DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20202149

West syndrome: clinic etiological profile, and response to treatment in Western India

Shah Nawaz, Mona P. Gajre, Arpita Adhikari, Shagufta .

Abstract


Background: Information on the profile of infants with West syndrome in developing countries is limited. This study was done to determine clinico-etiological profile and clinical response of infantile spasms to various medications in children with west syndrome in a developing country.

Methods: It was a retrospective cohort study from January 2017-january 2020 done in a tertiary care hospital in western India. Records of 39 children with west syndrome who visited this pediatric neurology division during study period were analysed. 6 were excluded in view of incomplete data. Detailed history, examination, investigations and medications given were noted. Follow up records were assessed to look for long term control of spasms, relapse rates after cessation, or progression to other seizure types.

Results: Mean age at onset of infantile spasms was found to be 8.12 months (1 - 36 months).  Mean lag time to treatment was 5.35 months. Etiology was found in 69.7% children with perinatal causes being most common. With oral prednisolone, 54.5% had complete cessation of spasms, and with ACTH also 54.5% had complete spasm cessation. Favourable clinical response at 6 months follow up was found in 8 (47.05%) of the 17 children. Surprisingly, lag time (p=0.381) and symptomatic etiology (p=1.00) did not have any significant impact on outcome.

Conclusions: This study highlights the developing country perspective of west syndrome. Increased lag time, different etiological profile and poor outcome are the challenges. High dose prednisolone is a good first line alternative treatment option in resource poor settings.


Keywords


Infantile spasms, Prednisolone, West syndrome

Full Text:

PDF

References


Lux AL. West and son: the origins of West syndrome. Brain Develop. 2001 Nov 1;23(7):443-6.

Güveli BT, Çokar Ö, Dörtcan N, Benbir G, Demirbilek V, Dervent A. Long-term outcomes in patients with West syndrome: an outpatient clinical study. Seizure. 2015 Feb 1;25:68-71.

Osborne JP, Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, et al. The underlying etiology of infantile spasms (West syndrome): Information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification 2. Epilepsia. 2010 Oct;51(10):2168-74.

Taghdiri MM, Nemati H. Infantile spasm: a review article. Iranian J Child Neurol. 2014;8(3):1.

Wirrell EC, Shellhaas RA, Joshi C, Keator C, Kumar S, Mitchell WG. Pediatric Epilepsy Research Consortium (PERC). How should children with West syndrome be efficiently and accurately investigated? Results from the National Infantile Spasms Consortium. Epilepsia. 2015 Apr;56(4):617-25.

Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 2010 Apr;51(4):676-85.

Nelson GR. Management of infantile spasms. Translat Pediatr. 2015 Oct;4(4):260.

Jeavons PM, Bower BD, Dimitrakoudi M. Long‐term prognosis of 150 cases of “West syndrome”. Epilepsia. 1973 Jun;14(2):153-64.

Matsumoto A, Watanabe K, Negoro T, Sugiura M, Iwase K, Hara K, et al. Long‐term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases. Develop Medi Child Neurol. 1981 Feb;23(1):51-65.

Sharma NL, Viswanathan V. Outcome in West syndrome. Indian Pediatr. 2008 Jul 1;45(7):559.

Hrachovy RA, Frost Jr JD, Kellaway P. Hypsarrhythmia: variations on the theme. Epilepsia. 1984 Jun;25(3):317-25.

Kaushik JS, Patra B, Sharma S, Yadav D, Aneja S. Clinical spectrum and treatment outcome of West Syndrome in children from Northern India. Seizure. 2013;22(8):617-621.

Kalra V, Passi GR. Analysis of childhood epileptic encephalopathies with regard to etiological and prognostic factors. Brain Develop. 1998 Jan 1;20(1):14-7.

Chandra S, Bhave A, Bhargava R, Kumar R. West syndrome: response to valproate. Frontiers Neurol. 2012 Nov 23;3:166.

Cohen‐Sadan S, Kramer U, Ben‐Zeev B, Lahat E, Sahar E, Nevo Y, et al. Multicenter long‐term follow‐up of children with idiopathic West syndrome: ACTH versus vigabatrin. Eur J Neurol. 2009 Apr;16(4):482-7.

Karvelas G, Lortie A, Scantlebury MH, Duy PT, Cossette P, Carmant L. A retrospective study on aetiology based outcome of infantile spasms. Seizure. 2009 Apr 1;18(3):197-201.

O’Callaghan FJ, Lux AL, Darke K, Edwards SW, Hancock E, Johnson AL, et al. 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 Jul;52(7):1359-64.

Iype M, Saradakutty G, Kunju PA, Mohan D, Nair MK, George B, et al. Infantile spasms: A prognostic evaluation. Ann Ind Acad Neurol. 2016 Apr;19(2):228.

Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, et al. Infantile spasms: a US consensus report. Epilepsia. 2010;51(10):2175-89.

Cowan LD, Hudson LS. The epidemiology and natural history of infantile spasms. J Child Neurol. 1991 Oct;6(4):355-64.

Trevathan E, Murphy CC, Yeargin‐Allsopp M. The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia. 1999 Jun;40(6):748-51.

Hrachovy RA, Frost Jr JD, Kellaway P, Zion TE. Double-blind study of ACTH vs prednisone therapy in infantile spasms. J Pediatr. 1983;103(4):641-5.

Snead OC, Benton JW, Myers GJ. ACTH and prednisone in childhood seizure disorders. Neurology. 1983 Aug 1;33(8):966.

Kossoff EH, Hartman AL, Rubenstein JE, Vining EP. High-dose oral prednisolone for infantile spasms: an effective and less expensive alternative to ACTH. Epilep Behav. 2009 Apr 1;14(4):674-6.

Baram TZ, Mitchell WG, Tournay A, Snead III OC, Hanson RA, Horton EJ. High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study. Pediatrics. 1996 Mar;97(3):375.

Mackay MT, Weiss SK, Adams-Webber T, Ashwal S, Stephens D, Ballaban-Gill K, et al. Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society. Neurology. 2004 May 25;62(10):1668-81.

Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, et al. 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 Jun 12;78(24):1974-80.

Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW, et al. The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial. Lancet Neurol. 2005 Nov 1;4(11):712-7.

Thiele EA. Managing epilepsy in tuberous sclerosis complex. J Child Neurol. 2004 Sep;19(9):680-6.