ʻO ka maʻi Creutzfeldt-Jakob kekahi o nā maʻi prion. He mau maʻi kakaʻikahi kēia i ʻike ʻia e ka degeneration o ka ʻōnaehana nūnū waena a ua kapa ʻia hoʻi nā subacute transmissible spongiform encephalopathies (TSE). Hoʻokumu ʻia lākou e ka hōʻiliʻili ʻana i loko o ka lolo o kahi protein maʻamau akā ʻano maikaʻi ʻole, ka protein prion (1). ʻO ka mea pōʻino, ʻike ʻia ka maʻi Creutzfeldt-Jakob e kahi ala wikiwiki a make a me ka ʻole o ka mālama ʻana. Aia 100 a 150 mau hihia i kēlā me kēia makahiki ma Farani (2).

Hoʻomaka pinepine ka maʻi me nā maʻi kīnā ʻole e like me ka insomnia a i ʻole ka hopohopo. Hoʻomaka mālie ka hoʻomanaʻo, ka hoʻonohonoho ʻana a me ka ʻōlelo. A laila hōʻike ʻia e nā maʻi psychiatric a me cerebellar ataxia (ka paʻa ʻole i ka wā e kū ʻole ai a i ka wā e hele pū ʻia me ka ʻona e like me ka ʻona). Aia kekahi mau maʻi maʻamau i loko o ka ʻōnaehana nūnū waena (nā plaques florid, nā waihona amyloid o PrPres i hoʻopuni ʻia e nā vacuoles).

Hoʻopilikia ʻia nā kāne ʻelua, akā naʻe me ke alapine kiʻekiʻe i nā ʻōpio ʻōpio.

ʻO ka mea pōʻino, ʻaʻohe hōʻike diagnostic pono. Hiki i ka electroencephalogram (EEG) ke hoʻomaopopo i nā pilikia kūikawā i ka hana o ka lolo. Hōʻike ʻo MRI i nā mea ʻino ma kekahi mau ʻāpana o ka lolo (basal ganglia, cortex) kahi liʻiliʻi o nā maʻi ʻokoʻa.

Inā hiki i kēia mau mea lapaʻau a me nā mea paraclinical ke hiki ke hana i kahi hōʻailona o ka maʻi Creutzfeldt-Jakob, he hōʻailona wale nō ia: ʻoiaʻiʻo, ʻo ka hoʻokolokolo wale ʻana o ka ʻiʻo o ka lolo, i hana pinepine ʻia ma hope o ka make e hiki ai ke hōʻoia i ka hōʻailona.

ʻO ka maʻi Creutzfeld-Jakob wale nō ka maʻi kanaka i hiki ke loaʻa i ke kumu genetic (ma muli o ka hoʻololi ʻana o ka gene e hoʻopili ana i ka protein prion, ʻo ka mutation E200K ka mea maʻamau), ke kumu maʻi (ke lua i ka contamination) a i ʻole ke ʻano sporadic (o ʻike ʻole ʻia, me ka ʻole o ka hoʻololi ʻana a i ʻole ka ʻike ʻana i kahi prion exogenous i loaʻa).

Eia nō naʻe, ʻo ke ʻano sporadic ka mea maʻamau: ʻo ia ka 85% o nā subacute transmissible spongiform encephalopathies (TSEs) i ʻike ʻia i kēlā me kēia makahiki. I kēia hihia, ʻike pinepine ʻia ka maʻi ma hope o 60 mau makahiki a holomua ma kahi o 6 mau mahina. Inā he genetic ka maʻi a maʻi paha, ʻoi aku ka maʻi o ka maʻi ma mua a ʻoi aku ka mālie. I nā ʻano infectious, hiki ke lōʻihi loa ka manawa incubation a ʻoi aku ma mua o 50 mau makahiki.

ʻO ka protein prion (PrPc) he pūmua physiological i loaʻa ma ke ʻano mālama ʻia i nā ʻano he nui. I loko o nā neurons lolo, hiki i ka prion protein ke lilo i pathogenic ma o ka hoʻololi ʻana i kona ʻano hoʻohālikelike ʻekolu-dimensional: piʻi paʻa iā ia iho, e lilo ia i hydrophobic, hiki ke hoʻoheheʻe liʻiliʻi a kūpaʻa i ka degradation. Ua kapa ʻia ʻo ia ka "scrapie" prion protein (PrPsc). Hoʻohui pū ʻia ʻo PrPsc me kekahi i kekahi a hana i nā waihona e hoʻonui ai i loko a i waho o nā pūnaʻi lolo, e hoʻopau ana i kā lākou hana a me nā hana ola.

Ma kēia ʻano ʻano ʻino, hiki i ka protein prion ke hoʻoili i kāna anomaly conformational: ma ka hoʻopili ʻana me kahi PrPsc, ʻo ka protein prion maʻamau i ka huli ʻana i kahi conformation abnormal. ʻO kēia ka hopena domino.

ʻO ka pilikia o ka lawe ʻana ma waena o nā kānaka

Hiki ke hoʻoili ʻia i waena o nā maʻi prion me ka hoʻololi ʻana i ke kiko a i ʻole ma hope o ka hoʻokele ʻana i nā hormones ulu. ʻO nā ʻiʻo koʻikoʻi loa e hele mai mai ke kikowaena kikowaena a me ka maka. I ka liʻiliʻi liʻiliʻi, hiki i ka wai cerebrospinal, ke koko a me kekahi mau ʻāpana (nā ʻāʻī, nā māmā, a me nā mea ʻē aʻe) hiki ke hoʻoili i ka prion maʻamau.

ʻO ka pilikia o ka meaʻai

 ʻO ka hoʻoili ʻia ʻana o kahi prion mai nā pipi i nā kānaka ma o ka ʻai ʻana i ka meaʻai i hoʻohaumia ʻia i manaʻo ʻia i ka makahiki 1996, i ka wā o ka pilikia nui o ka "bipi pupule". No kekahi mau makahiki i kēia manawa, ua loaʻa ka maʻi ahulau o ka bovine spongiform encephalopathy (BSE) i nā holoholona ma United Kingdom3. ʻO ka hoʻolaha ʻana o kēia maʻi prion, ka mea i hoʻopilikia i nā ʻumi kaukani holoholona i kēlā me kēia makahiki, ʻaʻole kānalua ma muli o ka hoʻohana ʻana i ka ʻai holoholona, ​​i hana ʻia mai nā kupapaʻu a lawa ʻole ka decontaminated. Ke hoʻopaʻapaʻa ʻia nei kona kumu.

I kēia lā, ʻaʻohe lāʻau lapaʻau kūikawā no nā maʻi prion. ʻO nā lāʻau lapaʻau wale nō i hiki ke kuhikuhi ʻia, ʻo ia ka mea hiki ke hōʻoluʻolu a kaupalena paha i nā hōʻailona like ʻole o ka maʻi. Hāʻawi ʻia ke kākoʻo lapaʻau, ka pilikanaka a me ka noʻonoʻo i nā maʻi a me ko lākou ʻohana e ka National CJD Support Unit. ʻO ka ʻimi ʻana i nā lāʻau lapaʻau e pili ana i ka pale ʻana i ka hoʻololi ʻana o PrPc, ka paipai ʻana i ka hoʻopau ʻana i nā ʻano ʻano like ʻole o ka protein a me ka hoʻopaʻa ʻana i kona hoʻolaha ʻana. Kuhi ʻia kahi alakaʻi hoihoi i ka PDK1, kekahi o nā mea hoʻolaha kelepona i pili i ka wā maʻi. ʻO kona hoʻopaʻa ʻana e hiki iā ia ke pale i ka hanana hoʻololi ʻana ma o ka hoʻolaha ʻana i ka cleavage o PrPc, a e hoʻemi i nā hopena o kāna hana hou ʻana i ke ola o nā neurons.