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eMedicine - Infantile Spasm (West Syndrome)

eMedicine - Infantile Spasm (West Syndrome)

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Published by: Rohit Vishal Kumar on Sep 29, 2008
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eMedicine - Infantile Spasm (West Syndrome) : Article by Tracy A Gl...http://www.emedicine.com/neuro/TOPIC171.HTM1 of 176/16/2008 5:39 PM
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AUTHOR AND EDITOR INFORMATION
Section 1 of 11
You are in:eMedicine Specialties>Neurology>Pediatric Neurology
Infantile Spasm (West Syndrome)
Article Last Updated: Apr 10, 2006
Authors and Editors Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Multimedia References
Author: 
Tracy A Glauser, MD,
Professor, Departments of Pediatrics and Neurology, University ofCincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital MedicalCenter of CincinnatiTracy A Glauser is a member of the following medical societies:American Academy of Neurology,American Academy of Pediatrics,American Epilepsy Society, andChild Neurology Society Coauthor(s):
Diego A Morita, MD,
Assistant Professor of Pediatrics and Neurology, Department ofPediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of CincinnatiEditors:
Robert Baumann, MD
, Program Director, Professor, Departments of Neurology and Pediatrics,University of Kentucky;
Francisco Talavera, PharmD, PhD,
Senior Pharmacy Editor, eMedicine;
Kenneth J Mack, MD, PhD,
Senior Associate Consultant, Department of Child and AdolescentNeurology, Mayo Clinic;
Selim R Benbadis, MD,
Professor, Director of Comprehensive EpilepsyProgram, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine,Tampa General Hospital;
Nicholas Y Lorenzo, MD,
Chief Editor, eMedicine Neurology; Consulting Staff,Neurology Specialists and Consultants
Author and Editor Disclosure
 
Synonyms and related keywords:
infantile spasms, hypsarrhythmia, developmental delay, Westsyndrome, mental retardation, epilepsy syndrome
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eMedicine - Infantile Spasm (West Syndrome) : Article by Tracy A Gl...http://www.emedicine.com/neuro/TOPIC171.HTM2 of 176/16/2008 5:39 PM
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INTRODUCTION
Section 2 of 11
Authors and Editors Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Multimedia References
 
eMedicine - Infantile Spasm (West Syndrome) : Article by Tracy A Gl...http://www.emedicine.com/neuro/TOPIC171.HTMof 176/16/2008 5:39 PM
CLINICAL
Section 3 of 11
Background
West syndrome is composed of the triad of infantile spasms, an interictal EEG pattern termed hypsarrhythmia, and mentalretardation, although the diagnosis can be made even if one of the 3 elements is missing (according to the internationalclassification). This severe epilepsy syndrome is an age-dependent expression of a damaged brain. The term "infantile spasms"has been used to describe the seizure type, the epilepsy syndrome, or both. In this article, the term "infantile spasms" issynonymous with West syndrome.The syndrome's namesake, Dr W J West, gave the first detailed description of infantile spasms, as they occurred in his child. In aletter to the editor of
The Lancet 
in 1841, West described the events as "bobbings" that "cause a complete heaving of the headforward towards his knees, and then immediately relaxing into the upright position … these bowings and relaxings would berepeated alternately at intervals of a few seconds, and repeated from 10 to 20 or more times at each attack, which attack wouldnot continue more than 2 or 3 minutes; he sometimes has 2, 3 or more attacks in the day."This detailed clinical description was followed approximately 100 years later by the report of the typical interictal EEG patterntermed hypsarrhythmia. Most patients with infantile spasms have some degree of developmental retardation.The eponym West syndrome was created in the early 1960s by Drs. Gastaut, Poirier, and Pampiglione.
Pathophysiology
Infantile spasms are believed to reflect abnormal interactions between the cortex and brainstem structures. Focal lesions early inlife may secondarily affect other sites in the brain, and hypsarrhythmia may represent this abnormal activity arising from multiplebrain sites. The frequent onset of infantile spasms in infancy suggests that an immature central nervous system may be importantin the pathogenesis. The brain-adrenal axis also may be involved. One theory states that the effect of different stressors in theimmature brain produces an abnormal excessive secretion of corticotropin-releasing hormone, causing spasms. The clinicalresponse to adrenocorticotropic hormone (ACTH) and glucocorticoids can be explained by suppression of corticotropin-releasinghormone (CRH) production.
Frequency
United States
Infantile spasm constitutes 2% of childhood epilepsies but 25% of epilepsy with onset in the first year of life. The rate of infantilespasm is estimated between 2.5 to 6.0 per 10,000 live births. Its prevalence rate is 1.5-2.0 per 10,000 children aged 10 years oryounger.
International
Infantile spasm occurs in 0.05 (Estonia) to 0.41 (Oulu, Finland) of 1000 live births and in 1.4% (Estonia), 4.2% (Odense,Denmark), and 7.6% (Tampere, Finland) of children with epilepsy.
Mortality/Morbidity
The premature death rate ranges from 5-31%. The upper limit comes from a study of 214 Finnish children with a history ofinfantile spasms who were followed for a mean of 25 years (range, 20-30 y). Most of the deaths (61%) occurred at or before age10 years, while only 10% occurred after age 20 years.
Sex
Although males are affected slightly more often than females, no significant gender difference is noted.
Age
Ninety percent of infantile spasms begin in those younger than 12 months. Peak onset is at age 4-6 months.
Authors and Editors Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Multimedia References
History
Ictal manifestationsSpasms begin with a sudden, rapid, tonic contraction of trunk and limb musculature that gradually relaxes over0.5-2 seconds.

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