Pathophysiological basis of West Syndrome: Literature review
DOI:
https://doi.org/10.33448/rsd-v13i4.45608Keywords:
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
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Vinícius Thomaz Pignatari; Giovanna Arjona Lamussi Silva; Luiza Lopes Cagliari; Maria Eduarda Valotto; Júlia Couto de Carvalho; Priscila Gomes Silva Serpa; Guilherme Kazama Tsujigushi
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.