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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KCNQ2 Cure Alliance

The mission of KCNQ2 Cure Alliance is to educate and to advance research leading to treatments or a cure for patients living with the genetic disorder KCNQ2 Epilepsy.

Last Updated: 12 Dec 2015

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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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KCNQ2 Cure Alliance

The mission of KCNQ2 Cure Alliance is to educate and to advance research leading to treatments or a cure for patients living with the genetic disorder KCNQ2 Epilepsy.

http://www.kcnq2cure.org/

Last Updated: 12 Dec 2015

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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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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 102 free, full-text research articles on human participants. First 3 results:

Obesity, Hypertension and Metabolic Syndrome in Children in West Virginia.
 

Author(s): Jeannie Co, Jamie Jeffrey, Mary Emmett, Asmita Modak, Stephen B Sondike

Journal: W V Med J. ;111(4):20-2, 24.

 

Last Updated: 5 Aug 2015

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Chikungunya virus infection amongst the acute encephalitis syndrome cases in West Bengal, India.
 

Author(s): D Taraphdar, B K Roy, S Chatterjee

Journal: Indian J Med Microbiol. 2015 Feb;33 Suppl():153-6.

 

Chikungunya virus (CHIKV) infection from the acute encephalitis syndrome cases is an uncommon form and has been observed in the year 2010-11 from West Bengal, India. The case-1 and case-2 had the acute encephalitis syndrome; case-3 was of acute disseminated encephalomyelitis whereas ...

Last Updated: 6 Feb 2015

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Long-term outcomes in patients with West syndrome: an outpatient clinical study.
 

Author(s): Betül Tekin Güveli, Özlem Çokar, Nimet Dörtcan, Gülçin Benbir, Veysi Demirbilek, Ayşın Dervent

Journal: Seizure. 2015 Feb;25():68-71.

 

Nearly half of all patients with seizure onset in the first year of life suffer from West syndrome (WS). The prognosis of epilepsy and psychosocial outcomes in children with WS are variable. This study was performed to examine the factors influencing the outcome of this patient population.

Last Updated: 3 Feb 2015

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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 7 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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Cognitive and behavioral outcome in West syndrome.
 

Author(s): Franco Guzzetta

Journal: Epilepsia. 2006 ;47 Suppl 2():49-52.

 

Cognitive and behavioral impairments in West syndrome are analyzed at the onset of the disease. Multimodal sensory impairments, namely of the visual function, that are usually associated with the acute stage of the disease, are stressed as possible mechanisms interfering with cognitive ...

Last Updated: 19 Nov 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: 6 Jan 2016

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