A ELA tem uma evolução progressiva sem intervalos de remissões, recidivas ou estabilização, causando deficiência progressiva e, por fim, morte. A doença varia muito entre os indivíduos afetados em termos de quadro clínico e de curso temporal. A sobrevida mediana é de 3 a 5 anos, mas foi relatada sobrevida de até 10 anos e até mais (em aproximadamente 10% a 20% dos pacientes).[21]van Es MA, Hardiman O, Chio A, et al. Amyotrophic lateral sclerosis. Lancet. 2017 Nov 4;390(10107):2084-98.
http://www.ncbi.nlm.nih.gov/pubmed/28552366?tool=bestpractice.com
[122]Turner MR, Parton MJ, Shaw CE, et al. Prolonged survival in motor neuron disease: a descriptive study of the King's database 1990-2002. J Neurol Neurosurg Psychiatry. 2003 Jul;74(7):995-7.
https://jnnp.bmj.com/content/74/7/995
http://www.ncbi.nlm.nih.gov/pubmed/12810805?tool=bestpractice.com
[123]Chiò A, Logroscino G, Hardiman O, et al; Eurals Consortium. Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):310-23.
http://www.ncbi.nlm.nih.gov/pubmed/19922118?tool=bestpractice.com
Fatores de prognóstico
Os fatores prognósticos associados a uma sobrevida mais prolongada incluem:
Tratamento com ventilação não invasiva (VNI)[123]Chiò A, Logroscino G, Hardiman O, et al; Eurals Consortium. Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):310-23.
http://www.ncbi.nlm.nih.gov/pubmed/19922118?tool=bestpractice.com
Nutrição enteral[84]Katzberg, HD, Benatar M. Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2011 Jan 19;(1):CD004030.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD004030.pub3/full
http://www.ncbi.nlm.nih.gov/pubmed/21249659?tool=bestpractice.com
Idade mais jovem no diagnóstico[124]Eisen A, Schulzer M, MacNeil M, et al. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve. 1993 Jan;16(1):27-32.
http://www.ncbi.nlm.nih.gov/pubmed/8423829?tool=bestpractice.com
[125]Spencer KR, Foster ZW, Rauf NA, et al. Neuropathological profile of long-duration amyotrophic lateral sclerosis in military veterans. Brain Pathol. 2020 Nov;30(6):1028-40.
https://onlinelibrary.wiley.com/doi/epdf/10.1111/bpa.12876
http://www.ncbi.nlm.nih.gov/pubmed/32633852?tool=bestpractice.com
Início nos membros[124]Eisen A, Schulzer M, MacNeil M, et al. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve. 1993 Jan;16(1):27-32.
http://www.ncbi.nlm.nih.gov/pubmed/8423829?tool=bestpractice.com
[126]Czaplinski A, Yen AA, Appel SH. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):390-2.
http://www.ncbi.nlm.nih.gov/pubmed/16484652?tool=bestpractice.com
Capacidade vital forçada (CVF) basal de >75% (sobrevida mediana de 5 anos)[126]Czaplinski A, Yen AA, Appel SH. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):390-2.
http://www.ncbi.nlm.nih.gov/pubmed/16484652?tool=bestpractice.com
Maior tempo desde o início dos sintomas até o diagnóstico.[125]Spencer KR, Foster ZW, Rauf NA, et al. Neuropathological profile of long-duration amyotrophic lateral sclerosis in military veterans. Brain Pathol. 2020 Nov;30(6):1028-40.
https://onlinelibrary.wiley.com/doi/epdf/10.1111/bpa.12876
http://www.ncbi.nlm.nih.gov/pubmed/32633852?tool=bestpractice.com
Os fatores prognósticos associados ao pior desfecho incluem:
Idade avançada no diagnóstico
Início bulbar
Comorbidade com demência frontotemporal
CVF basal de <75%
Perda de peso substancial.[127]Goutman SA, Boss J, Iyer G, et al. Body mass index associates with amyotrophic lateral sclerosis survival and metabolomic profiles. Muscle Nerve. 2022 Nov 2 [Epub ahead of print].
https://onlinelibrary.wiley.com/doi/10.1002/mus.27744
http://www.ncbi.nlm.nih.gov/pubmed/36321729?tool=bestpractice.com
A taxa de progressão dos sintomas é considerada um fator prognóstico independente.[123]Chiò A, Logroscino G, Hardiman O, et al; Eurals Consortium. Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):310-23.
http://www.ncbi.nlm.nih.gov/pubmed/19922118?tool=bestpractice.com