Wake wazibuza ukuthi kulula kangakanani ukwenza lezi zinto lapho sihamba, sikhuluma nomngane, noma sihlafuna ukudla kwethu? Ngemuva kwayo yonke intshukumo, isenzo ngasinye, kuneqembu lezithunywa ezibaluleke kakhulu emzimbeni wethu. Ziyilokho esikubiza ngokuthi ama-motor neurons. Zifana neqembu lezinceku ezivela ebuchosheni bethu ezitshela imisipha yethu ukuthi "yenzeni lokhu."
Kodwa, njengezinye izingxenye zomzimba wethu, lezi zithunywa nazo zingalimala. Yilapho-ke siba nezimo ezibizwa ngokuthi 'izifo ze-motor neuron'. Cishe uke wezwa nge-ALS. Leso ngesinye sezifo eziyinhloko kulesi sigaba. Kodwa kunezinye izinhlobo eziningana zezifo ze-motor neuron esingaziqondi kangako. Ngakho-ke namuhla, ake sixoxe ngazo ngendlela elula.
Bangobani laba 'bamangqamuzana ashukumisayo'?
Kalula nje, ama-motor neurons awuhlobo lweseli lemizwa. Umsebenzi wawo oyinhloko ukuthwala imiyalezo okudingeka imizimba yethu ihambe ngayo. Kunezinhlobo ezimbili eziyinhloko zalezi.
1. Ama-neuron e-motor angaphezulu: Lawa asebuchosheni bakho. Athumela imiyalezo esuka ebuchosheni bakho iye emgogodleni wakho. Njengomphathi ehhovisi elikhulu.
2. Ama-neuron aphansi: Lawa atholakala emgogodleni wakho. Athatha imiyalezo ebuchosheni bese etshela imisipha yethu ukuthi, "Kulungile, qala ukusebenza manje." Kufana nomphathi egatsheni.
Manje cabanga ngalokho okwenzekayo uma unesifo se-motor neuron. Lawa maseli ezinzwa aqala ukufa kancane kancane. Bese imiyalezo kagesi evela ebuchosheni ingakwazi ukufika emisipheni kahle. Ukudluliselwa komyalezo kuyama phakathi nendawo. Ngokuhamba kwesikhathi, imisipha iba buthakathaka futhi iqala ukushwabana ngoba ayinamsebenzi kuyo. Odokotela bakubiza lokhu ngokuthi 'ukuwohloka', noma ukulahlekelwa yimisipha.
Uma lokhu kwenzeka, silahlekelwa ukulawula ukunyakaza kwethu. Kuba nzima kakhulu ukuhamba, ukukhuluma, ukugwinya, futhi ekugcineni ngisho nokuphefumula.
Into ebalulekile ukuthi akuzona zonke izifo ze-motor neuron ezifanayo. Ezinye zithinta ama-motor neuron aphezulu kuphela, ezinye zithinta aphansi, kanti ezinye zithinta kokubili.
Yiziphi izinhlobo eziyinhloko zesifo se-motor neurone?
Manje ake sibheke ezinye zezifo ezaziwayo nezingaziwa kangako kulesi sigaba.
I-Amyotrophic Lateral Sclerosis (ALS)
Lesi yisifo esivame kakhulu se-motor neuron esibonakala kubantu abadala.I-ALS ithinta ama-neuron okunyakaza aphezulu naphansi. Lokhu kubangela ukuthi imisipha elawula ukuhamba, ukukhuluma, ukuhlafuna, ukugwinya, nokuphefumula ibe buthaka kancane kancane futhi iphele. Ukuqina kwemisipha nokunyakazisa kungase kwenzeke.
Esikhathini esiningi, akukho mbangela ethile engatholakala ye-ALS. Odokotela bayibiza ngokuthi 'iyenzeka ngezikhathi ezithile'. Lokhu kusho ukuthi noma ubani angaba nayo. Kodwa-ke, phakathi kuka-5% no-10% wamacala adluliselwa ngofuzo . Izimpawu zivame ukuvela phakathi kweminyaka engu-40 no-60.
I-Primary Lateral Sclerosis (PLS)
I-PLS ifana ne-ALS, kodwa ithinta kuphela ama-neuron aphezulu . Lokhu kwenza lesi sifo sihambe kancane kakhulu kune-ALS. Izimpawu zifaka phakathi ubuthakathaka nokuqina kwezingalo nemilenze, ukuhamba kancane, kanye nokulahlekelwa ibhalansi nokuxhumana. Inkulumo ingase ihambe kancane futhi ingacaci. Ayinzima njenge-ALS, futhi ayibulali abantu.
I-Progressive Bulbar Palsy (PBP)
Empeleni lolu uhlobo lwe-ALS. Abantu abaningi abane-PBP bagcina sebethola i-ALS, ethinta ama-motor neurons esiqwini sobuchopho, esitholakala phansi kobuchopho.
Lawa ma-neuron esiqwini sobuchopho asisiza ukuhlafuna, ukugwinya, nokukhuluma. Ngakho-ke lapho i-PBP ikhula, amagama ayaphela, ukudla kuba nzima ukugwinya, futhi kuba nzima ukulawula imizwa . Ungase uhleke noma ukhale ngokuzumayo ngaphandle kwesizathu.
Ukuwohloka Kwemisipha Okuqhubekayo
Lolu hlobo aluvamile njenge-ALS noma i-PBP. Luthinta kakhulu ama-neuron angaphansi kwe-motor . Ubuthakathaka buvame ukuqala ezandleni. Bese busakazekela kwezinye izingxenye zomzimba. Imisipha iba buthakathaka futhi ukuqaqamba kungavela. Lesi sifo singakhula sibe yi-ALS ngezinye izikhathi.
Ukuwohloka Kwemisipha Yomgogodla (SMA)
Lesi yisimo sofuzo . I-SMA ibangelwa yiphutha ku-gene ebizwa ngokuthi `SMN1`. Le gene ikhiqiza iphrotheni evikela ama-motor neuron. Uma leyo phrotheni ingekho, ama-motor neuron ayafa. Lokhu kuthinta ama-motor neuron aphansi. I-SMA ihlukaniswe ngezinhlobo eziningana kuye ngokuthi izimpawu zivela nini.
| Uhlobo lwe-SMA | Ubudala bokuqala kwezimpawu | Izici eziyinhloko |
|---|---|---|
| Uhlobo 1 (isifo sikaWerdnig-Hoffmann) | Cishe ezinyangeni eziyisithupha | Ingane ayikwazi ukuhlala yodwa noma ukuphakamisa ikhanda layo liqonde. Imisipha yayo ibuthakathaka kakhulu. Inenkinga yokugwinya nokuphefumula. |
| Uhlobo 2 | Phakathi kwezinyanga eziyi-6 neziyi-12 | Ingane ingakwazi ukuhlala, kodwa ayikwazi ukuma noma ukuhamba yodwa. Ingase ibe nobunzima bokuphefumula. |
| Uhlobo 3 (Kugelberg-Welander disease) | Phakathi kweminyaka engu-2 nengu-17 ubudala | Kuthinta imisebenzi efana nokuhamba, ukugijima, kanye nokukhwela izitebhisi. Kungase kube nokugoba komhlane. |
| Uhlobo 4 | Ngemva kweminyaka engama-30 | Lapho umuntu esemdala, ubuthakathaka bemisipha, ukuthuthumela, ukuqaqamba, kanye nobunzima bokuphefumula kungase kwenzeke. |
Isifo SikaKennedy
Lesi futhi yisifo esidluliselwa ngofuzo. Kodwa okukhethekile kwalesi sifo ukuthi lesi sifo sithinta abesilisa kuphela . Ngisho noma abesifazane benesakhi sofuzo esibangela lesi sifo (abathwali baso), ababi nezimpawu. Kodwa-ke, kunethuba elingu-50% lokuthi ingane yesilisa yalowo mama izozuza lesi sifo njengefa.
Amadoda analesi simo angase azizwe eqhaqhazela izandla, eqhaqhazela imisipha futhi eqaqamba, ebuthakathaka ebusweni, ezingalweni nasemilenzeni. Angase abe nobunzima bokugwinya nokukhuluma.
Uphila kanjani nesifo esinjengalesi?
Ikusasa lomuntu ophila nesifo se-motor neurone lincike ohlotsheni lwesifo. Njengoba sesibonile, ezinye izinhlobo zikhula kancane futhi azibi kakhulu kunezinye.
Okwamanje, akukho ukwelashwa okutholiwe okungakwelapha ngokuphelele isifo sezinzwa zomzimba, kodwa lokho akusho ukuthi kufanele silahlekelwe yithemba.
Imithi yamanje kanye nezindlela ezahlukene zokwelapha (isb., ukwelashwa ngokomzimba, ukwelashwa ngenkulumo) kungalawula izimpawu futhi kuthuthukise kakhulu ikhwalithi yokuphila . Ngakho-ke, uma wena noma othile omaziyo ebhekene nalezi zimpawu, into engcono kakhulu ongayenza ukubona udokotela ofanelekayo ngokushesha okukhulu ukuze uthole ukuxilongwa okunembile futhi uthuthukise uhlelo lokwelashwa.
Umlayezo Wokuya Nawe Ekhaya
- Isifo se-motor neurone (MND) yiqembu lezimo ezithinta amangqamuzana ezinzwa alawula ukunyakaza kwethu.
- Ubuthakathaka bemisipha, ukuqina, ukuthuthumela, kanye nobunzima bokukhuluma nokugwinya kungaba yizimpawu eziyinhloko.
- I-ALS uhlobo oluvame kakhulu lwalokhu, kodwa kunezinye izinhlobo eziningi.
- Ezinye izifo ze-motor neuron zingatholakala njengefa.
- Uma unezinye zalezi zimpawu, qiniseka ukuthi ubona udokotela . Gwema ukuzihlola.
- Nakuba lezi zifo zingelapheki ngokuphelele, kunezindlela zokwelapha ezingasiza ekulawuleni izimpawu futhi ziphile impilo enhle.
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