I-cerebellar ataxia

I-cerebellar ataxia

Kwenzenjani ?

I-cerebellar ataxia ibangelwa isifo noma ukulimala ku-cerebellum, etholakala ebuchosheni. Lesi sifo sibonakala ngokungahambisani nokunyakaza kwemisipha. (1)

I-Ataxia yigama elihlanganisa ndawonye inani lezinkinga ezithinta ukusebenzisana, ibhalansi nolimi.

Zonke izingxenye zomzimba zingathinteka yilesi sifo, kodwa abantu abane-ataxia ngokuvamile banokukhubazeka ku:

- ukulinganisa nokuhamba;

– ulimi;

- ukugwinya;

- ekwenzeni imisebenzi edinga izinga elithile lokulawula, njengokubhala noma ukudla;

– umbono.

Kunezinhlobo ezahlukene ze-ataxia ezibonakala ngezimpawu ezihlukahlukene kanye nobukhulu: (2)

I-ataxia etholakele iwuhlobo oluhambisana nokukhula kwezimpawu ngenxa yokulimala, unhlangothi, i-sclerosis, isimila ebuchosheni, ukuntuleka kokudla okunomsoco noma ezinye izinkinga ezilimaza ubuchopho nohlelo lwezinzwa;

- i-ataxia yofuzo, ihambisana nefomu lapho izimpawu zikhula kancane kancane (ngaphezu kweminyaka eminingana). Leli fomu liyimbangela yokungajwayelekile kofuzo kubazali. Leli fomu libizwa nangokuthi i-ataxia ka-Friedreich.

- idiopathic ataxia ngokuqala sekwephuzile kwe-cerebellar ataxia, lapho ubuchopho buthinteka kancane kancane ngokuhamba kwesikhathi ngenxa yezizathu ezivame ukungaziwa.

Ngokuphathelene ne-autosomal recessive cerebellar ataxia, iyingxenye yeqembu lezifo zemizwa ezingavamile ezithinta isimiso sezinzwa esimaphakathi nese-peripheral. Lawa ma-pathologies angathinta nezinye izitho. Umsuka walolu hlobo lwesifo ifa le-autosomal recessive. Noma ukudluliselwa kofuzo olushintshile lwentshisekelo oluvela kubazali. Ukuba khona kwekhophi eyodwa kuphela yofuzo kuyadingeka ekuthuthukiseni lesi sifo.

Ukuthuthukiswa kwalesi sifo kuvame ukuvela ngaphambi kweminyaka engama-20.

Kuyisifo esiyivelakancane, ukusabalala kwaso (inombolo yezigameko kubantu esinikeziwe ngesikhathi esinikeziwe) kuphakathi kwecala eli-1 kanye nelesi-4 kubantu abayi-100. (000)

Izimpawu

Izimpawu ezihambisana ne-cerebellar ataxia ziyi-neurological and mechanical.

I-Cerebellar ataxia ngokuvamile ithinta isiqu: kusukela entanyeni kuya ezinqulwini, kodwa futhi izingalo nemilenze.

Izimpawu ezijwayelekile ze-cerebellar ataxia zifaka: (1)

- Ukumiswa kwenkulumo okungaqondakali (i-dysarthria): ukuphazamiseka kwamalunga;

- i-nystagmus: ukunyakaza kwamehlo okuphindaphindiwe;

- ukunyakaza kwamehlo okungahlangene;

– ukuhambahamba okungazinzile.

Imvelaphi yalesi sifo

I-Cerebellar ataxia ihlasela kakhulu izingane ezincane ezineminyaka ephakathi kwe-3.

Lesi sifo singakhula ngemva kwamasonto ambalwa ngemva kokutheleleka ngegciwane. Lezi zifo ezibangelwa amagciwane okukhulunywa ngazo zihlanganisa: inkukhu, ukutheleleka ngegciwane le-Eptein-Barr, isifo sika-Coxsackie noma ukutheleleka nge-echovirus.

Eminye imvelaphi ingase ihlotshaniswe nalesi sifo, ikakhulukazi: (1)

- ithumba ku-cerebellum;

- ukuphuza utshwala, izidakamizwa ezithile noma ukuthintana nezibulala-zinambuzane;

- ukopha kwangaphakathi ku-cerebellum;

- i-multiple sclerosis: ukuthuthukiswa kwezicubu ezixhumene nesitho, okubangela ukuba lukhuni;

- ingozi ye-cerebral vascular;

– imithi yokugoma ethile.

I-Ataxia ivame ukuhlotshaniswa nokulimala kwe-cerebellum. Nokho, ukungahambi kahle kwezinye izingxenye zesimiso sezinzwa kungaba imbangela.

Lokhu kulimala kobuchopho kuhlotshaniswa nezimo ezithile, njengalezi: ukulimala ekhanda, ukuntuleka komoyampilo ebuchosheni noma ngisho nokuphuza kakhulu utshwala.

Ngaphezu kwalokho, ukudluliselwa kwalesi sifo kungenziwa futhi ngokudluliselwa kwefa lefomu elivelele le-autosomal. Noma, ukudluliswa kofuzo olushintshile lwentshisekelo, okukhona ku-chromosome engeyona ezocansi, kusukela kubazali. Ukuba khona kwekhophi eyodwa kuphela yamakhophi amabili ofuzo oluguquliwe kwanele ekuthuthukiseni i-cerebellar ataxia. (2)

Izici zengozi

Izici zobungozi ezihlobene ne-cerebellar ataxia ziwufuzo, kumongo wefa elibusayo le-autosomal. Esimweni sokugcina, ukudluliselwa kwekhophi eyodwa yofuzo oluguquguqukayo lwesithakazelo kwanele ekuthuthukiseni lesi sifo enzalweni. Ngalo mqondo, uma omunye wabazali ababili ethintwa yi-pathology, ingane inengozi engu-50% yalokho.

Ezinye izici nazo ziyavela ekuthuthukisweni kwalesi sifo. Lezi zihlanganisa izifo ezibangelwa amagciwane: inkukhu, ukutheleleka ngegciwane le-Eptein-Barr, isifo sika-Coxsackie noma ukutheleleka kwe-echovirus.

Isici esiyingozi esitholakala kakhulu ku-cerebellar ataxia ubuchopho nesistimu yezinzwa.

Ukuvimbela nokwelashwa

Ukuxilongwa okuyinhloko kwalesi sifo kuvame ukuhlanganiswa nokuxilongwa okuhlukile, lapho udokotela ebuza isiguli imibuzo eminingi ukuze anqume ukuthi usanda kugula yini. Leli phuzu lokuqala lokubuka futhi lenza kube nokwenzeka ukuqeda ezinye izimbangela ezingase zibe khona ezihlobene nokuba khona kwezimpawu.

Ngemva kwalokhu kubuka kokuqala, kuhlolwa ubuchopho nesimiso sezinzwa esimaphakathi ukuze kuhlonzwe izifunda ze-cerebral cortex ezithintwe yilesi sifo. Phakathi kwalezi zivivinyo, singabala:

- ukuskena kwekhanda;

– I-MRI (Magnetic Resonance Imaging) yekhanda.


Ukwelashwa kwalesi sifo kuncike ngqo kumbangela yaso: (1)

- ukuhlinzwa kuyadingeka uma i-ataxia ibangelwa ukopha kobuchopho;

- izidakamizwa ezacisa igazi ngesikhathi sokushaywa unhlangothi;

- ama-antibiotics kanye nama-antiviral ngesikhathi sokutheleleka;

- ama-steroid ekwelapheni ukuvuvukala kwe-cerebellum.


Ngaphezu kwalokho, uma kwenzeka i-ataxia ebangelwa ukutheleleka ngegciwane kwakamuva, akukho mithi edingekayo.

Ezimweni eziningi zalesi sifo, alikho ikhambi laso. Izindlela zokwelapha kuphela ezilawula futhi ezikhawulela izimpawu ezinqunyiwe uma kunesidingo.

Izinsiza zolimi zingabuye zihlotshaniswe, i-physiotherapy ekuxazululeni ukushiyeka kokunyakaza, izikhathi zokwelapha zomsebenzi ezivumela ukuphindaphinda kwezenzo zansuku zonke noma izidakamizwa ezivumela ukulawulwa kwemisipha eqinile, imisipha ye-caridaque, ukunyakaza ukulawulwa kwe-ocular kanye nomchamo. (2)

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