Isifo ALS: ebangela, iimpawu kunye ikhondo. Uxilongo kunye nonyango lateral sclerosis

amayeza Modern ke eguqukayo. Izazinzulu ukudala ngeendlela ezintsha zonyango lwezifo esinganyangekiyo ngaphambili. Noko ke, iingcali zingenako namhlanje ukunika unyango okwaneleyo zonke izifo. Esinye isifo eso sifo ALS. Izinto ezibangela le sifo nangoku unexplored, kwaye inani lezigulane kwandisa minyaka kuphela. Kweli nqaku sisebenzisa sihlolisise le ngezifo, iimpawu zayo engundoqo kunye neendlela zonyango.

ulwazi jikelele

lateral sclerosis (ALS, isifo Lou Gehrig kaThixo) i ngezifo kakhulu e-nervous system, apho ekuthiwa-ilahleko zemithambo-luvo mafutha apha emqolo ngokunjalo kwi uthinte ebuchotsheni. Sisifo okungapheliyo kunye esinganyangekiyo ngokuthe ngcembe kukhokelela ukonakala kwenkqubo luvo. Kumanqanaba okugqibela esi sifo umntu uba athalala, kodwa ezithanda ingqondo ecacileyo kunye nempilo yengqondo.

isifo ALS, oonobangela, yaye apho ukuphelisa Pathogenesis khange kufundwe, akukho iindlela ezahlukeneyo ezithile zokuxilonga kunye nonyango. Izazinzulu namhlanje kuqhubeka ukuphonononga ngenkuthalo kuyo. Ngoku kwakusazeka ukuba sifo senzeka ingakumbi abantu abaphakathi kweminyaka eli-50 ukuya kutsho kwiminyaka emalunga-70, kodwa ke kukho iimeko kunye ngokoyiswa ngaphambili.

ngezigaba

Kuxhomekeka indawo iimpawu eziphambili Iingcali sifo zezi zilandelayo ngokumila:

1. Lumbosacral ifomu (kukho kukwaphula umsebenzi lwemoto le nemilenze).
2. ifomu Bulbar (ezinye nuclei ubuchopho ochaphazelekayo, nto leyo ithetha ukushwabana umlinganiswa central).
3. ifomu wesibeleko-thoracic (iimpawu eziphambili baqhelene abacombululi umsebenzi lwemoto ye imilenze phezulu).

Kwelinye icala, iingcali ukwahlukanisa iindlela ezintathu isifo ALS:

1. uhlobo Mariana (iimpawu eziphambili kwenzeka kwangoko kakhulu, kukho ikhondo olucothayo kwesi sifo).
2. Uhlobo Classic manqapha (95% yazo zonke iimeko).
3. uhlobo Family (iyahluka imbonakaliso emva kanye nezici amafa).

Kutheni isifo khona ALS?

Izinto ezibangela le sifo, ngelishwa, Kusekho ayikaqondwa. Ngoku Izazinzulu ziye bodwa eziliqela, kubukho, kwenzeke ukuphuhlisa amaxesha awongezelelekileyo ezininzi:

• guquko protein ubikvina;
• ngokwaphula inyathelo neurotrophic factor;
• izakhi zofuzo ezithile;
• ukwanda igcwala olukhulu simahla kwizisele neuronal ngokwabo;
• phambi kwe-arhente esosulelayo;
• umsebenzi kwandisa kokubizwa-asidi excitatory acid.

isifo ALS. iimpawu

Photo kwezigulane nesi sifo ingafumaneka kwi kubalawuli ezikhethekileyo. Bonke bephela amanyene kuphela enye into - iimpawu zangaphandle kwesi sifo kwi zigaba emva kwexesha.

Ngokuphathelele iimpawu eziphambili kwezonyango izifo, kunqabile kakhulu ukuba ukuqapha kwicala kwizigulane. Ngaphezu koko, izigulane ezinokuba basoloko bechaza stress babo njalo okanye ukungabikho ukuphumla ukusuka routine emsebenzini. ezi zilandelayo iimpawu zesi sifo, ibakho usaqala:

• buthathaka kwezihlunu;
• dysarthria (kunzima ukuthetha);
• aqaqambayo rhoqo;
• imilenze ubuthakathaka imilenze;
• twitching ukukhanya izihlunu.

Yonke le miqondiso kufuneka wazise wonke ube isizathu sokuba udluliseiwe yincutshe. Kungenjalo, isifo baya kuqhubela phambili, nto leyo amaxesha amaninzi kwandisa amathuba iingxaki.

isifo

Njani ALS? Esi sifo, iimpawu eziye ezidweliswe ngasentla, ekuqaleni iqala nobuthathaka kwezihlunu kunye antsi kwi yesiqephu. Ukuba ngezifo eyenzeke imilenze, izigulana babe nzima ehamba, rhoqo kukhubeka.

Ukuba isifo kubonakala kunye ukuphazamiseka imilenze aphezulu, kukho iingxaki malunga nokuphunyezwa kwe imisebenzi eziziziqalelo (amaqhosha ihempe, khawulitshitshise iqhosha kwi lock).

njani enye uqaphela isifo ALS? Ubangelwa sifo-25% yamatyala zigutyungelwe izitshanguba le Oblongata Medulla. Ekuqaleni, ubunzima zentetho kwaye ke baginye. Konke oku kuphathelene iingxaki kunye ukuhlafuna ukutya. Ngenxa yoko, abantu akusekho nokutya ngokufanelekileyo kwaye ukuncipha. Kulo mba, izigulane ezininzi kweyela urhulumente edakumbisayo, kuba isifo ngokuqhelekileyo oku kuchaphazela umsebenzi kwengqondo.

Ezinye izigulane kufuneka ubunzima bokwakha amagama kunye nkqu yoxinaniso yesiqhelo. ephule Minor yale hlobo isikakhulu ngenxa ukuphefumla kakuhle ebusuku. ngoku iingcali zonyango kufuneka uxelele isigulana malunga iimpawu ukuhamba sifo, iinketho unyango, ukuze akwazi phambili ukwenza isigqibo ngekamva labo.

izigulane ezininzi kufa ukusilela zemiphunga okanye inyumoniya. Ngokuqhelekileyo, ukufa kwenzeka kwisithuba seminyaka emihlanu ukususela kumhla isiqinisekiso sifo.

ekuxilongweni

Qinisekisa phambi sifo ube yincutshe kuphela. Kulo mba, indima ephambili zokutyhila abanobuchule zemifanekiso ezikhoyo lwezonyango ngayo umntu othile. Kubaluleke kakhulu ezohlukileyo une isifo ALS.

1. Electromyography. Le ndlela yenza kube lula ukuqinisekisa ubukho fasciculations xa nisaqala yophuhliso lwayo. Ngethuba le nkqubo, ingcaphephe ehlola umsebenzi yombane izihlunu.
2. MRI utyhila nakujoliso olutsha engaqhelekanga nokuvavanya ukusebenza izakhiwo luvo.

Yintoni ekufuneka ziphathwe?

Ngelishwa, iyeza namhlanje singasondezi unyango olusebenzayo nxamnye nesi sifo. njani mna ndiloyisile sifo ALS? Unyango kufuneka ejoliswe ikakhulu kwikhosi isifo linyuke. la manyathelo alandelayo zisetyenziselwa le njongo:

• massage okukhethekile imilenze;
• xa kukho ukusilela izihlunu zemiphunga ubekwa yomatshini;
• kwimeko jini isincomo okudambisa kunye nemithi yokunciphisa. Kwimeko nganye, iziyobisi ezimiselwe kwi kwisiseko ngasinye;
• ubuhlungu bamalunga stoped steroidal amachiza-ukudumba ( "Finlepsin");
• iingcali Namhlanje zinika zonke izigulane ichiza "Riluzole". It has isenzo eqinisekisiwe kwaye i inhibitor zokukhutshwa ekuthiwa- glutamic acid. Xa tapeworm iziyobisi kunciphisa umonakalo neuronal. Noko ke, oku kuthetha ayikho nako ukumphilisa ngokupheleleyo isigulane, oko kubamba ALS sifo kuphela;
• ukuba kube lula ukuhamba isigulane usebenzisa izixhobo ezizodwa (imisimelelo, izitulo) ntsimbi namacici ukuba nitrogen epheleleyo entanyeni.

Isiqu therapy cell

Kumazwe amaninzi aseYurophu kwizigulane kunye ALS namhlanje babacukucezela iiseli zabo esiqwini, esiza kucothisa uphuhliso isifo wakhe. Olu hlobo unyango ijolise ekuphuculeni imisebenzi ephambili ebuchotsheni. iiseli stem kulinywa kuloo mmandla eyonakeleyo, luvo kuncitshiswa ukuphucula oxygenation ingqondo ukukhuthaza imbonakalo iimpahla ezintsha yegazi.

Ukuqhela iiseli stem ngokwabo abohlukeneyo zabo ngokuqhelekileyo kuqhutywa kwimeko kwesibhedlele. Emva unyango, isigulana isekhona iintsuku 2 esibhedlele, apho iingcali iliso imeko yakhe.

Ngexesha cell unyango elawulwa kwi-CSF yi empontshwa. Ngaphandle umzimba phantsi abavumelekanga ukuba bavelise ngokwabo, yaye reimplantation lwenziwa kuphela emva kokuba icocwe ngokucokisekileyo.

Okubalulekileyo kukuba, olu hlobo unyango ezinokucothisa kakhulu esi sifo ALS. izigulane Ifoto emva kweenyanga 5-6 emva kokuba inkqubo ibonisa ngokucacileyo le yinto. Ngokupheleleyo balahle isifo ukuxhulumana le akusoze uncedeke. Ngelishwa, iimeko ezinjalo abhalwe, kwaye xa unyango ayiniki nasonasiphi na isiphumo.

isiphelo

Abantu abane esi sifo siphantse sibe nezinzima. Progression ukuphazamiseka intshukumo ekugqibeleni ibandakanya iyabulala (iminyaka 2-6).

Kweli nqaku sithetha ngalo yokuba lo ngezifo, njengoko ALS. Isifo, ogama iimpawu zingabonakali ixesha elide, azikwazi okwangoku sinyangeke kude kube sekupheleni. Izazinzulu emhlabeni wonke bayaqhubeka ukufunda ngenkuthalo esi sifo, izizathu zalo kunye nophuhliso isantya, ezama ukufumana loncedo lwesiphumo.