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.
WestW. On a peculiar form of infantile convulsions. Lancet. 1841;1:724–725.
2.
GibbsFGibbsE. Atlas of Encephalography. Cambridge, MA: Addison-Wesley; 1952.
3.
GoCYMackayMTWeissSK. Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2012;78:1974–1980.
4.
KaushikJSPatraBSharmaS. Clinical spectrum and treatment outcome of West Syndrome in children from Northern India. Seizure. 2013;22:617–621.
5.
SinghiPRayM. Profile of West syndrome in North Indian children. Brain Dev. 2005;27:135–140.
6.
KalraVGulatiSPandeyRMMenonS. West syndrome and other infantile epileptic encephalopathies—Indian hospital experience. Brain Dev. 2002;24:130–139.
7.
BergATBerkovicSFBrodieMJ. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 2010;51:676–685.
8.
RiikonenR. ACTH therapy of West syndrome: Finnish views. Brain Dev. 2001;23:642–646.
9.
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. Washington, DC: American Psychiatric Association; 1994.
10.
HrachovyRAFrostJDJrKellawayP. Hypsarrhythmia: variations on the theme. Epilepsia. 1984;25:317–325.
11.
SharmaSSankhyanNGulatiSAgarwalaA. Use of the modified Atkins diet for treatment of refractory childhood epilepsy: a randomized controlled trial. Epilepsia. 2013;54:481–486.
12.
SharmaSSankhyanNGulatiSAgarwalaA. Use of the modified Atkins diet in infantile spasms refractory to first-line treatment. Seizure. 2012;21:45–48.
13.
PanayiotopoulosC. A Clinical Guide to Epileptic Syndromes and Their Treatment. 2nd ed.London: Springer; 2011.
14.
LuxALEdwardsSWHancockE. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet. 2004;364:1773–1778.
15.
LuxALEdwardsSWHancockE. The United Kingdom Infantile Spasms Study comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial. Lancet Neurol. 2005;4:712–717.
16.
PartikianAMitchellWG. Neurodevelopmental and epilepsy outcomes in a North American cohort of patients with infantile spasms. J Child Neurol. 2010;25:423–428.
17.
SharmaNLVishwanthanV. Outcome in West syndrome. Indian Pediatr. 2008;45:559–563.
18.
EisermannMMDeLaRaillereADellatolasG. Infantile spasms in Down syndrome—effects of delayed anticonvulsive treatment. Epilepsy Res. 2003;55:21–27.
19.
AryaRShinnarSGlauserTA. Corticosteroids for the treatment of infantile spasms: a systematic review. J Child Neurol. 2012;27:1284–1288.
KossoffEHHedderickEFTurnerZFreemanJM. A case-control evaluation of the ketogenic diet versus ACTH for new-onset infantile spasms. Epilepsia. 2008;49:1504–1509.
22.
HongAMTurnerZHamdyRFKossoffEH. Infantile spasms treated with the ketogenic diet: prospective single-center experience in 104 consecutive infants. Epilepsia. 2010;51:1403–1407.
23.
SharmaSSankhyanNGulatiSAgarwalaA. Use of the modified Atkins diet in infantile spasms refractory to first-line treatment. Seizure. 2012;21:45–48.
24.
DulacOPlouinPJambaqueI. Predicting favourable outcome in idiopathic West Syndrome. Epilepsia. 1993;34:747–756.
25.
O’CallaghanFJLuxALDarkeK. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia. 2011;52:1359–1364.
26.
IwataniYKagitani-ShimonoKTominagaK. Long-term developmental outcome in patients with West syndrome after epilepsy surgery. Brain Dev. 2012;34:731–738.
27.
KayaalpLDerventASaltikS. EEG abnormalities in West syndrome: correlation with the emergence of autistic features. Brain Dev. 2007;29:336–345.
