Sklerosisi ya kimiotrofia ya pembezoni

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Sklerosisi ya kimiotrofia ya pembezoni
Mwainisho na taarifa za nje
An MRI with increased signal in the posterior part of the internal capsule which can be tracked to the motor cortex consistent with the diagnosis of ALS.
ICD-10 G12.2
ICD-9 335.20
OMIM 105400
DiseasesDB 29148
MedlinePlus 000688
eMedicine neuro/14 emerg/24 pmr/10
MeSH D000690

Sklerosisi ya kimiotrofia ya pembezoni (ALS), ambayo pia hujulikana kama Ugonjwa wa Lou Gehrig na ugonjwa wa Charcot, ni hali inayohusisha kufa kwa nuroni.[1] Barani Ulaya jina ugonjwa wa nuroni mota (MND) hutumika sana,[2] ilhali wengine hutumia jina hilo kwa kundi la masharti ambapo ALS inapatikana sana.[3] ALS hubainishwa kwa misuli migumu, kushtuka kwa misuli, na udhaifu unaokithiri kutokana na kupungua kwa ukubwa wa misuli. Hii hupelekea ugumu katika kuongea, kumeza, na hatimaye kupumua.[4]

Visababishi na mienendo[hariri | hariri chanzo]

Kisababishi hakijulikani kwa asilimia 90 hadi 95 za visa.[4] Takribani asilimia 5-10 za visa ni vya Kurithiwa kutoka kwa wazazi.[5] Karibu nusu ya visa hivi vya jenetikia hutokana na moja ya jeni mbili maalum. Husababisha kufa kwa nuroni zinazodhibiti misuli ya hiari. Utambuzi hutegemea ishara na dalili za mtu kwa uchunguzi ili kuondoa visababishi vingine vinavyoweza kukisiwa.[6]

Matibabu, prognosis na epidemiolojia[hariri | hariri chanzo]

Hakuna tiba ya ALS.[4] Dawa iitwayo riluzole inaweza kurefusha maisha kwa takribani miezi miwili hadi mitatu.[7] Uingizaji hewa usio vamizi unaweza kupelekea maisha bora na marefu.[8] Ugonjwa huu huanza katika umri wa miaka 60 na katika visa vya kurithiwa katika umri wa miaka 50.[5] Muda wa wastani wa kuishi kutoka mwanzo hadi kufariki ni miaka mitatu hadi minne.[9] Takribani asilimia 10 huishi zaidi ya miaka 10.[4] Wengi hufariki kutokana na matatizo ya kupumua. Katika sehemu nyingi za dunia, viwango vya ALS havijulikani.[5] Barani Uropa na Marekani, ugonjwa huu huathiri takribani watu 2 kwa kila watu 100,000 kila mwaka.[5][10]

Historia[hariri | hariri chanzo]

Maelezo ya ugonjwa huu yalianza angalau mwaka wa 1824 na Charles Bell.[11] Katika mwaka wa 1869, uhusiano kati ya dalili na matatizo halisi ya kinurolojia ulielezwa kwa mara ya kwanza na Jean-Martin Charcot, aliyeanza kutumia jina sklerosisi ya kimiotrofia ya pembezoni katika mwaka wa 1874.[11] Ilijulikana vizuri Marekani ilipomuathiri mchezaji maarufu wa besiboli Lou Gehrig,[1] na katika karne ya 20 Stephen Hawking alipopata umaarufu wa ufanisi wa kisayansi.[12] Katika mwaka wa 2014 video za ice bucket challenge zilienea kwenye mtandao na kuongeza ufahamu wa umma.[13]

Marejeleo[hariri | hariri chanzo]

  1. 1.0 1.1 (2013) Encyclopedia of human genetics and disease. Santa Barbara, Calif.: Greenwood, 79–80. ISBN 9780313387135. 
  2. Motor neurone disease. Iliwekwa mnamo 2 January 2015.
  3. (2008) Greenfield's neuropathology, 8th ed., London: Hodder Arnold, 947. ISBN 9780340906811. 
  4. 4.0 4.1 4.2 4.3 Motor Neuron Diseases Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS). www.ninds.nih.gov. Iliwekwa mnamo 7 November 2010.
  5. 5.0 5.1 5.2 5.3 Kiernan, MC; Vucic, S; Cheah, BC; Turner, MR; Eisen, A; Hardiman, O; Burrell, JR; Zoing, MC (12 March 2011). "Amyotrophic lateral sclerosis.". Lancet 377 (9769): 942–55. doi:10.1016/s0140-6736(10)61156-7 . PMID 21296405 .
  6. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet (September 19, 2014). Iliwekwa mnamo 2 January 2015.
  7. Miller, RG; Mitchell, JD; Moore, DH (14 March 2012). "Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND).". The Cochrane database of systematic reviews 3: CD001447. doi:10.1002/14651858.CD001447.pub3 . PMID 22419278 .
  8. McDermott, CJ; Shaw, PJ (22 March 2008). "Diagnosis and management of motor neurone disease.". BMJ (Clinical research ed.) 336 (7645): 658–62. doi:10.1136/bmj.39493.511759.be . PMID 18356234 .
  9. (2008) Clinical neurology of the older adult, 2nd ed., Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 421. ISBN 9780781769471. 
  10. Epidemiology of Sporadic ALS. Iliwekwa mnamo 2 January 2015.
  11. 11.0 11.1 Rowland LP (March 2001). "How amyotrophic lateral sclerosis got its name: the clinical-pathologic genius of Jean-Martin Charcot". Arch. Neurol. 58 (3): 512–5. doi:10.1001/archneur.58.3.512 . PMID 11255459 . http://archneur.jamanetwork.com.proxy1.athensams.net/article.aspx?articleid=778657.
  12. (2004) Encyclopedia of family health, 3rd ed., Tarrytown, NY: Marshall Cavendish, 1256. ISBN 9780761474869. 
  13. 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): 94–6. doi:10.5582/irdr.2014.01015 . PMID 25364651 .