title: The Clinical Characteristics and Treatment Response in Children with West Syndrome in a Developing Country: A Retrospective Case Record Analysis.
abstract: This study describes the clinical characteristics, treatment, and outcome of children with West syndrome in a tertiary care hospital in north India. Overall, 310 case records diagnosed from January 2009 to June 2012 were reviewed. The median age of onset of spasms was 5 months (interquartile range = 2.5-7 months). The predominant underlying etiology was perinatal cerebral ischemia (55%). Adrenocorticotropic hormone or oral steroids were received by 92% children, of whom 43% became seizure free. Median lag time for appropriate treatment initiation was significantly less in patients who became seizure free as compared to those with persisting seizures (11 vs 15 months, P = .001) soon after receiving treatment of choice. Later age at onset of spasms was associated with a favorable seizure outcome (P = .03). In a resource-limited setting, unawareness along with treatment costs and repeated visits to the hospital are significant obstacles to optimum management.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India sheffaligulati@gmail.com.
authors.forename: Sheffali
authors.initials: S
authors.lastname: Gulati
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Puneet
authors.initials: P
authors.lastname: Jain
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Lakshminarayanan
authors.initials: L
authors.lastname: Kannan
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rachna
authors.initials: R
authors.lastname: Sehgal
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Biswaroop
authors.initials: B
authors.lastname: Chakrabarty
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage; D017668:Age of Onset; D000927:Anticonvulsants / Q000008:administration & dosage; D001921:Brain / Q000503:physiopathology; D003906:Developing Countries; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007194:India / Q000453:epidemiology; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D013256:Steroids / Q000008:administration & dosage; D062606:Tertiary Care Centers; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D013256:Steroids; D000324:Adrenocorticotropic Hormone
keywords: Lennox-Gastaut syndrome; West syndrome; epileptic encephalopathy; epileptic spasms; hypsarrhythmia