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Infantile Spasms/West Syndrome

  • Reference work entry
Encyclopedia of Autism Spectrum Disorders

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Synonyms

Blitz-Nick-Salaam Krämpfe; Infantile myoclonic epilepsy; Infantile spasms syndrome; Massive infantile spasms; Myoclonic spasms of infancy; West syndrome

Infantile spasms or West syndrome consists of infantile spasms, hypsarrhythmia, and mental retardation. The spasms are usually resistant to conventional antiepileptic drugs. The typical syndrome has its onset between 3 and 7 months of age and seldom after age of 1 year.

Now, however, it is known that there is a great variability of the features. Any feature of this classical triad may be missing.

Categorization

According to the up-to-date International League against Epilepsy (ILAE, 2001) Classification, West syndrome is an epileptic syndrome consisting of clinical spasms usually in clusters with epileptiform EEG, with onset usually less than 2 years of age. West syndrome is an epileptic encephalopathy where cognitive, motor and/or sensory functions are altered by the epilepsy.

Two main groups of the syndrome are recognized:...

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Authors and Affiliations

  1. Department of Child Neurology, University of Kuopio, P.O. Box 1627, Kuopio, FIN-70211, Finland

    Prof. Raili Riikonen (Professor of Child Neurology)

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  1. Prof. Raili Riikonen
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Correspondence to Raili Riikonen .

Editor information

Editors and Affiliations

  1. Irving B. Harris Professor of Child Psychiatry, Pediatrics and Psychology Yale University School of Medicine, Chief, Child Psychiatry Children's Hospital at Yale-New Haven Child Study Center, New Haven, CT, USA

    Fred R. Volkmar (Director, Child Study Center) (Director, Child Study Center)

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Riikonen, R. (2013). Infantile Spasms/West Syndrome. In: Volkmar, F.R. (eds) Encyclopedia of Autism Spectrum Disorders. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-1698-3_1832

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  • DOI: https://doi.org/10.1007/978-1-4419-1698-3_1832

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-1697-6

  • Online ISBN: 978-1-4419-1698-3

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