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Genetic and Rare Diseases Information Center (GARD)

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West syndrome


Other Names for this Disease

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

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 underlying disorders, such as birth injury, 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.[1]
Last updated: 3/28/2011

References

  1. National Institutes of Health. Infantile Spasms Information Page. National Institute of Neurological Disorders and Stroke. February 22, 2011; http://www.ninds.nih.gov/disorders/infantilespasms/infantilespasms.htm. Accessed 3/21/2011.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss West syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Infantile spasm
  • IS
  • Tonic spasms with clustering, arrest of psychomotor development and hypsarrhythmia on EEG
  • West's syndrome
  • X-linked infantile spasm syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.