I hriatrengnaah eng thil nge thleng? Creutzfeldt-Jakob Disease (CJD) chungchang hi i zir dawn em ni?

"Aw, hei pawh hi ka theihnghilh ve ta!" Thil tê tê theihnghilh leh a châng chuan inbuatsaih hi thil pangngai a ni thei. Mahse, hriatrengna hloh zauh zauh leh nungchang inthlak danglam ang chi thilte pawh hi natna khirh tak chhinchhiahna a ni thei bawk. Vawiin hian natna hmuh tur awm lo, mahse natna khirh tak, thluak tibuaitu chungchang kan sawi dawn a ni. Chu chu Creutzfeldt-Jakob natna, a nih loh leh a tawi zawnga kan sawi angin CJD a ni.

He CJD hi eng nge ni? A awlsam zawngin...

Ngaihtuah teh, kan thluak hi machine complex tak a ni. He machine hian hna a thawh that theih nan a peng te te, cell an tih te hi a hrisel a ngai a ni. CJD hi natna hmuh tur awm lo, na tak a ni a, zawi zawiin kan thluak cell te a tichhia a ni. Hei hi a chhan ber chu protein chi danglam tak ``prions`` an tih hi a ni.

Tunah chuan heng ‘prion’ te hi eng nge an nih i ngaihtuah mai thei. Kan taksaah, a bikin thluak ah hian protein an tih te hi a awm a. Hengte hi machine te tak te ang mai an ni a, an pianzia bik an nei a, chutiang pianziaah chuan an awm chauhvin an hna chu an thawk tha thei a ni. Origami ang maiin han ngaihtuah teh. I fold dik chuan rannung emaw pangpar mawi tak emaw i siam thei a ni. Mahse, i fold dik loh chuan? A thawk lo. Chu chu heng protein te hi chutiang chu a ni. A chang chuan heng protein te hi an fold dik lo thin. Chutiang protein fold dik lo chu ‘prions’ kan ti thin.

Heng misfolded ‘prions’ te hi thluak cell chhungah an pung khawm tan thin. Chu cell te chu an khawlkhawm a, an tichhia a ni. Nitin bawlhhlawh bawm khat ang mai a ni. Hei vang hian CJD vei te hian dementia symptoms , hriatrengna hloh leh buaina ang chi an nei thin. Tin, nungchang inthlak danglamna leh kal harsa an tawk thei bawk.

A pawimawh ber chu CJD hi a thihpui fo thin hi a ni. Damdawi a la awm lo va, natna zual zel tur tihkhawtlai dan tur a awm lo. Amaherawhchu, damlo nun nuam zawk leh natna tihziaawm thei tur enkawlna leh enkawlna a awm.

Hei hi natna hmuh tur awm lo tak a ni. Khawvel pumah he natna hi kum tin mi maktaduai khat zinga mi pakhat atanga pahnih velin a vei thin. United States ang ramah pawh kum tin CJD vei mi 350 vel chauh an awm.

Hei hi a danglamna pakhat a awm a, chu chu ``variant CJD`` (or ``vCJD``) tih a ni. A tlem hle bawk. Bawnghnute ei hian natna ``bovine spongiform encephalopathy`` (BSE) a nei a, hei hi bawnghnute natna a ni.

CJD natna lan chhuah dante chu engte nge ni? A bul a\angin han thlir ila.

CJD symptoms hi a lang zauh zauh tlangpui. Mahse, natna a zual zel chuan heng symptoms te hi a pung chak thei hle. Natna lanchhuahna bulpui ber, natna lan chhuah tirh atanga a hnu lam thlenga kan thlir ho ang u:

• Theihnghilh leh hriatrengna lama harsatna:Thil tenau te theihnghilh atanga tan a ni. I thian hming emaw, kawngpui emaw, hna i thawh lai emaw ang chi thil. Zawi zawiin hei hi a na zual sauh sauh a.
• Buaina leh ngaihtuahna buai: I awmna hmun, engtik lai nge tih, tute nge i bula awm tih i hre thei lo.
• Nungchang leh mize inthlak danglamna: I nihna ang kha i ni lo mai thei. I thinrim awlsam a, i lungngai a, a nih loh leh rilru natna zawng zawng i hloh thei bawk.
• Mit lama harsatna, i thil hmuh hriatthiam harsa: I mit a chak lo a, i thil hmuh chu i hre thei lo.
• Hallucinations emaw delusions: Thil awm lo hmuh emaw hriat emaw, mi pakhatin a tihnat tum che tih rinna dik lo neih emaw.
• Coordination lama harsatna (ataxia): I kal tha thei lo a, i ke leh ke i thunun harsa a, zu rui ang maiin i inher thei bawk.
• Taksa inthlauhna lama harsatna: Ding lai emaw, kal lai emawa tlu.
• Muscle contraction (`myoclonus`) leh muscle stiffness (`dystonia`): Kut emaw ke emaw a hrual nghal vat a, taksa ruh a tight ang.
• Seizures: Fit ang maiin a lo thleng thei.
• Paralysis: Taksa peng thenkhat chu a paralyze thin.
• Muscle atrophy: Taksa thau (muscle mass) a bo.

Heng symptoms te hi pakhat hnu pakhatin a lang thei a, engemaw zat chu a awm dun thei bawk. A pawimawh ber chu heng symptoms te hi i neih chuan a rang thei ang bera doctor pan hi a ni.

Eng thilin nge CJD hi a thlen? Chu 'prion' te kha i hrechhuak leh ang u

Kan sawi tawh ang khan CJD thlentu ber chu proteins fold dik lo ``prions'' an tih hi a ni.Heng ``prions'' chungchanga hlauhawm ber chu protein pangngai, hrisel tak tak pawh an fold dik lo thei tih hi a ni. Apple tha lo pakhatin apple dang zawng zawng a ti chhe thei ang bawkin.

Thluak cell te hian heng ‘prion’ fold dik lo te hi an paih chhuak thei lo. Chuvângin, an pung khâwm a, a tâwpah chuan thluak cell-te chu an tichhia a ni. Heng ‘prions’ te hi thluak zawng zawnga an darh zel chuan natna hi a zual chak hle.

CJD chi hrang hrang a awm em?

Ni e, CJD hi chi hrang hrang lian tak tak a awm a:

• Sporadic CJD: Hei hi CJD chi hrang hrang zingah chuan a tam ber a ni. A chhan dik tak erawh hriat a la ni lo. A lo thleng nghal a, a chhan chiang tak a awm lo.
• Genetic CJD: Hei hi rochun a ni. Nu leh pa pakhat emaw, nu leh pa pahnih emaw atanga rochun gene chhia avanga lo awm a ni. Hei hi chi hnih a awm a, chungte chu Gerstmann-Sträussler-Scheinker (GSS) syndrome leh fatal family insomnia . Hengte hi a tricky deuh a, mahse condition bik tak tak an ni.
• CJD lei tawh (`CJD lei`): 1.1.Hei hi pawn lam thil avanga thleng thei a ni. Entirnan, CJD vei atanga taksa peng thlak emaw, tissue thlak emaw, surgical instrument bawlhhlawh hman emaw hmangin a thleng thei a ni. Mahse hei hi a tlem hle. Tunlai hian hemi chungchangah hian ngaihtuahna a tam hle.
• Variant CJD (vCJD): Hemi chungchang hi kan sawi tawh a. Mad cow disease (BSE) vei tawh bawnghnute ei avanga lo awm a ni. BSE thlentu prions hian mihring bakah rannung dangte pawh a kai thei a ni.

Tute nge CJD vei theihna chance sang zawk?

Tu pawhin CJD an la thei. A chang chuan, i taksaa ‘prions’ te hi kum tam tak chhung chu natna lan chhuah lohvin a awm thei. Mahse, symptoms a lan chhuah chuan thluak a chhiat avangin natna chu a na nghal thei a ni.

He natna hian kum 50 leh 80 inkar mi a vei tlangpui a, mahse, genetic CJD type hi kum 30 leh 50 inkar a lo lang thei tlangpui.

CJD hi natna hrik kai theih a ni em?

Hei hi mi tam tak tan chuan harsatna a ni. CJD hi casual contact hmanga darh a ni lo va, kut inthlak, inbiakna, ei dun ang chi te hi a ni. CJD hi mi pakhat atanga mi dang hnena a darh theihna awmchhun chu CJD vei atanga taksa peng emaw tissue emaw transplant emaw, a vei tawh atanga hormone thenkhat hman emaw a ni.

`vCJD` an tih chi hi thisen pekna hmanga kai theih niin an sawi a, mahse chu chu a tlem hle, a tlem hle. `vCJD` an tih natna ngei pawh hi a tlem hle.

Doctor-te hian engtin nge CJD hi an hriat theih?

Doctor chuan CJD hi i symptoms leh signs te uluk taka enfiahin a hmuchhuak thin. Taksa endikna bakah hian neurological exam pawh an nei dawn a ni. I thluak hnathawhna lama harsatna awmte hriat tumna atan hna awlsam tak tak thawk turin an ti ang che.

Specialist-te chuan CJD hi ‘transmissible spongiform encephalopathy’ tiin an dah a ni. He hming hi a buaithlak deuhin a lang thei a, mahse ‘prions’-in a tihchhiat thluak chu microscope hmanga a lan dan a kawk a ni. Chu chu sponge bumps nei ang mai a ni tihna a ni.

CJD hmuhchhuah a nih leh nih loh finfiah nan MRI scan emaw, spinal tap (lumbar puncture) emaw ang chi test an ti thin.

Chu bâkah, doctor-te chuan CJD ang chi natna dangte chu paih chhuah nân leh CJD zirchian belh nân hetiang test hi an hmang thei bawk:

• Thisen test a ni
• Genetic testing - a bik takin type pakhat chu hereditary a nih leh nih loh enfiah nan
• Electroencephalogram (EEG) hmanga tih a ni.
• Urinalysis tih a ni
• Brain biopsy - Hei hi tih a ni tlem hle a, test dang zawng zawngin diagnosis chiang tak a pe thei lo a nih chauhvin tih a ni.

CJD enkawlna tur a awm em?

Vanduaithlak takin CJD damdawi a awm lo va, natna darh zel tur venna tur damdawi a awm lo va, a kal chakna tikhawtlai thei tur damdawi a awm lo bawk . Doctor-te tih theih awmchhun chu a natna lan chhuah dan avanga lungawi lohna tihziaawmna tura tanpui hi a ni. Hei hi palliative care an ti a .

Entîr nân, damdawi chu seizure, nungchang inthlâk danglamna, emaw, thunun theih loh taksa ruh tliak enkawlna atân emaw pêk theih a ni. Mahse, heng enkawlnate hlawkna hi a tlem hle.

Hetiang dinhmun hi a na zual nghal vat thei. Chuvangin, nang leh i hmangaih tak takte tan supportive care options hi a pawimawh hle. He natna hi a tawp berah chuan hospice service hian a pui thei hle.

A châng chuan, i tlin chuan i medical team chuan enkawlna thar zir chianna clinical trial-a tel tûrin an rawt thei che a ni.

CJD vei hian eng ang hmalam hun nge a beisei theih ang?

CJD hi tihdam theih loh a nih avangin damdawi a neih loh avangin he natna vei tan chuan a hmalam hun chu a thim hle. Symptoms a lo lan tan chuan rei vak lovah mahni inrintawkna neia hnathawh theihna te, che vel theihna te, leh thusawi theihna te chu a bo ta a ni.

Hrilhfiahna hi a thleng thuai thei a, chuvangin i duh leh mamawh phuhruk a nih theih nan i medical team te nen ruahmanna siam hmasak a pawimawh hle. Dik tak chuan, damdawi lam harsatna i neih leh neih loh pawh ngaihtuah lovin, mi zawng zawng tan `advance directive` neih hi a tha hle. Chumi awmzia chu i duhzawng i sawi theih loh chuan eng damdawi lam enkawlna nge i duh tih i ziak lawk tihna a ni. A bik takin CJD ang natna darh chak takah hian he ruahmanna hi a tir lamah neih a pawimawh hle.

He natna hi a nasat zia ngaihtuah chuan, nang leh i chhungte chu heng inthlak danglamnate hmachhawn tur leh rilru lama chak taka awm theihna tura pui turin rilru lam hriselna lama mithiamte hnen atanga thurawn lak hi a tha hle.

CJD vei dam rei zawng chu engzat nge ni ang?

CJD vei tam zawk hi an hmuhchhuah atanga thla engemaw zat emaw kum khat emaw chhungin an thi thin. A danglamna awm chhun chu CJD genetic form a ni a, miten an natna lan chhuah tirh atanga kum khat atanga kum sawm thleng an dam thei a ni.

I doctor chuan i dinhmun chungchangah thu chiang tak, tunlai tak tak a pe thei che a ni. Statistics hian mi zawng zawng hi a nghawng inang vek lo tih hre reng ang che.

CJD hi ven theih a ni em?

CJD chi tam zawk hi chu ven theih a ni lo. Exception awm chhun chu `vCJD` tih type hi a ni. `BSE` (mad cow disease) vei bawnghnute ei atanga lo chhuak a ni.

Ran test hian BSE vei bawngte ei tur supply chain-a an luh loh nan a pui a ni. Mahse, sa test loh leh siam dik loh chuan hlauhawmna a la nei thei tho. Hriselna tur chuan sa test loh leh inrelbawl loh, a bik takin thluak peng emaw ruhro emaw ei loh tur a ni.

A tawp berah chuan thil hriat reng tur

CJD diagnosis hi nun tidanglam thei thil a ni. Nang leh i chhehvel khawvel chu mit khap kar lek chhungin i bo tawh ang maiin a lang thei. Chutiang inthlâk danglamna chu nang leh i hmangaihte tân chuan tuar harsa tak a ni thei a.

Eng nge i beisei ang tih leh engtin nge i hmachhawn ang tih i hriat chian loh chuan i medical team te chu tanpui turin an awm a ni. Anni hian ruahmanna siam lawk leh thil lo thleng tur atana inbuatsaih turin an pui thei che a ni. Nangmah chauh i ni lo tih hre reng ang che.

` CJD, Creutzfeldt-Jakob Natna, prion, thluak natna, thluak lam natna, hriatrengna hloh, dementia, mad cow natna