Isifo seCreutzfeldt-Jakob

Isifo seCreutzfeldt-Jakob (CJD) luhlobo lokonakala kwengqondo olukhokelela ekunciphiseni okukhawulezayo kokuhamba kunye nokuphulukana nokusebenza kwengqondo.

I-CJD ibangelwa yiprotein ebizwa ngokuba yi-prion. I-prion ibangela ukuba iiproteni eziqhelekileyo zisonge ngokungaqhelekanga. Oku kuchaphazela ukusebenza kwezinye iiproteni.

I-CJD inqabile kakhulu. Kukho iintlobo ezininzi. Iindidi zodidi lweCJD zezi:

• I-CJD engapheliyo yenza uninzi lweemeko. Yenzeka ngaphandle kwesizathu esaziwayo. Iminyaka yobudala eqala ngayo ngama-65.
• I-CJD eyaziwayo yenzeka xa umntu ezuza ilifa ngokungaqhelekanga kumzali (le fomu yeCJD inqabile).
• Ukufunyanwa kweCJD kubandakanya ukwahluka kweCJD (vCJD), ifom enxulumene nesifo seenkomo eziphambeneyo. Iatrogenic CJD ikwayindlela efunyenwe sesi sifo. Ngamanye amaxesha kudluliswa imveliso yegazi, ukufakelwa, okanye izixhobo zotyando ezingcolileyo ngamanye amaxesha.

Uhlobo lwe-CJD lubangelwa kukutya inyama eyosulelekileyo. Usulelo olubangela esi sifo ezinkomeni kukholelwa ukuba lolunye lubangela i-vCJD ebantwini.

Ukwahluka kwe-CJD kubangela ngaphantsi kwepesenti enye yazo zonke iimeko ze-CJD. Ihlala ichaphazela abantu abancinci. Abantu abangaphantsi kwama-200 kwihlabathi lonke baye banesi sifo. Phantse onke amatyala enzeka eNgilani naseFrance.

I-CJD inokunxulumana nezinye izifo ezininzi ezibangelwa ziipron, kubandakanya:

• Isifo esinganyangekiyo (esifumaneka kwixhama)
• I-Kuru (ichaphazele ikakhulu abasetyhini baseNew Guinea abatya iingqondo zezalamane zabo ezifileyo njengenxalenye yesiko lokungcwaba)
• I-Scrapie (ifunyenwe kwiigusha)
• Ezinye izifo ezinqabileyo zabantu, ezinje ngesifo sikaGerstmann-Straussler-Scheinker kunye nokulala okungalunganga kosapho

Iimpawu zeCJD zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Isifo sengqondo esixhalabisayo esiba mandundu ngokukhawuleza kwiiveki okanye kwiinyanga ezimbalwa
• Umbono ongenanto (ngamanye amaxesha)
• Utshintsho kuhambo (ukuhamba)
• Ukudideka, ukudideka
• Imibono (ukubona okanye ukuva izinto ezingekhoyo)
• Ukungabikho kolungelelwaniso (umzekelo, ukukhubeka nokuwa)
• Ukuqina kwemisipha, ukujija
• Ndiziva ndothukile, ndiyaxhuma
• Ubuntu buyatshintsha
• Ukulala
• Ukuhamba ngokukhawuleza okanye ukuxhuzula
• Ingxaki yokuthetha

Kwasekuqaleni kwesi sifo, inkqubo yeemvakalelo kunye novavanyo lwengqondo ziya kubonisa inkumbulo kunye neengxaki zokucinga. Emva kwexesha kwesi sifo, kuvavanyo lwenkqubo yemoto (kuvavanyo lokuvavanya ukubonwa kwemisipha, amandla, ulungelelwaniso, kunye neminye imisebenzi ebonakalayo) inokubonisa:

• Ukucinga okungaqhelekanga okanye ukwanda kweempendulo eziqhelekileyo zokucinga
• Ukwandisa ithoni yemisipha
• Ukudibanisa izihlunu kunye nokuqaqamba
• Impendulo eyomeleleyo yokothuka
• Ubuthathaka kunye nelahleko yemisipha (ukutshabalalisa izihlunu)

Kukho ilahleko yokulungelelanisa kunye notshintsho kwi-cerebellum. Lo ngummandla wengqondo olawula ukulungelelanisa.

Ukuvavanywa kwamehlo kubonisa iindawo zobumfama umntu anokungaziboni.

Uvavanyo olusetyenziselwa ukufumanisa le meko lunokubandakanya:

• Uvavanyo lwegazi ukukhupha ezinye iindlela zesifo sengqondo esixhalabisayo kunye nokujonga iziphawuli ngamanye amaxesha ezenzeka nesi sifo
• Ukuvavanywa kwe-CT kwengqondo
• I-Electroencephalogram (i-EEG)
• I-MRI yengqondo
• Impompo yomqolo ukuvavanya iprotheni ebizwa ngokuba yi-14-3-3

Esi sifo sinokuqinisekiswa kuphela nge-biopsy yengqondo okanye nge-autopsy. Namhlanje, kunqabile ukuba kwenziwe i-biopsy yengqondo ukukhangela esi sifo.

Akukho lunyango lwaziwayo lwale meko. Amayeza ahlukeneyo azamile ukusicothisa isifo. Oku kubandakanya amayeza okubulala iintsholongwane, amayeza okuxhuzula, ukunciphisa igazi, ukuthomalalisa uxinzelelo kunye ne-interferon. Kodwa akukho namnye usebenza kakuhle.

Injongo yonyango kukubonelela ngendawo ekhuselekileyo, ukulawula ubundlongondlongo okanye indlela ocaphuka ngayo, kwaye uhlangabeze iimfuno zomntu lowo. Oku kunokufuna ukubekwa esweni kunye noncedo ekhaya okanye kwindawo yokhathalelo. Iingcebiso kusapho zinokunceda usapho ukumelana notshintsho olufunekayo lokhathalelo lwasekhaya.

Abantu abakule meko banokufuna uncedo lokulawula isimilo esingamkelekanga okanye esiyingozi. Oku kubandakanya ukuvuza isimilo esihle kunye nokungahoyi isimilo esibi (xa sikhuselekile). Banokudinga uncedo lokufumana ulwazi ngokujongwa kwindawo ebangqongileyo. Ngamanye amaxesha, amayeza ayafuneka ukunceda ukulawula ubundlongondlongo.

Abantu abane-CJD kunye neentsapho zabo banokufuna iingcebiso zomthetho kwangethuba kwinkqubo yokuphazamiseka. Ukuyalelwa kwangaphambili, amandla egqwetha, kunye nezinye izinto ezisemthethweni zinokwenza kube lula ukwenza izigqibo malunga nokukhathalelwa komntu one-CJD.

Iziphumo zeCJD zimbi kakhulu. Abantu abane-CJD ye-sporadic abakwazi ukuzikhathalela kwiinyanga ezi-6 okanye ngaphantsi emva kokuqala kweempawu.

Esi sifo siyabulala ngexesha elifutshane, ngesiqhelo kwiinyanga ezisi-8. Abantu abane-CJD eyahlukileyo baya becotha kancinci, kodwa imeko isabulala. Bambalwa abantu abaphila ixesha elide njenge-1 okanye 2 iminyaka. Unobangela wokufa uhlala esosuleleko, ukusilela kwentliziyo, okanye ukusilela ekuphefumlweni.

Inkqubo ye-CJD yile:

• Usulelo kwesi sifo
• Ukungondleki kakhulu
• Isifo sengqondo esiyingozi kwezinye iimeko
• Ukuphulukana nokukwazi ukunxibelelana nabanye
• Ukuphulukana nokukwazi ukusebenza okanye ukuzikhathalela
• Ukufa

I-CJD ayisiyongxamiseko kwezonyango. Nangona kunjalo, ukuxilongwa kunye nokunyangwa kwangoko kunokwenza ukuba kube lula ukulawula iimpawu, kunika abaguli ixesha lokwenza izikhokelo kwangaphambili kunye nokulungiselela ukuphela kobomi, kunye nokunika iintsapho ixesha elongezelelekileyo lokuya kule meko.

Izixhobo zonyango ezinokungcoliseka kufuneka zisuswe kwinkonzo zize zilahlwe. Abantu abaziwayo ukuba bane-CJD akufuneki banikele nge-cornea okanye ezinye izicubu zomzimba.

Uninzi lwamazwe ngoku anezikhokelo ezingqongqo zokulawula iinkomo ezosulelekileyo ukunqanda ukuhambisa i-CJD ebantwini.

I-encephalopathy enesifo esosulelayo; vCJD; I-CJD; Isifo sikaJacob-Creutzfeldt

• Isifo seCreutzfeldt-Jakob
• Inkqubo ye-nervous central kunye ne-peripheral system

I-Bosque PJ, iTyler KL. Iipron kunye nesifo se-prion senkqubo ye-nervous system (izifo ezinokuhanjiswa zi-neurodegenerative). Ku: Bennett JE, Dolin R, Blaser MJ, ii-eds. Mandell, Douglas, kunye neBennett's Principles and Practice of Infectious Diseases. Umhla we-9. IPhiladelphia, PA: Elsevier; 2020: isahl. 179.

MD yeGeschwind. Izifo zePrion. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 94.