West Syndrome

Synonyms

• Generalized Flexion Epilepsy
• Infantile Epileptic Encephalopathy
• Infantile Myoclonic Encephalopathy
• Jackknife Convulsion
• Massive Myoclonia
• Salaam Spasms
• Infantile Spasms
• hypsarrhythmia
• mental retardation
• epilepsy syndrome
• X-linked infantile spasms
• ISSX
• X-linked infantile spasm syndrome
• IS

Disorder Subdivisions

• None

General Discussion

West Syndrome is a rare form of infantile spasm that occurs very early in the development of an infant. Unusual brain wave patterns occur (hypsarrhythmia) and possibly mental retardation. The spasms that occur may range from violent jackknife or "salaam" movements where the whole body bends in half, or they may be no more than a mild twitching of the nose or mouth. These spasms usually begin in the early months after birth and can often be helped with medication. There may be many different causes for the spasms. Neurological testing will be helpful in determining the cause.

Resources

Epilepsy Foundation
4351 Garden City Drive
Landover, MD 20785
Tel: (301)459-3700
Fax: (301)577-2684
Tel: (800)332-1000
TDD: (800)332-2070
Email: postmaster@efa.org
Internet: http://www.epilepsyfoundation.org

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 8/30/2007
Copyright 1993, 1995, 1996, 2000, 2002, 2007 National Organization for Rare Disorders, Inc.