Epidemiological and clinical factors impact on survival in ALS/ MND: a cohort study
Keywords:epidemiology, amyotrophic lateral sclerosis, motor neuron disease, survival,
Background Motor neuron diseases (MND) are rare yet severe neurodegenerative disorders with peculiar characteristics of unknown etiologies that cannot be prevented. They are irreversible and incurable, but treatable diseases. Treatment can improve the quality of life and increase survival. This study aimed to investigate the impact of epidemiological and clinical factors in individuals with motor neuron diseases, particularly amyotrophic lateral sclerosis (ALS). This study aimed to analyse the survival of patients with ALS in relation to clinical and epidemiological aspects. Methods Data were collected from charts in the Division of Clinical Investigation on Neuromuscular Diseases (DCINM), (from 1999 to 2011). Kaplan-Meier curves illustrated the survival time. Results A total of 1146 records of patients with MND were investigated and 578 records, in accordance with the inclusion criteria, were included. For analyzing epidemiological and clinical aspects 231 records were included and survival curves were plotted. Conclusion The survival of patients with MND was not influenced by sex. Younger patients live longer. Too much time passes between initial symptoms and 1st appointment. Patients who survive longest are those who were not diagnosed on the 1st appointment, or did not show any clinical and electromyographic findings, and presented with initial symptoms in the lower limbs
Brooks BR. World Federation of Neurology Research Group on Neuromuscular Diseases. El Escorial: World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J. Neurol Sci. 1994; 124 (Suppl 1): 96-107.
Swash M, Desai J. Motor Neuron Disease: classification and nomenclature. Amyotrophic Lateral Sclerosis and other Motor Neuron Disorders. 2000; 1:105-112.
Meininger V, Shefner J, Cudkowicz M. Lithium therapy in ALS. Amyotrophic lateral sclerosis: official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases. 2008; 9 (2):122.
Rothstein JD. Excitotoxic mechanisms in the pathogenesis of amyotrophic lateral sclerosis. Adv Neurol. 1995; 68:7-20.
Cifra A, Nani F, Nistri A. Eur J Neurosci. Riluzole is a potent drug to protect neonatal rat hypoglossal motoneurons in vitro from excitotoxicity due to glutamate uptake block. 2011 Mar; 33(5).
Shaw PJ, Ince PG. Glutamate, excitotoxicity and amyotrophic lateral sclerosis. J Neurol. 1997; 244: Suppl 2:S3-S14.
Akhlaq A. Farooqui, Wei-Yi Ong, Lloyd Horrocks. in Neurochemical Aspects of Excitotoxicity. Springer, 2008.
Morahan JM, Yu B, Trent Ronald J, Pamphlett R. Genetic susceptibility to environmental toxicants in ALS. American Journal of Medical Genetics. Part B, Neuropsychiatric genetics: the official publication of the International Society of Psychiatric Genetics. 2007;144B(7):885-90.
Bradley W. New aspects of the cyanobacteria/BMAA hypothesis and future directions. Amyotrophic Lateral Sclerosis.2008; 9 (Suppl1): 40.
Chen A, Montes J, Mitsumoto H. The role of exercise in amyotrophic lateral sclerosis. Physical medicine and rehabilitation clinics of North America. 2008; 19(3):545-57.
Piazza O, Siren AL, Ehrenreich H. Soccer, neurotrauma and amyotrophic lateral sclerosis: is there a connection? Current Medical Research and Opinion. 2004;20:( 4):505-508.
Armon C. An Evidence-Based Medicine Approach to the Evaluation of the Role of Exogenous Risk Factors in Spopradic Amyotrophic Lateral Sclerosis. Neuroepidemiology. 2003;22:217-228.
Taioli E. All causes mortality in male professional soccer players. European Journal of Public Health. 2007;17:(6):600-604. doi:10.1093/eurpub/ckm035.
ChiÃ³ A, Traynor BJ, Swingler R, Mitchell D, Hardiman O, Mora G, et al. Amyotrophic lateral sclerosis and soccer: a different epidemiological approach strengthen the previous findings. Journal of the Neurological Sciences. 2008; 269(1-2):187-8; author reply 188-9.
Sutedja NA, Veldink JH, Fischer K, Kromhout H, Wokke JHJ, Huisman MHB, et al. Lifetime occupation, education, smoking, and risk of ALS. Neurology. 2007; 69(15):1508-14.
Horner RD, Kamins KG, Feussner JR, Grambow SC, Hoff-Lindquist J, Harati Y, et al. Occurrence of amyotrophic lateral sclerosis among Gulf War veterans. Neurology . 2003; 61(6):742-9.
Ellis CM, Simmons A. Diffusion tensor MRI assesses corticospinhal tract damage in ALS. Neurology. 1999; 53: 1051-1058.
Brooks Br. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic lateral sclera other motor neuron disord. 2000;1:293-299.
Jacob S. Diffusion tensor imaging for long-term follow-up of corticospinhal tract degeneration in amyotrophic lateral sclerosis. Neuroradiology. 2003; 45: 598-600.
Grahan JM. Diffusion tensor imaging for the assessment of upper motor neuron integrity in ALS. Neurology. 2004;63:2111-2119.
Rocha AJ, Oliveira ASB. Detection of corticospinhal tract compromise in amyotrophic lateral sclerosis with brain MR Imaging: Relevance of the T1-Weighted spin-echo magnetization transfer contrast sequence. AJNR Am J Neuroradiol. 2004; 25: 1509-1515.
Sach M, Winkler G. Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. 2004; 127:340-350.
Oliveira ASB, Pereira RDB. Amyotrophic Lateral Sclerosis - Three Letters that change the peopleâ€™s Life For Ever. Arq. Neuropsiquiatr. 2009;67(3A):750-82.
Mitsumoto H, Rabkin JG. Palliative care for patients with amyotrophic lateral sclerosis: "prepare for the worst and hope for the best". JAMA: the journal of the American Medical Association. 2007; 298(2):207-16.
Faria DC, FÃ¡vero FM, Fontes SV, Quadros AAJ, Oliveira ASB. Perfil clÃnico de pacientes com doenÃ§a do neurÃ´nio motor no ambulatÃ³rio da Unifesp. Rev Neurocienc.2008;16(3):189-93.
Forbes R.B.; Colville S.; Cran G.W.; Swingler R.J. Unexpected decline in survival from amyotrophic lateral sclerosis/motor neurone disease. J. Neurol. Neurosurg. Psychiatry 2004;75: 1753-1755.
Matos SE, Conde MTRP, FÃ¡vero FM, Taniguchi M, Quadros AAJ, Fontes SV, Oliveira ASB. Mortality rates due to amyotrophic lateral sclerosis in SÃ£o Paulo City from 2002 to 2006. Arq Neuropsiquiatr. 2011; 69 (6): 861-866.
Lima J. M. B., Mesquita N., Duro L. A. A., Furtado A. B. Epidemiological aspects of amyotrophic lateral sclerosis in Rio de Janeiro city. Rev Brasileira de Neurologia 1983; 19(3): 75-78.
Â¬Dietrich-Neto F., Callegaro D., Dias-Tasta E., Silva H. A., Ferraz M. E.,Lima J. M. B., Oliveira A. S. B. Amyotrophic Lateral Sclerosis in Brazil: 1998 national survey. Arq neuropsiquiatr. 2000: 58(3-A): 607-615.
Castro-Costa C. et al. Amyotrophic Lateral Sclerosis: Clinical Analysis Of 78 Cases from Fortaleza (Northeastern Brazil). Arq Neuropsiquiatr. 1999;57(3-B): 761-774.
Moraes L, Goldbaum M, Silva HCA, Callegaro D. Incidence rate of amyotrophic lateral sclerosis (ALS) in Sao Paulo city, Brazil, 1991-1997. Arq Neuropsiquiatr. 1998;56(Supl 1):343.
Werneck LC, Bezerra R, Silveira Neto O, Scola RH. A clinical epidemiological study of 251 cases of amyotrophic lateral sclerosis in the south of Brazil. Arq. Neuro-Psiquiatr. 2007;65(2A):189-95.
SEADE. FundaÃ§Ã£o Sistema Estadual de AnÃ¡lise de Dados. PopulaÃ§Ã£o e estatÃsticas de saÃºde. SÃ£o Paulo; 2004. [acesso em 12 de fev 2008]. DisponÃvel em: http://www.seade.gov.br/produtos/idr/dem/dem_pop_04.xls
Turner MR, Brockington A, Scarber J, Hollinger H, Marsden R, Shaw PJ, Talbot K. Pattern of spread and prognosis in lower limb-onset ALS. Amyotrophic Lateral Scler.2010;11(4):369-73.
KÃµrner S, Kollewe K, Fahlbusch M, Zapf A, Dengler R, Krampfl K, Petri S. Onset and spread patternsof upper and lower motor neuron symptons in amyotrophic lateral sclerosis. Muscle Nerve.2011; 43(5):636-42.
ChiÃ² A, Calvo A, Moglia C, Mazzini L, Mora G, Parals study group. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J. Neurol Neurosurg Psychiatry. 2011; 82(7):740-6.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under aÂ Creative Commons Attribution LicenseÂ that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- 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.
- 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 (SeeÂ The Effect of Open AccessÂ and Benefits of Publishing Open Access).