Amyotrophic lateral sclerosis

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Causes

By Mayo Clinic staff

In ALS, the nerve cells that control the movement of your muscles gradually die, so your muscles progressively weaken and begin to waste away. Up to 1 in 10 cases of ALS is inherited. But the remainder appear to occur randomly.

Researchers are studying several possible causes of ALS, including:

  • Gene mutation. Various genetic mutations can lead to inherited forms of ALS, which appear nearly identical to the non-inherited forms.
  • Chemical imbalance. People who have ALS typically have higher than normal levels of glutamate, a chemical messenger in the brain, around the nerve cells in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.
  • Disorganized immune response. Sometimes a person's immune system begins attacking some of his or her body's own normal cells, and scientists have speculated that this may trigger the process that results in ALS.
  • Protein mishandling. There's evidence that mishandled proteins within the nerve cells can lead to a gradual accumulation of abnormal forms of these proteins in the cells, eventually causing the nerve cells to die.
Risk factors Symptoms
References
  1. Amyotrophic lateral sclerosis fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm. Accessed Sept. 28, 2010.
  2. Elman LB, et al. Clinical features of amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Accessed Sept. 28, 2010.
  3. Maragakis NJ, et al. Epidemiology and pathogenesis of amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Accessed Sept. 28, 2010.
  4. McCluskey L. Familial amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Accessed Sept. 28, 2010.
  5. Neurological diagnostic tests and procedures. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/misc/diagnostic_tests.htm. Accessed Sept. 28, 2010.
  6. ALS in the military: Unexpected consequences of military service. ALS Association. http://www.alsa.org/files/pdf/als_military_paper.pdf. Accessed Sept. 28, 2010.
  7. Elman LB, et al. Diagnosis of amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Accessed Sept. 28, 2010.
  8. Galvez-Jimenez N, et al. Symptom-based management of amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Accessed Sept. 28, 2010.
  9. Choudry RB, et al. Pharmacologic treatment of amyotrophic lateral sclerosis. http://www.uptodate.com/home/index.html. Sept. 28, 2010.
  10. Why is exercise important? In: Everyday Life With ALS: A Practical Guide. Muscular Dystrophy Association. http://www.als-mda.org/publications/everydaylifeals/ch8/. Accessed Sept. 28, 2010.
  11. Miller RG, 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;73:1227.
  12. Miller RG, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73:1227.
  13. Covering all the bases. ALS Association. http://www.alsa.org/coveringallthebases/. Accessed Oct. 21, 2010.
  14. Armon C. Smoking may be considered an established risk factor for sporadic ALS. Neurology. 2009;73:1693.
  15. Gallo V, et al. Smoking and risk for amyotrophic lateral sclerosis: Analysis of the EPIC cohort. Annals of Neurology. 2009;65:378.
  16. Mayadev AS, et al. The amyotrophic lateral sclerosis center: A model of multidisciplinary management. Physical Medicine and Rehabilitation Clinics of North America. 2008;19:619.
  17. Crum BA (expert opinion). Mayo Clinic, Rochester, Minn. Oct. 6, 2010.
DS00359 Dec. 2, 2010

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