West syndrome

Common Name(s)

West syndrome, Infantile spasms

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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "West syndrome" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

Last Updated: 29 Apr 2014

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Pediatric Brain Foundation

Pediatric Brain Foundation's Mission is Three-fold: 1. Expedite scientific research to find treatments and cures for ALL of the more than 14 million children, in the U.S. alone, living with some form of neurological disorder 2. Provide families and health care professionals with up-to-date information and resources on the latest discoveries in pediatric neurology 3. Educate the public and public officials on the critical importance of funding pediatric neurological research

Last Updated: 22 Apr 2015

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "West syndrome" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

http://www.caringvoice.org

Last Updated: 29 Apr 2014

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Pediatric Brain Foundation

Pediatric Brain Foundation's Mission is Three-fold: 1. Expedite scientific research to find treatments and cures for ALL of the more than 14 million children, in the U.S. alone, living with some form of neurological disorder 2. Provide families and health care professionals with up-to-date information and resources on the latest discoveries in pediatric neurology 3. Educate the public and public officials on the critical importance of funding pediatric neurological research

http://www.pediatricbrainfoundation.org

Last Updated: 22 Apr 2015

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "West syndrome" returned 61 free, full-text research articles on human participants. First 3 results:

Temporal lobe impairment in West syndrome: event-related potential evidence.
 

Author(s): Klaus Werner, Tangunu Fosi, Stewart G Boyd, Torsten Baldeweg, Rod C Scott, Brian G Neville

Journal: Ann. Neurol.. 2015 Jan;77(1):47-57.

 

This study investigates auditory processing in infants with West syndrome (WS) using event-related potentials (ERPs).

Last Updated: 13 Jan 2015

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Opsoclonus-myoclonus syndrome attributable to West Nile encephalitis: a case report.
 

Author(s): Victoria Bîrluţiu, Rareş Mircea Bîrluţiu

Journal:

 

Opsoclonus-myoclonus syndrome is a very rare neurological disorder associated with some viral infections and exceptionally with the West Nile virus.

Last Updated: 11 Jul 2014

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Re: NPHS2 gene in steroid-resistant nephrotic syndrome: prevalence, clinical course, and mutational spectrum in south-west Iranian children.
 

Author(s): Muhammed Mubarak, Aiysha Abid

Journal: Iran J Kidney Dis. 2014 May;8(3):258-9.

 

Last Updated: 2 Jun 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "West syndrome" returned 5 free, full-text review articles on human participants. First 3 results:

[Differential diagnoses of West syndrome].
 

Author(s): Natalio Fejerman

Journal: Rev Neurol. 2013 Sep;57 Suppl 1():S125-8.

 

This study describes the clinical and electroencephalographic characteristics of epileptic spasms, and more especially those that occur during the first two years of life (infantile spasms). West syndrome has been clearly defined as the association between infantile spasms with an ...

Last Updated: 30 Jul 2013

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Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.
 

Author(s): James W Wheless, Patricia A Gibson, Kari Luther Rosbeck, Maria Hardin, Christine O'Dell, Vicky Whittemore, John M Pellock

Journal:

 

Infantile spasms (IS; West syndrome) is a severe form of encephalopathy that typically affects infants younger than 2 years old. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant ...

Last Updated: 6 Aug 2012

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Epidemiology of primary Sjögren's syndrome in north-west Greece, 1982-2003.
 

Author(s): Y Alamanos, N Tsifetaki, P V Voulgari, A I Venetsanopoulou, C Siozos, A A Drosos

Journal: Rheumatology (Oxford). 2006 Feb;45(2):187-91.

 

To investigate the incidence and prevalence, as well as the mortality and survival rates, of primary Sjögren's syndrome (pSS) in a defined area of north-west Greece with a population of about 500 000 inhabitants.

Last Updated: 20 Jan 2006

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Prevention of West Syndrome With Low-dose Adrenocorticotropin Hormone (ACTH)
 

Status: Recruiting

Condition Summary: West Syndrome

 

Last Updated: 3 Feb 2015

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Evaluation of Neuroinflammation in Children With Infantile Spasms
 

Status: Recruiting

Condition Summary: Infantile Spasms

 

Last Updated: 17 Dec 2014

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