Cutar Creutzfeldt-Jakob na ɗaya daga cikin cututtukan prion. Waɗannan cututtuka ne da ba kasafai ake samun su ba waɗanda ke da lalacewa na tsarin jijiya na tsakiya kuma ana kiran su da ake kira subacute transmissible spongiform encephalopathies (TSE). Suna haifar da su ta hanyar tarawa a cikin kwakwalwa na furotin na yau da kullun amma mara kyau, furotin prion (1). Abin baƙin ciki shine, cutar Creutzfeldt-Jakob tana da saurin sauri da mutuwa da kuma rashin magani. Akwai lokuta 100 zuwa 150 kowace shekara a Faransa (2).

Cutar sau da yawa tana farawa da cututtuka marasa takamaiman kamar rashin barci ko damuwa. A hankali, ƙwaƙwalwar ajiya, daidaitawa da rikice-rikicen harshe sun shiga. Daga nan sai ya bayyana ta hanyar cututtuka na psychiatric da kuma cerebellar ataxia (rashin kwanciyar hankali lokacin da yake tsaye ba motsi da kuma lokacin tafiya wanda yake tare da abin mamaki kamar na buguwa). Hakanan akwai raunuka na yau da kullun a cikin tsarin juyayi na tsakiya (filayen florid, ajiyar amyloid na PrPres kewaye da vacuoles).

Dukkanin jinsin suna da tasiri, duk da haka tare da yawan mita a cikin samari.

Abin takaici, babu ingantaccen gwajin gano cutar. Electroencephalogram (EEG) na iya gano takamaiman rikice-rikice a cikin ayyukan kwakwalwa. MRI yana bayyana ƙayyadaddun ƙayyadaddun ƙayyadaddun abubuwa a wasu yankuna na kwakwalwa (basal ganglia, cortex) wanda akwai 'yan kaɗan na ganewar asali.

Idan duk wadannan na asibiti da paraclinical abubuwa iya sa shi yiwuwa a yi wani ganewar asali na Creutzfeldt-Jakob cuta, shi ne kawai mai yiwuwa ganewar asali: a gaskiya ma, kawai jarrabawa na kwakwalwa nama, da za'ayi sau da yawa bayan mutuwa damar tabbatar da ganewar asali. ganewar asali.

Cutar Creutzfeld-Jakob ita ce kawai cutar ɗan adam da za ta iya zama sanadin kwayoyin halitta (saboda maye gurbi a cikin kwayar halittar da ke ɓoye furotin na prion, maye gurbin E200K shine ya fi kowa), sanadin kamuwa da cuta (na biyu zuwa kamuwa da cuta) ko na ɗan lokaci (na faruwa bazuwar, ba tare da maye gurbi ko fallasa ga wani exogenous prion samu).

Duk da haka, nau'in nau'i na wucin gadi shine ya fi kowa: yana da kashi 85 cikin 60 na duk wani nau'i na spongiform encephalopathies (TSEs) da aka gano kowace shekara. A wannan yanayin, cutar yawanci tana bayyana bayan shekaru 6 kuma tana ci gaba a cikin kusan watanni 50. Lokacin da cutar ta kasance ta kwayoyin halitta ko kamuwa da cuta, alamun sun kasance a baya kuma suna ci gaba a hankali. A cikin nau'ikan kamuwa da cuta, lokacin shiryawa na iya zama tsayi sosai kuma ya wuce shekaru XNUMX.

Protein prion (PrPc) furotin ne na ilimin lissafi wanda aka samo shi ta hanyar kiyayewa sosai a yawancin nau'ikan. A cikin jijiyoyi na kwakwalwa, furotin na prion zai iya zama pathogenic ta hanyar canza yanayinsa mai girma uku: yana ninka kansa sosai, wanda ya sa ya zama hydrophobic, mai narkewa da juriya ga lalacewa. Sannan ana kiransa “scrapie” prion protein (PrPsc). PrPsc suna taruwa tare da juna kuma suna samar da adibas waɗanda ke ninka ciki da wajen ƙwayoyin kwakwalwa, suna rushe aikinsu da hanyoyin tsira.

A cikin wannan nau'i mara kyau, furotin prion shima yana da ikon watsa nau'in halittarsa: akan hulɗa da PrPsc, furotin prion na al'ada yana ɗaukar wani abu mara kyau. Wannan shine tasirin domino.

Haɗarin watsawa tsakanin mutane

Watsawa tsakanin mutum-mutumi na cututtukan prion yana yiwuwa tare da dashen nama ko bin gudanar da kwayoyin girma. Mafi yawan ƙwayoyin cuta suna fitowa daga tsarin kulawa na tsakiya da ido. A ɗan ƙarami, ruwan cerebrospinal, jini da wasu gabobin (ƙoda, huhu, da sauransu) kuma na iya watsa prion mara kyau.

Hadarin abinci

 An yi zargin watsa prion daga shanu zuwa ga mutane ta hanyar cin gurɓataccen abinci a cikin 1996, a lokacin rikicin "mahaukacin saniya". Shekaru da yawa yanzu, annobar cutar sankarar mahaifa (BSE) ta afkawa garke a cikin Burtaniya3. Yaduwar wannan cuta ta prion, wacce ta shafi dubun dubatar dabbobi a kowace shekara, babu shakka ta kasance ne saboda amfani da abincin dabbobi, da ake samu daga gawa da kuma rashin gurbacewa. Asalin sa, duk da haka, ana ci gaba da muhawara.

A yau, babu takamaiman magani ga cututtukan prion. Magungunan da za a iya ba da izini kawai su ne waɗanda za su iya sauƙaƙe ko iyakance nau'ikan alamun cutar. Ana ba da tallafin likita, zamantakewa da tunani ga marasa lafiya da danginsu ta Sashin Tallafi na CJD na ƙasa. Neman magunguna da nufin hana jujjuyawar PrPc, inganta kawar da nau'ikan furotin mara kyau da iyakance yaduwar sa yana da bege. Wani jagora mai ban sha'awa yana hari PDK1, ɗayan masu shiga tsakani na salula da ke da hannu yayin kamuwa da cuta. Hana shi zai sa ya yiwu duka biyu su hana al'amuran juzu'i ta hanyar haɓaka ɓarna na PrPc, da kuma rage sakamakon maimaitawar sa akan rayuwa na neurons.