West Syndrome – What is it, Symptoms and Treatments!

West Syndrome – What it is, Symptoms and Treatments of this disease that many are unaware of. Furthermore, West Syndrome  is a severe form of epilepsy that develops in people when they are still children. The name was given in honor of the English physician 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. West Syndrome  It has several causes, the most common being linked to organic changes in the brain caused prenatally, perinatally or postnatally.

It has been statistically proven that boys are more affected with West Syndrome than  girls. Clinical signs involve spasms, developmental delay and electroencephalographic tracing with a hypsarrhythmia pattern.

The seizures provoke flexion of the head , abduction of the upper limbs and bending of the legs. Small cries during spasms are also frequent. Each crisis lasts about a few seconds, enough to drive parents crazy.

Causes of West Syndrome:  People who are carriers of these symptomatic spasms are those who have an identifiable etiologic factor that is responsible for West Syndrome . These factors are multiple forms of aggression to the brain in the pre- or postnatal period, such as infections , trauma, etc. About 70 to 75% of children with infantile spasms have the symptomatic form of the disorder.

Tuberous sclerosis, an autosomal dominant hereditary condition, is the most common prenatal cause of infantile spasms. In those with so-called cryptogenetic spasms, no specific cause is identified, although it can be said that it exists and that the 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 infantile spasms is uncommon, but about 17% of patients may have a history of other forms of epilepsy . An arrest or regression of psychomotor development accompanies the spasms in 70 to 95% of patients.

Most of the time the causes of the disease are not known, although in some circumstances it may be due to:

• Previous injuries.
• Hypoxia (lack of oxygen) at birth.
• Infections during childbirth or pregnancy (toxoplasmosis, cytomegalovirus, rubella, herpes, syphilis, etc.).
• Meningitis.

Symptoms of West Syndrome: This disorder comprises a triad of clinical manifestations involving spasms, developmental delay, and electroencephalographic tracing with a hypsarrhythmia pattern. The child has constant seizures that last on average a few seconds, which are worse at night or right after waking up. The characteristics of the crisis are as follows:

• Extension and sudden elevation of the head ;
• Flexion of the legs;
• Abduction of the upper limbs;
• Scream or cry during spasms.

Contractions are symmetrical and brief. The upper limbs move forward and out, while the abdominal muscles contract. The seizures decrease with the child’s maturation, however, they tend to return on average in the fourth or fifth year of life.

Given these signs, it is extremely important to seek medical help. A professional needs to assess the child’s health and determine the best treatment approach. The vast majority of diseases, when detected early, have a positive prognosis.

West Syndrome Treatments:  The goal of treatment for people with West Syndrome is to eliminate spasms with the fewest medications and side effects possible. Medications such as conventional antiepileptics and ACTH are the main pillars of West Syndrome therapy , but there are other drug possibilities as well.

In some patients, resection of a localized brain area can prevent seizures. Physiotherapy aims to treat or try to reduce the sequelae.

Another objective of physical therapy is to try to avoid deformities, using passive mobilization and stretching. Hydrotherapy can also be of help in solving existing spasticities.

How to Prevent West Syndrome? There is almost always no way to prevent West Syndrome . In symptomatic cases, one should try to avoid possible causal factors.