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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CNS Foundation

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

Last Updated: 12 Oct 2013

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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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CNS Foundation

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

http://www.cnsfoundation.org

Last Updated: 12 Oct 2013

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

Incidence of Japanese encephalitis among acute encephalitis syndrome cases in West Bengal, India.
 

Author(s): Bhaswati Bandyopadhyay, Indrani Bhattacharyya, Srima Adhikary, Saiantani Mondal, Jayashree Konar, Nidhi Dawar, Asit Biswas, Nemai Bhattacharya

Journal: Biomed Res Int. 2013 ;2013():896749.

 

Japanese encephalitis (JE) is the most important cause of acute and epidemic viral encephalitis. Every year sporadic JE cases are reported from the various districts of West Bengal, indicating its endemicity in this state. JE vaccination programme has been undertaken by the State ...

Last Updated: 9 Dec 2013

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NPHS2 gene in steroid-resistant nephrotic syndrome: prevalence, clinical course, and mutational spectrum in South-West Iranian children.
 

Author(s): Mitra Basiratnia, Majid Yavarian, Simin Torabinezhad, Asma Erjaee

Journal: Iran J Kidney Dis. 2013 Sep;7(5):357-62.

 

Mutations in podocin (NPHS2) gene have the key role in the pathogenesis of steroid-resistant nephrotic syndrome (SRNS) in children, but data is scarce regarding their prevalence and natural course among different all ethnic groups. This study was aimed to demonstrate the spectrum ...

Last Updated: 27 Sep 2013

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Occipital lobe epilepsy secondary to posterior reversible encephalopathy syndrome (PRES) during a post-partum eclampsia in Mali (West Africa).
 

Author(s): Maïga Youssoufa, Kuate Tegueu Callixte, Napon Christian

Journal:

 

Eclampsia is known to cause posterior reversible encephalopathy syndrome (PRES) that is often associated with an extensive neurovascular damage affecting preferably posterior regions, often leading to reversible cortical blindness. In spite the magnitude of these lesions, post eclamptic ...

Last Updated: 16 Aug 2013

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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 6 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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[Research advance in the etiology of West syndrome].
 

Author(s): Zhan-Li Liu, Li-Ping Zou

Journal: Zhongguo Dang Dai Er Ke Za Zhi. 2010 Feb;12(2):159-61.

 

Last Updated: 4 Mar 2010

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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: 25 Sep 2012

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Evaluation of Neuroinflammation in Children With Infantile Spasms
 

Status: Recruiting

Condition Summary: Infantile Spasms

 

Last Updated: 18 Mar 2014

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Genetic Studies in Patients and Families With Infantile Spasms
 

Status: Recruiting

Condition Summary: Infantile Spasms

 

Last Updated: 25 Mar 2013

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