Machado-Joseph chirwere (MJD), inozivikanwawo se spinocerebellar ataxia rudzi rwechitatu (SCA3), inotorwa neAatia disorder. Ataxia inogona kuchinja masimba ekutungamirira, zvichikonzera kusava nekuenzanisa uye kubatana. Kunyanya, MJD inokonzera kusava nekufambira mberi kwekubatana muzvanza nemakumbo. Vanhu vane chirwere chinowanzova nekufamba kwakasiyana, zvakafanana nekudhakwa kunodhakwa.
Vanogonawo kuva nekutambudzika kutaura uye kumedza.
MJD yave yakabatanidzwa kune chiremera chemajini mumutambo weAXX3 pane chromosome 14. Icho chinyorwa chinotyisa, chinoreva kuti mubereki mumwe chete anofanira kuva neine jeni rekuti mwana agone kuchinjwa. Kana iwe uine mamiriro acho, mwana wako ane 50 muzana yekana yekuwana nhaka. Mamiriro acho anowanzoonekwa kuvanhu vePutukezi kana Azorean desccent. Pachitsuwa cheFlorres muAzores, 1 mu 140 vanhu vanobatwa. Zvisinei, MJD inogona kuitika mune imwe dzinza reboka.
Zviratidzo
Kune zvitatu zvakasiyana zveMJD. Ndeupi rudzi rwaunofunga kubva apo zviratidzo zvinotanga uye kuoma kwezviratidzo izvozvo. Hezvino kutarisa pane zvakajairika zviratidzo uye zviratidzo zveizvi zvitatu:
| Tora | Zera Rokutanga | Chirwere Chisimba uye Kufambira mberi | Zviratidzo |
|---|---|---|---|
| Tora I (MKD-I) | Pakati pe10-30 wemakore | Chiratidzo chakasimba chinokurumidza kukurumidza |
|
| Rudzi rwechipiri (MJD-II) | Pakati pemakore makumi maviri nemakumi mashanu | Zviratidzo zvinowedzera kuwedzera zvishoma nezvishoma |
|
| Rudzi rwe III (MJD-III) | Pakati pemakore makumi mana nemakore makumi manomwe | Zviratidzo zvishoma nezvishoma zvinowedzera kupfuura nguva |
|
Vanhu vakawanda vane MJD vanewo zvirwere zvekuona zvakadai sekuonekwa kaviri (diplopia) uye kusakwanisa kudzora kufamba kweziso, pamwe nekudedera mumaoko avo uye nezvinetso nekuenzanisa nekubatana. Vamwe vanogona kukura maziso kana kuti matambudziko ekudhonza.
Maitiro aJe Anozivikanwa
MJD inorwara ichibva pane zviratidzo zvauri kuona.
Sezvo chirwere ichi chiri nhaka , zvinokosha kutarisa mumhuri yako nhoroondo. Kana hama dzine zviratidzo zveMJD, bvunza kana zviratidzo zvavo zvatanga uye kuti zvakakurumidza sei kukura. A definitive diagnosis inogona kungobva kuine genetic test, iyo inotarisa kukanganisa mu chromosome yako yechi14. Kune avo vanogara nekutanga kwekutanga MJD, tarisiro yehupenyu inogona kuva shoma semakore ekuma30. Vaya vane MJD dzakanaka kana rudzi rwekupedzisira-rwemazuva ose, kazhinji vane tarisiro yeupenyu hunowanzoitika.
Mishonga
Parizvino, hapana mushonga weMachado-Joseph. Isu hatina nzira yekudzivisa zviratidzo zvayo kuti zvifambire mberi. Kune, zvisinei, mishonga inogona kubatsira kubvisa zviratidzo. Baclofen (Lioresal) kana kuti botulinum toxin (Botox) inogona kubatsira kuderedza kusvibiswa kwemisumbu uye dystonia. Iroodopa therapy, chirwere chinoshandiswa kuvanhu vane chirwere chePasinson, chinogona kubatsira kuderedza kuoma uye kunonoka. Kurapa kwepanyama uye midziyo yekubatsira zvinogona kubatsira vanhu vane kufamba uye mabasa ezuva nezuva. Kune zviratidzo zvinoratidzwa, magirazi ejeri anogona kubatsira kuderedza kupfava kana kuonekwa kaviri.
Sources:
"Machado-Joseph Fact Sheet." Matambudziko. 16 Apr 2014. National Institute of Neurological Disorders uye Stroke.
"Machado-Joseph Disease." Index of Rare Diseases. National Organisation for Rare Disorders. 15 Feb, 2007.