Matenda a Creutzfeldt-Jakob ndi amodzi mwa matenda a prion. Awa ndi matenda osowa omwe amadziwika ndi kuwonongeka kwa minyewa yapakati komanso amatchedwanso subacute transmissible spongiform encephalopathies (TSE). Zimayamba chifukwa cha kudzikundikira muubongo kwa puloteni yabwinobwino koma yosagwirizana bwino, mapuloteni a prion (1). Tsoka ilo, matenda a Creutzfeldt-Jakob amadziwika ndi njira yofulumira komanso yakupha komanso kusowa kwa chithandizo. Pali milandu 100 mpaka 150 chaka chilichonse ku France (2).

Matendawa nthawi zambiri amayamba ndi matenda osadziwika bwino monga kusowa tulo kapena nkhawa. Pang'onopang'ono, kukumbukira, kukhudzidwa ndi kusokonezeka kwa chinenero kumayikidwa. Kenaka amawonetseredwa ndi matenda a maganizo komanso cerebellar ataxia (kusakhazikika pamene atayima osasunthika komanso panthawi yoyenda yomwe imatsagana ndi kugwedeza kofanana ndi kuledzera). Palinso zotupa zapakati pa mitsempha yapakati (zolemba zamaluwa, ma amyloid deposits a PrPres atazunguliridwa ndi vacuoles).

Amuna ndi akazi amakhudzidwa, komabe ndi mafupipafupi a achinyamata akuluakulu.

Tsoka ilo, palibe mayeso odalirika a matenda. Electroencephalogram (EEG) imatha kuzindikira kusokonezeka kwapadera muzochitika zaubongo. MRI imawulula zolakwika zina m'magawo ena aubongo (basal ganglia, cortex) komwe kulibe matenda osiyanasiyana.

Ngati zinthu zonsezi zachipatala komanso za paraclinical zingapangitse kuti adziwe matenda a Creutzfeldt-Jakob, ndiye kuti n'zotheka kuzindikira matenda: Ndipotu, kufufuza kokha kwa minofu ya ubongo, yomwe imachitika nthawi zambiri pambuyo pa imfa imalola kutsimikizira matenda.

Matenda a Creutzfeld-Jakob ndi matenda okhawo a anthu omwe amatha kukhala chifukwa cha majini (chifukwa cha kusintha kwa jini yomwe imasunga mapuloteni a prion, kusintha kwa E200K kukhala kofala kwambiri), chifukwa choyambitsa matenda (chachiwiri mpaka kuipitsidwa) kapena mawonekedwe a sporadic (wa kuchitika mwachisawawa, popanda kusintha kapena kukhudzana ndi prion yachilendo yomwe yapezeka).

Komabe, mawonekedwe a sporadic ndi omwe amapezeka kwambiri: amawerengera 85% ya subacute transmissible spongiform encephalopathies (TSEs) omwe amapezeka chaka chilichonse. Pamenepa, matendawa nthawi zambiri amawonekera pambuyo pa zaka 60 ndipo amapitirira pafupifupi miyezi isanu ndi umodzi. Matendawa akakhala chibadwa kapena kupatsirana, zizindikiro zimayamba msanga ndipo zimakula pang'onopang'ono. Mu mawonekedwe opatsirana, nthawi yoyamwitsa imatha kukhala yayitali kwambiri ndikupitilira zaka 6.

Puloteni ya prion (PrPc) ndi mapuloteni a thupi omwe amapezeka m'njira yotetezedwa kwambiri m'mitundu yambiri. Mu ma neuron a muubongo, mapuloteni a prion amatha kukhala a pathogenic posintha mawonekedwe ake amitundu itatu: imadzipindika yokha mwamphamvu kwambiri, zomwe zimapangitsa kuti ikhale ya hydrophobic, yosungunuka pang'ono komanso yosagwirizana ndi kuwonongeka. Kenako imatchedwa "scrapie" prion protein (PrPsc). PrPsc imaphatikizana wina ndi mzake ndikupanga madipoziti omwe amachulukana mkati ndi kunja kwa ma cell aubongo, kusokoneza ntchito yawo ndi njira zopulumutsira.

Mu mawonekedwe osazolowereka, puloteni ya prion imathanso kufalitsa kusagwirizana kwake: pokhudzana ndi PrPsc, mapuloteni abwinobwino a prion nawonso amatenga mawonekedwe osagwirizana. Izi ndiye zotsatira za domino.

Kuopsa kwa kufala pakati pa anthu

Kupatsirana kwapawiri kwa matenda a prion ndikotheka ndi kupatsirana kwa minofu kapena kutsatira kasamalidwe ka mahomoni okula. Minofu yoopsa kwambiri imachokera ku dongosolo lapakati la mitsempha ndi diso. Pang'onopang'ono, cerebrospinal fluid, magazi ndi ziwalo zina (impso, mapapo, ndi zina zotero) zimatha kufalitsa prion yodabwitsa.

Kuopsa kwa chakudya

 Kupatsirana kwa prion kuchokera ku ng'ombe kupita kwa anthu kudzera mukudya zakudya zoyipitsidwa kunkaganiziridwa kuti mu 1996, panthawi yovuta kwambiri ya "ng'ombe yamisala". Kwa zaka zingapo tsopano, mliri wa bovine spongiform encephalopathy (BSE) wakhudza ziweto ku United Kingdom3. Kufalikira kwa matenda a prion, omwe amakhudza nyama masauzande makumi ambiri chaka chilichonse, mosakayikira anali chifukwa chogwiritsa ntchito chakudya cha nyama, chopangidwa kuchokera ku mitembo komanso chosakanizidwa bwino. Komabe, anthu amakambitsirana za chiyambi chake.

Masiku ano, palibe mankhwala enieni a matenda a prion. Mankhwala okhawo omwe angaperekedwe ndi omwe amatha kuchepetsa kapena kuchepetsa zizindikiro zosiyanasiyana za matendawa. Thandizo lachipatala, chikhalidwe ndi maganizo limaperekedwa kwa odwala ndi mabanja awo ndi National CJD Support Unit. Kufufuza kwa mankhwala omwe cholinga chake ndi kuteteza kutembenuka kwa PrPc, kulimbikitsa kuthetsa mitundu yachilendo ya mapuloteni ndi kuchepetsa kufalikira kwake ndi chiyembekezo. Chotsogolera chosangalatsa chimayang'ana PDK1, m'modzi mwa oyimira ma cellular omwe amakhudzidwa ndi matenda. Kuletsa kwake kungapangitse kuti zonse zitheke kuletsa kutembenuka mwa kulimbikitsa kugawanika kwa PrPc, ndikuchepetsa zotsatira za kubwereza kwake pa kupulumuka kwa ma neuron.