West Syndrome – Infantile Spasms: A Rare Paediatric Case Report
West syndrome case report
Abstract
West syndrome (WS) is a rare, severe form of epilepsy with onset in infancy and early childhood. It involves clustered epileptic spasm episodes, the aberrant interictal electroencephalogram pattern known as hypsarrhythmia, and neuropsychomotor delay. In this view, we present a case of a 6-year-old female child a known case of west syndrome, global developmental delay, hypothyroidism, and post-operative laparoscopic gastrotomy performed under general anaesthesia on March 31/2022, in view of recurrent aspiration and came with the chief complaints of seizure 6-7 episodes per day, decreased urine output, constipation for two days, straining to pass urine, and three episodes of vomiting. The history included that the baby cried immediately after birth, seizure on day 5 of life, and was shifted to the Neonatal intensive care unit (NICU), admitted there for 5 days because of respiratory distress syndrome and hyperalbuminemia. During their stay in the hospital, the child has been prescribed Lamotrigine 50mg, Levocarnitine 500mg, Levetiracetam 250mg, Clobazam 5mg, Phenobarbital 60mg, Sodium Valproate 100mg, Thyronorm 50mcg. Now the patient is symptomatically better. This case report intends to sensitize physicians in this region toward this rare neurological syndrome.
Keywords: Hypsarrhythmia, Infantile, Seizure, West syndrome.
Keywords:
Hypsarrhythmia, Infantile, Seizure, West syndromeDOI
https://doi.org/10.22270/jddt.v13i4.6009References
Shields WD. Infantile spasms: little seizures, big consequences. Epilepsy Currents. 2006; 6(3):63-69. http://dx.doi.org/10.1111/j.1535-7511.2006.00100.x
Carvalho GDA, Lima OB, Melo VC, Silva KL. Actions of nursing care in the infant with west syndrome: a case report. J Res Fundamental Care Online. 2014; 6(4):1525–1533. http://dx.doi.org/10.9789/2175-5361.2014.v6i4.1525-1533
Khatri A, Kalra N, Tyagi R, Baweja M, Khandelwal D. Dental findings in patients with West syndrome: a report of two cases. J Indian Soc Pedod Prev Dent. 2014; 32(2):168–171. http://dx.doi.org/10.4103/0970-4388.130988
Cowan LD, Hudson LS. The epidemiology and natural history of infantile spasms. J Child Neurol. 1991; 6(4):355–364. http://dx.doi.org/10.1177/088307389100600412
Sidenvall R, Eeg-Olofsson O. Epidemiology of infantile spasms in Sweden. Epilepsia. 1995; 36(6):572–574. http://dx.doi.org/10.1111/j.1528-1157.1995.tb02569.x
Cowan LD, Bodensteiner JB, Leviton A, Doherty L. Prevalence of the epilepsies in children and adolescents. Epilepsia. 1989; 30(1):94–106. http://dx.doi.org/10.1111/j.1528-1157.1989.tb05289.x
Riikonen R. Epidemiological data of West syndrome in Finland. Brain Dev. 2001;2 3(7):539–541. http://dx.doi.org/10.1016/s0387-7604(01)00263-7
Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi group. Epilepsia. 2004; 45(11):1416–1428.http://dx.doi.org/10.1111/j.00139580.2004.02404.x
Hrachovy RA, Frost JD Jr. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol. 2003; 20(6):408–425. http://dx.doi.org/10.1097/00004691-200311000-00004
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; 62(10):1668–1681. http://dx.doi.org/10.1212/01.wnl.0000127773.72699.c8
West WJ: On a peculiar form of infantile convulsions. Lancet. 1841; 1:724–725. http://dx.doi.org/10.1016/s0140-6736(00)40184-4
Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O’Dell C, Whittemore V. Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents. BMC Pediatr. 2012; 12(1):108. http://dx.doi.org/10.1186/1471-2431-12-108
Prats JM, Garaizar C, Rua MJ, Garcia-Nieto ML, Madoz P. Infantile spasms treated with high doses of sodium valproate: initial response and follow-up. Dev Med Child Neurol. 1991; 33(7):617–25. http://dx.doi.org/10.1111/j.1469-8749.1991.tb14932.x
Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion. Epileptic Disord. 2007; 9(4):353–412. http://dx.doi.org/10.1684/epd.2007.0144
Chamberlain MC. Nitrazepam for refractory infantile spasms and the Lennox-Gastaut syndrome. J Child Neurol. 1996; 11(1):31–34. http://dx.doi.org/10.1177/088307389601100107
Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomized controlled trial. Lancet 2004; 364(9447):1773–1778.
Nelson GR. Management of infantile spasms. Transl Pediatr. 2015; 4(4):260-70. http://dx.doi.org/10.3978/j.issn.2224-4336.2015.09.01
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