Clinical and epidemiological characteristics of patients with Amyotrophic Lateral Sclerosis (ALS) in central Brazil
DOI:
https://doi.org/10.11606/issn.2176-7262.rmrp.2022.192620Keywords:
Amyotrophic Lateral Sclerosis, Brazil, Cross-sectional study, Epidemiology, Photographic panelAbstract
Objective: This study aimed to evaluate the clinical-epidemiological characteristics of patients with Amyotrophic Lateral Sclerosis (ALS) in the State of Goiás, Brazil. Methods: We conducted a descriptive cross-sectional study to assess medical records of patients with ALS followed-up at the State Rehabilitation and Readaptation Medical Center Dr. Henrique Santillo, Goiânia, GO, Brazil, between 2005 and 2018. In addition, we registered and created a photographic panel with the main clinical findings of ALS cases. Results: From 224 investigated patients, 51.8% were male, and 67.4% manifested the classic form of the disease. Initial symptoms were more frequent in the lower limbs (37.9%), and complications resulted in 45.5% of tracheostomy, 60.3% of gastrostomy, and 49.1% of deaths. Most patients had a five-year survival from the onset of symptoms, and no significant association between the use of non-invasive ventilation and increased survival were found. The analysis of the clinical-epidemiological characteristics showed a more extended time between the first symptoms and the diagnosis of the disease was observed. Conclusion: In this study, the time between the first symptoms and diagnosis was longer than in the literature, resulting in late treatments. In addition, there was no satisfactory result regarding survival with the use of non-invasive ventilation. Therefore, clinical-epidemiological studies of the disease in Brazil, as well as public awareness and training of professionals in recognition of ALS clinical signs will assist in early and more efficient interventions.
Downloads
References
Ghasemi M, Brown Jr RH. Genetics of Amyotrophic Lateral Sclerosis. Cold Spring Harb Perspect Med. 2017; 8(5): 1–38.
Raymond J, Oskarsson B, Mehta P, Horton K. Clinical characteristics of a large cohort of US participants enrolled in the National Amyotrophic Lateral Sclerosis (ALS) Registry, 2010–2015. Amyotroph Lateral Scler Frontotemporal Degener. 2019; 26(5-6): 413-420.
Swinnen B, Robberecht W. The phenotypic variability of amyotrophic lateral sclerosis. Nat Rev Neurol. 2014; 10(11): 661-670.
Yu X, Zhao Z, Shen H, Bing Q, Li N, Hu J. Clinical and Genetic Features of Patients with Juvenile Amyotrophic Lateral Sclerosis with Fused in Sarcoma (FUS) Mutation. Med Sci Monit. 2018; 24: 8750-8757.
Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, et al. Amyotrophic lateral sclerosis. Lancet. 2011; 377(9769): 942–955.
Masrori P, Damme PV. Amyotrophic lateral sclerosis: A clinical review. Eur J Neurol. 2020; 27(10): 1918-1929.
Van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, et al. Amyotrophic lateral sclerosis. Lancet. 2017; 390(10107): 2084-2098.
Nicolas A, Kenna KP, Renton AE, Ticozzi N, Faghri F, Chia R, et. al. Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron. 2018; 97(6): 1268-1283.
Moore A, Young CA, Hughes DA. Health Utilities and Costs for Motor Neurone Disease. Value in Health. 2019; 22(11): 1257-1265.
Jaiswal MK. Riluzole and edaravone: A tale of two amyotrophic lateral sclerosis drugs. Med Res Rev. 2019; 39(2): 733-748.
Calzada NG, Soro EP, Gomez LM, Bulta EG, Izquierdo AC, Panades MP, et al. Factors predicting survival in amyotrophic lateral sclerosis patients on non-invasive ventilation. Amyotroph Lateral Scler Frontotemporal Degener. 2016; 17(5-6): 337-342.
Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, et al. Amyotrophic lateral sclerosis. Nat Rev Dis Primers. 2017; 3: 170-185.
Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000; 1(5): 293-299.
Vasconcelos K, Oliveira ASB, Fuchs LFP, Simões RS, Girão MJBC, Soares Jr. JM, et al. Estrogens: possible protection against Amyotrophic Lateral Sclerosis? Rev Assoc Med Bras. 2019; 65(5): 576-578.
Prado LGR, Bicalho ICS, Vidigal-Lopes M, Ferreira CJA, Barbosa LSM, Gomez RS, et al. Amyotrophic lateral sclerosis in Brazil: Case series and review of the Brazilian literature. Amyotroph Lateral Scler Frontotemporal Degener. 2016; 17(3-4): 282-288.
Shibuya K, Misawa S, Uzawa A, Sawai S, Tsuneyama A, Suzuki Y-I, et al. Split hand and motor axonal hyperexcitability in spinal and bulbar muscular atrophy. J Neurol Neurosurg Psychiatry. 2020; 91(11).
Korner S, Kollewe K, Fahlbusch M, Zapf A, Dengler R, Krampfl K, et al. Onset and spreading patterns of upper and lower motor neuron symptoms in Amyotrophic lateral sclerosis. Muscle Nerve. 2011; 43: 636-642.
Lindauer E, Dupuis L, Müller H-P, Neumann H, Ludolph AC, Kassubek J. Adipose tissue distribution predicts survival in Amyotrophic lateral sclerosis. PloS ONE. 2013; 8(6): e67783.
Park YJ, Lee J, Kim SH, Ko SH, Shin MJ, Chang JH, et al. Care status of the ALS patients with long-term use of tracheostomy tube. Ann Rehabil Med. 2015; 39(6): 964-970.
Kak M, Issa NP, Roos RP, Sweitzer BJ, Gottlieb O, Guralnick A, et al. Gastrostomy tube placement is safe in advanced Amyotrophic lateral sclerosis. Neurol Res. 2017; 39(1): 16-22.
Ryan M, Heverin M, Doherty MA, Davis N, Corr EM, Vajda A, et al. Determining the incidence of familiality in ALS A study of temporal trends in Ireland from 1994 to 2016. Neurol Genet. 2018; 4(3): e239.
Logroscino G, Traynor BJ, Hardiman O, Chiò A, Mitchell D, Swingler RJ, et al. Incidence of Amyotrophic Lateral Sclerosis in Europe. J Neurol Neurosurg Psychiatry. 2010; 81(4): 385–390.
Abe T, Madotto F, Pham T, Nagata I, Uchida M, Tamiya N, et al. Epidemiology and patterns of tracheostomy practice in patients with acute respiratory distress syndrome in ICUs across 50 countries. Crit Care. 2018; 22(1): 195.
Kirstein MM, Körner S, Schneider A, Manns MP, Petri S, Voigtländer T. Percutaneous endoscopic gastrostomy with and without jejunal extension in patients with amyotrophic lateral sclerosis. Eur J Gastroenterol Hepatol. 2018; 30(3): 257-262.
Carvalho M, Gooch CL. The yin and yang of gastrostomy in the management of ALS Friend or foe? Neurology. 2017; 89(14): 1435-1436.
Benjaminsen E, Alstadhaug KB, Gulsvik M, Baloch FK, Odeh F. Amyotrophic lateral sclerosis in Nordland county, Norway, 2000–2015: prevalence, incidence, and clinical features. Amyotroph Lateral Scler Frontotemporal Degener. 2018; 19(7-8): 522-527.
Longinetti E, Fang F. Epidemiology of amyotrophic lateral sclerosis: an update of recent literature. Curr Opin Neurol. 2019; 32(5): 771-776.
Rosenbohm A, Peter RS, Erhardt S, Lulé D, Rothenbacher D, Ludolph AC, et al. Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol. 2017; 264(4): 749-757.
Jun KY, Park J, Oh K-W, Kim EM, Bae JS, Kim I, et al. Epidemiology of ALS in Korea using nationwide big data. J Neurol Neurosurg Psychiatry. 2019; 90(4): 395-403.
Hirose T, Kimura F, Tani H, Ota S, Tsukahara A, Sano E, et al. Clinical characteristics of long‐term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis. Muscle Nerve. 2018; 58(6): 770-776.
Downloads
Published
Issue
Section
License
Copyright (c) 2022 Dhiogo da Cruz Pereira Bento, Rayana Pereira Dantas de Oliveira, Kamilla de Faria Santos, Rômulo Morais Azevedo, Lays Bernardes Minasi, Yves Mauro Fernandes Ternes, Angela Adamski da Silva Reis, Rodrigo da Silva Santos
This work is licensed under a Creative Commons Attribution 4.0 International License.
