West syndrome

West syndrome's disease overview

What is

West syndrome?

West syndrome is a special form of secondary epilepsy occurring in young children, also known as Constitutional syndrome in newborns. Strong>, accounting for 9% of epilepsy in children. British doctor William James West (1793-1848) was the first to describe the disease in 1841 so his name was named for the name of the disease.

In general, West syndrome is very rare but common in children under one year of age, with the incidence of <6/10,000 children. The disease usually comes from 3 to 6 months of age. Boys have a higher incidence of disease than women, accounting for about 60% of the total number of cases.

West syndrome is divided into 3 groups: West syndrome has no causes, hidden causes and symptoms. West syndrome has no cause of the best prognosis.

Causes of West syndrome's disease

West syndrome may appear after any brain damage, including the cause of before birth and after birth, can be determined in 70-75% of the affected children. Brain damage may be one or more structural lesions, periodic lesions, infections, brain oxygen, intracranial hemorrhage, chromosomes such as Down syndrome, rarer than Due to metabolic diseases such as vitamin B6 deficiency, diabetes ...

tuberous screrosis complex, TSC) is the leading cause of West syndrome. Scleroderma is not a malignant disease. This is a genetic mutation related to seizures, tumors in the eyes, heart, kidneys and abnormal manifestations in the skin.

In addition, West syndrome can also be caused by a CDKL5 gene or ARX gene on the X chromosome X, so it greatly affects boys. During a pregnancy, a woman carrying a disease gene is likely to give birth to a girl with a disease gene with a rate of 25%, giving birth to a girl without a disease gene at a rate of 25%, a boy. Diseases with 25% and a boy who does not get sick is also 25%. If a man has a West syndrome, all their daughters will receive a disease gene from their father. The boys will not receive the disease gene because the father only transmits the sex chromosome for his son.

In 8-42% of cases, the cause of West syndrome is not found.

Symptoms of West syndrome's disease

The manifestation of West syndrome usually appears in the first year of the child. The average diseases of the disease is 6 months old. The outstanding features of the West syndrome include:

Human -folded seizures, easily confused with startled attacks. The movements unconsciously onset suddenly, and usually lasts for a few seconds, sometimes lasting many minutes. Each cycle usually appears when children are awake or after feeding with unintentional contraction of the head, neck, body, hands, legs folded and crossed together. In some children, the back is often bent and stretched limbs. The time, level and position of muscle groups changes between children.

Mental retardation , exercise. Most of the sick children will gradually retreat or do not achieve the skills that need the coordination of many muscles and psychological retardation. Children are often indifferent to the surrounding environment, quiet, little laugh, and dazed face that does not respond to external stimuli. Gradually, children could not perform the movements that they had previously been able to do it, they could not hold their heads, could not sit, could not crawl, could no longer laugh, ...

Transmission route of West syndrome's diseaseWest syndrome

West syndrome is not infectious, no transmission from patients to healthy people.

People at risk for West syndrome's disease

Any factor that increases the likelihood of brain damage can increase the risk of West syndrome, such as:

  • Children's brain birth defects
  • Children suffocating at birth

    • Infectious infections, especially those who have to be treated at the newborn resuscitation unit.

    Chromosomes disorders

  • Family history with relatives with West syndrome or disorders of the nervous system such as epilepsy.

    • Prevention of West syndrome's disease

    There is no measure to prevent West syndrome. Parents need to monitor the physical, physical and mental development of children to immediately detect abnormalities as soon as possible. Pregnant women need to eat and rest properly, listen to the advice of a doctor to minimize the bad obstetrics outcome, avoid affecting the health of newborns in general and the brain speaking. Particularly.

    Diagnostic measures for West syndrome's disease

    West syndrome diagnosis needs to be combined between history, clinical examination and subclinical measures, of which brain electrical (EEG) plays a decisive role.

    Children's brain electrocardine has West syndrome has characteristics such as: abnormal waves with high wave amplitude, chaotic waves model. The abnormalities are evident when they sleep.

    Some other tests can also be indicated to help find the cause of the disease and distinguish it from other diseases such as:

  • Blood formula
  • Urine analysis test
  • Cerebrospinal fluids
  • CT scan of brain
  • MRI of the brain

    • West syndrome's disease treatments

    Treatment goals in children with West syndrome include:

  • Improve the quality of life of children, control of epilepsy symptoms
  • Minimize the side effects of treatment
  • Use the least possible drugs.
    • Treatment of West syndrome with drugs

    drugs such as ACTH, oral corticosteroids, vigabatrin, and classic anti -dynamic drugs are commonly prescribed in the treatment regimen. West syndrome. In particular, Vigabatrin is the first drug accepted to be used in the treatment of West syndrome. The drug has an unwanted effect on the user's vision, so the patient is absolutely not allowed to use the drug without the permission of a doctor.Treatment of West syndrome with surgery

    on some patients, localized cortex surgery can help cure seizures.

    Diet

    a ketogenic -rich diet can be applied to successfully treat many epilepsy. Many studies also believe that this diet should be considered as part of the West syndrome treatment regimen.

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