Isifo seCreutzfeldt-Jakob

Isifo seCreutzfeldt-Jakob (i-CJD) siyindlela yokulimala kobuchopho okuholela ekwehleni okusheshayo kokuhamba nokulahleka kokusebenza kwengqondo.

I-CJD ibangelwa yi-protein ebizwa nge-prion. I-prion ibangela ukuthi amaprotheni ajwayelekile agoqeke ngokungavamile. Lokhu kuthinta amandla amanye amaprotheni okusebenza.

I-CJD ayivamile kakhulu. Kunezinhlobo eziningana. Izinhlobo zakudala ze-CJD yilezi:

• I-Sporadic CJD yenza amacala amaningi. Kwenzeka ngaphandle kwesizathu esaziwayo. Isilinganiso seminyaka lapho iqala khona ingama-65.
• I-CJD ejwayelekile yenzeka lapho umuntu ezuza njengefa i-prion engajwayelekile kumzali (leli fomu le-CJD alivamile).
• I-CJD etholakalayo ifaka i-CJD ehlukile (vCJD), ifomu elihlobene nesifo senkomo esile. Iatrogenic CJD nayo iyindlela etholakele yalesi sifo. Iatrogenic CJD kwesinye isikhathi idluliswa ngokumpontshelwa umkhiqizo wegazi, ukufakelwa kabusha, noma okokuhlinza okungcolisiwe.

I-CJD ehlukile ibangelwa ukudla inyama enegciwane. Ukutheleleka okubanga lesi sifo ezinkomeni kukholakala ukuthi yikhona kanye okudala i-vCJD ebantwini.

Ukuhlukahluka kwe-CJD kubangela ngaphansi kwamaphesenti ayi-1 wawo wonke amacala e-CJD. Kuvame ukuthinta abantu abasha. Bangaphansi kuka-200 abantu emhlabeni jikelele abake baba nalesi sifo. Cishe wonke amacala enzeka eNgilandi naseFrance.

I-CJD ingahle ihlobene nezinye izifo eziningana ezibangelwa ama-prions, kufaka phakathi:

• Ukugula okungapheli (okutholakala kuzinyamazane)
• UKuru (othinteke kakhulu abesifazane baseNew Guinea abadle ubuchopho bezihlobo ezifile njengengxenye yesiko lomngcwabo)
• I-Scrapie (etholakala ezimvwini)
• Ezinye izifo ezingavamile kakhulu zabantu, ezifana nesifo sikaGerstmann-Straussler-Scheinker nokuqwasha komndeni okubulalayo

Izimpawu ze-CJD zingafaka noma yikuphi okulandelayo:

• Ukuwohloka komqondo okuya ngokuya kube kubi masonto ambalwa noma izinyanga ezimbalwa
• Umbono ofiphele (kwesinye isikhathi)
• Izinguquko ku-gait (ukuhamba)
• Ukudideka, ukudideka
• Imibono (ukubona noma ukuzwa izinto ezingekho)
• Ukungabi nokuxhumana (ngokwesibonelo, ukukhubeka nokuwa)
• Ukuqina kwemisipha, ukudikiza
• Uzizwa unovalo, uyagxuma
• Ubuntu buyashintsha
• Ukulala
• Ukunyakaza okungazelelwe noma ukuquleka okungazelelwe
• Inkinga yokukhuluma

Ekuqaleni kwalesi sifo, uhlelo lwezinzwa nokuhlolwa kwengqondo kuzokhombisa izinkinga zememori nezokucabanga. Kamuva kulesi sifo, ukuhlolwa kwesistimu yemoto (isivivinyo sokuhlola ukucabanga kwemisipha, amandla, ukuxhumana, neminye imisebenzi ebonakalayo) kungakhombisa:

• Ukucabanga okungajwayelekile noma izimpendulo ezijwayelekile ze-reflex
• Khulisa ithoni yemisipha
• Ukudikiza kwemisipha nokuqaqamba kwemisipha
• Impendulo eqinile yokwethuka
• Ubuthakathaka nokulahleka kwezicubu zemisipha (ukuncipha kwemisipha)

Kukhona ukulahleka kokuxhumanisa nezinguquko ku-cerebellum. Le yindawo yobuchopho elawula ukuhlangana.

Ukuhlolwa kwamehlo kukhombisa izindawo zobumpumputhe umuntu angazibona.

Ukuhlolwa okusetshenziselwa ukuxilonga lesi simo kungafaka:

• Ukuhlolwa kwegazi ukukhipha ezinye izinhlobo zokuwohloka komqondo nokubheka omaka kwesinye isikhathi abenzeka ngalesi sifo
• I-CT scan yobuchopho
• I-Electroencephalogram (EEG)
• I-MRI yobuchopho
• Thepha umgogodla ukuhlola iprotheni ebizwa nge-14-3-3

Lesi sifo singaqinisekiswa kuphela nge-biopsy yobuchopho noma ngokuhlolwa kwesidumbu. Namuhla, kuyaqabukela kakhulu ukuthi kwenziwe i-biopsy yobuchopho ukubheka lesi sifo.

Alikho ikhambi elaziwayo lalesi simo. Imithi ehlukene izamile ukunciphisa isifo. Lokhu kufaka phakathi ama-antibiotic, izidakamizwa zesifo sokuwa, izinciphisi zegazi, imishanguzo yokulwa nokudangala, kanye ne-interferon. Kepha akukho okusebenza kahle.

Inhloso yokwelashwa ukuhlinzeka ngendawo ephephile, ukulawula ukuziphatha okunolaka noma okuthukuthele, nokuhlangabezana nezidingo zomuntu. Lokhu kungadinga ukuqashwa nokusizwa ekhaya noma endaweni yokunakekela. Ukwelulekwa ngomndeni kungasiza umndeni ukubhekana nezinguquko ezidingekayo ekunakekelweni kwasekhaya.

Abantu abanalesi simo bangadinga usizo ekulawuleni isimilo esingamukeleki noma esiyingozi. Lokhu kufaka ukuvuza kokuziphatha okuhle nokungazinaki izindlela ezimbi zokuziphatha (uma kuphephile). Bangadinga futhi usizo lokuthola ukuqondiswa endaweni ebazungezile. Kwesinye isikhathi, imithi iyadingeka ukusiza ukulawula ulaka.

Abantu abane-CJD kanye nemindeni yabo kungadingeka ukuthi bafune izeluleko zomthetho ekuqaleni kwesifo. I-Advance directive, amandla abameli, nezinye izinyathelo zomthetho kungenza kube lula ukwenza izinqumo ngokunakekelwa komuntu one-CJD.

Umphumela we-CJD mibi kakhulu. Abantu abane-CJD engajwayelekile abakwazi ukuzinakekela ezinyangeni eziyisithupha noma ngaphansi ngemuva kokuqala kwezimpawu.

Lesi sifo siyabulala ngesikhathi esifushane, imvamisa kungakapheli izinyanga eziyi-8. Abantu abane-CJD ehlukile baya ngokuya behamba kancane, kepha isimo sisabulala. Bambalwa abantu abasinda isikhathi esingangonyaka owodwa noma emibili. Imbangela yokufa imvamisa ukutheleleka, ukwehluleka kwenhliziyo, noma ukwehluleka ukuphefumula.

Inkambo ye-CJD yile:

• Ukutheleleka ngalesi sifo
• Ukungondleki okukhulu
• Ukuwohloka komqondo kwezinye izimo
• Ukulahlekelwa amandla okuxhumana nabanye
• Ukulahlekelwa yikhono lokusebenza noma ukuzinakekela
• Ukufa

I-CJD ayisona isimo esiphuthumayo sezokwelapha. Kodwa-ke, ukuxilongwa nokwelashwa kusenesikhathi kungenza izimpawu kube lula ukuzilawula, kunikeze iziguli isikhathi sokwenza izinkomba kusengaphambili nokulungiselela ukuphela kwempilo, nokunikeza imindeni isikhathi esengeziwe sokubhekana nalesi simo.

Imishini yezokwelapha engangcoliswa kufanele isuswe ekusebenzeni bese ilahlwa. Abantu abaziwa ukuthi bane-CJD akufanele banikele nge-cornea noma ezinye izicubu zomzimba.

Amazwe amaningi manje anemihlahlandlela eqinile yokuphatha izinkomo ezinegciwane ukugwema ukudlulisela i-CJD kubantu.

I-transceissible spongiform encephalopathy; vCJD; CJD; Isifo sikaJacob-Creutzfeldt

• Isifo seCreutzfeldt-Jakob
• Isistimu yezinzwa emaphakathi nesistimu yezinzwa ezungezayo

I-Bosque PJ, uTyler KL. Izifo ze-Prion kanye ne-prion zesistimu yezinzwa emaphakathi (izifo ezithathelwanayo ze-neurodegenerative). Ku: Bennett JE, Dolin R, Blaser MJ, ama-eds. Mandell, Douglas, kanye neBennett's Principles and Practice of Infectious Diseases. Umhlaka 9. IPhiladelphia, PA: Elsevier; 2020: isahluko 179.

IGeschwind MD. Izifo ze-Prion. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 94.