I-Amyotrophic Lateral Sclerosis

I-Amyotrophic lateral sclerosis (ALS), ngamanye amaxesha eyaziwa ngokuthi isifo sikaLou Gehrig emva komdlali odumile webhola yebhola, yimeko edala umntu ukuba athatyatheke kancane kancane kwaye aqhubeke buthaka. Olu buthakathaka obunokuqhubekayo lubangelwa ukuguqulwa kwamathambo kwiphondo lwangaphakathi lomgudu wokugcoba , oludlulisela ulwazi kwingqondo kwiimisipha zomzimba.

Njengoko la maseli esiswini afa, izihlunu zixubana nazo ziqala ukuhlaselwa. Ukongezelela, i-neurons engqondweni iyafa, nangona i-neurons efa ngokuqhelekileyo ayinxulumene nendlela umntu acinga ngayo, ngoko ke umntu uya kuhlala eyazi indlela esi sifo siphuthuka ngayo. Kwiimeko ezininzi, i-ALS ibangela ukukhubazeka nokufa phakathi neminyaka emihlanu. Phantse ipesenti yeshumi yexesha, abantu abane-ALS basinda ixesha elide.

I-ALS idla ngokuchaphazela abantu xa iphakathi kweminyaka engama-40 no-70 ubudala; nangona kunjalo, inokuthi iyenze ngamanye amaxesha ebomini bomntu. Amadoda athathwe ngokuphindaphindiweyo kunabesifazane. Ngenhlanhla, i-ALS ayinqabile, ixhatshaza abantu abangaba ngu-30 000 e-United States, malunga nama-5 600 amatyala amatsha e-ALS afunyaniswa rhoqo ngonyaka.

Iimpawu ze-ALS

Iimpawu ze-ALS zivame ukuqala ngobuthathaka. Olu buthathaka lunokuqala ngelo linye linye. Imisipha inganciphisa, iyanamathele okanye ifumaneke ngokuthi "i-fasciculations." Ukuba imilenze ichaphazeleka okokuqala, loo mntu unokuqala ukuphawula ukuba baqhekeza ngokuphindaphindiweyo okanye banokuziva bexhamla.

Ukuba iimpawu ziqala ezandleni, kunokuqala ukuba nobunzima bokusingatha izinto ezincinci, ezifana nokuqosha iqela okanye ukujika ikhiye. Okungaqhelekanga, izihlunu zokuqala ezichaphazelekayo zizo ebusweni nasemqaleni, ezikhokelela ekubeni kunzima ukuthetha okanye ukugwinya. Akukho ukubetha okanye ukuphazamiseka okunxulumene nobuthathaka.

Njengoko eso sifo siyaqhubeka, ubuthathaka buya kuba bubi kwaye buya kufakelwa kwezinye iindawo zomzimba. Umntu uya kulahlekelwa ukuthetha njengoko elahlekelwa ukulawulwa kolwimi kunye nemilomo. Ekugqibeleni, umntu unokufuna i- tube yokutya . Njengoko izidumbu zifuna ukuphefumula, ukuxhaswa kunokunikezelwa, okokuqala ngomshini we- CPAP uze ube ne- air ventical. Ngenxa yokuba abanalo amandla okukhwehlela okanye ukucima imiphefumlo yabo, abantu abane-ALS eziphambili bazimisele ukufuna ukuphefumlelwa kwepneumonia . Enyanisweni, abaninzi abantu abane-ALS baya kugqitywa ngenxa yokusukela okanye ukungaphumeleli kokuphefumula .

Ngamanye amaxesha, abantu abane-ALS banesifo sengqondo esifanelekileyo . Kwakhona, abanye abantu bahlakulela i-pseudobulbar palsy, eyenza kube nzima kubo ukulawula iimvakalelo zabo.

Yintoni ebangela ii-ALS?

Izizathu ezisemthethweni ze-ALS zisacatshungulwa. Isifo ngokuqhelekileyo sibonakala sitsho ngokukhawuleza, nangona malunga neepesenti ezili-10 zeemeko ziyimfuza. I-gene egqithisa i-superoxide dismutase (SOD1), i-enzyme ephula i-radicals yamahhala, yafunyanwa ngo-2001. Ezinye iigulo - kuquka neprotheni ebopha i-DNA (TARDBP, yaziwa nangokuthi yi-TDP43); i-fused-in-sarcoma (FUS), ukungafaneki kwemfuyo kwi-chromosome 9 (C9ORF72); kunye ne-UBQLN2, edibanisa iphrotheni ene-ubiquitin enjenge-ubiquitin? - 2 - zonke zidibene ne-ALS.

Ngenxa yolu tshintsho olusimangqeleko lwamaselula, iiseli zesisongululo uphondo oluphambene nomtya womgogodla kunye neeseli kwi-cortex ye-cerebral ziqala ukufa.

Abanye abantu baye baqaphela ukuxhamla okunokwenzeka phakathi kokulimala kwentloko kunye nobungozi obuninzi be-ALS, nangona ezi ziko zingabonakalisa ingxaki eyahlukileyo eyaziwa njenge- encephalopathy engapheliyo . Amaqhawe aseMkhosini, ngakumbi abo bakhonza kwi-Gulf War, banomngcipheko wokwanda kweempawu ze-ALS, njengabadlali abathile. Ukubonakaliswa kwiibhox kwakhona kuphandwe, nangona akukho nto ecacileyo ayizange ivele.

I-ALS ichongwa njani?

Ukuxilongwa kwe-ALS kufanele kwenziwe yintliziyo ye-neurologist.

Iingcali ze-neurologist ezivandlakisayo izifo ze-neuron ezifana ne-ALS zingathetha ngokudibanisa "iimpawu eziphezulu nezisezantsi ze-neuron" ezifunekayo ukwenzela ukuba zixilongwe. Iziphumo ezithile zeziphumo zokuhlola, ezinjenge-tendon deep reflexes, zibonisa ukuba ubuthathaka bubangelwa zizifo emgodleni okanye kwiingqondo. Ezinye iziphumo zeemvavanyo, ezifana ne-fasciculations, ngokuqhelekileyo zibhekiselele kumonakalo kwimbilini emva kokuba ushiye umgca wesipelini. Ngenxa yokuba izifo ze-neuron ezifana ne-ALS ziwonakalisa indawo apho i-motor neurons eziphezulu zihla ukusuka kwingqondo zixubusha kunye ne-neurons eziphantsi eziphuma emthonjeni, zombini iimpawu ze-neuron eziphezulu kunye nezantsi ziyabonakala kwi-ALS kwaye zifuneka ukuba zixilongwe.

Ukubaluleka kokuxilongwa kwe-ALS ngokuqhelekileyo kubangela ukuvavanya okongeziweyo ukuba kungabandakanyi ezinye, ezinokuphulukiswa ngakumbi, izifo ezinokulinganisa ii-ALS. I- electromyogram (EMG) kunye nesifundo sokuqhutyelwa kwentsholongwane singenziwa ukwenzela ukuba singabandakanyi ukuba izifo ezifana ne-myasthenia gravis okanye i-peripheral neuropathy. Isiskripthi se-MRI singenziwa ukuba singabandakanyi ezinye izifo zentambo yomgulane, njengezicubu okanye i-sclerosis.

Ngokuxhomekeke kwimbali yomntu ngamnye kunye nokuhlolwa komzimba, iimvavanyo ezongezelelweyo ngezifo ezifana ne-HIV, i- Lyme okanye i- syphilis inokwenziwa. Izigulane ezifunyenwe nge-ALS kufuneka ziqikelele ngokugqithiseleyo ukufumana umbono wesibini.

I-ALS ithathwa njani?

Iyeza elilodwa kuphela, uRiluzole, liboniswe ukuba liphumelele ekuphuculeni ukusinda kwezigulane ezine-ALS. Ngelishwa, isiphumo sithobekile, sandisa ixesha lokusinda kuphela kumyinge weenyanga ezintathu ukuya ezintlanu.

Kodwa kukho uncedo. Ukusebenza neqela labaqeqeshi bezonyango kunokunceda ukukhulula ezininzi iimpawu ze-ALS. Iqela elinjalo lingabandakanya i-neurologist, i-therapists, intetho kunye neengcali zengcali kunye neengcali kwizinto zokutya ezinomsoco kunye nokuphefumula.

Abasebenzi bezentlalo bangabandakanyeka ekuncediseni amaqela enkxaso kunye neemfuno zomthetho, ezifana nokuthanda nokuphila kwamagqwetha . Ngokukhethekileyo kufuphi nokuphela kobomi, izigulane ezininzi ziyazuza ngokusebenza kunye neengcali ekunyamekeleni nokunyamekela .

Ukusebenza kunye nabaqeqeshi abaqeqeshiweyo banokukunceda izigulane ze-ALS zihlala ubomi babo bonke ngokuzimeleyo kwaye zithuthuzele ngokusemandleni.

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AC McKee, BE Gavett, RA Stern, CJ Nowinski, RC Cantu, NW Kowall, DP Perl, ET Hedley-Whyte, B Price, C Sullivan, P Morin, HS Lee, CA Kubilus, DH Daneshvar, M Wulff, AE Budson. I-TDP-43 Iproteinopathy kunye ne-Neuron Izifo kwi-Chronic Encephalopathy Chronicle. J Neuropathol Exp Neurol. Agasti 2010

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