Pathophysiological basis of West Syndrome: Literature review

Authors

DOI:

https://doi.org/10.33448/rsd-v13i4.45608

Keywords:

Neurodevelopmental disorders; Spasm; Epilepsy.

Abstract

West Syndrome is an epileptic encephalopathy composed of a triad: muscle spasms, predominantly in clusters, delay in neuropsychomotor development and hypsarrhythmia observed on the electroencephalogram. The pathophysiology is not yet fully understood. The most commonly used diagnostic methods today are EEG, which is performed in all cases, computed tomography and transfontanellar ultrasound. Studies on preferred drug therapy are inconclusive, suggesting multiple treatment and dosage options. The lack of knowledge of West Syndrome on the part of the primary health care medical team leads to late diagnosis, which delays treatment, culminating in delayed neuropsychomotor development that may be irreversible. This article aims to analyze the intrinsic characteristics of the disease, as well as pathophysiology, clinical picture, diagnostic and therapeutic advances. The methodology consists of an integrative literature review. Therefore, the development of research on the pathophysiology of the disease to determine new therapies is essential, leading to the search for improving the restricted prognosis and quality of life, guaranteeing better survival of patients with West syndrome.

References

Anne-Lise Poulat, G. L., Sanlaville, D., Blanchard, G., Lion-François, L., Christelle Rougeot, Vincent des Portes, & Ville, D. (2014). A proposed diagnostic approach for infantile spasms based on a spectrum of variable aetiology. European Journal of Paediatric Neurology, 18(2), 176–182. https://doi.org/10.1016/j.ejpn.2013.11.005

Arce Portillo, E., Rufo Campos, M., Muñoz Cabello, B., Blanco Martínez, B., Madruga Garrido, M., Ruiz del Portal, L., & Candau Fernández-Mensaque, R. (2011). Síndrome de West: etiología, opciones terapéuticas, evolución clínica y factores pronósticos. Revista de Neurología, 52(02), 81. https://doi.org/10.33588/rn.5202.2010392

Calderón Romero, M., Arce Portillo, E., López Lobato, M., Muñoz Cabello, B., Blanco Martínez, B., Madruga Garrido, M., & Alonso Luego, O. (2018). Síndrome de West criptogénico: perfil clínico, respuesta al tratamiento y factores pronósticos [Cryptogenic West syndrome: Clinical profile, response to treatment and prognostic factors]. Anales de pediatria, 89(3), 176–182. https://doi.org/10.1016/j.anpedi.2017.10.012

Campistol Plana, J., & García Cazorla, À. (2003). Síndrome de West. Análisis, factores etiológicos y opciones terapéuticas. Revista de Neurología, 37(04), 345. https://doi.org/10.33588/rn.3704.2003181

Garcia, T. R., Cruz, M. C. A., Silva, G. de O. A., Cardoso, E. F., & Arruda, J. T. (2020). Canabidiol para o tratamento de pacientes com Síndrome de West e epilepsia. Research, Society and Development, 9(9), e420997267. https://doi.org/10.33448/rsd-v9i9.7267

Grubits Freire, H. B., de Andrade, P. R., & Motti, G. S. (2016). Equoterapia como recurso terapêutico no tratamento de crianças autistas. Multitemas, (32). https://www.multitemas.ucdb.br/multitemas/article/view/709

Güveli, B. T., Çokar, Ö., Dörtcan, N., Benbir, G., Demirbilek, V., & Dervent, A. (2015). Long-term outcomes in patients with West syndrome: An outpatient clinical study. Seizure - European Journal of Epilepsy, 25, 68–71. https://doi.org/10.1016/j.seizure.2015.01.001

Haeser Martignago, F., de Oliveira Pinho, G. K., Baumer, J. M., Chirolli, M. J., Cony Quinteiro, S., Freitas Paniz, V. L., & da Silva Granez, M. V. (2015). Benefícios da equoterapia em crianças com deficiência intelectual e múltipla. Extensão Tecnológica: Revista De Extensão Do Instituto Federal Catarinense, (3), 75–82. https://publicacoes.ifc.edu.br/index.php/RevExt/article/view/99

Igor Costa Santos, Bernardo Machado Bernardes, Madalena, L., Elisa, A., & Eduarda, M. (2023). Síndrome de west em neonatos: avaliação neurológica. Revista Ibero-Americana de Humanidades, Ciências E Educação, 9(9), 846–858. https://doi.org/10.51891/rease.v9i9.11230

Jones, K., O. Carter Snead, Boyd, J., & Go, C. (2014). Adrenocorticotropic Hormone Versus Prednisolone in the Treatment of Infantile Spasms Post Vigabatrin Failure. Journal of Child Neurology, 30(5), 595–600. https://doi.org/10.1177/0883073814533148

Knight, E. M. P., & Wyllie, E. (2022). West Syndrome and the new classification of epilepsy. The Lancet Neurology, 21(8), 689. https://doi.org/10.1016/s1474-4422(22)00267-8

Liberalesso, P. (2018). Síndromes epilépticas na infância. Uma abordagem prática. 8(0), 56–63. https://doi.org/10.25060/residpediatr-2018.v8s1-10

Neto, J. M. R., & Marques, D. K. A., Fernandes, M. G. M., & Nóbrega, M. M. L. (2016). Análise de teorias de enfermagem de Meleis: revisão integrativa. Revista Brasileira de Enfermagem. 69 (1). 162-168.

Pavone, P., Polizzi, A., Marino, S. D., Corsello, G., Falsaperla, R., Marino, S., & Ruggieri, M. (2020). West syndrome: a comprehensive review. Neurological Sciences, 41(12), 3547–3562. https://doi.org/10.1007/s10072-020-04600-5

Pozo Alonso, A. J., Pozo Lauzán, D., & Pozo Alonso, D. (2002). Síndrome de West: etiología, fisiopatología, aspectos clínicos y pronósticos. Revista Cubana de Pediatría, 74(2), 151–161. http://scielo.sld.cu/scielo.php?script=sci_abstract&pid=S0034-75312002000200009

Silva, J. F., Borges, T. M., Guimarães, D. V., Ferro, L. C. de C., Albuquerque, É. F. de, Leal, B. M., Rodrigues, A. F. P., Ribeiro, G. G. C., Bitencourt, A. M., Braga, Y. C., Pinheiro, M. M., Fleury, C. L., Barbosa, R. P., & Oliveira, M. P. (2023). Síndrome de West: uma abordagem diagnóstica, evolução clínica e revisão. Brazilian Journal of Health Review, 6(5), 21567–21575. https://doi.org/10.34119/bjhrv6n5-186

Souza, J. R., Ribeiro, C. da C., & Lamônica, D. A. C. (2020). Características clínicas e do neurodesenvolvimento da Síndrome de West. In Anais. Bauru: Faculdade de Odontologia de Bauru, Universidade de São Paulo.

Souza, M. T., Silva, M. D., & Carvalho, R. (2010). Revisão integrativa: o que é e como fazer. Revista Einstein, 8 (1),102-106

Teixeira, C. C. C., Inácio, G. B., Lima, J. L., Freitas, A. J. O., Costa, M. I. V. da, Dias, L. H. R., & Nardy, M. J. M. (2022). O efeito do uso do Canabidiol em crianças com Epilepsia Refratária. Brazilian Journal of Development, 8(7), 54307–54327. https://doi.org/10.34117/bjdv8n7-347

Ware, T. L., Mackay, M. T., Harvey, A. S., & Freeman, J. L. (2012). Epileptic spasms: Experience with a high-dose oral corticosteroid protocol. Journal of Paediatrics and Child Health, 48(11), 985–989. https://doi.org/10.1111/j.1440-1754.2012.02582.x

Wheless, J. W., Gibson, P. A., Rosbeck, K. L., Hardin, M., O’Dell, C., Whittemore, V., & Pellock, J. M. (2012). Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents. BMC Pediatrics, 12(1). https://doi.org/10.1186/1471-2431-12-108

Published

22/04/2024

How to Cite

PIGNATARI, V. T. .; SILVA, G. A. L. .; CAGLIARI, L. L. .; VALOTTO, M. E.; CARVALHO, J. C. de .; SERPA, P. G. S. .; TSUJIGUSHI, G. K. . Pathophysiological basis of West Syndrome: Literature review. Research, Society and Development, [S. l.], v. 13, n. 4, p. e8613445608, 2024. DOI: 10.33448/rsd-v13i4.45608. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/45608. Acesso em: 22 dec. 2024.

Issue

Section

Health Sciences