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

BMC Pediatrics

Research Article

Maria Hardin¹  John M Pellock²  Patricia A Gibson³  James W Wheless⁴  Christine O’Dell⁵  Kari Luther Rosbeck⁶  Vicky Whittemore⁶ 

[1] 7 Carpenter Close, 06877, Ridgefield, CT, USA;Division of Child Neurology, Department of Neurology, Virginia Commonwealth University School of Medicine, 1001 East Marshall Street, 1st Floor, 23298, Richmond, VA, USA;Epilepsy Information Service, Comprehensive Epilepsy Program, Wake Forest University, Medical Center Boulevard, 27157, Winston-Salem, NC, USA;Professor and Chief of Pediatric Neurology, LeBonheur Chair in Pediatric Neurology, University of Tennessee Health Science Center, Memphis, TN, USA;Director, LeBonheur Comprehensive Epilepsy Program & Neuroscience Institute, LeBonheur Children’s Medical Center, Memphis, TN, USA;Clinical Chief and Director of Pediatric Neurology, St. Jude Children’s Research Hospital, 777 Washington Avenue, P335, 38105, Memphis, TN, USA;The Comprehensive Epilepsy Management Center, Montefiore Medical Center, 111 East 210th Street, 10467, Bronx, NY, USA;Tuberous Sclerosis Alliance, 801 Roeder Road, Suite 750, 20910, Silver Spring, MD, USA;

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

DOI  :  10.1186/1471-2431-12-108

 received in 2011-07-07, accepted in 2012-07-25,  发布年份 2012

来源: Springer

【 摘 要 】

BackgroundInfantile 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.MethodsTreatment 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.ResultsEarly 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.ConclusionsGiven 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.

【 授权许可】

CC BY   
© Wheless et al.; licensee BioMed Central Ltd. 2012