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I-Creutzfeldt-Jakob (KROITS-felt YAH-kobe) isifo, esaziwa nangokuthi yi-CJD, yisifo esingavamile sobuchopho esibangela ukuqwasha. Siyingxenye yeqembu lezinye izifo zabantu nezilwane ezibizwa ngokuthi yizifo zeprion. Izimpawu ze-Creutzfeldt-Jakob zingafana nalezo ze-Alzheimer. Kodwa-ke, i-Creutzfeldt-Jakob iyasabela ngokushesha futhi iholele ekufeni.

I-Creutzfeldt-Jakob disease (CJD) yaduma ngawo-1990 lapho abantu base-United Kingdom baqala ukugula ngalolu hlobo lwesifo. Baphuthuma i-variant CJ, eyaziwa ngokuthi yi-vCJD, ngemuva kokudla inyama yezilwane ezigula. Kodwa-ke, izinye izimo ze-Creutzfeldt-Jakob azixhunyaniswanga nokudla inyama yenkomo.

Zonke izinhlobo ze-CJD zinzima kodwa zingevamile kakhulu. Cishe kuphikiswa icala elilodwa noma amabili e-CJD ngabantu bezigidi eziyizigidi emhlabeni jikelele minyaka yonke. Lesi sifo sivame ukubathinta abantu abadala.

I-Creutzfeldt-Jakob isifo sibonakala ngokushintsha kwamandla engqondo. Izimpawu ziyaqina ngokushesha, ngokuvamile ezinyangeni ezimbalwa kuya ezinyangeni ezimbalwa. Izimpawu zokuqala zihlanganisa:

• Ukushintsha komuntu siqu.
• Ukukhohlwa.
• Ukucabanga okonakele.
• Ukubona okungacaci noma ubuthakathaka.
• Ukungalali ubuthongo.
• Izinkinga zokuxhumanisa.
• Ukukhuluma okunzima.
• Ukugwinya okunzima.
• Ukunyakaza okungazelelwe, okuqhumayo.

Ukufa kuvame ukuvela phakathi nonyaka. Abantu abane-Creutzfeldt-Jakob isifo bavame ukufa ngezinkinga zezokwelapha ezihlobene nesifo. Zingase zihlanganise ukugwinya okunzima, ukuwa, izinkinga zenhliziyo, ukwehluleka kwemiphunga, noma i-pneumonia noma ezinye izifo.

Kubantu abane-CJD ehlukile, ukushintsha kwamandla engqondo kungabonakala kakhulu ekuqaleni kwesifo. Emacaleni amaningi, ukuqothuka kwengqondo kuvela kamuva ekuguleni. Izimpawu zokuqothuka kwengqondo zihlanganisa ukulahleka kwamandla okucabanga, ukubonisana nokukhumbula.

I-CJD ehlukile ithonya abantu besemncane kunaku-CJD. I-CJD ehlukile ibonakala ihlala izinyanga eziyi-12 kuya kweziyi-14.

Enye indlela engavamile yesifo se-prion ibizwa ngokuthi i-variably protease-sensitive prionopathy (VPSPr). Ingalingana nezinye izindlela zokuqothuka kwengqondo. Ibangele ukushintsha kwamandla engqondo nezinkinga zokukhuluma nokucabanga. Inkambo yesifo isikhathi eside kunamanye ama-prion ama-prion - cishe izinyanga eziyi-24.

I-Creutzfeldt-Jakob nezinye izimo ezihlobene kubonakala sengathi zibangelwa yizinguquko ohlotsheni lwe-protein ebizwa ngokuthi yi-prion. Lezi zinhlobo ze-protein zivame ukukhiqizwa emzimbeni. Kodwa uma zihlangana ne-prion ezithathelwanayo, ziyaqina futhi zibe yisimo esingavamile. Zingasakazeka futhi zithinte izinqubo emzimbeni.

Amacala amaningi e-Creutzfeldt-Jakob disease avela ngezizathu ezingaziwa. Ngakho-ke izimbangela zingozi azikwazi ukunqunywa. Kodwa izici ezimbalwa zibonakala zihambisana nezinhlobo ezahlukene ze-CJD.

• Ubudala. I-Sporadic CJD ivame ukuthuthuka kamuva ekuphileni, ngokuvamile cishe eminyakeni engama-60. Ukuqala kwe-familial CJD kwenzeka ngokushesha. Futhi i-variant Creutzfeldt-Jakob disease (vCJD) ithonye abantu abancane kakhulu, ngokuvamile eminyakeni yabo engama-20 kamuva.
• I-Genetics. Abantu abane-familial CJD banokuguqulwa kwe-genetic okubangela leyo gciwane. Ukuthuthukisa le ndlela yesifo, umntwana kufanele abe nokukopisha okukodwa kwe-gene ebangela i-CJD. I-gene ingadluliselwa kubazali bobabili. Uma unayo i-gene, ithuba lokudlulisela kubantwana bakho lingama-50%.
• Ukuvezwa kuma-tissue angcolile. Abantu abamukelwe i-human growth hormone engcolile bangase babe sengozini ye-iatrogenic CJD. Ukwamukela ukuhlinzwa kwe-tissue ehlanganisa ubuchopho, okuthiwa yi-dura mater, kumuntu onale CJD nakho kungafaka umuntu engozini ye-iatrogenic CJD.

Ubungozi bokuthola i-vCJD ngokudla inyama yenkomo engcolile buphansi kakhulu. Emazweni asebenzise izindlela zempilo yeningi eziphumelelayo, ubungozi abukho nhlobo. I-Chronic wasting disease (CWD) yisifo se-prion esithinta izimvu, izimbali, izimpondo nezinqama. Litholwe kwamanye amazwekazi eNyakatho Melika. Kuze kube manje, azikho izimo ezibhalwe phansi ze-chronic wasting disease (CWD) eziye zabangela isifo kubantu.

Isifo seCreutzfeldt-Jakob sinezimpikiswano ezinkulu ebuchosheni nasezimweni zomzimba. Leli gciwane liqhubeka ngokushesha. Ngokuhamba kwesikhathi, abantu abane-CJD bayazidela kubangani nasemhlabeni. Baphelelwa nakho ukuzinakekela. Abaningi bayagqwala ekomeni. Leli gciwane liyabafa kakhulu.

Ayikho indlela eyaziwayo yokuvimbela i-CJD eyenzeka ngezikhathi ezithile. Uma unemlando womndeni wesifo sezitho zenu, ungase uzuze ngokukhuluma nodokotela we-genetics. Udokotela angakusiza ukuthi uhlukanise ingozi yakho.

Ukuhlolwa kwe-biopsy yengqondo noma ukuhlolwa kwezicubu zengqondo ngemva kokufa, okwaziwa ngokuthi yi-autopsy, yiyona ndlela eqinisekisa ukuba kukhona isifo se-Creutzfeldt-Jakob, esaziwa nge-CJD. Kodwa-ke, abahlinzeki bezempilo bavame ukwenza ukuxilongwa okuchane ngaphambi kokufa. Baxhomeke ekuxilongweni kwakho emlandweni wakho wezokwelapha nomlando wakho siqu, ekuhlolweni kwezonyango, nakwimisebenzi ethile yokuxilonga.

Ukuhlolwa kwezonyango kungabonisa isifo se-Creutzfeldt-Jakob (CJD) uma ubhekene nalokhu:

Ngaphezu kwalokho, abahlinzeki bezempilo bavame ukusebenzisa le miqondo yokusiza ekutholeni i-CJD:

Izivivinyo ze-spinal fluid. I-spinal fluid izungezile futhi ivikela ubuchopho nomgogodla. Ekuhlolweni okuthiwa yi-lumbar puncture, okwaziwa nangokuthi yi-spinal tap, inani elincane le-spinal fluid lithathiwe ukuze lihlolwe. Lolu vivinyo lungakwazi ukukhipha ezinye izifo ezibangela izimpawu ezifanayo ne-CJD. Lingakwazi futhi ukuthola amazinga amaprotheni angabonisa i-CJD noma isifo se-Creutzfeldt-Jakob esiguqukayo (vCJD).

Uvivinyo olusha oluthiwa yi-real-time quaking-induced conversion (RT-QuIC) lungathola ukuba khona kwamaprotheni eprion abangela i-CJD. Lolu vivinyo lungaxilonga i-CJD ngaphambi kokufa, ngokungafani ne-autopsy.

• Ukukhahlela kwemisipha nokubamba.

• Ukushintsha kwe-reflexes.

• Izinkinga zokuxhumanisa.

• Izinkinga zobumhlophe.

• Ubuthakathaka.

• I-Electroencephalogram, eyaziwa nangokuthi yi-EEG. Lolu vivinyo luqapha umsebenzi wamagetsi wobuchopho. Lwenziwa ngokubeka ama-discs amancane ensimbi okuthiwa yi-electrodes ekhanda. Imiphumela ye-electroencephalogram (EEG) yabantu abane-CJD ne-variant CJD ibonisa iphethini engavamile.

• I-Magnetic resonance imaging (MRI). Lolu hlobo lwe-imaging lusebenzisa amaza omsakazo nendlela yamagnethi ukudala izithombe eziningi zekhanda nomzimba. I-MRI ilusizo kakhulu ekubukeni izifo zengqondo. I-MRI yenza izithombe ezinokuqina okuphezulu. Abantu abane-CJD banokushintsha okuqondene okungatholakala ekuhlolweni okuthile kwe-MRI.

• Izivivinyo ze-spinal fluid. I-spinal fluid izungezile futhi ivikela ubuchopho nomgogodla. Ekuhlolweni okuthiwa yi-lumbar puncture, okwaziwa nangokuthi yi-spinal tap, inani elincane le-spinal fluid lithathiwe ukuze lihlolwe. Lolu vivinyo lungakwazi ukukhipha ezinye izifo ezibangela izimpawu ezifanayo ne-CJD. Lingakwazi futhi ukuthola amazinga amaprotheni angabonisa i-CJD noma isifo se-Creutzfeldt-Jakob esiguqukayo (vCJD).

A newer test called real-time quaking-induced conversion (RT-QuIC) can detect the presence of the prion proteins that cause CJD. This test can diagnose CJD before death, unlike an autopsy.

Ayikho impilo ephumelelayo yeCreutzfeldt-Jakob disease noma yiziphi izinhlobo zayo. Imithi eminingi iye yahlolwa futhi ayibonisi izinzuzo. Abahlinzeki bezempilo bagxila ekunciphiseni ubuhlungu nezinye izimpawu, nokwenza abantu abanesifo esinjalo baphumule.