances it may be due to:
- Previous trauma.
- Hypoxia (lack of oxygen) at birth.
- Infections during childbirth or pregnancy (toxoplasmosis, cytomegalovirus, rubella, herpes, syphilis, etc.).
Symptoms of West Syndrome:This disorder consists of a triad of clinical manifestations involving spasms, developmental delay and electroencephalographic tracing with hypsarrhythmia pattern. The child has constant crises that last in average a few seconds, being these worse at night or soon to wake up.The characteristics of the crisis are as follows:
- Extension and sudden raising of the head;
- Flexion of the legs;
- Abduction of upper limbs;
- I scream or cry during the spasms.
The contractions are symmetrical and brief. The upper limbs move forward and out, while the muscles of the abdomen contract. The crises decrease with maturation of the child, however, tend to return on average in the fourth or fifth year of life. In the face of these signs it is extremely important to seek medical help. A professional needs to assess the child's health and check the best approach to treatment. The vast majority of diseases, when detected early, have a positive prognosis..
West Syndrome Treatments:The goal of treatment for people withWest Syndromeis to eliminate spasms with fewer medications and fewer possible side effects. Medications such as conventional antiepileptics and ACTH are the mainWest Syndrome, but there are also other drug possibilities.
In some patients resection of a localized brain area may prevent seizures. Physical therapy is intended to treat or attempt to reduce sequelae. Another goal of physical therapy is to try to avoid deformities by doing passive mobilization and stretching. Hydrotherapy can also help in the solution of existing spasticity..
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How to Prevent West Syndrome?Almost always there is no way to preventWest Syndrome. In symptomatic cases, one must avoid possible causal factors..
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