User:Mr. Ibrahem/Amyotrophic lateral sclerosis

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Amyotrophic lateral sclerosis
Other namesLou Gehrig's disease; Charcot's disease; motor neurone disease (MND)[1]
An MRI with increased signal in the posterior part of the internal capsule that can be tracked to the motor cortex, consistent with the diagnosis of ALS
SpecialtyNeurology
SymptomsStiff muscles, muscle twitching, gradually worsening weakness[2]
ComplicationsDifficulty in speaking, swallowing, breathing[2]
Usual onset50s–60s[3]
CausesUnknown (most), inherited (few)[3][4]
Diagnostic methodBased on symptoms[4]
TreatmentNon-invasive ventilation[5]
MedicationRiluzole, edaravone[6][7]
PrognosisLife expectancy 2–4 years[5]
Frequency2.6/100,000 per year (Europe)[8]

Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease, is a disease that causes the death of neurons that control voluntary muscles.[4][9] Some also use the term motor neuron disease for a group of conditions of which ALS is the most common.[2] ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size.[2] It may begin with weakness in the arms or legs, or with difficulty speaking or swallowing.[10] About half of the people affected develop at least mild difficulties with thinking and behavior and most people experience pain.[11][12] Most eventually lose the ability to walk, use their hands, speak, swallow, and breathe.[5]

The cause is not known in 90% to 95% of cases,[4] but is believed to involve both genetic and environmental factors.[13] The remaining 5–10% of cases are inherited from a person's parents.[3] About half of these genetic cases are due to one of two specific genes.[4] The underlying mechanism involves damage to both upper and lower motor neurons.[2] The diagnosis is based on a person's symptoms, with testing done to rule out other potential causes.[4]

No cure is known.[4] The goal of treatment is to improve symptoms.[11] A medication called riluzole may extend life by about two to three months.[6] Non-invasive ventilation may result in both improved quality and length of life.[5] Mechanical ventilation can prolong survival but does not stop disease progression.[14] A feeding tube may help.[15] The disease can affect people of any age, but usually starts around the age of 60 and in inherited cases around the age of 50.[3] The average survival from onset to death is two to four years, though this can vary, and about 10% survive longer than 10 years.[5][16][4] Most die from respiratory failure.[3] In Europe, the disease affects about two to three people per 100,000 per year.[8] Rates in much of the world are unclear.[17] In the United States, it is more common in white people than black people.[18]

Descriptions of the disease date back to at least 1824 by Charles Bell.[19] In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis.[19] It became well known in the United States in the 20th century when in 1939 it affected baseball player Lou Gehrig and later worldwide following the 1963 diagnosis of cosmologist Stephen Hawking.[20][21] The first ALS gene was discovered in 1993 while the first animal model was developed in 1994.[22][23] In 2014, videos of the Ice Bucket Challenge went viral on the Internet and increased public awareness of the condition.[24]

References[edit]

  1. ^ Wijesekera LC, Leigh PN (February 2009). "Amyotrophic lateral sclerosis". Orphanet Journal of Rare Diseases. 3 (4): 3. doi:10.1186/1750-1172-4-3. PMC 2656493. PMID 19192301.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  2. ^ a b c d e "Motor Neuron Diseases Fact Sheet". National Institute of Neurological Disorders and Stroke. Archived from the original on 13 April 2014. Retrieved 7 November 2010.
  3. ^ a b c d e Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC (March 2011). "Amyotrophic lateral sclerosis". Lancet. 377 (9769): 942–55. doi:10.1016/s0140-6736(10)61156-7. PMID 21296405.
  4. ^ a b c d e f g h "Amyotrophic Lateral Sclerosis (ALS) Fact Sheet". National Institute of Neurological Disorders and Stroke. 19 September 2014. Archived from the original on 5 January 2017. Retrieved 2 January 2015.
  5. ^ a b c d e Hobson EV, McDermott CJ (September 2016). "Supportive and symptomatic management of amyotrophic lateral sclerosis" (PDF). Nature Reviews. Neurology. 12 (9): 526–38. doi:10.1038/nrneurol.2016.111. PMID 27514291. Archived (PDF) from the original on 1 December 2020. Retrieved 1 August 2020.
  6. ^ a b Miller RG, Mitchell JD, Moore DH (March 2012). "Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)". The Cochrane Database of Systematic Reviews. 3 (3): CD001447. doi:10.1002/14651858.CD001447.pub3. PMID 22419278.
  7. ^ "FDA approves drug to treat ALS". U.S. Food and Drug Administration. 5 May 2017. Archived from the original on 8 May 2017.
  8. ^ a b Hardiman O, Al-Chalabi A, Brayne C, Beghi E, van den Berg LH, Chio A, Martin S, Logroscino G, Rooney J (July 2017). "The changing picture of amyotrophic lateral sclerosis: lessons from European registers". Journal of Neurology, Neurosurgery, and Psychiatry. 88 (7): 557–63. doi:10.1136/jnnp-2016-314495. PMID 28285264. Archived from the original on 21 December 2019. Retrieved 1 August 2020.
  9. ^ "Motor neurone disease". NHS Choices. Archived from the original on 29 December 2014. Retrieved 2 January 2015.
  10. ^ Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, Shaw PJ, Simmons Z, van den Berg LH (October 2017). "Amyotrophic lateral sclerosis" (PDF). Nature Reviews. Disease Primers. 3 (17071): 17071. doi:10.1038/nrdp.2017.71. PMID 28980624. Archived (PDF) from the original on 1 December 2020. Retrieved 1 August 2020.
  11. ^ a b van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, van den Berg LH (November 2017). "Amyotrophic lateral sclerosis". Lancet. 390 (10107): 2084–2098. doi:10.1016/S0140-6736(17)31287-4. PMID 28552366.
  12. ^ Chiò A, Mora G, Lauria G (February 2017). "Pain in amyotrophic lateral sclerosis". The Lancet. Neurology. 16 (2): 144–57. arXiv:1607.02870. doi:10.1016/S1474-4422(16)30358-1. PMID 27964824.
  13. ^ Wingo TS, Cutler DJ, Yarab N, Kelly CM, Glass JD (2011). "The heritability of amyotrophic lateral sclerosis in a clinically ascertained United States research registry". PLOS ONE. 6 (11): e27985. Bibcode:2011PLoSO...627985W. doi:10.1371/journal.pone.0027985. PMC 3222666. PMID 22132186.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  14. ^ Soriani M, Desnuelle C (May 2017). "Care management in amyotrophic lateral sclerosis". Revue Neurologique. 173 (5): 288–89. doi:10.1016/j.neurol.2017.03.031. PMID 28461024.
  15. ^ Connolly S, Galvin M, Hardiman O (April 2015). "End-of-life management in patients with amyotrophic lateral sclerosis". The Lancet. Neurology. 14 (4): 435–42. doi:10.1016/S1474-4422(14)70221-2. PMID 25728958.
  16. ^ Swinnen B, Robberecht W (November 2014). "The phenotypic variability of amyotrophic lateral sclerosis". Nature Reviews. Neurology. 10 (11): 661–70. doi:10.1038/nrneurol.2014.184. PMID 25311585. Archived from the original on 31 December 2018. Retrieved 1 August 2020.
  17. ^ Al-Chalabi A, Hardiman O (November 2013). "The epidemiology of ALS: a conspiracy of genes, environment and time". Nature Reviews. Neurology. 9 (11): 617–28. doi:10.1038/nrneurol.2013.203. PMID 24126629.
  18. ^ Mehta P, Kaye W, Raymond J, Punjabi R, Larson T, Cohen J, Muravov O, Horton K (November 2018). "Prevalence of Amyotrophic Lateral Sclerosis – United States, 2015". Morbidity and Mortality Weekly Report. 67 (46): 1285–1289. doi:10.15585/mmwr.mm6746a1. PMC 5858037. PMID 30462626.
  19. ^ a b Rowland LP (March 2001). "How amyotrophic lateral sclerosis got its name: the clinical-pathologic genius of Jean-Martin Charcot". Archives of Neurology. 58 (3): 512–15. doi:10.1001/archneur.58.3.512. PMID 11255459.
  20. ^ Kelly, Evelyn B. (2013). Encyclopedia of human genetics and disease. Santa Barbara, CA: Greenwood. pp. 79–80. ISBN 978-0-313-38713-5. Archived from the original on 8 September 2017.
  21. ^ Youngson, David B. Jacoby, Robert M. (2004). Encyclopedia of family health (3rd ed.). Tarrytown, NY: Marshall Cavendish. p. 1256. ISBN 978-0-7614-7486-9. Archived from the original on 8 September 2017.{{cite book}}: CS1 maint: multiple names: authors list (link)
  22. ^ Renton AE, Chiò A, Traynor BJ (January 2014). "State of play in amyotrophic lateral sclerosis genetics". Nature Neuroscience. 17 (1): 17–23. doi:10.1038/nn.3584. hdl:2318/156177. PMC 4544832. PMID 24369373.
  23. ^ Lutz C (August 2018). "Mouse models of ALS: Past, present and future". Brain Research. 1693 (Part A): 1–10. doi:10.1016/j.brainres.2018.03.024. PMID 29577886.
  24. ^ Song P (August 2014). "The Ice Bucket Challenge: The public sector should get ready to promptly promote the sustained development of a system of medical care for and research into rare diseases". Intractable & Rare Diseases Research. 3 (3): 94–96. doi:10.5582/irdr.2014.01015. PMC 4214244. PMID 25364651.
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