Sclerosis Lateral Primary (Pls)

Primary lateral sclerosis (PLS) imhando yechirwere chemotor neuron. Chirwere chemotor neuron chinokanganisa masero etsinga ari muuropi anodzora kufamba. MuPLS, kuparara kwemasero etsinga kunokonzera kushaya simba mumhasuru dzinodzora makumbo, maoko nerurimi. Zviratidzo zvinowanzotanga nekutadza kudzikama. Vanhu vane PLS vanogona kufamba zvishoma nezvishoma uye vachitadza kufamba zvakanaka. Nekufamba kwenguva, zviratidzo zvinoshanduka kusanganisira kutadza kushandisa maoko nemaoko, zvichiteverwa nematambudziko ekutsenga, kumedza uye kutaura. Zvishoma, chirwere ichi chinogona kutanga nematambudziko ekutaura nekumedza. Chirwere chisingawanzoitiki ichi chinogona kutanga pazera ripi neripi, asi chinowanzoitika pakati pemakore 40 kusvika 60. PLS inowanzoitika kuvarume kupfuura kuvakadzi. Zvisingawanzoitika, PLS inogona kutanga muhuduku hwepwere. Rudzi urwu rwunozivikanwa sejuvenile primary lateral sclerosis. Juvenile PLS inokonzerwa nekushanduka kwejeni rinopihwa kubva kuvabereki vese kumwana. PLS inowanzokanganiswa nechimwe chirwere chemotor neuron chinonyanya kuzivikanwa chinonzi amyotrophic lateral sclerosis (ALS). Kunyangwe zvichida chine hukama neALS, PLS inokura zvishoma nezvishoma kupfuura ALS. Muvanhu vazhinji, PLS haisi inouraya.

Zviratidzo zve primary lateral sclerosis zvinowanzo kutora makore kuti zvibude uye zviwedzere kuipa. Zviratidzo kazhinji zvinovamba mumakumbo. Zvisingawanzoitika, PLS inotanga nekushaya simba mururimi kana maoko. Ipapo kushaya simba kunofamba zvishoma nezvishoma pasi pechigunwe chemuviri kuenda kumakumbo. Zvisinei, zviratidzo zvinosiyana kubva kumunhu kuenda kune mumwe munhu. Zviratidzo zvinogona kusanganisira:

• Kuomarara, kushaya simba uye kuita ma spasms emhasuru mumakumbo. Izvi zvinozivikanwa se spasticity. Zvisingawanzoitika, spasticity inotanga mumukumbo mumwe chete woenda kumaoko, maoko, rurimi nemuromo.
• Kufamba kwakaderera.
• Kukakavadzana, kuva munhu asinganyatsoziva kufamba uye kuva nematambudziko ekururamisa muviri.
• Kushaya hunyanzvi mumaoko.
• Inzwi rakapfava, pamwe nekukurumidza kutaura kwakaderera uye kuyerera kwemashure.
• Matambudziko ekukwesha uye kukwenya.
• Dzimwe nguva, kuchinja-chinja kwakawanda, kukurumidza uye kwakasimba kwemanzwiro.
• Zvisingawanzoitika, matambudziko ekufema nematambudziko ebladder pakupera kwechirwere.

Mirawo dokita kana iwe uine matambudziko asingaperi ekushomeka kana kushaya simba mumakumbo ako kana maoko. Onawo nyanzvi yehutano kana iwe uine dambudziko rekumera kana kutaura.

Enda nachiremba mwana wako kana mwana wako akaita kukuvara kwemhasuru kana achiratidza kurasikirwa nekusimba kazhinji kupfuura zvakajairwa.

Mukushaya kwemasero ane chekuita nekuita kwemuviri (primary lateral sclerosis), masero ane chekuita nekuita kwemuviri ari muuropi anodzora kufamba anoputsika zvishoma nezvishoma uye anorega kushanda. Aya masero ane chekuita nekuita kwemuviri anonzi upper motor neurons.

Somugumisiro, maturakisi haakwanisi kubatanidza motor neurons ari muchikamu chemuviri (spinal cord), anodzora magaro anofamba. Kukanganisika uku kunokonzera matambudziko ekufamba. Vanhu vane PLS vangave nematambudziko ekururamisira, kushaya simba, kufamba kuri kunonoka uye kusakwanisa kuita zvinhu zvakanaka. PLS inogona zvakare kukonzera matambudziko ekukurukura uye kudya.

Chikonzero chePLS chinotanga pakuva mukuru hachizivikanwi. Kuvanhu vazhinji, chirwere ichi hachina kuwanikwa kubva kuvabereki. Hazvizivikanwi kuti nei kana sei chinotanga.

Juvenile PLS inokonzerwa nekuchinja kwegene rinonzi ALS2.

Vatsvakurudzi havazive kuti gene iri rinokonzera sei chirwere ichi. Asi vanoziva kuti gene re ALS2 rinopa mirairo yekugadzira protein inonzi alsin. Alsin iri mumasero e motor neuron. Kana mirairo ichichinjwa mumunhu ane juvenile PLS, protein ye alsin inoshanduka uye haishande zvakanaka. Izvi zvinoita kuti basa remagaro risashande zvakanaka.

Juvenile PLS chirwere chinowanikwa kubva kuvabereki, umo vabereki vese vanofanirwa kuva vanotakura gene kuti vaipfuurire kumwana wavo. Zvisinei, havafanirwe kuva nechirwere ichocho ivo pachavo.

Hapana zvinhu zvakagadzwa zvine ngozi zvakatikomberedza zve primary lateral sclerosis.

Zvinogona kutora makore anenge makumi maviri kuti primary lateral sclerosis ifambe zvishoma nezvishoma uye iwedzere kuipa. Zviratidzo zvinosiyana kubva kumunhu kuenda kune mumwe munhu. Vamwe vanhu vanoramba vachifamba, asi vamwe pakupedzisira vanoda kushandisa michina yekufambisa kana mamwe maturusi anobatsira.

Kune vanhu vakawanda, PLS inotanga pakuva mukuru haifungidzirwi kuti inodzikisira nguva yekurarama. Asi inogona zvishoma nezvishoma kukanganisa mhando yehupenyu hwako sezvo minyoro yakawanda ichikanganisika. Minyoro isina simba inogona kukonzera kuti uwirike, izvo zvinogona kukonzera kukuvara. Matambudziko ekukwesha nekumera anogona kukonzera kushomeka kwezvinovaka muviri. Kuita mabasa ezuva nezuva, akadai sekushambira nekukweshera, kunogona kuva kwakaoma.

Vanhu vane PLS vanogona kuwana matambudziko nepfungwa dzavo, anozivikanwa sekuora kwepfungwa. Kana kuti vangave vane shanduko mumaitiro avo. Kune vamwe vanhu, zviratidzo zvavo zvinowirirana nezviratidzo zve frontotemporal dementia.

Hapana bvunzo imwe chete inosimbisa chirwere che primary lateral sclerosis (PLS). PLS inogona kuva nezviratidzo zvakafanana nezvimwe zvirwere zvehuropi senge multiple sclerosis neALS. Nechikonzero ichi, ungada bvunzo dzakawanda kuti ubvise zvimwe zvirwere.

Munhu ane hutano anotanga kunyora nhoroondo yako yehutano nemhuri yako zvakanyatsonaka uye anoita bvunzo yehuropi. Ipapo unogona kuita bvunzo idzi:

• Kuongororwa kweropa. Kuongororwa kweropa kunotarisa hutachiona kana zvimwe zvinokonzera kushaya simba kwemhasuru.
• MRI. MRI kana mamwe bvunzo dzepfungwa dzako kana musana dzinogona kuratidza zviratidzo zvekuti masero aneta akaputsika. MRI inogonawo kuratidza zvimwe zvinokonzera zviratidzo, senge matambudziko emuviri, multiple sclerosis kana zvirwere zvemusana.
• Electromyogram (EMG). Bvunzo iyi inotarisa simba remagetsi emhasuru kana dzichidzoka uye kana dziri kuzorora. Bvunzo iyi inoyeresawo kuita kwe lower motor neurons. Izvi zvinogona kubatsira kusiyanisa pakati pePLS ⸺ chirwere che upper motor neuron ⸺ neALS, inokanganisa upper ne lower motor neurons.

Panguva yeEMG, chiremba wako anopinda needle electrode kuburikidza neganda rako kuenda kumhasuru dzakasiyana.

• Kudzidza kwekuita kwematumbu. Bvunzo iyi inogona kuona kana uine kukuvara kwetumbu. Huwandu hwakaderera hwemasimba emagetsi hunoyeresa kugona kwetumbu dzako kutumira zviratidzo kumhasuru munzvimbo dzakasiyana dzemuviri wako.
• Lumbar puncture, inozivikanwawo se spinal tap. Spinal tap inogona kubatsira kubvisa multiple sclerosis, hutachiona nezvimwe zvirwere. Needle yakatetepa, isina chinhu inopinda mumusana wako inobvisa zvidimbu zvidiki zve cerebrospinal fluid. Iri ndiro mvura rinopoteredza pfungwa dzako nemusana wako. Zvidimbu zvinoongororwa murabhoritari.

Electromyogram (EMG). Bvunzo iyi inotarisa simba remagetsi emhasuru kana dzichidzoka uye kana dziri kuzorora. Bvunzo iyi inoyeresawo kuita kwe lower motor neurons. Izvi zvinogona kubatsira kusiyanisa pakati pePLS ⸺ chirwere che upper motor neuron ⸺ neALS, inokanganisa upper ne lower motor neurons.

Panguva yeEMG, chiremba wako anopinda needle electrode kuburikidza neganda rako kuenda kumhasuru dzakasiyana.

Dzimwe nguva zvinotora makore matatu kusvika mana usati wagona kuziva chirwere. Izvi ndizvo nekuti ALS yekutanga inogona kutaridzika sePLS kusvika zvimwe zviratidzo zvibude makore mashoma gare gare. Ungakumbirwa kudzoka kuti uite EMGs dzakawanda kwemakore matatu kusvika mana usati wasimbiswa chirwere chePLS.

Kuongororwa kwemajini kunogona kuitwa kana PLS yevechidiki ichifungidzirwa. Zano remajini rinogonawo kukurudzirwa.