Ngaba wakha wazibuza ukuba kulula kangakanani ukwenza ezi zinto xa sihamba, sithetha nomhlobo, okanye sihlafuna ukutya kwethu? Emva kwayo yonke intshukumo, isenzo ngasinye, kukho iqela labathunywa ababaluleke kakhulu emzimbeni wethu. Bangabantu esibabiza ngokuba zii-motor neurons. Bafana neqela labakhonzi abavela kwingqondo yethu abaxelela izihlunu zethu ukuba "zenze oku."
Kodwa, njengezinye iindawo zomzimba wethu, ezi zithunywa zinokonakala. Kulapho ke siphuhla khona iimeko ezibizwa ngokuba 'zizifo ze-motor neuron'. Mhlawumbi ukhe weva nge-ALS. Le yenye yezifo eziphambili kolu didi. Kodwa kukwakho nezinye iintlobo ezininzi zezifo ze-motor neuron esingazivayo kangako ngazo. Ngoko ke namhlanje, masithethe ngazo ngendlela elula.
Ngoobani la 'ma-motor neurons'?
Ngamafutshane, ii-motor neurons luhlobo lweeseli zemithambo-luvo. Umsebenzi wazo ophambili kukuthwala imiyalezo ekufuneka imizimba yethu ihambe ngayo. Kukho iintlobo ezimbini eziphambili zezi.
1. Ii-neurons ze-motor eziphezulu: Ezi zikwingqondo yakho. Zithumela imiyalezo esuka engqondweni yakho iye kwi-spinal cord yakho. Njengomphathi eofisini enkulu.
2. Ii-neurons ezisezantsi: Ezi zifumaneka kwi- spinal cord yakho. Zithatha imiyalezo evela engqondweni zize zixelele izihlunu zethu, "Kulungile, qala ukusebenza ngoku." Kufana nomphathi kwisebe.
Ngoku cinga ngento eyenzekayo xa unesifo se-motor neuron. Ezi seli zemithambo-luvo ziqala ukufa kancinci kancinci. Emva koko imiyalezo yombane evela ebuchotsheni ayikwazi ukufikelela kwimisipha ngokufanelekileyo. Ukudluliselwa kwemiyalezo kuyema phakathi. Ekuhambeni kwexesha, izihlunu ziba buthathaka kwaye ziqale ukuncipha kuba azinamsebenzi kuzo. Oogqirha bakubiza oku ngokuthi 'yi-atrophy', okanye ukuphelelwa yimisipha.
Xa oku kusenzeka, siyayeka ukulawula iintshukumo zethu. Kuba nzima ngakumbi ukuhamba, ukuthetha, ukuginya, kwaye ekugqibeleni nokuphefumla.
Into ebalulekileyo kukuba ayizizo zonke izifo ze-motor neuron ezifanayo. Ezinye zichaphazela kuphela ii-motor neuron eziphezulu, ezinye zichaphazela ezisezantsi, kwaye ezinye zichaphazela zombini.
Ziziphi iintlobo eziphambili zesifo se-motor neurone?
Ngoku makhe sijonge ezinye zezifo ezaziwayo nezingaziwa kangako kolu didi.
I-Amyotrophic Lateral Sclerosis (ALS)
Esi sesona sifo sixhaphakileyo se-motor neuron esibonwa ngabantu abadala.I-ALS ichaphazela zombini ii-neurons ze-motor eziphezulu nezisezantsi. Oku kubangela ukuba izihlunu ezilawula ukuhamba, ukuthetha, ukuhlafuna, ukuginya, kunye nokuphefumla zibe buthathaka kancinci kancinci kwaye ziphele. Ukuqina kwemisipha kunye nokushukuma nazo zinokwenzeka.
Amaxesha amaninzi, akukho sizathu sithile sifumanekayo se-ALS. Oogqirha bayibiza ngokuba 'yi-sporadic'. Oku kuthetha ukuba nabani na angayihlakulela. Nangona kunjalo, phakathi kwe-5% kunye ne-10% yamatyala adluliselwa kwilifa . Iimpawu zihlala zibonakala phakathi kweminyaka engama-40 kunye nama-60.
Isifo sePrimary Lateral Sclerosis (PLS)
I-PLS ifana ne-ALS, kodwa ichaphazela kuphela ii-neurons eziphezulu . Oku kwenza esi sifo sicothe kakhulu kune-ALS. Iimpawu ziquka ubuthathaka kunye nokuqina kweengalo nemilenze, ukuhamba kancinci, kunye nokulahlekelwa kukulinganisela kunye nokulungelelanisa. Intetho isenokucotha kwaye ingacaci. Ayinzima njenge-ALS, kwaye ayibulali abantu.
I-Progressive Bulbar Palsy (PBP)
Olu luhlobo lwe-ALS. Abantu abaninzi abane-PBP ekugqibeleni baba ne-ALS, echaphazela ii-motor neurons kwi-brain stem, ekwisiseko sobuchopho.
Ezi neurons ezikwisiqu sobuchopho zisinceda sihlafune, siginye, size sithethe. Ngoko ke xa i-PBP ikhula, amagama ayadideka, ukutya kuba nzima ukuginya, kwaye kuba nzima ukulawula iimvakalelo . Ungahleka ngequbuliso okanye ukhale ngaphandle kwesizathu.
Ukuwohloka kweMisipha okuqhubekekayo
Olu hlobo aluxhaphakanga njenge-ALS okanye i-PBP. Luchaphazela kakhulu ii-neurons ezisezantsi . Ubuthathaka budla ngokuqala ezandleni. Emva koko busasazeka kwezinye iindawo zomzimba. Izihlunu ziba buthathaka kwaye ukuqaqamba kunokubakho. Esi sifo ngamanye amaxesha sinokukhula sibe yi-ALS.
Ukuwohloka kweMisipha yoMgogodla (i-SMA)
Le yimeko yemfuza . I-SMA ibangelwa sisiphene kwi-gene ebizwa ngokuba yi-`SMN1`. Le gene ivelisa iproteni ekhusela ii-motor neurons. Xa loo proteni ingekho, ii-motor neurons ziyafa. Oku kuchaphazela ii-motor neurons ezisezantsi. I-SMA yahlulwe yaba ziintlobo ezahlukeneyo ngokuxhomekeke kwiminyaka apho iimpawu zibonakala khona.
| Uhlobo lwe-SMA | Ubudala bokuqala kweempawu | Iimpawu eziphambili |
|---|---|---|
| Uhlobo 1 (isifo sikaWerdnig-Hoffmann) | Malunga neenyanga ezi-6 | Umntwana akanakuhlala yedwa okanye aphakamise intloko yakhe ithe nkqo. Izihlunu zakhe zibuthathaka kakhulu. Unengxaki yokuginya nokuphefumla. |
| Uhlobo 2 | Phakathi kweenyanga ezi-6 nezi-12 | Umntwana angakwazi ukuhlala phantsi, kodwa akanakuma okanye ahambe yedwa. Usenokuba nobunzima bokuphefumla. |
| Uhlobo 3 (isifo saseKugelberg-Welander) | Phakathi kweminyaka emi-2 kunye ne-17 ubudala | Ichaphazela imisebenzi efana nokuhamba, ukubaleka, kunye nokunyuka izinyuko. Umqolo ugobile. |
| Uhlobo 4 | Emva kweminyaka engama-30 | Xa umntu emdala, ubuthathaka bemisipha, ukungcangcazela, ukuqaqamba, kunye nobunzima bokuphefumla bunokubakho. |
Isifo sikaKennedy
Esi sisifo esidluliselwa kwilifa. Kodwa into ekhethekileyo yaso kukuba esi sifo sichaphazela amadoda kuphela . Nokuba amabhinqa ane-gene ebangela esi sifo (abathwali baso), awabi nazimpawu. Nangona kunjalo, kukho amathuba angama-50% okuba umntwana oyinkwenkwe waloo mama azuze esi sifo njengelifa.
Amadoda anale meko anokufumana ukungcangcazela kwezandla, ukushukuma kwemisipha kunye nokuqaqamba, ubuthathaka ebusweni, ezingalweni nasemilenzeni. Anokuba nobunzima bokuginya nokuthetha.
Uphila njani nesifo esinje?
Ikamva lomntu ophila nesifo se-motor neurone lixhomekeke kuhlobo lwesifo. Njengoko sibonile, ezinye iintlobo zikhula kancinci kwaye azibi kakhulu kunezinye.
Okwangoku, akukho nyango lwesifo se-motor neurone, kodwa oko akuthethi ukuba masilahle ithemba.
Amayeza akhoyo ngoku kunye neendlela ezahlukeneyo zonyango (umz., unyango lomzimba, unyango lokuthetha) zinokulawula iimpawu kwaye ziphucule kakhulu umgangatho wobomi . Ke ngoko, ukuba wena okanye umntu omaziyo uhlangabezana nezi mpawu, eyona nto ingcono onokuyenza kukubona ugqirha ofanelekileyo ngokukhawuleza ukuze ufumane uxilongo oluchanekileyo kwaye uphuhlise isicwangciso sonyango.
Umyalezo Wokuya Ekhaya
- Isifo se-motor neurone (MND) liqela leemeko ezichaphazela iiseli zemithambo-luvo ezilawula iintshukumo zethu.
- Ubuthathaka bemisipha, ukuqina, ukungcangcazela, kunye nobunzima bokuthetha nokuginya zisenokuba zezona mpawu ziphambili.
- I-ALS lolona hlobo luqhelekileyo lwale nto, kodwa kukho nezinye iintlobo ezininzi.
- Ezinye izifo ze-motor neuron zinokufunyanwa njengelifa.
- Ukuba unayo nayiphi na kwezi mpawu, qiniseka ukuba ubonana nogqirha . Kuphephe ukuzixilonga.
- Nangona ezi zifo zingenakunyangwa ngokupheleleyo, kukho unyango olunokunceda ukulawula iimpawu kunye nokuphila ubomi obulungileyo.
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