Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with undetermined etiology that primarily involves the motor neurons in the cerebral cortex, brainstem and spinal cord.
There is no cure and the mean duration of survival is 2-5 years without tracheostomy and ventilator support.
Clinical hallmarks of ALS are: presence of upper & lower motor neuron features involving the brainstem and spinal cord and progressive limb weakness, respiratory insufficiency, spasticity, hyperreflexia, and bulbar symptoms such as dysarthria and dysphagia.
  1. Jackson M, Lladó J, Rothstein JD. Therapeutic developments in the treatment of amyotrophic lateral sclerosis. Expert Opin Investig Drugs. 2002 Oct;11(10):1343-1364. PMID: 12387699
  2. Andersen PM, Borasio GD, Dengler R, et al. EFNS Task Force on Management of Amyotrophic Lateral Sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol. 2005 Dec;12(12):921-938. PMID: 16324086
  3. 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;73(15):1227-1233. PMID: 19822873
  4. Kiernan MC, Vucic S, Cheah BC, et al. Amyotrophic lateral sclerosis. Lancet. 2011 Mar;377(9769):942-955. PMID: 21296405
  5. Kühnlein P, Gdynia HJ, Sperfeld AD, et al. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol. 2008 Jul;4(7):366-374. PMID: 18560390
  6. Miller RG, Mitchell JD, Lyon M, et al. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2002(2):CD001447. PMID: 12076411
  7. Rowland LP, Shneider NA. Amyotrophic lateral sclerosis. N Engl J Med. 2001 May;344(22):1688-1700. PMID: 11386269
  8. Quality Standard Subcommittee of the American Academy of Neurology. Practice advisory on the treatment of amyotrophic lateral sclerosis with riluzole: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 1997 Sep;49(3):657-659. PMID: 9305318
  9. Swash M. An algorithm for ALS diagnosis and management. Neurology. 1999;53(8)(Suppl 5):S58-S62. PMID: 10560641
  10. Walling AD. Amyotrophic lateral sclerosis: Lou Gehrig’s disease. Am Fam Physician. 1999 Mar;59(6):1489-1496. PMID: 10193591
  11. Yunusova Y, Green JR, Lindstrom MJ, et al. Kinematics of disease progression in bulbar ALS. J Commun Disord. 2010 Jan;43(1):006-20. PMID: 19683250
  12. Amyotrophic lateral sclerosis and other motor neuron disorders. Harrison's Online: McGraw-Hill Companies; 2003.
  13. MedWorm
  14. Elman LB, McCluskey L. Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease. UpToDate. Feb 2011. Accessed 01 Oct 2011
  15. Galvez-Jimenez N. Symptom-based management of amyotrophic lateral sclerosis. UpToDate. Feb 2011. Accessed 01 Oct 2011.
  16. Choudry RB, Galvez-Jimenez N, Cudkowicz ME. Pharmacologic treatment of amyotrophic lateral sclerosis. UpToDate. Jun 2011. Accessed 01 Oct 2011
  17. Brooks BR, Miller RG, Swash M; World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. A consensus conference at Airlie House, Warrenton. Apr 1998
  18. Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat Rev Neurol. 2011;7:639-649. doi: 10.1038/nrneurol.2011.153 . Accessed 16 Feb 2015. PMID: 21989247
  19. Rusina R, Ridzon P, Keller O, et al. Relationship between ALS and the degree of cognitive impairment, markers of neurodegenration and predictors of poor outcome: a prospective study. Eur J Neurol. 2010 Jan;17(1):23-30. doi: 10.1111/j.1468-1331.2009.02717.x. PMID: 19572947
  20. Salvioni CC, Stanich P, Almeida CS, et al. Nutritional care in motor neurone disease/amyotrophic lateral sclerosis. Arq Neuropsiquiatr. 2014 Feb;72(2):157-163. doi: 10.1590/0004-282X20130185. PMID: 24604371
  21. Choudry RB, Galvez-Jimenez N, Cudkowicz ME. Disease modifying treatment of amyotrophic lateral sclerosis. UpToDate. Jun 2017.
Editor's Recommendations
Most Read Articles
17 Apr 2019
The ketogenic and Modified Atkin’s diets are effective treatments for children with refractory epilepsy of genetic aetiology, a new study suggests.
Audrey Abella, 15 Aug 2018
Supervised aerobic-strength training improved cognitive and motor functions among elderly individuals with mild cognitive impairment (MCI), according to studies presented at AAIC 2018.
Stephen Padilla, 02 Apr 2019
Exposure to ambient pesticides within 2,000 m of the mother’s residence during pregnancy increases the risk of autism spectrum disorder (ASD) in offspring, suggests a study. Moreover, risks for ASD with comorbid intellectual disability are further increased when infant is exposed to pesticides.
2 days ago
Lidocaine prolongs seizure remission without resulting in harmful side effects in children with cluster seizures, a recent study has found.