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Condition

West Syndrome

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Condition 101

What is the definition of West Syndrome?

West syndrome is characterized by a specific type of seizure (infantile spasms) seen in infancy and childhood. This syndrome leads to developmental regression and causes a specific pattern, known as hypsarrhythmia (chaotic brain waves), on electroencephalography (EEG) testing. The infantile spasms usually begin in the first year of life, typically between 4-8 months. The seizures primarily consist of a sudden bending forward of the body with stiffening of the arms and legs; some children arch their backs as they extend their arms and legs. Spasms tend to occur upon awakening or after feeding, and often occur in clusters of up to 100 spasms at a time. Infants may have dozens of clusters and several hundred spasms per day. Infantile spasms usually stop by age five, but may be replaced by other types of seizures. Many disorders leading to brain injury, such as birth problems, cerebral anomalies, metabolic disorders, and genetic disorders can lead to these spasms, making it important to identify the underlying cause. In some children, no cause can be found. The goals of treatment are to reduce or eliminate seizures, and include several medications, such as corticoids, avigabatrin, and antiepileptic drugs. Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.

What are the alternative names for West Syndrome?

  • Infantile spasm
  • IS
  • X-linked infantile spasm syndrome
  • X-linked infantile spasms
  • West's syndrome
  • Tonic spasms with clustering, arrest of psychomotor development and hypsarrhythmia on EEG

Top Global Doctors

Latest Research

Latest Advance
Study
  • Condition: Infantile Spasms
  • Journal: Pediatric neurology
  • Treatment Used: Adrenocorticotropic Hormone (tetracosactide; ACTH)
  • Number of Patients: 189
  • Published —
This study analyzed the treatment options for infantile spasms, especially the adrenocorticotropic hormone dosage and the formulation (natural versus synthetic), and their effectiveness.
Latest Advance
Study
  • Condition: COVID-19
  • Journal: BMJ open respiratory research
  • Treatment Used: Continuous Positive Airway Pressure (CPAP) in Prone Position
  • Number of Patients: 24
  • Published —
This study tested the safety and efficacy of using a CPAP machine on patients in a conscious prone position to treat their COVID-19 infections.

Clinical Trials

Clinical Trial
Other
  • Status: Not yet recruiting
  • Participants: 250
  • Start Date: September 14, 2020
KArukera Study of Ageing in Foster Families
Clinical Trial
Drug
  • Status: Recruiting
  • Study Type: Drug
  • Participants: 40
  • Start Date: August 10, 2020
A Prospective, Case-control Evaluation of Ketogenic Dietary Therapy for New-onset Childhood Absence Epilepsy