Genetic risk factors; age; male sex; heavy metals; organic chemicals; smoking; electric shock; physical exercise; head injury[2]
Diagnostic method
Clinical diagnosis of exclusion based on progressive symptoms of upper and lower motor neuron degeneration in which no other explanation can be found. Supportive evidence from electromyography, genetic testing, and neuroimaging
Life expectancy highly variable but typically 2–4 years after diagnosis[6]
Frequency
Incidence: 1.6/100,000 individuals per year[6]
Prevalence: 4.4/100,000 living individuals[6]
Lifetime risk: 1 in 400 individuals[7]
Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease in the United States, is a rare but terminal neurodegenerative disorder that results in the progressive loss of both upper and lower motor neurons that normally control voluntary muscle contraction.[3] ALS is the most common form of the motor neuron diseases.[8] ALS often presents in its early stages with gradual muscle stiffness, twitches, weakness, and wasting.[3] Motor neuron loss typically continues until the abilities to eat, speak, move, and, lastly, breathe are all lost.[3] While only 15% of people with ALS also fully develop frontotemporal dementia, an estimated 50% face at least some minor difficulties with thinking and behavior.[9] Depending on which of the aforementioned symptoms develops first, ALS is classified as limb-onset (begins with weakness in the arms or legs) or bulbar-onset (begins with difficulty in speaking or swallowing).[10]
Most cases of ALS (about 90% to 95%) have no known cause, and are known as sporadic ALS.[3][11] However, both genetic and environmental factors are believed to be involved.[12] The remaining 5% to 10% of cases have a genetic cause, often linked to a history of the disease in the family, and these are known as familial ALS (hereditary).[6][13] About half of these genetic cases are due to disease-causing variants in one of four specific genes.[14] The diagnosis is based on a person's signs and symptoms, with testing conducted to rule out other potential causes.[3]
There is no known cure for ALS.[3] The goal of treatment is to slow the disease progression, and improve symptoms.[9] Treatments that slow ALS include riluzole (extends life by two to three months) and sodium phenylbutyrate/ursodoxicoltaurine (extends life by around seven months).[15][16] 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.[17] A feeding tube may help maintain weight and nutrition.[18] Death is usually caused by respiratory failure.[19] The disease can affect people of any age, but usually starts around the age of 60.[19] The average survival from onset to death is two to four years, though this can vary, and about 10% of those affected survive longer than ten years.[20]
Descriptions of the disease date back to at least 1824 by Charles Bell.[21] In 1869, the connection between the symptoms and the underlying neurological problems was first described by French neurologist Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis.[21]
^Cite error: The named reference Orphanet2009 was invoked but never defined (see the help page).
^ abcdCite error: The named reference Masrori-2020 was invoked but never defined (see the help page).
^ abcdefgh"Amyotrophic Lateral Sclerosis (ALS) Fact Sheet". National Institute of Neurological Disorders and Stroke. Archived from the original on 5 January 2017. Retrieved 22 October 2020.
^ abHobson EV, McDermott CJ (September 2016). "Supportive and symptomatic management of amyotrophic lateral sclerosis" (PDF). Nature Reviews. Neurology. 12 (9): 526–538. doi:10.1038/nrneurol.2016.111. PMID 27514291. S2CID 8547381. Archived (PDF) from the original on 1 December 2020. Retrieved 20 December 2019.
^ abcdGoutman SA, Hardiman O, Al-Chalabi A, Chió A, Savelieff MG, Kiernan MC, Feldman EL (May 2022). "Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis". The Lancet. Neurology. 21 (5): 480–493. doi:10.1016/S1474-4422(21)00465-8. PMC 9513753. PMID 35334233.
^Ryan M, Heverin M, McLaughlin RL, Hardiman O (November 2019). "Lifetime Risk and Heritability of Amyotrophic Lateral Sclerosis". JAMA Neurology. 76 (11): 1367–1374. doi:10.1001/jamaneurol.2019.2044. PMC 6646974. PMID 31329211.
^"Motor Neuron Diseases Fact Sheet". www.ninds.nih.gov. National Institute of Neurological Disorders and Stroke. Archived from the original on 10 October 2020. Retrieved 27 October 2020.
^ abvan 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. S2CID 24483077.
^Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, et al. (October 2017). "Amyotrophic lateral sclerosis" (PDF). Nature Reviews. Disease Primers. 3 (17071): 17071. doi:10.1038/nrdp.2017.71. PMID 28980624. S2CID 1002680. Archived (PDF) from the original on 1 December 2020. Retrieved 20 December 2019.
^"Understanding ALS". The ALS Association. Archived from the original on 26 October 2020. Retrieved 28 October 2020.
^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.
^"Amyotrophic lateral sclerosis". MedlinePlus Genetics. Retrieved 7 August 2023.
^Cite error: The named reference Goutman-2022 was invoked but never defined (see the help page).
^Cite error: The named reference alsa1 was invoked but never defined (see the help page).
^Heo YA (September 2022). "Sodium Phenylbutyrate and Ursodoxicoltaurine: First Approval". CNS Drugs. 36 (9): 1007–1013. doi:10.1007/s40263-022-00945-x. PMID 35907175. S2CID 251162676.
^Soriani MH, Desnuelle C (May 2017). "Care management in amyotrophic lateral sclerosis". Revue Neurologique. 173 (5): 288–299. doi:10.1016/j.neurol.2017.03.031. PMID 28461024.
^Connolly S, Galvin M, Hardiman O (April 2015). "End-of-life management in patients with amyotrophic lateral sclerosis". The Lancet. Neurology. 14 (4): 435–442. doi:10.1016/S1474-4422(14)70221-2. PMID 25728958. S2CID 34109901.
^ abKiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, et al. (March 2011). "Amyotrophic lateral sclerosis". Lancet. 377 (9769): 942–955. doi:10.1016/s0140-6736(10)61156-7. PMID 21296405.
^Pupillo E, Messina P, Logroscino G, Beghi E (February 2014). "Long-term survival in amyotrophic lateral sclerosis: a population-based study". Annals of Neurology. 75 (2): 287–297. doi:10.1002/ana.24096. PMID 24382602. S2CID 205345019.
^ abRowland LP (March 2001). "How amyotrophic lateral sclerosis got its name: the clinical-pathologic genius of Jean-Martin Charcot". Archives of Neurology. 58 (3): 512–515. doi:10.1001/archneur.58.3.512. PMID 11255459.
Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease in the United States, is a rare but terminal neurodegenerative...
The Ice Bucket Challenge, sometimes called the ALS Ice Bucket Challenge, is an activity involving the pouring of a bucket of ice water over a person's...
Hilton Als (born 1960) is an American writer and theater critic. He is a teaching professor at the University of California, Berkeley, an associate professor...
sclerosis (ALS). He is credited with helping the ALS Ice Bucket Challenge gain national attention, which raised approximately $220 million for ALS research...
Project ALS is a non-profit organization whose mission is to identify and support leading scientific research toward the first effective treatments and...
Alsing is a given name and surname. Notable people with the name include: Alsing Andersen (1893–1962), Danish politician Peter Alsing Nielsen (1907–1985)...
The ALS Association is an American nonprofit organization that funds global amyotrophic lateral sclerosis (ALS) research, provides care services and programs...
voluntary muscles of the body. This includes amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular...
amyotrophic lateral sclerosis (ALS) research and patient advocate. He became known for his activism after being diagnosed with ALS in 2017. Since then, Wallach...
The Battle of Als (Danish: Slaget om Als; German: Übergang nach Alsen) was fought on 29 June 1864 during the Second Schleswig War between Denmark and Prussia...
The ALS Society of Canada is a registered, not-for-profit Canadian organization. ALS Canada, founded in 1977, is a national voluntary health organization...
ALS – Aircraft Leasing Services is a regional airline based in Nairobi, Kenya. ALS started operations in 1985 with a single two-seat Cessna 150 which was...
Rolf Adam Engelbrekt Alsing (12 October 1968 – 15 April 2020) was a Swedish television and radio presenter, best known for presenting Big Brother Sweden...
"Politics as a Vocation" (German: Politik als Beruf) is an essay by German economist and sociologist Max Weber (1864–1920). It originated in the second...
Alsing Emanuel Andersen (5 February 1893 – 5 December 1962) was a Danish social democrat politician. Andersen served as the Minister of Defense (1935–1940)...
(ALS). With a staff including more than 30 scientists, it operates a research and development program centered on ALS. ALS TDI was founded as the ALS Therapy...
Peder Als (16 May 1725 – 8 July 1775) was a Danish historical and portrait painter. Als was born at Copenhagen in 1725, and studied under Carl Gustaf...
Roald Als (born April 2, 1948) is a Danish cartoonist best known for his editorial cartoons in Danish newspapers Weekendavisen and Politiken. Als was born...
MPEG-4 Audio Lossless Coding, also known as MPEG-4 ALS, is an extension to the MPEG-4 Part 3 audio standard to allow lossless audio compression. The extension...
ALS Limited (ASX: ALQ) is a company headquartered in Brisbane, Australia, which provides testing, inspection, certification, and verification services...
ALS Awareness Month is a campaign to spread awareness of and raise funds for research for a cure for ALS (amyotrophic lateral sclerosis, also known as...
As We Were Dreaming (German: Als wir träumten) is a 2015 German film directed by Andreas Dresen, based on the novel of the same name by author Clemens...
Sebbe Als is a replica of a Viking ship, Skuldelev wreck no. 5. She is the oldest sailing 'fiver' in Denmark. A sailing trip with the Imme Gram [da] and...
Advanced Life Support (ALS) is a set of life saving protocols and skills that extend basic life support to further support the circulation and provide...
her death. On 19 December 2020, Roxette's longtime drummer Per "Pelle" Alsing died at the age of 60. In 2021, Roxette released a 30th-anniversary edition...
A Woman Like Eve (Dutch: Een vrouw als Eva) is a 1979 Dutch drama film directed by Nouchka van Brakel, about a woman who leaves her husband for another...
Als in een Roes is a 1986 Dutch film directed by Pim de la Parra. The story revolves around a theater group that is looking for a drama to play. The director...