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Open Access Highly Accessed Research article

Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents

James W Wheless123*, Patricia A Gibson4, Kari Luther Rosbeck5, Maria Hardin6, Christine O’Dell7, Vicky Whittemore5 and John M Pellock8

Author Affiliations

1 Professor and Chief of Pediatric Neurology, LeBonheur Chair in Pediatric Neurology, University of Tennessee Health Science Center, Memphis, TN, USA

2 Director, LeBonheur Comprehensive Epilepsy Program & Neuroscience Institute, LeBonheur Children’s Medical Center, Memphis, TN, USA

3 Clinical Chief and Director of Pediatric Neurology, St. Jude Children’s Research Hospital, 777 Washington Avenue, P335, Memphis, TN, 38105, USA

4 Epilepsy Information Service, Comprehensive Epilepsy Program, Wake Forest University, Medical Center Boulevard, Winston-Salem, NC, 27157, USA

5 Tuberous Sclerosis Alliance, 801 Roeder Road, Suite 750, Silver Spring, MD, 20910, USA

6 7 Carpenter Close, Ridgefield, CT, 06877, USA

7 The Comprehensive Epilepsy Management Center, Montefiore Medical Center, 111 East 210th Street, Bronx, NY, 10467, USA

8 Division of Child Neurology, Department of Neurology, Virginia Commonwealth University School of Medicine, 1001 East Marshall Street, 1st Floor, Richmond, VA, 23298, USA

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BMC Pediatrics 2012, 12:108  doi:10.1186/1471-2431-12-108

Published: 25 July 2012

Abstract

Background

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 with IS. The objective of this article is to assist pediatric health care providers in the detection of the disease and in the counseling and guidance of families who have an infant with IS.

Methods

Treatment guidelines, consensus reports, and original research studies are reviewed to provide an update regarding the diagnosis and treatment of infants with IS. Web sites were searched for educational and supportive resource content relevant to providers and families of patients with IS.

Results

Early detection of IS and pediatrician referral to a pediatric neurologist for further evaluation and initiation of treatment may improve prognosis. Family education and the establishment of a multidisciplinary continuum of care are important components of care for the majority of patients with IS. The focus of the continuum of care varies across diagnosis, initiation of treatment, and short- and long-term needs. Several on-line educational and supportive resources for families and caregivers of patients with IS were identified.

Conclusions

Given the possibility of poor developmental outcomes in IS, including the emergence of other seizure disorders and cognitive and developmental problems, early recognition, referral, and treatment of IS are important for optimal patient outcomes. Dissemination of and access to educational and supportive resources for families and caregivers across the lifespan of the child with IS is an urgent need. Pediatric health care providers are well positioned to address these needs.

Keywords:
West syndrome; Encephalopathic epilepsy; ACTH; Vigabatrin; Infantile spasms; Treatment; Continuum of care; Community resources; Seizures; Infants