Prion Disease hi eng nge ni? Eng nge a nih chiang takin i zawng chhuak ang u!

He 'Prion Disease' hi i hre ngai em? He hming hi i tan chuan a thar deuh pawh a ni mai thei. A chhan chu hei hi khawvela natna chi hrang hrang awm lo tak tak pawl a nih vang a ni. Amaherawhchu, he natna hi kan hriat chian a pawimawh hle a, kan thluak hi direct-in a nghawng a, a symptoms te chu a na nghal vat thin. Mihringah pawh a awm thei a, rannungah pawh a awm thei.

Prion Disease hi eng nge ni?

A awlsam zawngin sawi ila, prion natna hi kan thluaka protein pangngai atanga lo chhuak natna pawl a ni a, chu chu a pangngai lo takin protein tha lo ``prions'' an tih ah a inthlak a ni.Kan taksaa cell tha tak chu rang takin cell tha lo, inthlak danglam takah a chang ta ang tih han ngaihtuah teh.

Heng prion protein pangngai lo te hi thluaka an lo awm khawm chuan thluak cell te chu an tichhe tan thin. A dik tak chuan hei hi neurodegenerative disease a ni . Hun a kal zel a, he chhiatna hian thluak hnathawh a ti tlahniam zauh zauh thei a, chu chuan dementia ang chi dinhmun a thlen thei a ni. Chumi awmzia chu i hriatrengna a hloh a, ngaihtuah harsa i ti a, i hna i thawk thei lo tihna a ni. He natna hlauhawm ber chu heng symptoms te hi a intan thut a, a kal chak hle a ni. He natna hian khawvel pumah mi maktaduai khat zinga pakhat vel a vei a ni. Hei hian a tlem hle tihna a ni.

Prion natna hi vanduaithlak takin thihna thlen thei natna a ni . Chumi awmzia chu vawikhat natna a lo awm tawh chuan tihdam a harsa tihna a ni. Doctor-te chuan a natna lan chhuah dan thunun an tum a, damlo chu a nuam thei ang bera siam an tum a ni. Tin, damlo chu natnain an nunah, an chhungkua leh anmahni enkawltute chunga nghawng a neihte hmachhawn turin an pui bawk.

Engtin nge heng prion natna te hi an lo awm?

Miin prion natna a vei theih dan tlangpui pathum a awm a.

1. Genetic mutation (hei hi familial prion disease an ti a) rochun hmangin.

2. Infection hmangin (hei hi acquired prion disease an ti a).

3. Mahse, a tam zawkah chuan he natna hi genetic cause emaw pawn lam infection emaw awm lovin a lo awm thin . Researcher leh doctor te chuan hei hi ‘sporadic prion disease’ an ti a.

Tunah chuan heng hmanrua tinte hi chipchiar deuhin han thlir ila.

Prion natna chi hrang hrang

Hei hi prion natna vei tam ber a ni. Heta thil thleng chu, chhan hriat lohvin, thluaka protein pangngai chu a chunga kan sawi tak ‘prion’ tha lo takah a chang nghal vek a ni. Hetiang thil a thlen chhan dik tak hi hriat a la ni lo.

He category-a tel natna lian ber berte chu:

• Creutzfeldt-Jakob disease (CJD) : Hei hi sporadic prion natna zinga 85% a ni. A chi hman tlanglawn ber a ni.
• Sporadic thihna thlen thei mut theih lohna: Hei hi a tlem hle. Hereditary fatal insomnia aiin a tlem zawk.
• Variable protease-sensitive prionopathy : Hei hi prion natna sporadic tak a ni bawk.

Chhungkaw Prion natna te

Hetiang chi prion natna hi kan gene-a `PRNP` an tih gene a mutation vang a ni. He natna hi mutation hi nu emaw pa emaw atanga rochun a nih pawhin a awm thei (hei hi `autosomal dominant inheritance` an ti a ni). `PRNP` gene-ah hian mutation hrang hrang 50 chuang hmuhchhuah a ni tawh a, mutation tin hian hereditary prion disease chi hrang hrang a thlen thei a ni.

Hetiang category-a tel natna thenkhat:

• Chhungkaw Creutzfeldt-Jakob natna (CJD) .
• Gerstmann-Sträussler-Scheinker (GSS) syndrome : Hei hi a awm lo hle a, a tlem hle. Khawvel puma mi maktaduai 100 zinga mi pakhat atanga mi sawm velah a awm thin.
• Fatal familiar insomnia : Hei hi GSS syndrome aiin a tlem zawk. Khawvel pumah chhungkaw 50 atanga 70 vel chauhvin he natna thlentu genetic mutation hi an keng tel a ni.
• PRNP gene-a mutations avanga lo awm prion natna dang pawh an sawi tel bawk. Entirnan, British chhungkaw pakhata mi 11 chuan ril\am, sensory autonomic neuropathy, seizures, leh dementia ang chi natna lan chhuahna an nei a. He natna hi autonomic neuropathy tia koh hian i thisen sang, heart rate, leh bowel leh urinary incontinence te ang chi thil a nghawng a ni.

Acquired Prion natna a ni

Hei hi prion natna vei theihna kawng awm lo ber a ni. Hetah hian mi pakhatin prions (entir nan, ‘mad cow disease’ vei bawng sa) ei emaw, damdawi hmanraw bawlhhlawh (entir nan, surgery lai) hmanga natna hi a kai thei a ni.

• Kuru hi hetiang chi infectious neurodegenerative disease vei hmuhchhuah leh zirchian hmasak ber a ni. Papua New Guinea rama Fore mite zingah hmuh a ni. An ritual thenkhat (mihring sa ei ang chi) hmanga thehdarh niin an ngai.

Vanneihthlak takin tunlai damdawi hmanraw tihfai dan khauh tak leh ei leh in himna ngaihsakna hian hetiang hian prion natna darh zel hi nasa takin a ti tlem a ni.

Prion natna zingah eng nge hlauhawm ber?

Dik tak chuan prion natna zawng zawng hi thihna thlen thei a ni . Chu chu natna i kai chuan i thi ngei ang tihna a ni. A tlangpuiin damlo chu prion natna lan chhuah tirh atanga thla engemaw zat atanga kum thum chhungin a thi thin.

Prion natna lan chhuah dan chu engte nge ni?

Prion natna lan chhuah dan hi a inang lo thei a, chu chu prion natna chi hrang hrang leh thluak peng eng nge a nghawng dan a zirin a ni. Mahse a tlangpuiin prion natna vei te hian an nervous system-ah harsatna an nei a, chu chu zawi zawiin a na zual zel a ni.

Hengte hi a lan chhuah dan tlangpui thenkhat chu an ni:

• Kal harsa, tluk (`Ataxia`) .
• Thusawi harsa, tawngkam slurring (`Aphasia`) .
• Buaina, ngaihtuahna buai (disorientation).
• Mu thei lo
• Hriatrengna hloh, ngaihtuahna leh thutlukna siam harsa
• Zawi zawia che emaw, taksa ruh a nghet lo emaw (`Hypokinetic movement disorders`) .
• A rilru a buai em em a, a rilru a buai em em bawk a. (`Myoclonus`) .
• Mihring nun inthlak danglam (e.g., thinrim, buai) .
• Rilru lama harsatna (e.g. lungkhamna, lungngaihna, leh a chang chuan mit lama thil hmuh theih loh pawh) .

Ngaihtuah teh, i hnaih bertu mi pakhat chu a lo danglam ta thut mai a. Thil a theihnghilh thin a, a sawi thin angin a tawng thei lova, kal a harsat thin. Heng symptoms te hi engtiang chiahin nge a lungchhiatthlak?

Prion natna hian eng harsatna nge a thlen?

Prion natna avanga harsatna awmte hi a inzawm tlat thei a, chu chuan harsatna hrang hrang a siam a, chu chuan damlo chauh ni lovin, anmahni enkawltu chhungte leh ṭhiante chungah pawh nghawng thui tak a nei thei a ni.

Heng harsatnate hi natna lan chhuah tirh atanga thla engemaw zat emaw, kum khat emaw chhungin a lo awm thei a ni:

• Mahni hna thawh theih lohna
• Dementia ( hriatrengna hloh vek tawh mai) .
• Thusawi thiam lohna (`Mutism`) .
• Coma (hei hi final stage a ni) .

Miin prion natna a neih chuan mi dangah a innghat thuai thin. Chhungkaw leh midangte chu anmahni enkawl tur leh pui turin an awm reng a ni. Chutih laiin, an hmangaih tak takte chuan an hriatrengna a hloh a, an tawng thei lo va, an mizia a inthlak danglam lai en chu enkawltute tan chuan lungkhamna nasa tak a ni.

Engvangin nge heng prion natna te hi a lo awm?

Hei hi scientific lam thil deuh a ni a, mahse a awlsam zawngin ka sawi ang. Prion natna hi kan thluaka prion protein pangngai (`PrPc` tia koh) chu protein pangngai lo, misfolded (`PrPSc` tia koh - chu chu prion a ni)-ah a chantir atanga intan a ni.

Heng prions pangngai lo (`PrPSc`) te hian prion protein pangngai (`PrPc`) te chu an inzawm khawm tan a, a nih loh leh an inzawm khawm tan a, prion pangngai lo ah an chantir ta a ni. Apple tha lo tak chu apple tha tak tak pile-a paih ang mai a ni a, a bak zawng pawh a chhe vek ang. Hun a kal zel a, heng prion protein pangngai lo te hian thluaka nerve cell te chu a tichhe emaw, a tichhia emaw a ni. Chu chu i kal thei lo va, i ngaihtuah thei lo va, i tawng mumal thei lo bawk.

Engtin nge prion natna hi hriat chhuah a nih?

Doctor-te chuan prion natna an hriat theih nan a hnuaia test te hi an hmang thei ang:

• Thisen test leh lumbar puncture : Genetic mutation nei, inherited prion diseases nei te chu an thisen emaw cerebrospinal fluid (thluak leh spinal cord vel tui)-a natna emaw chhiatna emaw biomarker an neih leh neih loh enfiah thin an ni.
• Brain MRI scan : Hei hian thluak thlalak chiang tak tak a la thei a, chuvangin doctor ten prion natna chhinchhiahna an zawng thei a ni.
• Electroencephalogram (EEG) : Hei hian thluaka electric hnathawh dan a teh a.
• Real-time quaking-induced conversion assay (RT-QuIC) : He test-ah hian pathologist-te chuan spinal fluid-ah prions an zawng a. Hei hi test thar tak, dik tak a ni.

Prion natna damdawi a awm em?

Vanduaithlak takin doctor-te chuan prion natna damdawi emaw, natna kal zel dan tur thunun thei tur damdawi emaw an la hmu lo. Prion natna hi nunna atana hlauhawm natna a ni . Mi tam zawk chu prion natna an vei tih hmuhchhuah a nih atanga thla engemaw zat atanga kum tam tak chhungin an thi thin.

Enkawlnaah hian palliative care lam a ngaih pawimawh ber a ni . Hei hian symptoms control a, damlo chu a nuam thei ang bera leh natna nei lo tura siam tihna a ni. Entirnan, doctor-te chuan damdawi an pe thei a, chu chu:

• Myoclonus tan antiseizure damdawi emaw muscle relaxant emaw a ni.
• Natna tur opioids.

Prion natna vei hian eng ang hmalam hun nge a neih?

Hetih lai hian prion natna tihdamna leh enkawlna a la awm lo. Amaherawhchu, zirchiangtute chuan hei hi kawng hnihin an chhui mek a, hei hian natna hi a hmaa hriatchhuah theihna tur leh nakin lawka enkawl dan tur a thlen thei a ni.

A chunga kan sawi tak `RT-QuIC` test hian thluaka chhiatna tihdanglam theih loh a thlen hmain prion natna a hriat theih nan a pui a ni. Zirchiangtute chuan heng protein pangngai lo siam chhuah tihtawp dan tur leh protein pangngai lo awm lo tura ven dan tur zawngin prions an zirchiang mek bawk.

Prion natna ka neih chuan engtin nge ka tih ang?

Prion natna hi a zual chak em em a, nangmah ngeiin hna thawh chu i tan a harsa mai thei. He natna hi i neih chuan ``advance directive'' tihfel a tha.

Advance directive hi dan anga document a ni. Entirnan:

• Living wills : Prion natna ang chi natna hlauhawm tak i neih chuan he document hian i chunga thil thleng tur i duh (e.g. enkawlna thenkhat i duh leh duh loh) a tarlang thei a ni.
• Durable power of attorney for healthcare (DPA): He document hian nangmah ngeiin i thusawi theih tawh loh chuan i healthcare chungchanga thutlukna siamtu tur hming a tarlang thei a ni.

Hetiang thilte hi ruahmanna siam lawk hian, nakin lawka nang leh i chhungte’n an tawn tur lungkhamna chu i tihziaawm thei a ni.

Chhungkaw zinga mi tu emaw hian hereditary prion disease a neih chuan engtin nge ni ang?

A châng chuan, miten an genetic mutations an rochun a, chu chuan prion natna vei theihna a tipung a ni. Genetic test hmangin prion natna thlentu gene mutation pakhat emaw a aia tam emaw i nei em tih a hriat theih. Heng test te hian i risk bik a lantir thei bawk.

Natna thihna thlen thei genetic test neih hi mimal thutlukna a ni. Mi thenkhat chuan hlauhawm an nih leh nih loh hriat an duh lo. Genetic test neih i duh chuan doctor hnenah referral dil rawh. Entirnan, United States-ah hian hei hi ``National Prion Disease Pathology Surveillance Center`` ang chi institution a awm a. Sri Lanka-ah pawh hemi chungchangah hian thurawn pe thei specialist doctor an awm bawk.

Chhungkaw member prion natna vei chu engtin nge i enkawl?

Hetiang natna hian i nitin nun zawng zawng a tidanglam thei a ni. I hmangaih tak chuan natna hlauhawm tak a nei tih hriat avanga mangang leh lungngaihna i hmachhawn lai hian, i chhungte i enkawl dan tur pawh i ruahman a ngai bawk. Rawtna thenkhat chu hetiang hi a ni:

• Enkawlna ruahmanna siam rawh : I dinhmun azirin i chhungte chuan chenna inah enkawl an mamawh thei a, a nih loh leh in lamah enkawl an mamawh thei a, chhungte, ṭhiante leh mimal hriselna lama ṭanpuitute rinchhanin. I hmangaih tak chuan ni tin hna thawhnaah ṭanpuina a mamawh mai thei a, chu chu bathroom-a kal leh chaw ei te hi a ni.
• Hospice care ruahmanna siam rawh : Prion natna hi natna hlauhawm tak a ni a, a lo awm nghal thei a, a na zual nghal vat thei bawk. Hospice care i ruahman lawk chuan i hmangaih tak chuan an mamawh hunah a dawng thei ang.
• Environment muang tak siam rawh : Prion natna vei te hi thil thleng nghal (e.g., mi khawih, thawm ri nasa tak hriat, mipui tamna hmuna awm) ah hian a danglam bik hle. An buai emaw, an thinrim emaw pawh a ni thei. An chhehvel enkawl hian an rilru hahdam leh hahdam taka awm turin a pui thei a ni.

Hetiang hunah hian enkawltu tan pawh mahni inngaihtuah a pawimawh hle. Chawlh hahdam leh puihna i mamawh bawk. He phurrit hi nangmah chauhin phur tum suh.

Engtikah nge Doctor ka hmuh ang?

Prion natna lanchhuahna hi a na zual nghal vat tlangpui. Nangmah emaw, i chhungte zingah emaw prion natna i neih chuan i doctor-te nen thawkdunin natnain a nghawng dan tur i enkawl tur a ni.

A bik takin, a hmaa kan sawi tawh ang chi (hriatrengna hloh, kal harsa, tawng harsa, mize inthlak danglam) te hi a lo intan nghal a, a na zual nghal vat a nih chuan damdawi lam thurawn zawng nghal rawh.

Doctor hnenah eng zawhna nge ka zawh ang?

Nangmah emaw, i chhungte zinga tu emaw chuan prion natna a vei tih hriat chu thil mak tak a ni thei. Tunah hian nang leh i chhungte hian harsatna tam tak in nei a, natna a zual zel chuan thil tam zawk a lo chhuak ang. Eng nge i beisei tih hriat theihna tûr zawhna ṭhenkhat chu hetiang hi a ni:

• Engtin nge prion natna ka nei tih i hriat?
• Eng prion natna nge ka neih?
• Chu chuan engtin nge min nghawng?
• Eng nge ka tih leh ang?
• Ka chhungte enkawl turin eng nge ka tih theih ang?
• Ka chhungte hian genetic counseling leh testing an nei tur em ni?

A tawp berah chuan thil hriat reng tur

Prion natna hi natna hlauhawm tak, thihna thlen thei pawl pakhat a ni a, i thluak a nghawng a ni. Prion symptoms hi a lo lang nghal thei a, a zual nghal vat thei bawk. Nangmah emaw, i hmangaih tu emaw hian a tihdam theih lohna tur damdawi leh enkawlna nei lo natna a nei tih hriat chu a lungchhiatthlak thei hle.

Mahse, symptoms enkawl dan tur enkawlna a awm . Tin, prion natna vei te leh an enkawltute tanpui nan programme leh service hrang hrang a awm bawk. Nangmah emaw, i hmangaih tu emaw hian prion natna a neih chuan zawhna zawh leh tanpuina zawng hlau suh. Nangmah chauh i ni lo.

` Prion natna, Thluak natna, Thluak lam natna, Dementia, CJD, Genetic Disease