What is West Syndrome?

What is West Syndrome?

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West syndrome, a type of epilepsy in infants, is characterized by infantile spasms that usually occur between 4 and 8 months after birth. The syndrome is characterized by seizures, convulsions and other symptoms that occur suddenly in children. Treatment usually includes antiepileptic drugs, corticosteroids and other specialized therapies. Hormone therapy and dietary changes may also sometimes be used. Early diagnosis and treatment can have a positive impact on a child's development.

West syndrome varies according to each patient's situation. The treatment plan is determined by a specialized doctor and adapted to individual needs. Knowing about this syndrome allows parents and health professionals to intervene more effectively in children's health.

What are the Symptoms of West Syndrome?

West syndrome is a type of epilepsy that occurs in infancy and is characterized by various symptoms. Symptoms usually appear between 4 and 8 months after birth. Symptoms of West syndrome can be as follows:

Sudden Seizures (Infantile Spasms): Seizures that occur between 4 and 8 months after birth and are usually brief but severe.

Grumpiness and Loss of Appetite: West syndrome in infants can often be associated with moodiness and loss of appetite.

Sudden Jumping at Night when Falling asleep: When babies suddenly exhibit motor movements such as jumping while falling asleep at night.

Head Tilt Forward: Babies' heads tilt forward during infantile spasms.
Contraction of the Arms and Legs: Contracting or trembling movements in the arms and legs.

Small Head Circumference: Babies with West syndrome may often have a small head circumference.

Slow Development: Significant delays in language development, motor skills and other developmental areas.
Delayed Speaking and Walking: Significant delays in children reaching key developmental milestones such as speaking and walking.

What Causes West Syndrome?

West syndrome is a childhood epilepsy syndrome that can occur due to various causes. Although the cause is not fully understood, some factors may contribute to the development of this syndrome. Here are the possible causes of West syndrome:

Structural Changes in the Brain: Abnormalities in the brain, especially structural changes that occur before or during birth, can cause West syndrome.

Brain Infections: Infections of the baby's brain, especially viral infections, can cause West syndrome.

Oxygen Deficiency (Hypoxia): Lack of oxygen during or after birth can affect the normal development of the brain and cause epileptic seizures.

Genetic Mutations: Certain genetic factors and genetic syndromes may play a role in the development of West syndrome.

Metabolic Disorders: Metabolic diseases or disorders, especially problems with energy metabolism, can lead to West syndrome.

Abnormal Brain Development (Malformation): Abnormalities or malformations that occur during brain development can cause West syndrome.

How is West syndrome diagnosed?

West syndrome is usually diagnosed by a neurologist or pediatric neurologist. The diagnostic process involves a physical examination, history taking and neurological tests, especially electroencephalography (EEG). By measuring the electrical activity of the brain, EEG can detect an abnormal pattern called "hypsarrhythmia", which is specific to West syndrome.

Brain imaging methods such as magnetic resonance imaging (MRI) or computed tomography (CT) scans are also used to look for structural abnormalities in the brain.

Other tests such as blood tests, urine tests and cerebrospinal fluid analysis can also help determine the cause of the syndrome. This comprehensive diagnostic process helps to create the right treatment plan and support the child's development.

How is West syndrome treated?

Treatment for West syndrome usually involves a multidisciplinary approach and the treatment plan is determined based on the child's specific condition. This treatment plan is managed by neurologists, pediatricians and other health professionals. Some common methods used in the treatment of West syndrome include

Antiepileptic drugs: Medication is often the mainstay of West syndrome treatment. Specially selected antiepileptic drugs work to reduce and control the frequency of seizures.

Corticosteroids: Steroid medications, especially ACTH (adrenocorticotropic hormone) and prednisone, can be used to control infantile spasms and regulate neurologic activity.

Dietary Modifications: Special diets, especially the ketogenic diet, may be a treatment option in some cases. This diet attempts to control epileptic activity by increasing the body's production of ketones.

Hormone Therapy: Hormone therapy with ACTH (adrenocorticotropic hormone) or corticotropin can help control infantile spasms.
Surgical Interventions: In rare cases, surgical interventions (neurosurgery) may be considered. However, such interventions usually involve special circumstances and are carefully evaluated.

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CreatorNP Istanbul Hospital Editorial Board
Updated At05 March 2024
Created At23 January 2024
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