Survival in patients with Amyotrophic Lateral Sclerosis
Keywords:
esclerosis lateral amiotrófica, enfermedad de la motoneurona, sobrevida, factores de riesgo, formas clínicas, mortalidad, riluzol.Abstract
Introduction. Amyotrophic lateral sclerosis (ALS) is the most frequent disease in the heterogeneous group of disorders with motor neuron diseases.
Objective: To characterize the survival of patients diagnosed with amyotrophic lateral sclerosis considering factors related to their clinical behavior at "Dr. Jose Rafael Estrada Gonzalez” National Institute of Neurology and Neurosurgery, Havana, Cuba.
Material and Methods: A descriptive and retrospective research was conducted. The study included a case series of 147 patients diagnosed with ALS by clinical and neurophysiological confirmation and images. The patients were treated in the multidisciplinary consultation in the period from October 2005 to October 2015. A total of 110 of them already died.
Results: The disease most often occurs between the ages of 51 and 60. In the first 40 months, most of the patients in the series died, for a total of 80 people. The spinal clinical form predominated in males who had higher survival; the bulbar form prevailed in women. Most of the patients had no risk factors. Diabetes, arterial hypertension, ischemic cerebrovascular disease, neoplasms, hepatitis C, head trauma, bronchial asthma and ischemic heart disease stand out as comorbidities. There were cases of the disease within a single family.
Conclusions The greatest survival from the diagnosis of the disease was observed in the group between 51 and 60 years of diagnosis of the disease, some of them reaching up to 10 years. The general average of survival was between 2 and 5 years. It was lower in patients with comorbidities, family history and bulbar form. After evaluating the effectiveness of the treatment with Riluzole, the survival was not significant.
Keywords: amyotrophic lateral sclerosis, motor neuron disease, survival, risk factors, clinical forms, mortality, Riluzole.
Downloads
References
1. Brown Robert H. Jr. Esclerosis lateral amiotrófica. En: Harrison. Principios de Medicina Interna. 19 a ed. McGraw-Hill Interamericana Editores, S.A. México 2016; p: 2631-36.
2. Saiz A. Enfermedades de la motoneurona. Esclerosis Lateral Amiotrófica. En: Farreras-Rozman. Medicina Interna, 18 a ed. Elsevier, España, 2016; p: 1379-82
3. Shaw Pamela J. Esclerosis Lateral Amiotrófica. En: Cecil. Tratado de Medicina Interna. 25 a ed. Elsevier España 2016; p: 2347-52
4. Pritzker D.N, Abbas, A.K, Aster J.C. Esclerosis lateral amiotrófica. En: Robbins. Patología Humana; 10ma edición, Elsevier España, 2017; p: 841-48
5. Colectivo de autores. Amyotrophic lateral sclerosis. In: Neurological disease. Davidson's Principles and Practice of Medicine 22 Ed. Elsevier Limited. 2014; p: 2230-2280
6. Raina Gabriela. Esclerosis Lateral amiotrófica. En: Enfermedades de la neurona motora. Micheli-Pardal Neurología. 2da. Ed. Panamericana, Buenos Aires 2010; p: 291-99
7. Nunes Gonçalo, Santos Carla Adriana, Grunho Miguel, Fonseca Jorge. Enteral feeding through endoscopic gastrostomy in amyotrophic lateral sclerosis patients. Nutr. Hosp. [Internet]. 2016 Oct [citado 2018 Jul 06]; 33(5): 1015-1021. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0212-16112016000500002&lng=es. http://dx.doi.org/10.20960/nh.561
8. Rianchoa J,Gonzalob I, Ruiz-Sotob M, Bercianoa J. ¿Por qué degeneran las motoneuronas? Actualización en la patogenia de la esclerosis lateral amiotrófica. Neurología. [Internet] 2015. [citado 22 may 2016]. Disponible en: http://dx.doi.org/10.1016/j.nrl.2015.12.001
9. Zapata-Zapata, C. H., Franco-Dáger, E., Solano-Atehortúa, J. M., Ahunca-Velásquez, L. F. Esclerosis lateral amiotrófica: actualización. Iatreia, [Internet] 2016; 29(2): 194-205. Acceso 8 feb 2018 Disponible en: https://www.redalyc.org/pdf/1805/180544647008.pdf
10. Camacho A, Esteban J, Paradas C. Report by the Spanish Foundation for the Brain on the social impact of amyotrophic lateral sclerosis and other neuromuscular disorders. Neurología [Internet] 2018; 33(1): 35-46 [citado 8 Mar 2018] Disponible en: https://www.sciencedirect.com/science/article/pii/S0213485315000341
11. Ysasi, G. C. G., Prado, F., Herrero, M. V., Bach, J. R. Alternativas de tratamiento en pacientes con patologías neuromusculares y afecciones respiratorias. In Anales de la Facultad de Ciencias Médicas [Internet] 2017; 50(2): 79-88. Acceso 3 dic 2017 Disponible en: http://revistascientificas.una.py/index.php/RP/article/view/1438
12. Pinchak Catalina, Salinas Pamela, Prado Francisco, Herrero María, Giménez Gloria, García Carolina et al. Actualización en el manejo respiratorio de pacientes con enfermedades neuromusculares. Arch. Pediatr. Urug. [Internet]. 2018 Feb [citado 2018 Jul 05]; 89(1): 40-51. Disponible en: http://www.scielo.edu.uy/scielo.php?script=sci_arttext&pid=S1688-12492018000100040&lng=es. http://dx.doi.org/10.31134/ap.89.1.8
13. Akly, M. P., Schiava, M., Melcom, M., Rodríguez, G., Gargiulo, G., Bettini, M., de Ambrosi, B. Estudio epidemiológico multicéntrico sobre esclerosis lateral amiotrófica en la Ciudad de Buenos Aires. Neurología Argentina [Internet] 2017, 9(4), 225-230. Acceso 8 ene 2018 Disponible en: https://www.sciencedirect.com/science/article/abs/pii/S185300281730068X
14. Díaz-Gómez, M. F., Ortiz-Corredor, F. Diseño y validación de un sistema de clasificación para evaluar el grado de discapacidad de los pacientes con esclerosis lateral amiotrófica. Rev Neurol, [Internet] 2017; 64:112-8. [citado 8 Mar 2018] Disponible en: https://www.researchgate.net/profile/Fernando_Ortiz-Corredor/publication/279463912_Diseno_de_un_sistema_de_clasificacion_para_evaluar_el_grado_de_discapacidad_de_los_pacientes_con_esclerosis_lateral_amiotrofica/links/5922a499a6fdcc4443f61ac9/Diseno-de-un-sistema-de-clasificacion-para-evaluar-el-grado-de-discapacidad-de-los-pacientes-con-esclerosis-lateral-amiotrofica.pdf
15. Riancho, J., Gonzalo, I., Ruiz-Soto, M., Berciano, J. ¿Por qué degeneran las motoneuronas? Actualización en la patogenia de la esclerosis lateral amiotrófica. Neurología. [Internet] 2019; 34(1):27-37 [citado 8 Mar 2019] Disponible en: https://www.sciencedirect.com/science/article/pii/S0213485316000025
16. Hardiman O. Amyotrophic Lateral Sclerosis. In: Hardiman O, Doherty C, editors. Neurodegenerative Disorders. London: Springer-Verlag 2011. p. 143-66
17. Enriquez-Marulanda, A., Uribe-Becerra, J. P., Granados, A. M., Shinchi, M. High-intensity signal in the corticospinal tract in MRI as a sign of upper motor neuron lesion in amyotrophic lateral sclerosis: Case report and literature review. Acta Neurológica Colombiana [Internet] 2017; 33(2): 99-103. [citado 8 dic 2017] Disponible en: http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0120-87482017000200099
18. Brooks B, Miller R, Swash M. For the world federation of neurology research group on motor neuron diseases. El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic lateral Sclerosis [Internet] 2000; 1: 293-9. [citado 8 dic 2017] Disponible en: https://www.tandfonline.com/doi/abs/10.1080/146608200300079536?journalCode=iafd18
19. Mandrioli J. Epidemiology of ALS in Emilia Romagna Region. A population based Study. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, [Internet] 2014; 15(3-4):262-268. [citado 10 dic 2017]. Disponible en: https://www.tandfonline.com/doi/abs/10.3109/21678421.2013.865752
20. Silva, L. P., Gusmão, C. A., Pithon, K. R., Gomes, T. B. P., & Junior, E. P. P. Esclerose lateral amiotrófica: descrição de aspectos clínicos e funcionais de uma série de casos numa região de saúde do nordeste do Brasil. Journal of Health & Biological Sciences 2018 [citado 2018 Oct 05]; 6(3), 293-298. Disponible en: periodicos.unichristus.edu.br/jhbs/article/viewFile/1811/702
21. Labra J, Menon P, Byth K, Morrison S, Vucic S. Rate disease progression: a prognostic biomarker of ALS. J Neurol Neurosurg Psychiatry [Internet] 2016; 87:628-632. [citado 20 dic 2017]. Disponible en: https://jnnp.bmj.com/content/87/6/628.short
22. Camacho Assef, J. A., Camacho Escalante, L., Gómez Mantilla, N., Camacho Assef, V., López Borroto, K., García Garriga, H. Development of regenerative medicine in Cuba. Mediciego [Internet] 2017; 23(4):55-60. [citado 2 dic 2018] Disponible en: https://www.medigraphic.com/pdfs/mediciego/mdc-2017/mdc174i.pdf
23. Carmona Espinazo, F., Perteguer Huertas, I., Alarcón Manoja, M., García Polo, C., & Moya Molina, A. (. Atención clínica, toma de decisiones y soporte nutricional en pacientes con esclerosis lateral amiotrófica. Nutr. clin. diet. hosp, [Internet] 2016; 36(2): 200-205. [citado 10 dic 2017]. Disponible en: https://revista.nutricion.org/PDF/carmonaespinazo.pdf
24. Gallardo, N., Arantzamendi, M., Carvajal, A. (). Revisión narrativa sobre la calidad de vida relacionada con la salud en personas con esclerosis lateral amiotrófica. Medicina Paliativa [Internet] 2018; 25(2):105-113. [citado 20 dic 2018] Disponible en: https://www.sciencedirect.com/science/article/pii/S1134248X17300885
25. Ingre Caroline, Roos Per M, Piehl Fredrik, Kamel Freya, Fang Fang. Factores de riesgo para esclerosis lateral amiotrófica. Clinical Epidemiology [Internet] 2015; 7: 181–193. [citado 10 dic 2017] Disponible en: http://dx.doi.org/10.2147/CLEP.S37505
26. Schuster C, Hardiman O, Bede P. Survival prediction in Amyotrophic lateral sclerosis based on MRI measures and clinical characteristics. BMC Neurology [Internet] 2017; 17:73-83 [citado 22 dic 2018]. Disponible en: DOI 10.1186/s12883-017-0854-x https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-017-0854-x
27. Grigorakos L, Lazarescu D, Myrinathefs P, Antonopoulos G, Agathonikou A, Sotiriou E, et al. Patients with amyotrophic lateral sclerosis in the intensive care unit. Curr Opin Neurol Sci. [Internet] 2016; 1:12–18. [citado 22 dic 2018] Disponible en: https://scientiaricerca.com/srcons/SRCONS-01-00004.php
28. Cordesse V, Sidorok F, Schimmel P, Holstein J, Meininger V. Coordinated care affects hospitalization and prognosis in amyotrophic lateral sclerosis: a cohort study. BMC Health Serv Res. [Internet] 2015; 15:134. [citado 22 dic 2018] Disponible en: https://bmchealthservres.biomedcentral.com/articles/10.1186/s12913-015-0810-7
29. Scull Torres, M. Cuidados paliativos en esclerosis lateral amiotrófica. Medimay [Internet], 2018; 25(1):68-76. [citado 22 dic 2018]. Disponible en: https://www.medigraphic.com/pdfs/revciemedhab/cmh-2018/cmh181h.pdf
30. Pisa F.E. Hospitalizations due to respiratory failure in patients with Amyotrophic Lateral Sclerosis and their impact on survival: a population based cohort Study. BMC Pulmonary Medicine [Internet] 2016; 16:136-45 [citado 12 dic 2019] Disponible en: https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-016-0297-y
31. Gonzalez-Bermejo, J., Morélot-Panzini, C., Tanguy, M. L., Meininger, V., Pradat, P. F., Lenglet, T., .Desnuelle, C.. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial. The Lancet Neurology [Internet], 2016; 15(12):1217-1227. [citado 12 dic 2019]. Disponible en: https://www.sciencedirect.com/science/article/abs/pii/S1474442216302332
32. Talman, P., Duong, T., Vucic, S., Mathers, S., Venkatesh, S., Henderson, R., Macdonnell, R. Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort. BMJ open[Internet]. 2016; 6(9):e012054. [citado 12 dic 2019]. Disponible en: https://bmjopen.bmj.com/content/6/9/e012054?utm_term=usage-012019&utm_content=consumer&utm_campaign=bmjopen&utm_medium=cpc&utm_source=trendmd
33. Knibb JA, et al. Una herramienta clínica para predecir supervivencia en la ELA. J Neurol Neurosurg Psychiatry [Internet] 2016; 87:1361–1367. [citado 12 dic 2019]. doi:10.1136/jnnp-2015-312908
34. Ong M-L, Tan PF, Holbrook JD Predicting functional decline and survival in amyotrophic lateral sclerosis. PLoS ONE [Internet] 2017; 12(4): e0174925. [citado 4 ene 2019]. https://doi.org/10.1371/journal
35. Farrero E, Antón A, Egea CJ, Almaraz MJ, Masa JF, Utrabo I, et al. Normativa sobre el manejo de las complicaciones respiratorias de los pacientes con enfermedad neuromuscular. Sociedad Española de Neumología y Cirugía Torácica (SEPAR). Arch Bronconeumol. [Internet] 2013; 49:306–13. [citado 4 ene 2019] Disponible en: https://dialnet.unirioja.es/servlet/revista?codigo=2926
36. Schuster. Survival prediction in Amyotrophic lateral sclerosis based on MRI measures and clinical characteristics. BMC Neurology [Internet] 2017; 17:73-83 [citado 4 ene 2019]. Disponible en: https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-017-0854-x
37. Domínguez LO, Ramos LM, Toledo L, Montes de Oca TM Esclerosis lateral amiotrófica: un reto actual para las neurociencias. 16 de Abril [Internet]. 2018; 57(267):55-63 [citado 4 ene 2019]. Disponible en: https://www.medigraphic.com/pdfs/abril/abr-2018/abr18267j.pdf
Download PDF
