DOI: https://dx.doi.org/10.18203/2349-3291.ijcp20221066
Published: 2022-04-25

Correlation of neuroimaging findings and outcome in West syndrome

Bina Prajapati Manandhar, Divya K. C., Najala Khatun

Abstract


Background: West syndrome (WS), an infantile epileptic encephalopathy which occurs in clusters with developmental regression and hypsarrhythmia. The classification refers to genetic, structural and metabolic cause. Neuroimaging can help to identify various lesion in the brain leading to West syndrome and outcomes can be measured in form of seizure control and neurodevelopmental improvement. We aimed to study the correlation of neuroimaging in West syndrome with the outcomes.

Methods: This was a retrospective cross-sectional study done among patients who presented to International friendship children’s hospital. All the cases diagnosed with WS and between 4 months to 5 years of age according to ILAE (International league against epilepsy) were included in this study. The study duration was three years. Fisher’s exact test was used to see correlation of underlying neuroimaging findings with outcome in children with West syndrome.

Results: Of 37 children diagnosed with WS, 65% were male with a mean age of presentation 17±7 months and mean age of onset of seizure 5±1 months. Thirty patients had regular follow up and neurodevelopmental outcome could be determined. There was no significant relation between neuroimaging finding and neurodevelopmental outcome along with seizure remission (p>0.05). Improvement in developmental milestones was seen in 17 patients (56.6%) and 1 patient (3.3%) had normal milestone as per age.

Conclusions: Neuroimaging is an important diagnostic tool to determine etiology of WS but it’s not the sole component to predict the neurodevelopmental outcome and seizure remission.


Keywords


West syndrome, Neuroimaging, Neurodevelopment

Full Text:

PDF

References


Mikati MA, Tchapyjnikov D. Generalized seizures and related epilepsy syndromes. In: Kliegman RM, Geme JWS, Blum NJ, eds. Nelson text book of Pediatrics. 21st ed. Philadelphia: Elsevier; 2019: 3098-9.

Khatami A, Sell E, Aggag M, Miller E. Brain MRI findings in infantile spasm: outcome correlations in a patient COHORT. Open J Med Imag. 2016;6:80-92.

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

Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Develop. 2014;36(9):739-51.

Pellock JM, Hrachovy R, Shinnar S. Infantile spasms: a U.S. consensus report. Epilepsia. 2010;51(10):2175-89.

Lúthvígsson P, Olafsson E, Sigurthardóttir S, Hauser WA. Epidemiologic features of infantile spasms in Iceland. Epilepsia. 1994;35(4):802-5.

Sidenvall R, Eeg-Olofsson O. Epidemiology of infantile spasms in Sweden. Epilepsia. 1995;36(6):572-4.

Jia JL, Chen S, Sivarajah V, Derek S, Cortez MA. Latitudinal differences on the global epidemiology of infantile spasms: systematic review and meta-analysis. Orphan J Rare Dis.2018;13(216):1-17.

Frost JD, Hrachovy RA. Pathogenesis of infantile spasms: a model based on developmental desynchronization. J Clin Neurophysiol. 2005;22(1):25-36.

Berg AT, Berkovic SF, Brodie MJ. Revised terminology and concepts for organization of seizures and epilepsies. Report of the ILAE commission on classification and terminology. 2005-2009. Epilepsia. 2010;51:676-85.

Akter N, Rahman MM, Akhter S, Fatema K. Neuroimaging findings as a predictor of choice of antiepileptic drugs for seizure control in West syndrome. Bangladesh J Child Health. 2018;42(1):9-14.

Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, west syndrome and related phenotypes: what we know in 2013. Brain Develop. 2014;36:739-51.

Okumura A, Watanabe K, Negoro T, Aso K, Natsumo J, Matsumoto A, et al. Evolutional changes and outcome of west syndrome: correlation with magnetic resonance imaging findings. Epilepsia. 1998;39(5):46-9.

Wong M, Trevathan E. Infantile spasms. Pediatric Neurol. 2001;24:89-98.

Lagie L, Verhelst H, Ceulemans B. Treatment and longterm outcome in West syndrome: the clinical reality. A multicentre follow up study. Seizure. 2010;19:159-64.

Barbarrosa EP, Ferrer ICP, Tovani-Palone MR. West syndrome: clinical characteristics, therapeutics, outcomes and prognosis. Electron J Gen Med. 2020;17(2):190.

Sharma NL, Viswanathan V. Outcome in West syndrome. Ind Pediatr. 2008;45:559-63.

Pavone P, Polizzi A, Marino SD, Corsello G, Falsaperla R, Marino S, et al. West syndrome: a comprehensive review. Neurol Sci. 2020;41(12):3547-62.

Castano G, Lyons CG, Jan JE, Connolly M. Cortical visual impairment in children with infantile spasms. J AAPOS. 2000;4(3):175-8.

Gupta J, Sharma S, Mukherjee SB, Jain P, Aneja S. Neuro-developmental and epilepsy outcomes of children with West syndrome: a cross-sectional study from North India. Ann Indian Acad Neurol. 2020;23(2):177-81.

Lombroso CT. A prospective study of infantile spasms: clinical and therapeutic correlations. Epilepsia. 1983;24(2):135-58.

Riikonen R. Long-term outcome of patients with West syndrome. Brain Dev. 2001;23(7):683-7.