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.

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.

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

Prevalence and association of metabolic syndrome and vitamin D deficiency among postmenopausal women in a rural block of West Bengal, India.
 

Author(s): Soumi Srimani, Indranil Saha, Debnath Chaudhuri

Journal:

 

Prevalence of metabolic syndrome (MS) and vitamin D deficiency was reported among postmenopausal women (PMW) in India. However, no report is available regarding the association of MS and 25-hydroxyvitamin D [25(OH)D] among PMW in India. This study aimed to find out the prevalence ...

Last Updated: 31 Dec 1969

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Rapid whole-genome sequencing identifies a novel variant associated with West syndrome.
 

Author(s): Lauge Farnaes, Shareef A Nahas, Shimul Chowdhury, James Nelson, Serge Batalov, David M Dimmock, Stephen F Kingsmore,

Journal:

 

A 9-mo-old infant was admitted with infantile spasms that improved on administration of topiramate and steroids. He also had developmental delay, esotropia, and hypsarrhythmia on interictal electroencephalogram (EEG), and normal brain magnetic resonance imaging (MRI). West syndrome ...

Last Updated: 31 Dec 1969

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Existence of a Strong Correlation of Biomarkers and miRNA in Females with Metabolic Syndrome and Obesity in a Population of West Virginia.
 

Author(s): Perrine Goguet-Rubio, Rebecca L Klug, Dana L Sharma, Krithika Srikanthan, Nitin Puri, Vishal H Lakhani, Alexandra Nichols, Kathleen M O'Hanlon, Nader G Abraham, Joseph I Shapiro, Komal Sodhi

Journal:

 

Metabolic syndrome causes complications like cardiovascular disease and type 2 diabetes mellitus (T2DM). As metabolic syndrome develops, altered levels of cytokines and microRNAs (miRNA) are measurable in the circulation. We aimed to construct a panel detecting abnormal levels of ...

Last Updated: 31 Dec 1969

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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:

Systematic Review of Metabolic Syndrome Biomarkers: A Panel for Early Detection, Management, and Risk Stratification in the West Virginian Population.
 

Author(s): Krithika Srikanthan, Andrew Feyh, Haresh Visweshwar, Joseph I Shapiro, Komal Sodhi

Journal:

 

Metabolic syndrome represents a cluster of related metabolic abnormalities, including central obesity, hypertension, dyslipidemia, hyperglycemia, and insulin resistance, with central obesity and insulin resistance in particular recognized as causative factors. These metabolic derangements ...

Last Updated: 31 Dec 1969

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[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: 31 Dec 1969

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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: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Novel Approach to Infantile Spasms
 

Status: Recruiting

Condition Summary: Infantile Spasm

 

Last Updated: 26 Feb 2018

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Trial of Cannabidiol (CBD; GWP42003-P) for Infantile Spasms (GWPCARE7)
 

Status: Recruiting

Condition Summary: Infantile Spasms

 

Last Updated: 20 Oct 2017

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Prednisolone Versus Vigabatrin in the First-line Treatment of Infantile Spasms
 

Status: Recruiting

Condition Summary: Infantile Spasms

 

Last Updated: 19 Apr 2018

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