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AMYOTROPHIC LATERAL SCLEROSIS
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.
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  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
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  12. Amyotrophic lateral sclerosis and other motor neuron disorders. Harrison's Online: McGraw-Hill Companies; 2003. http://harrisonsonline.com
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  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
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