Learn About West Syndrome

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. 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. Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.
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What are the alternative names for West Syndrome?
  • West syndrome
  • IS
  • Infantile spasm
  • Tonic spasms with clustering, arrest of psychomotor development and hypsarrhythmia on EEG
  • West's syndrome
  • X-linked infantile spasm syndrome
  • X-linked infantile spasms
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What are the latest West Syndrome Clinical Trials?
A Phase I Open-Label Pilot Study to Investigate the Feasibility, Safety, Tolerability and Efficacy of Daily Administration of Tricaprilin in Subjects With Infantile Spasms

Summary: The purpose of this study is to assess the safety, tolerability, and efficacy of tricaprilin in subjects with infantile spasms. This is a single-arm, open-label, pilot study in up to 10 subjects with infantile spasms.

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A Phase 3, Randomized, Open-Label Study to Evaluate the Efficacy and Safety of AMZ002, Compared to Vigabatrin, in the Treatment of Infantile Spasms

Summary: The main purpose of this study is to evaluate the efficacy of AMZ002 compared to Vigabatrin in participants with newly diagnosed infantile spasms (IS).

Who are the sources who wrote this article ?

Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for West Syndrome?
Overview of therapeutic options for epilepsy.
Efficacy of pulse intravenous methylprednisolone in epileptic encephalopathy: a randomised controlled trial.
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Case Report: Interventional therapy for portal venous stenosis caused by systemic vasculitis.