amyotrophic lateral sclerosis (ALS); Lou Gerig's disease

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Etiology

Epidemiology

  • 5.0 per 100,000 Americans, 1.7 per 100,000[24]
  • 20 per 100,000 among Americans in their 70s[21]

Pathology

Genetics

Clinical manifestations

Laboratory

Diagnostic procedures

Radiology

Complications

Differential diagnosis

Management

* mildly obese patients with ALS have longer survival[10]

More general terms

More specific terms

Additional terms

References

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    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. 3.0 3.1 Chemical & Engineering News, Aug 26, 2002
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    Renton AE et al A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011 Oct 20;72(2):257-68. Epub 2011 Sep 21. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21944779
  6. 6.0 6.1 PubMed Health Dextromethorphan and Quinidine http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0016263/
  7. 7.0 7.1 7.2 Hardiman O. Management of respiratory symptoms in ALS. J Neurol. 2011 Mar;258(3):359-65 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21082322
  8. Bedlack RS. Amyotrophic lateral sclerosis: current practice and future treatments. Curr Opin Neurol. 2010 Oct;23(5):524-9 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20613515
  9. Miller RG, Jackson CE, Kasarskis EJ et al Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73(15):1227-33 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19822873
  10. 10.0 10.1 10.2 Wills AM et al Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo- controlled phase 2 trial. The Lancet, Early Online Publication, 28 February 2014 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/24582471 <Internet> http://www.thelancet.com/journals/lancet/article/PIIS0140-6736%2814%2960222-1/abstract
  11. 11.0 11.1 Toro J and Reyes S Tongue Fasciculations in Amyotrophic Lateral Sclerosis. N Engl J Med 2014; 371:e7. July 31, 2014 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25075854 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1309849
  12. Radunovic A, Mitsumoto H, Leigh PN. Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurol. 2007 Oct;6(10):913-25. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17884681
  13. Kiernan MC, Vucic S, Cheah BC et al Amyotrophic lateral sclerosis. Lancet. 2011 Mar 12;377(9769):942-55 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21296405
  14. 14.0 14.1 14.2 Su FC, Goutman SA, Chernyak S et al Association of Environmental Toxins With Amyotrophic Lateral Sclerosis. JAMA Neurol. Published online May 09, 2016 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27159543 <Internet> http://archneur.jamanetwork.com/article.aspx?articleid=2519875
    Cragg JJ, Cudkowicz ME, Weisskopf MG The Role of Environmental Toxins in Amyotrophic Lateral Sclerosis Risk. JAMA Neurol. Published online May 09, 2016 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27158795 <Internet> http://archneur.jamanetwork.com/article.aspx?articleid=2519869
  15. Geriatric Review Syllabus, 9th edition (GRS9) Medinal-Walpole A, Pacala JT, Porter JF (eds) American Geriatrics Society, 2016
  16. de Carvalho M, Dengler R, Eisen A et al Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol. 2008 Mar;119(3):497-503. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18164242
  17. 17.0 17.1 Gonzalez-Bermejo J et al. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): A randomised controlled triple-blind trial. Lancet Neurol 2016 Nov; 15:1217 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27751553 <Internet> http://www.thelancet.com/journals/laneur/article/PIIS1474-4422(16)30233-2/abstract
  18. 18.0 18.1 Fiore K FDA Okays Antioxidant Drug for ALS - Drug may work best in patients with preserved function. MedPage Today. May 5, 2017 https://www.medpagetoday.com/Neurology/GeneralNeurology/65087
  19. Brown RH, Al-Chalabi A. Amyotrophic Lateral Sclerosis. N Engl J Med 2017; 377:162-172. July 13, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28700839 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMra1603471
  20. 20.0 20.1 Kneisel K Relatives of ALS Patients More Apt to Have Mental Health Issues. Irish study suggests ties between pleiotropic genes, ALS, and neuropsychiatric disease. MedPage Today. October 18, 2017 https://www.medpagetoday.com/Psychiatry/Dementia/68620
    Chuquilin M, Wymer S, Wymer J Increasing Evidence for an Association Between Amyotrophic Lateral Sclerosis and Psychiatric Disorders. JAMA Neurol. Published online October 16, 2017 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29049449 https://jamanetwork.com/journals/jamaneurology/article-abstract/2657320
  21. 21.0 21.1 MedPage Today Staff ALS Prevalence Put at 5.0 per 100,000 (MMWR). Rate hit 20 per 100,000 among Americans in their 70s. MedPage Today. February 22, 2018 https://www.medpagetoday.com/neurology/generalneurology/71322
    Mehta P, Kaye W, Raymond J, et al. Prevalence of Amyotrophic Lateral Sclerosis - United States, 2014. MMWR Morb Mortal Wkly Rep 2018;67:216-218 https://www.cdc.gov/mmwr/volumes/67/wr/mm6707a3.htm
  22. 22.0 22.1 George J, Greater ALS Risk in Men Exposed to Diesel Exhaust. Findings may have implications for people living in high- traffic areas. MedPage Today. February 27, 2018 https://www.medpagetoday.com/meetingcoverage/aan/71413
    Dickerson A et al Amyotrophic lateral sclerosis and exposure to diesel exhaust in Denmark. American Academy of Neurology (AAN) 2018.
  23. 23.0 23.1 Visser AE, Rooney JPK, D'Ovidio F et al Multicentre, cross-cultural, population-based, case-control study of physical activity as risk factor for amyotrophic lateral sclerosis. Neurology, Neurosurgery, & Psychiatry. April 2018 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/29685899 <Internet> http://jnnp.bmj.com/content/early/2018/04/24/jnnp-2017-317724
  24. 24.0 24.1 24.2 Herman AO, Sadoughi S, Saitz R Professional Soccer Players May Face Increased Risk for ALS Physician's First Watch, Feb 28, 2018 David G. Fairchild, MD, MPH, Editor-in-Chief Massachusetts Medical Society http://www.jwatch.org
    Do Soccer Players Have an Increased Risk of ALS? American Academy of Neurology. 71st Annual Meeting, May 4-10, 2019, Philadelphia https://www.aan.com/PressRoom/Home/PressRelease/2697
  25. Radunovic A, Annane D, Rafiq MK, Brassington R, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2017 Oct 6;10:CD004427. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28982219
  26. 26.0 26.1 Paganoni S Trial of Sodium Phenylbutyrate - Taurursodiol for Amyotrophic Lateral Sclerosis. N Engl J Med 2020; 383:919-930. Sept 3 PMID: https://www.ncbi.nlm.nih.gov/pubmed/32877582 https://www.nejm.org/doi/full/10.1056/NEJMoa1916945
    George J Early Data for ALS Drug Show Survival Benefit - Mortality cut 44% over follow-up period MedPage Today October 16, 2020 https://www.medpagetoday.com/neurology/generalneurology/89172
  27. 27.0 27.1 27.2 George J Novel ALS Risk Gene Identified. Findings suggest apoptosis in motor neurons play an important role. MedPage Today June 17, 2021 https://www.medpagetoday.com/neurology/generalneurology/93170
    Russell KL et al Pathogenic effect of TP73 Gene Variants in People With Amyotrophic Lateral Sclerosis. Neurology 2021. June 16. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34135078 https://n.neurology.org/content/early/2021/06/15/WNL.0000000000012285
  28. 28.0 28.1 28.2 Anderson P Lipid Levels Tied to ALS Risk Medscape. September 14, 2021 https://www.medscape.com/viewarticle/958730
    Thompson AG, Talbot K, Turner MR Higher blood high density lipoprotein and apolipoprotein A1 levels are associated with reduced risk of developing amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2021. Sept 13. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34518331 Free article https://jnnp.bmj.com/content/early/2021/08/16/jnnp-2021-327133
  29. 29.0 29.1 Tran H, Moazami MP, Yang H et al Suppression of mutant C9orf72 expression by a potent mixed backbone antisense oligonucleotide. Nature Medicine 2021. Dec 23 PMID: https://www.ncbi.nlm.nih.gov/pubmed/34949835 https://www.nature.com/articles/s41591-021-01557-6
  30. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet
    NINDS Amyotrophic Lateral Sclerosis Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Amyotrophic-Lateral-sclerosis-ALS-Information-Page

Patient information

amyotrophic lateral sclerosis patient information

Database