Print friendly version
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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.
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]
References
- National Institutes of Health. Infantile Spasms Information Page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/infantilespasms/infantilespasms.htm. Accessed March 21, 2011.
Your Questions Answered
by the Genetic and Rare Diseases Information Center1 question(s) from the public on West syndrome have been answered. See questions and answers. You can also submit a new question.
On this page
General 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.
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- 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.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss West syndrome. Click on the link to go to OMIM and review these resources.