Abstract
West syndrome is an infantile epileptic encephalopathy with infantile spasms that are characteristic seizures associated with electrodecremental responses, hypsarrhythmia, and intellectual disabilities. There is a need to develop better therapies for West syndrome. Several rodent models of infantile spasms have been developed recently to elucidate the pathogenesis, and help identify new treatments. These are acute models, in which spasms occur only immediately after induction, and chronic models of infantile spasms that recapitulate many features of the syndrome. In this chapter, we are summarizing the main features and findings stemming from these models, as well as discuss their utility in the development of treatments for infantile spasms, other associated seizures, and their comorbidities.
Original language | English (US) |
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Title of host publication | Models of Seizures and Epilepsy |
Subtitle of host publication | Second Edition |
Publisher | Elsevier Inc. |
Pages | 977-993 |
Number of pages | 17 |
ISBN (Print) | 9780128040669 |
DOIs | |
State | Published - Jan 1 2017 |
Keywords
- Adenopolyposis colon
- APC
- Aristaless X linked homeobox
- ARX
- Chronic early stress
- Down syndrome
- Electrodecremental response
- Epilepsy
- Hypsarrhythmia
- Infantile spasms
- Multiple-hit model of infantile spasms
- NMDA
- Prenatal betamethasone
- Stress
- Tetrodotoxin
- Tuberous sclerosis
- West syndrome
ASJC Scopus subject areas
- Neuroscience(all)