Sclerosis Ya Mbere Ya Ubwonko (Pls)

Sclerosis ya mbere ibura imbarutso (PLS) ni ubwoko bw'indwara y'uturemangingo two mu mikaya. Indwara y'uturemangingo two mu mikaya igira ingaruka ku turemangingo tw'imikaya mu bwonko bugenzura imitsi. Muri PLS, kwangirika kw'uturemangingo tw'imikaya bituma imitsi igenzura amaguru, amaboko n'ururimi ibura imbaraga.

Ibishimisho bikunze gutangira n'ikibazo cyo kubura umutekano. Abantu bafite PLS bashobora kugenda buhoro kandi bagasa n'abadashoboye. Uko igihe gihita, ibimenyetso bikuramo kugira ibibazo mu biganza n'amaboko, bikurikirwa n'ibibazo byo kuruma, kwishima no kuvuga. Gake, iyi ndwara ishobora gutangira n'ibibazo byo kuvuga no kwishima.

Iyi ndwara idasanzwe ishobora kuza mu myaka yose, ariko ikunze kuba hagati y'imyaka 40 na 60. PLS igaragara cyane mu bagabo kuruta mu bagore. Gake, PLS ishobora gutangira mu buto. Uyu mubare uzirikanwa nka sclerosis ya mbere ibura imbarutso y'abana. PLS y'abana iterwa n'impinduka mu gene iherwa ku babyeyi bombi ku mwana.

PLS ikunze kwitiranywa n'indi ndwara y'uturemangingo two mu mikaya, izwi cyane, yitwa sclerosis ya amyotrophic lateral (ALS). Nubwo ishobora kuba ifitanye isano na ALS, PLS irushaho kuba mbi kurusha ALS. Mu bantu benshi, PLS ntiyahitana.

Ibimenyetso bya sclerosis laterale primaire bisanzwe bimamara imyaka kugira ngo bigaragara kandi bikomeze kuba bibi. Ibimenyetso muri rusange bitangira mu birenge. Gake, PLS itangira n'intege nke mu rurimi cyangwa mu ntoki. Hanyuma intege nke iragenda yinjira mu mugongo yerekeza mu birenge. Ariko, ibimenyetso bihinduka ukurikije umuntu ku wundi. Ibimenyetso bishobora kuba birimo:

• Ubugufi, intege nke n'amavunja y'imitsi mu birenge. Ibi bizwi nka spasticity. Gake, spasticity itangira mu kirenge kimwe gusa ikagenda yerekeza mu maboko, mu ntoki, mu rurimi no mu munwa.
• Kubuza umuvuduko w'imigirire.
• Kugwa, kuba umunyerere kandi ugira ikibazo cyo kubura umutekano.
• Ubugufi bw'intoki.
• Ijwi ridafite imbaraga, kimwe no kugira imvugo ipfubye, idasobanutse no gucika amavangingo.
• Kugira ikibazo cyo kuruma no kwishima.
• Rimwe na rimwe, impinduka zikabije, zihutirwa kandi zikomeye mu mimerere y'amarangamutima.
• Gake, ibibazo byo guhumeka n'ibibazo by'umwimerere mu gihe cy'indwara.

Fata rendez-vous kugira ngo ubone umuhanga mu buvuzi niba ufite ibibazo bidashira byo kugira ubugufi cyangwa intege nke mu maguru yawe cyangwa mu maboko. Nanone reba umuhanga mu buvuzi niba ugira ikibazo cyo kwishima cyangwa kuvugira.

Jyana umwana wawe kwa muganga niba umwana wawe arwaye spasms z'imitsi cyangwa asa n'aho atakaza umutekano kenshi ugereranije n'ibisanzwe.

Mu sclerosis y'uruhande rw'ibanze, uturemangingo tw'imbere mu bwonko tugenzura imiyoborere y'umubiri buce buce bigenda bisenya kandi bigahagarara gukora. Uturingo tw'imbere tw'imbere twitwa upper motor neurons.

Kubera ibyo, imitsi ntishobora gukora uturemangingo tw'imikorere mu kibero, bigenzura imikaya yigenga. Iyi ngaruka iterwa n'ikibazo cyo kugenda. Abantu bafite PLS bashobora kugira ibibazo byo kubura umubano, intege nke, kugenda buhoro kandi kugira ubushishozi buke. PLS kandi ishobora gutera ibibazo byo kuvuga no kwishima.

Intandaro ya PLS itangira mu bwana ntiyamenyekanye. Mu bantu benshi, indwara ntiyagenewe. Ntibiramenyekana impamvu cyangwa ukuntu itangira.

PLS y'abana iterwa n'impinduka mu gene yitwa ALS2.

Abashakashatsi ntibamenya ukuntu iyi gene iterwa n'indwara. Ariko bazi ko gene ya ALS2 itanga amabwiriza yo gukora protein yitwa alsin. Alsin iboneka mu uturemangingo tw'imikorere y'imitsi. Iyo amabwiriza ahindutse mu muntu ufite PLS y'abana, protein alsin igira akazi kayo kandi ntikora neza. Ibi bigira ingaruka ku mikorere y'imitsi.

PLS y'abana ni indwara igenewe mu buryo bw'umuryango bw'abana. Ibi bisobanura ko ababyeyi bombi bagomba kuba abatwaye iyi gene kugira bayimugezeho umwana wabo. Ariko ntibikeneye kugira iyi ndwara ubwabo.

Nta bimenyetso by'ibyago by'ibidukikije byashyizweho ku bw'indwara ya sclerosis ya mbere.

Indwara ya sclerosis ya mbere itera uburibwe ishobora gutinda imyaka 20 kugira ngo itere imbere kandi irusheho kuba mbi. Ibimenyetso bihinduka ukurikije umuntu ku wundi. Bamwe bakomeza kugenda, ariko abandi amaherezo bagomba gukoresha amaperekezo cyangwa ibindi bikoresho bifasha.

Kuri benshi, indwara ya PLS itangira mu bukure ntibyumvikana ko igabanya igihe cyo kubaho. Ariko ishobora kugira ingaruka buhoro buhoro ku mibereho yawe uko imitsi myinshi igenda ikomeretsa. Imitsi idakomeye ishobora gutuma ugura, ibyo bikaba byatera imvune. Ibibazo byo kuruma no kwishima bishobora gutera imirire mibi. Gukora ibikorwa bya buri munsi, nko koga no kwambara, bishobora kugorana.

Abantu barwaye PLS bashobora kugira ibibazo byo gutekereza, bizwi nka cognitive decline. Cyangwa bashobora kugira impinduka mu myitwarire yabo. Kuri bamwe, ibimenyetso byabo bihuza nibimenyetso bya frontotemporal dementia.

Nta kizami kimwe cyemeza burundu uburwayi bwa sclerosis ya mbere itera uburibwe (PLS). PLS ishobora kugaragaza ibimenyetso bisa n’iby’izindi ndwara zifata imitsi y’ubwonko nka sclerosis nyinshi (multiple sclerosis) na ALS. Kubw’ibyo, ushobora gukenera ibizami byinshi kugira ngo habeho gukuraho izindi ndwara.

Umuhanga mu buvuzi bw’indwara zifata imitsi y’ubwonko azabanza kwandika neza amateka y’uburwayi bwawe n’ay’umuryango wawe, hanyuma akore isuzuma ry’imitsi y’ubwonko. Hanyuma ushobora gukora ibizami bikurikira:

• Isuzuma ry’amaraso. Ibizami by’amaraso bireba niba hari indwara zandura cyangwa ibindi bishobora gutera intege nke z’imitsi.
• MRI. MRI cyangwa ibindi bizami byifashisha amashusho by’ubwonko bwawe cyangwa umugongo bishobora kugaragaza ibimenyetso by’uko uturemangingo tw’imitsi twangiritse. MRI kandi ishobora kugaragaza ibindi bintu bitera ibimenyetso, nko guhindagurika kw’imiterere y’umubiri, sclerosis nyinshi cyangwa uburibwe bw’umugongo.
• Electromyogram (EMG). Iki kizami kihera ku gusuzuma imikorere y’amashanyarazi y’imitsi iyo ikoranye n’iyo iruhukiye. Iki kizami kinapima uruhare rw’uturemangingo tw’imitsi yo hasi. Ibi bishobora gufasha gutandukanya PLS ⸺ indwara ifata uturemangingo tw’imitsi yo hejuru ⸺ na ALS, ifata uturemangingo tw’imitsi yo hejuru n’ayo hasi.

Mu gihe cy’EMG, muganga wawe azashyira electrode y’igishishwa mu ruhu rwawe mu mitsi itandukanye.

• Isuzuma ry’uburyo bwo gutwara ubutumwa bw’imiterere y’imitsi. Iki kizami gishobora kumenya niba ufite ikibazo cy’imitsi. Ingano nke y’umuriro w’amashanyarazi ipimira ubushobozi bw’imitsi yawe bwo kohereza ubutumwa ku mitsi mu bice bitandukanye by’umubiri wawe.
• Gusukura umuti mu mugongo, bizwi kandi nko gusukura umuti mu muhogo w’umugongo. Gusukura umuti mu mugongo bishobora gufasha gukuraho sclerosis nyinshi, indwara zandura n’ibindi bibazo. Igishishwa gito, cyuzuye umwobo gishyirwa mu muhogo w’umugongo gikuramo ibice bito by’amazi yo mu muhogo w’umugongo. Aya mazi akikije ubwonko bwawe n’umugongo wawe. Ibice byakuruwe hanyuma bigasesengurwa muri laboratwari.

Electromyogram (EMG). Iki kizami kihera ku gusuzuma imikorere y’amashanyarazi y’imitsi iyo ikoranye n’iyo iruhukiye. Iki kizami kinapima uruhare rw’uturemangingo tw’imitsi yo hasi. Ibi bishobora gufasha gutandukanya PLS ⸺ indwara ifata uturemangingo tw’imitsi yo hejuru ⸺ na ALS, ifata uturemangingo tw’imitsi yo hejuru n’ayo hasi.

Mu gihe cy’EMG, muganga wawe azashyira electrode y’igishishwa mu ruhu rwawe mu mitsi itandukanye.

Rimwe na rimwe bisaba imyaka 3 kugeza kuri 4 kugira ngo habeho kuvura. Ibi biterwa nuko ALS yo mu ntangiriro ishobora kumera nka PLS kugeza ibindi bimenyetso bigaragaye nyuma y’imyaka mike. Ushobora gusabwa kugaruka kugira ngo ukore EMG zisubiramo mu myaka 3 kugeza kuri 4 mbere y’uko uburwayi bwa PLS bwemezwa.

Isuzuma rya gene zishobora gukorwa iyo hakekwa PLS y’abakiri bato. Inama ku bijyanye n’indwara zishobora kuzanwa na gene zishobora kandi kugirwa inama.