West Syndrome
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Learn About West Syndrome

What is the definition of West Syndrome?
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. Many disorders leading to brain injury, such as birth problems, cerebral anomalies, 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. Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.
What are the alternative names for West Syndrome?
  • West syndrome
  • IS
  • Infantile spasm
  • Tonic spasms with clustering, arrest of psychomotor development and hypsarrhythmia on EEG
  • West's syndrome
  • X-linked infantile spasm syndrome
  • X-linked infantile spasms
Who are the top West Syndrome Local Doctors?
Aristea S. Galanopoulou
Elite in West Syndrome
Elite in West Syndrome

Montefiore Medical Center

111 East 210th Street, 
Bronx, NY 
Languages Spoken:
English, Greek

The maturation of GABAA receptor-mediated signaling from depolarizing to inhibitory is an age-related process controlled by cation chloride cotransporters, such as KCC2. As a result, GABA exerts dual functions, being an important neurotrophic factor during early development and the principal inhibitory neurotransmitter of the mature central nervous system. In our laboratory we have been investigating the age and mechanisms through which early life stressors and seizures may disrupt the normal patterns of brain development, by disrupting the neurotrophic effects of GABA. We are also studying methods to reverse these adverse processes. Furthermore, we are very interested in understanding how epileptogenesis proceeds in the developing brain and what is the specific role of GABAA receptors in this process. Dr. Galanopoulou is rated as an Elite provider by MediFind in the treatment of West Syndrome. Her top areas of expertise are West Syndrome, Spasmus Nutans, Post-Traumatic Epilepsy, and Epilepsy.

Solomon L. Moshe
Elite in West Syndrome
Pediatric Neurology
Elite in West Syndrome
Pediatric Neurology

The Children's Hospital At Montefiore

3415 Bainbridge Avenue, 
Bronx, NY 
Languages Spoken:
English

Solomon L. Moshé, MD, is Vice Chair of Neurology, Director of the Isabelle Rapin Child Neurology Division and Director of Clinical Neurophysiology at Montefiore. He is also the Charles Frost Chair in Neurosurgery and Neurology, Vice Chair, Saul R. Korey Department of Neurology, and Professor of Neurology, Neuroscience and Pediatrics at the Albert Einstein College of Medicine (Einstein). Dr. Moshé’s clinical focus is on epilepsy and electroencephalography with special emphasis on age-related influences on the expression, control and consequences of seizures. Dr. Moshe is rated as an Elite provider by MediFind in the treatment of West Syndrome. His top areas of expertise are West Syndrome, Spasmus Nutans, Post-Traumatic Epilepsy, and Epilepsy.

 
 
 
 
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Elia P. Knight
Elite in West Syndrome
Elite in West Syndrome

Cleveland Clinic Main Campus

9500 Euclid Avenue, 
Cleveland, OH 
Languages Spoken:
English

Elia Knight is a Pediatrics provider in Cleveland, Ohio. Dr. Knight is rated as an Elite provider by MediFind in the treatment of West Syndrome. Her top areas of expertise are CDKL5 Deficiency Disorder, West Syndrome, Seizures, and Epilepsy.

What are the latest West Syndrome Clinical Trials?
Phase 1 Study of Ketogenic Diet for Prevention of Epileptic Spasms in Infantile Onset Genetic Epilepsies

Summary: Epileptic spasms (ES) are a predominantly infantile seizure type observed frequently in certain genetic disorders. Ketogenic diet (high ratio of fat to carbohydrate/protein) is an established non-medication treatment for difficult to control seizures, including ES. Because ES are associated with worse developmental and cognitive outcomes if not detected or treated quickly and effectively, this tri...

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A Machine Learning Approach to Infantile Spasms Recognition in Video Recordings

Summary: Infantile spasms are a type of seizure linked to developmental issues. Unfortunately, they are often misdiagnosed, causing delays in treatment. The purpose of this study is to develop a computer program that can reliably differentiate infantile spasms from similar, yet benign movements in videos. This computer program will learn from videos taken by parents of study participants. Quickly recognizi...

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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center