Amiotrofik lateral skleroz sahifasining manbasini koʻrish

{{Tibbiy holat bilgiqutisi
| nomi                   = Amiotrofik lateral skleroz
| tasvir                 =  ALS Affected neurons and muscles.png
| izoh                   =  ALS ta'siri ostida bo'lgan [[nerv sistemasi]] tanadagi skelet mushaklarida progressiv simptomlarga sabab boʻladi
| soha                   = [[Nevrologiya]]
| belgilari               = '''Erta''': Mushaklar rigidligi, mushak qaltirashi, kuchning kamayishi<ref name="NINDS2020" /><br />'''Kechki''': [[Dizartriya|Nutqning buzilishi]], [[Disfagiya|yutishning qiyinlashuvi]], [[nafas qisishi]], nafas yetishmovchiligi
| asoratlari            = Yiqilish (baxtsiz hodisa), nafas yetishmovchiligi, [[pnevmoniya]], [[och qolish]] 
| boshlanish vaqti       =45–75yosh<ref name="Masrori-2020">{{cite journal | vauthors = Masrori P, Van Damme P | title = Amyotrophic lateral sclerosis: a clinical review | journal = European Journal of Neurology | volume = 27 | issue = 10 | pages = 1918–1929 | date = October 2020 | pmid = 32526057 | pmc = 7540334 | doi = 10.1111/ene.14393 }}</ref>
| davomiyligi =
| turlari =
| sabablari = Nomaʼlum (taxminan 85%), genetik (taxminan 15%)
| xavf omillari = Genetik xavf omillar; yosh; [[erkak|erkak jinsi]]; ogʻir metallar; organik kimyoviy moddalar; chekish; elektr toki urishi; jismoniy mashqlar; bosh miya jarohati
| tashxis = Boshqa hech qanday tushuntirish topilmagan holda yuqori va pastki motor neyron degeneratsiyasining progressiv simptomlariga asoslangan tashxis. [[Elektromiografiya]], [[genetik test]] va [[neyrovizualizatsiya]]
| differensial tashxis = Multifokal motor neyropatiya, Kennedi kasalligi, [[Umurtqa spastik paraplegiyasi]], Nerv qisilishi sindromi, [[Diabetik neyropatiya]], Post-polio sindromi, [[Miasteniya]], Multi skleroz<ref>{{cite journal | vauthors = Kwan J, Vullaganti M | title = Amyotrophic lateral sclerosis mimics | journal = Muscle & Nerve | volume = 66 | issue = 3 | pages = 240–252 | date = September 2022 | pmid = 35607838 | doi = 10.1002/mus.27567 | s2cid = 249014375 }}</ref>
| oldini olish =
| davolash = Nogironlar aravachasi; noinvaziv ventilyatsiya; oziqlantirish naychasi; kengaytirilgan va doimiy muloqot; simptomatik davolash
| dorilar = Riluzol, edaravon, fenilbutirat eritmasi, tofersen, dekstrometorfan/xinidin
| prognoz = Oʻrtacha umr koʻrish hammada har xil, lekin odatda tashxisdan keyin 2-4 yil
| takrorlanishi =
| oʻlim =
}}

'''Amiotrofik lateral skleroz''' ('''ALS'''), '''motor neyron kasalligi''' ('''MND''') yoki AQShda '''Lou Gehrig kasalligi''' sifatida tanilgan, nisbatan kam uchraydigan, terminal [[neyrodegenerativ kasallik]] boʻlib, ixtiyoriy mushak qisqarishini nazorat qiluvchi yuqori va pastki [[motor neyron]]larning progressiv yoʻqolishiga olib keladi<ref name="NINDS2020" />. ALS motor neyron kasalliklarining eng keng tarqalgan shaklidir<ref>{{Cite web |title=Motor Neuron Diseases Fact Sheet |url=https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/motor-neuron-diseases-fact-sheet |url-status=live |archive-url=https://web.archive.org/web/20201010105651/https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Motor-Neuron-Diseases-Fact-Sheet |archive-date=10 October 2020 |access-date=27 October 2020 |website=www.ninds.nih.gov |publisher=National Institute of Neurological Disorders and Stroke}}</ref>. ALS dastlabki bosqichlarida asta-sekin mushaklar qattiqlashishi, titroq va kuchsizlik bilan namoyon boʻladi<ref name="NINDS2020">{{Cite web |title=Amyotrophic Lateral Sclerosis (ALS) Fact Sheet |url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet |url-status=live |archive-url=https://web.archive.org/web/20170105002627/http://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet |archive-date=5 January 2017 |access-date=22 October 2020 |publisher=National Institute of Neurological Disorders and Stroke}}</ref>. Bemor motor neyronlarning nobud boʻlishi bilan, oxir-oqibat ovqatlanish, gapirish, harakat qilish va nihoyat, nafas olish imkoniyatlarini yoʻqotadi<ref name="NINDS2020" />. ALS bilan kasallangan odamlarning 15% [[frontotemporal demensiya]]ning toʻliq rivojlanishini boshdan kechiradi, taxminan 50% esa fikrlash va [[xulq|xulq-atvor]] bilan bogʻliq baʼzi kichik qiyinchiliklarni boshdan kechiradi<ref name="Lancet2017">{{cite journal |vauthors=van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, van den Berg LH |date=November 2017 |title=Amyotrophic lateral sclerosis |journal=Lancet |volume=390 |issue=10107 |pages=2084–2098 |doi=10.1016/S0140-6736(17)31287-4 |pmid=28552366 |s2cid=24483077}}</ref>. Yuqorida aytib oʻtilgan alomatlardan qaysi biri birinchi paydo boʻlishiga qarab, ALS ''limb-onset'' (qoʻl yoki oyoqlarda zaiflik bilan boshlanuvchi) yoki ''bulbar-onset'' ([[Dizartriya|gapirish]] yoki [[disfagiya|yutish]]da qiyinchilik bilan boshlanuvchi) turlarga boʻlinadi<ref name="Nature2017">{{cite journal |display-authors=6 |vauthors=Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, Shaw PJ, Simmons Z, van den Berg LH |date=October 2017 |title=Amyotrophic lateral sclerosis |url=http://eprints.whiterose.ac.uk/122838/1/WR_ALS-Hardiman_V3_EDITED_FOR_AUTHOR_APPROVAL_FINAL_APPROVED-1.pdf |url-status=live |journal=Nature Reviews. Disease Primers |volume=3 |issue=17071 |pages=17071 |doi=10.1038/nrdp.2017.71 |pmid=28980624 |s2cid=1002680 |archive-url=https://web.archive.org/web/20201201185718/http://eprints.whiterose.ac.uk/122838/1/WR_ALS-Hardiman_V3_EDITED_FOR_AUTHOR_APPROVAL_FINAL_APPROVED-1.pdf |archive-date=1 December 2020 |access-date=20 December 2019}}</ref>.

ALSning aksariyat holatlari (taxminan 90-95%) maʼlum sababga ega emas va ''sporadik ALS'' deb ataladi<ref name="NINDS2020" /><ref>{{cite web |title=Understanding ALS |url=https://www.als.org/understanding-als |publisher=The ALS Association |language=en |access-date=28 October 2020 |archive-date=26 October 2020 |archive-url=https://web.archive.org/web/20201026133033/https://www.als.org/understanding-als |url-status=live}}</ref>. Biroq, [[ekologik omillar|atrof-muhit omillari]] ham sabab boʻlishi mumkin<ref name="Wingo2011">{{cite journal | vauthors = Wingo TS, Cutler DJ, Yarab N, Kelly CM, Glass JD | title = The heritability of amyotrophic lateral sclerosis in a clinically ascertained United States research registry | journal = PLOS ONE | volume = 6 | issue = 11 | pages = e27985 | year = 2011 | pmid = 22132186 | pmc = 3222666 | doi = 10.1371/journal.pone.0027985 | bibcode = 2011PLoSO...627985W | doi-access = free }}</ref>. Qolgan 5-10% holatlari esa [[genetik]] sababga ega boʻlib, koʻpincha kasallikning oilaviy tarixi bilan bogʻliq va ''oilaviy ALS'' (avloddan avlodga oʻtadigan) deb ataladi<ref name="Goutman 480–493" /><ref>{{cite web |title=Amyotrophic lateral sclerosis |url=https://medlineplus.gov/genetics/condition/amyotrophic-lateral-sclerosis/ |website=MedlinePlus Genetics |access-date=7 August 2023 |language=en}}</ref>. Ushbu genetik holatlarning taxminan yarmi toʻrt muayyan [[gen]]ning kasallik keltiruvchi variantlari tufayli yuzaga keladi<ref name="Goutman-2022">{{cite journal | vauthors = Goutman SA, Hardiman O, Al-Chalabi A, Chió A, Savelieff MG, Kiernan MC, Feldman EL | title = Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis | journal = The Lancet. Neurology | volume = 21 | issue = 5 | pages = 465–479 | date = May 2022 | pmid = 35334234 | pmc = 9513754 | doi = 10.1016/S1474-4422(21)00414-2 }}</ref>. [[Diagnostika]] shaxsning belgilari va simptomlariga asoslanadi, boshqa mumkin boʻlgan sabablarni istisno qilish uchun testlar oʻtkaziladi<ref name="NINDS2020" />.

ALS uchun maʼlum davolanish usuli yoʻq<ref name="NINDS2020" />. Davolash maqsadi kasallikning rivojlanishini sekinlashtirish va simptomlarni yengillashdir<ref name="Lancet2017" />. ALSni sekinlashtiradigan davolashlar orasida riluzol (hayotni ikki yoki uch oyga uzaytiradi) va natriy fenilbutirat/ursodoksikoltaurin (hayotni taxminan yetti oyga uzaytiradi) mavjud<ref name="alsa1">{{Cite web |title=FDA-Approved Drugs for Treating ALS |url=https://www.als.org/navigating-als/living-with-als/fda-approved-drugs |access-date=25 April 2023 |website=The ALS Association |language=en |archive-date=25 April 2023 |archive-url=https://web.archive.org/web/20230425205601/https://www.als.org/navigating-als/living-with-als/fda-approved-drugs |url-status=live}}</ref><ref>{{cite journal | vauthors = Heo YA | title = Sodium Phenylbutyrate and Ursodoxicoltaurine: First Approval | journal = CNS Drugs | volume = 36 | issue = 9 | pages = 1007–1013 | date = September 2022 | pmid = 35907175 | doi = 10.1007/s40263-022-00945-x | s2cid = 251162676 | url = https://figshare.com/articles/online_resource/Sodium_Phenylbutyrate_and_Ursodoxicoltaurine_First_Approval/20327265 }}</ref>. Noinvaziv ventilyatsiya hayot sifatini va davomiyligini yaxshilaydi<ref name="Support2016">{{cite journal | vauthors = Hobson EV, McDermott CJ | title = Supportive and symptomatic management of amyotrophic lateral sclerosis | journal = Nature Reviews. Neurology | volume = 12 | issue = 9 | pages = 526–538 | date = September 2016 | pmid = 27514291 | doi = 10.1038/nrneurol.2016.111 | url = http://eprints.whiterose.ac.uk/104445/1/16.pdf | access-date = 20 December 2019 | url-status = live | s2cid = 8547381 | archive-url = https://web.archive.org/web/20201201183335/http://eprints.whiterose.ac.uk/104445/1/16.pdf | archive-date = 1 December 2020 }}</ref>. Mexanik ventilyatsiya esa hayot davomiyligini uzaytirishi mumkin, ammo kasallikning rivojlanishini toʻxtatmaydi<ref name="Care2017">{{cite journal | vauthors = Soriani MH, Desnuelle C | title = Care management in amyotrophic lateral sclerosis | journal = Revue Neurologique | volume = 173 | issue = 5 | pages = 288–299 | date = May 2017 | pmid = 28461024 | doi = 10.1016/j.neurol.2017.03.031 }}</ref>. Oziqlanish naychasi [[vazn]] va oziqlanishni saqlashda yordam berishi mumkin<ref name="Conn2015">{{cite journal | vauthors = Connolly S, Galvin M, Hardiman O | title = End-of-life management in patients with amyotrophic lateral sclerosis | journal = The Lancet. Neurology | volume = 14 | issue = 4 | pages = 435–442 | date = April 2015 | pmid = 25728958 | doi = 10.1016/S1474-4422(14)70221-2 | s2cid = 34109901 }}</ref>. Oʻlim odatda [[Nafas qisishi|nafas yetishmovchiligi]] sababli yuzaga keladi<ref name="Lancet2011">{{cite journal | vauthors = Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC | display-authors = 6 | title = Amyotrophic lateral sclerosis | url = https://archive.org/details/sim_the-lancet_march-12-18-2011_377_9769/page/942 | journal = Lancet | volume = 377 | issue = 9769 | pages = 942–955 | date = March 2011 | pmid = 21296405 | doi = 10.1016/s0140-6736(10)61156-7 | doi-access = free }}</ref>. Kasallik har qanday yoshdagi insonlarga taʼsir koʻrsatishi mumkin, lekin odatda, 60 yosh atrofida boshlanadi<ref name="Lancet2011" />. Kasallikning boshlanishidan oʻlimgacha oʻrtacha hayot davomiyligi 2-4 yilni tashkil etadi, lekin baʼzi holatlarda kasallanganlarning taxminan 10% dan koʻpi 10 yildan ortiq yashaydi<ref name="Pupillo-2014">{{cite journal | vauthors = Pupillo E, Messina P, Logroscino G, Beghi E | title = Long-term survival in amyotrophic lateral sclerosis: a population-based study | url = https://archive.org/details/sim_annals-of-neurology_2014-02_75_2/page/286 | journal = Annals of Neurology | volume = 75 | issue = 2 | pages = 287–297 | date = February 2014 | pmid = 24382602 | doi = 10.1002/ana.24096 | s2cid = 205345019 }}</ref>.

Kasallik haqida ilk taʼriflar 1824-yilda Charles Bell tomonidan berilgan<ref name="Rowland2001" />. 1869-yilda [[fransuz]] nevrologi Jean-Martin Charcot kasallik simptomlari va asosiy neyrologik muammolar oʻrtasidagi bogʻlanishni birinchi marta aniqladi. 1874-yildan boshlab, u ''amiotrofik lateral skleroz'' atamasidan foydalanishni boshladi<ref name="Rowland2001">{{cite journal | vauthors = Rowland LP | title = How amyotrophic lateral sclerosis got its name: the clinical-pathologic genius of Jean-Martin Charcot | journal = Archives of Neurology | volume = 58 | issue = 3 | pages = 512–515 | date = March 2001 | pmid = 11255459 | doi = 10.1001/archneur.58.3.512 }}</ref>.
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== Tasnifi ==
ALS motor neyron kasalligi boʻlib, bu motor neyronlariga tanlab taʼsir qiladigan neyrologik kasalliklar guruhidir. Motor neyronlari tanadagi [[skelet muskuli|skelet mushaklarini]] boshqaradigan [[hujayra]]lardir<ref name="NINDS2020" />. Boshqa motor neyron kasalliklariga birlamchi lateral skleroz (PLS), progressiv muskul atrofiyasi (PMA), progressiv bulbar paralich, psevdobulbar paralich va monomelik amiotrofiya (MMA) kiradi<ref>{{cite web|url=https://icd.who.int/browse11/l-m/en#/http://id.who.int/icd/entity/661720689|title=8B60 Motor neuron disease|website=ICD-11 for Mortality and Morbidity Statistics|publisher=World Health Organization|access-date=24 January 2019|archive-date=1 August 2018|archive-url=https://web.archive.org/20180801205234/https://icd.who.int/browse11/l-m/en%23/http://id.who.int/icd/entity/294762853#/http://id.who.int/icd/entity/661720689|url-status=live}}</ref>.

ALS kasalligi motor neyronlarining qaysi qismi taʼsirlanganiga, tananing qaysi aʼzosi birinchi boʻlib zararlanganiga, genetik boʻlishiga va kasallikning boshlanish yoshiga qarab bir necha turlarga klassifikatsiya qilnadi . Ushbu kasallik bilan tashxislangan har bir inson bu murakkab va bir-birini toʻldiruvchi subturlarning kesishgan joyida oʻziga xos oʻrinda boʻladi, bu esa diagnostika, tushunish va prognozlashda qiyinchiliklar tugʻdiradi<ref>{{cite journal | vauthors = van Eenennaam RM, Koppenol LS, Kruithof WJ, Kruitwagen-van Reenen ET, Pieters S, van Es MA, van den Berg LH, Visser-Meily JM, Beelen A | display-authors = 6 | title = Discussing Personalized Prognosis Empowers Patients with Amyotrophic Lateral Sclerosis to Regain Control over Their Future: A Qualitative Study | journal = Brain Sciences | volume = 11 | issue = 12 | pages = 1597 | date = November 2021 | pmid = 34942899 | pmc = 8699408 | doi = 10.3390/brainsci11121597 | doi-access = free }}</ref>.

=== Motor neyron kasalligining subturlari ===
[[File:Illustration of the motor neuron tract descending from primary motor cortex, via spinal cord, to skeletal muscle.jpg|thumb|Klassik ALS miya va umurtqa pogʻonasidagi neyronlarni (yuqori motor neyronlari, qizil rangda koʻrsatilgan) hamda umurtqa pogʻonasidan muskullargacha boradigan pastki motor neyronlarni (koʻk rangda koʻrsatilgan) oʻz ichiga oladi<ref name="Cold Spring Harbor 2017">{{cite journal | vauthors = Grad LI, Rouleau GA, Ravits J, Cashman NR | title = Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS) | journal = Cold Spring Harbor Perspectives in Medicine | volume = 7 | issue = 8 | page = a024117 | date = August 2017 | pmid = 28003278 | pmc = 5538408 | doi = 10.1101/cshperspect.a024117 | doi-access = free }}</ref>. ]]
ALS motor neyronlarning taʼsirlanish turiga qarab klassifikatsiya qilinadi<ref name="Masrori-2020" />. Tanadagi har qanday skelet muskulini muvaffaqiyatli boshqarish uchun bosh miya poʻstlogʻi harakat markazidan yuqori motor neyron orqali [[orqa miya]] bel qismiga signal yuborilishi kerak. Bu yerda u [[sinaps]] orqali pastki motor neyronga ulanadi, u esa muskulga bogʻlanadi. Miya va muskul oʻrtasidagi yuqori yoki pastki motor neyronining zararlanishi turli xil simptomlarni keltirib chiqaradi<ref name="Cold Spring Harbor 2017" />. Yuqori motor neyronlarning zararlanishi [[spazm]], qattiqlik va tendon reflekslarining ortishi yoki klonusni; pastki motor neyronining zararlanishi esa zaiflik, mushak atrofiyasi va fassikulatsiyalar (mushaklarning oʻz-oʻzidan qisqarishi) kabi simptomlarni keltirib chiqaradi<ref name="arora-2023">{{cite book | vauthors = Arora RD, Khan YS | chapter = Motor Neuron Disease |date=2023 | chapter-url = http://www.ncbi.nlm.nih.gov/books/NBK560774/ | title = StatPearls |access-date=27 August 2023 |place=Treasure Island (FL) |publisher=StatPearls Publishing |pmid=32809609 }}</ref>.

Klassik ALS bosh va orqa miyadagi yuqori va pastki motor neyronlarning [[degeneratsiya]]sini oʻz ichiga oladi<ref name="Goutman 480–493">{{cite journal | vauthors = Goutman SA, Hardiman O, Al-Chalabi A, Chió A, Savelieff MG, Kiernan MC, Feldman EL | title = Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis | journal = The Lancet. Neurology | volume = 21 | issue = 5 | pages = 480–493 | date = May 2022 | pmid = 35334233 | pmc = 9513753 | doi = 10.1016/S1474-4422(21)00465-8 }}</ref><ref name="Masrori-2020" />. Birlamchi lateral skleroz (PLS) faqat yuqori motor neyronlarning degeneratsiyasini, progressiv mushak atrofiyasi (PMA) esa faqat pastki motor neyronlarning degeneratsiyasini anglatadi. PLS va PMA alohida kasalliklar yoki ALSning turli xil variantlari ekanligi haqida munozaralar bor<ref name="Lancet2017" />.
{| class="wikitable"
|+
! ALS asosiy subturlari
!Yuqori motor neyron degeneratsiyasi
!Pastki motor neyron degeneratsiyasi
|-
|Klassik ALS
|Bor
|Bor
|-
|Birlamchi lateral skleroz (PLS)
|Bor
|Yoʻq
|-
|Progressiv mushak atrofiyasi (PMA)
|Yoʻq
|Bor
|}
== Tarqalishi ==
[[File:Prevalence of ALS in the United States by age group, 2012-2015.jpg|thumb|upright=1.5|2012–2015 yillarda Amerikada ALSning yosh guruhlari boʻyicha taxminiy tarqalishi<ref name="MMWR 2015">{{cite journal | vauthors = Mehta P, Kaye W, Raymond J, Punjani R, Larson T, Cohen J, Muravov O, Horton K | display-authors = 6 | title = Prevalence of Amyotrophic Lateral Sclerosis – United States, 2015 | journal = MMWR. Morbidity and Mortality Weekly Report | volume = 67 | issue = 46 | pages = 1285–1289 | date = November 2018 | pmid = 30462626 | pmc = 5858037 | doi = 10.15585/mmwr.mm6746a1 }}</ref>]]. ALS – bu kattalar orasida eng keng tarqalgan motor neyron kasalligi boʻlib, [[Alsgeymer kasalligi|Alsgeymer]] va [[Parkinson kasalligi|Parkinson kasalligidan]] keyin uchinchi eng keng tarqalgan neyrodegenerativ kasallikdir<ref name="Neuroscience2014">{{cite journal | vauthors = Renton AE, Chiò A, Traynor BJ | title = State of play in amyotrophic lateral sclerosis genetics | journal = Nature Neuroscience | volume = 17 | issue = 1 | pages = 17–23 | date = January 2014 | pmid = 24369373 | pmc = 4544832 | doi = 10.1038/nn.3584 | hdl = 2318/156177 }}</ref><ref>{{cite book | vauthors = Checkoway H, Lundin JI, Kelada SN | year = 2011 | chapter = Chapter 22: Neurodegenerative diseases | veditors = Rothman N, Hainaut P, Schulte P, Smith M, Boffetta P, Perera F | title = Molecular Epidemiology: Principles and Practices | url = https://archive.org/details/molecularepidemi0000unse_e6j0 | publisher = International Agency for Research on Cancer | pages = 408–409 | isbn = 978-9283221630}}</ref>. Butun dunyo boʻylab yiliga 100,000 kishidan taxminan 1.9 nafari ALS ga chalingan, hozirda esa bu koʻrsatkich 100,000 kishidan 4.5 nafarni tashkil qilmoqda<ref>{{cite journal | vauthors = Chiò A, Logroscino G, Traynor BJ, Collins J, Simeone JC, Goldstein LA, White LA | title = Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature | journal = Neuroepidemiology | volume = 41 | issue = 2 | pages = 118–130 | date = 2013 | pmid = 23860588 | pmc = 4049265 | doi = 10.1159/000351153 }}</ref>. Yevropada yangi holatlar soni yiliga 100,000 kishidan 2.6 nafarni, kasallikdan aziyat chekayotganlar esa 100,000 kishidan 7–9 nafarni tashkil etadi<ref>{{cite journal | vauthors = Hardiman O, Al-Chalabi A, Brayne C, Beghi E, van den Berg LH, Chio A, Martin S, Logroscino G, Rooney J | display-authors = 6 | title = The changing picture of amyotrophic lateral sclerosis: lessons from European registers | journal = Journal of Neurology, Neurosurgery, and Psychiatry | volume = 88 | issue = 7 | pages = 557–563 | date = July 2017 | pmid = 28285264 | doi = 10.1136/jnnp-2016-314495 | url = https://kclpure.kcl.ac.uk/portal/en/publications/the-changing-picture-of-amyotrophic-lateral-sclerosis(93e2a283-3125-4464-88ed-009897be906c).html | url-status = live | access-date = 22 August 2018 | s2cid = 52871105 | archive-url = https://web.archive.org/web/20191221151144/https://kclpure.kcl.ac.uk/portal/en/publications/the-changing-picture-of-amyotrophic-lateral-sclerosis(93e2a283-3125-4464-88ed-009897be906c).html | archive-date = 21 December 2019 | hdl = 2318/1633611 | hdl-access = free | archivedate = 2019-12-21 | archiveurl = https://web.archive.org/web/20191221151144/https://kclpure.kcl.ac.uk/portal/en/publications/the-changing-picture-of-amyotrophic-lateral-sclerosis(93e2a283-3125-4464-88ed-009897be906c).html }}</ref>. Yevropalik erkaklar uchun ALS rivojlanishining xavfi 1:350, ayollar uchun esa 1:400 ni tashkil etadi. Erkaklarda kasallikka chalinish xavfi yuqoriroq boʻlib, orqa miya zararlanishi bilan boshlanuvchi ALS kasalligi ayollarga nisbatan erkaklarda koʻproq uchraydi. 2015-yilda Amerikada ALS bilan ogʻriganlar soni 100,000 kishidan 5.2 nafarga teng boʻlib, bu koʻrsatkich oq tanlilar, erkaklar va 60 yoshdan katta insonlar orasida yuqoriroq edi. Sharqiy Osiyoda yangi holatlar soni yiliga 100,000 kishidan 0.8 nafarni, Janubiy Osiyoda esa 100,000 kishidan 0.7 nafarni tashkil qiladi. ALS epidemiologiyasi boʻyicha tadqiqotlarning taxminan 80% Yevropa va Amerikada oʻtkazilgan boʻlib, asosan, shimoliy yevropaliklar oʻrtasida amalga oshirilgan<ref name="Nature2017" />. Dunyoning koʻp qismlarida, jumladan [[Afrika]], Osiyoning ayrim qismlari, [[Hindiston]], [[Rossiya]] va [[Janubiy Amerika]]da ALSning tarqalishi haqida yetarli maʼlumotlar mavjud emas. Gʻarbiy Tinch okeanida, masalan, [[Guam]], Yaponiya Kii yarim oroli va [[Gʻarbiy Yangi Gvineya]] kabi hududlarda ALS tarqalishi 50–100 baravar yuqori boʻlishi xabar qilingan. Bu hududlardagi kasallik holatlari 1960-yillardan beri kamaygan<ref name="Orphanet2009">{{cite journal | vauthors = Wijesekera LC, Leigh PN | title = Amyotrophic lateral sclerosis | journal = Orphanet Journal of Rare Diseases | volume = 4 | issue = 4 | pages = 3 | date = February 2009 | pmid = 19192301 | pmc = 2656493 | doi = 10.1186/1750-1172-4-3 | doi-access = free }}</ref>, lekin sabab hali ham nomaʼlum<ref name="Conspiracy">{{cite journal | vauthors = Al-Chalabi A, Hardiman O | title = The epidemiology of ALS: a conspiracy of genes, environment and time | journal = Nature Reviews. Neurology | volume = 9 | issue = 11 | pages = 617–628 | date = November 2013 | pmid = 24126629 | doi = 10.1038/nrneurol.2013.203 | s2cid = 25040863 }}</ref>.
== Belgilari ==
Bu kasallik yuqori va pastki motor neyronlarining degeneratsiyasi natijasida tanada mushak zaifligi, atrofiyasi va spazmni keltirib chiqaradi. [[Retseptorlar|Sensor nervlar]] va [[vegetativ nerv sistemasi|avtonom nerv tizimi]], odatda, zararlanmaydi, bu esa ALS bilan ogʻrigan koʻpchilik bemorlarning [[eshitish]], [[ko'rish]], sezish, [[hid bilish]] va [[taʼm bilish]] qobiliyatlari saqlab qolishini anglatadi<ref name="NINDS2020" />.

ALSning dastlabki alomatlari juda noaniq boʻlib, koʻpincha eʼtibordan chetda qoladi. ALSning ilk belgilari, odatda, tananing bir tomonida mushak kuchsizligi yoki mushak atrofiyasi hisoblanadi. Boshqa alomatlar sifatida nafas olishda va yutishda qiyinchilik, mushaklar spazmi, nutqning buzilishi kuzatiladi<ref>{{cite journal | vauthors = Ravits J, Appel S, Baloh RH, Barohn R, Brooks BR, Elman L, Floeter MK, Henderson C, Lomen-Hoerth C, Macklis JD, McCluskey L, Mitsumoto H, Przedborski S, Rothstein J, Trojanowski JQ, van den Berg LH, Ringel S | display-authors = 6 | title = Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis | journal = Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration | volume = 14 | issue= Suppl 1 | pages = 5–18 | date = May 2013 | pmid = 23678876 | pmc = 3779649 | doi = 10.3109/21678421.2013.778548 }}</ref>.
== Sabablari ==
Sporadik ALSni keltiruvchi sabablar nomaʼlum boʻlib, shuning uchun bu kasallik [[idiopatik kasallik]] sifatida tavsiflanadi<ref name="Lancet2011" />. Uning aniq kelib chiqish sababi nomaʼlum boʻlsa-da, genetik va atrof-muhit omillari muhim rol oʻynaydi, degan taxminlar bor<ref name="Wingo2011" />. Genetik omillar atrof-muhit omillaridan yaxshiroq tushuntirilib, hech qanday aniq atrof-muhit omili ALSni keltirib chiqarish uchun qatʼiyan koʻrsatilmagan. ALS rivojlanayotgan koʻpchilik odamlar 40 dan 70 yoshgacha, tashxis qoʻyilganda oʻrtacha yosh 55 ni tashkil qiladi<ref name="auto">{{Cite web |title=Who Gets ALS? |url=https://www.als.org/understanding-als/who-gets-als |access-date=6 April 2022 |website=The ALS Association |language=en |archive-date=6 April 2022 |archive-url=https://web.archive.org/web/20220406153827/https://www.als.org/understanding-als/who-gets-als |url-status=live}}</ref>. ALS erkaklarda ayollarga nisbatan 20% koʻproq uchraydi, lekin 70 yoshdan keyin boshlanadigan bemorlarda jins bilan bogʻliq tarqalishdagi farq yoʻqoladi<ref name="Mayo Clinic">{{Cite web |title=Amyotrophic lateral sclerosis (ALS) - Symptoms and causes |url=https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022 |access-date=6 April 2022 |website=Mayo Clinic |language=en |archive-date=6 April 2022 |archive-url=https://web.archive.org/web/20220406073949/https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022 |url-status=live}}</ref>.
== Tashxis ==
[[File:ALS cross.jpg|thumb|upright=1.2|Bosh miya MRT tasvirida T2 signalining kuchayganini tasvirning markazi atrofidagi ichki kapsulaning orqa qismidagi kichik oq hudud sifatida koʻrsatadi, bu ALS tashxisiga mos keladi.]]ALS (Amiotrofik lateral skleroz) ning aniq tashxisi uchun bitta test yetarli emas<ref name="NINDS2020" />. Tashxis shifokorning klinik baholashiga asoslanadi<ref name="NINDS2020" />. Shifokor dastlab, bemorning toʻliq kasallik tarixini toʻplaydi va simptomlarini baholash uchun nevrologik tekshiruvlarni muntazam ravishda oʻtkazadi. Bu jarayonda mushak zaifligi, mushak atrofiyasi, giperefleksiya, Babinskiy simptomi va spastiklik kabi belgilar nazorat qilinadi<ref name="NINDS2020" />. Bir qator biomarkarlar ushbu holat uchun oʻrganilmoqda, lekin 2023-yil holatiga koʻra, ular umumiy tibbiyotda qoʻllanilmaydi<ref>{{cite journal | vauthors = Verber NS, Shepheard SR, Sassani M, McDonough HE, Moore SA, Alix JJ, Wilkinson ID, Jenkins TM, Shaw PJ | display-authors = 6 | title = Biomarkers in Motor Neuron Disease: A State of the Art Review | journal = Frontiers in Neurology | volume = 10 | pages = 291 | date = 2019 | pmid = 31001186 | pmc = 6456669 | doi = 10.3389/fneur.2019.00291 | doi-access = free }}</ref>.
== Davolash ==
ALSning davosi topilmagan<ref name="Lancet2017" />. Davolash, asosan, belgilarni yengillashtirish va qoʻllab-quvvatlovchi yordam berishga qaratilgan boʻlib, maqsad hayot sifatini yaxshilash va umrni uzaytirishdir<ref name="Lancet2017" />.
== Manbalar ==
{{manbalar}}
[[Turkum:Tibbiyot]]
[[Turkum:Nevrologik kasalliklar]]
[[Turkum:Nevrologiya]]