Ese hari umuntu wa hafi yawe ufite izi mpinduka? Reka twige ku ndwara ya Pick

Akenshi twumva dutangaye iyo tubonye impinduka zitunguranye mu myitwarire no mu mvugo bya bamwe mu bo dukunda, si byo se? Uyu munsi tugiye kuvuga ku ndwara ishobora kuba ari yo yatumye habaho izo mpinduka. Nubwo iyi ari ingingo igoye, reka tugerageze kuyisobanukirwa mu buryo bworoshye.

Indwara ya Pick ni iki? Itandukaniye he n'indwara ya Niemann-Pick?

Mu magambo make, indwara ya Pick ni indwara igira ingaruka ku bwonko, ikica uturemangingo buhoro buhoro. Ni ubwoko bwa dementia bwitwa frontotemporal dementia (FTD) . Ikunze kugaragara cyane ku bantu bari munsi y'imyaka 65. Mu bihe byashize, dementia yo mu gihe cyo kuruhuka ubwayo yitwaga indwara ya Pick. Ariko ubu, abaganga bakoresha iri zina gusa mu bihe bimwe na bimwe byujuje ibisabwa.

Noneho ushobora kuba urimo kwibaza uti "Ese ... indwara ya Pick ni imwe n'indwara ya Niemann-Pick?" Oya, ni indwara ebyiri zitandukanye rwose. Nubwo igice cya "Pick" cy'izina ari kimwe, indwara zombi ntabwo zifitanye isano.

• Indwara ya Pick: Yitiriwe Arnold Pick, inzobere mu ndwara zo mu mutwe akaba n'umuganga wo mu mutwe wo muri Tchèque wamenye iyi ndwara bwa mbere mu 1892. Ni ubwoko bwa FTD (dementia yo mu mutwe imbere n'imbere), bivuze ko igira ingaruka ku bwonko bwawe gusa.
• Indwara ya Niemann-Pick: Yitiriwe abaganga babiri b'Abadage, Albert Niemann na Ludwig Pick, iyi ndwara irangwa no kwirundanya kw'ibinure mu mubiri ndetse n'ikibazo mu buryo bigenzurwa. Iyo ibinure byirundanyije, bishobora kugira ingaruka ku ngingo nyinshi, harimo ubwonko, umwijima, urwagashya, umworera w'amagufwa n'ibihaha.

Urabyumva? Kimwe ni ikintu kigarukira ku bwonko, ikindi ni ikintu kigira ingaruka ku bice bitandukanye by'umubiri.

Ni nde urwara iyi ndwara? Ni ku rugero rungana iki?

Indwara ya Pick ikunze kugaragara mu bantu bakiri bato kurusha izindi ndwara nka dementia. Ikunze kugaragara mu bantu bari hagati y’imyaka 50 na 60. Ariko, rimwe na rimwe ishobora kugaragara mu bantu bafite imyaka 20 cyangwa 80.

Hari ibimenyetso bimwe na bimwe bigaragaza ko iyi ndwara ishobora kugaragara mu miryango. Abashakashatsi bagaragaje nibura impinduka eshatu zijyanye nayo mu ngirabuzimafatizo. Ariko, akenshi iyi ndwara iba rimwe na rimwe. Ibi bivuze ko umuntu ashobora kuyirwara nta mateka y’umuryango.

Biragoye kuvuga neza uburyo iyi ndwara ikunze kugaragara. Impuguke zivuga ko hari abantu bari hagati ya 15 na 22 bandura kuri buri muntu 100.000. Ariko, hari gushidikanya ku kuri kw'iyi mibare, kuko iyo ndwara igoye cyane kuyisuzuma mu buzima bwe, kandi rimwe na rimwe ndetse no nyuma y'urupfu, biragoye kuyisuzuma. Bityo rero umubare nyawo w'abarwayi ushobora kuba munini.

Ni gute indwara ya Pick igira ingaruka ku mubiri?

Indwara ya Pick ni indwara itera indwara zo mu bwonko zifata imitsi, ubwoko bwa dementia bwitwa frontotemporal dementia (FTD). Muri make, uturemangingo tw’imitsi (neurons) mu bwonko dutangira guhagarara gukora buhoro buhoro. Ibice by’ubwonko byagizweho ingaruka bitangira kugabanuka (atrophy). Hanyuma, utakaza ubushobozi ibyo bice byakoreshaga mu kugenzura. Nubwo bifite aho bisa n’indwara ya Alzheimer, indwara ya Pick ikunze gutangirira mu bwana, kandi hari itandukaniro rikomeye.

Kubera ko indwara ya Pick ifata ibice bimwe na bimwe by'ubwonko gusa, ibimenyetso by'ingenzi ni impinduka mu myitwarire cyangwa mu bumenyi bw'ururimi . Akenshi, abantu barwaye indwara ya Pick ntibashobora kumenya ko bafite ikibazo cyangwa indwara. Ibi biterwa nuko ubwonko bwabo budashobora gutunganya amakuru yerekeye indwara yabo. Ibi byitwa "kubura ubushishozi."

Ni ibihe bimenyetso by'indwara ya Pick?

Indwara ya Pick ibaho mu buryo bubiri bw'ingenzi: bumwe ni indwara yo kwiheba mu buryo bw'imyitwarire (bvFTD) , ubundi ni indwara yo kwiheba mu buryo bw'ibanze (PPA), ifata ibice by'ubwonko bifitanye isano n'ururimi.

Indwara yo kwibagirwa indwara yo mu mutwe (bvFTD)

Ibimenyetso by'iyi ndwara ya bvFTD bishobora kugabanywamo ibyiciro bitandatu by'ingenzi:

• Kubura ubushobozi bwo kwirinda: "Kudakora ibintu" ni igice cy'ubwonko kitubwira ngo "ntugakore ibi." Iyo ibi bice byangiritse, ntidushobora kwiyima kuvuga cyangwa gukora ibintu bidakwiye.
• Kutagira "akayunguruzo" k'ibyo uvuga: Kuvuga ibiri mu mutima wawe, rimwe na rimwe mu buryo bubabaza abandi, ni ukutagira ikinyabupfura, cyangwa ntibikwiye. Urugero, gutuka umuntu mu buryo butunguranye mu nama y'umuryango.
• Kutubaha abandi: Bashobora kutubaha ubuzima bwite bw'abandi, bakabakoraho batabishaka, bashobora kugerageza kwitwara mu buryo budakwiriye mu mibonano mpuzabitsina, kandi bagahita barakara cyangwa bagahangayika.
• Ibikorwa n'imyitwarire idahwitse: Ibi bishobora kuba birimo gukoresha amafaranga mu buryo butajyanye n'igihe, ndetse wenda no kwiba mu maduka.

• Uburangare: Ibi bishobora kugaragara nk'ihungabana, ariko bitandukanye gato.
• Gutakaza ubushake mu kintu icyo ari cyo cyose: Gutakaza ubushake mu bintu wakundaga (ibyo ukunda, kumarana umwanya n'inshuti).
• Kwitandukanya n'abandi: Kwirinda kuba uri kumwe n'inshuti n'umuryango.
• Kugabanuka k'ubwitaho: Gutakaza ubushake bwo kwiyuhagira, guhindura imyenda no kugumana isuku.

• Gutakaza impuhwe:Ibi kandi byitwa "gutungurana mu buryo bw'amarangamutima." Biragoye kumva ibyiyumvo by'abandi. Ntibagaragaza inkunga mu kababaro cyangwa mu byishimo by'abandi nkuko byahoze mbere.

• Imyitwarire isaba imbaraga: Abantu bafite iyi ndwara bagaragaza imyitwarire itandukanye cyane n'iy'abandi.
• Ingendo zisubiramo: Gukora ingendo ntoya kenshi, nko gukoma amashyi, gukandagira ibirenge, no kugenda usubira inyuma.
• Imyitwarire igoye cyangwa ishingiye ku migenzo: kureba filime zimwe kenshi, gusoma ibitabo bimwe, gukusanya ubwoko bumwe bw'ibintu. Ibi birimo no kubika ibintu.
• Gusubiramo: Gusubiramo amajwi amwe, amagambo amwe n'interuro zimwe inshuro nyinshi.

• Impinduka mu mirire cyangwa imyitwarire ishingiye ku kanwa: Abantu barwaye iyi ndwara bashobora kugira ikibazo cyitwa "hyperorality," ari cyo gitera amatsiko adasanzwe ku bintu bifitanye isano n'akanwa.
• Impinduka mu mirire: Abantu bakunda kurya ibiryo bakunda cyane cyane ibyo kurya biryoshye, ibiryo biryoshye n'inzoga. Ibi bishobora gutuma umuntu yongera ibiro.
• Imyitwarire yo kunywa itabi rirenze urugero: Kunywa itabi rirenze urugero, kurya byinshi, nibindi. Nanone, gushakisha mu kanwa. Nubwo ibi ari ibisanzwe ku bana bato mu gihe bashakisha isi, ntabwo bikunze kugaragara ku bantu bakuru.
• Pica: Uku ni ukurya ibintu bidashobora kuribwa kandi bitari iby'intungamubiri (ibiceri, umwanda, utubuye).

• Gutakaza imikorere y'ubuyobozi ariko ubundi bushobozi ntibugire ingaruka mbi: "Imikorere y'ubuyobozi" yerekeza ku bushobozi bwo gutegura umunsi, gukemura ibibazo, no kurangiza ibintu. Nubwo ubwo bushobozi bugira ingaruka mbi ku bantu barwaye iyi ndwara, ubundi bushobozi, nko kwibuka no gutunganya amaso, ntabwo bugira ingaruka mbi cyane mu ntangiriro. Iki ni ikintu cy'ingenzi gitandukanya indwara yo kwibagirwa imbere mu gihe cy'amaso n'indwara ya Alzheimer.

Ahasia y'ibanze ikomeza iterambere (PPA)

PPA ishobora kandi guterwa n'indwara ya Pick. Ibi bituma umuntu atakaza ubumenyi bwe mu rurimi buhoro buhoro, nko kuvuga no kumva ibyo abandi bavuga. Nubwo hari ubwoko butatu bw'ingenzi bwa PPA, ubwoko bubiri bw'ingenzi bushobora kwifashishwa n'indwara ya Pick ni:

• Itandukaniro ridasanzwe rya PPA (nfvPPA): Muri ubu bwoko, biragoye guhuza amagambo no gukoresha ikibonezamvugo gikwiye. Nubwo amagambo amwe n'interuro zoroshye bishobora kumvikana, interuro zigoye kumva.
• Ubwoko bw'inyuguti za PPA (svPPA):Ubu bwoko bugorwa no guhitamo ijambo rikwiye cyangwa kumva icyo amagambo asobanura. Ibintu bimwe na bimwe bivugwa bishobora kutumvikana, kandi bikagorana kumva icyo abandi bavuga. Hashobora kubaho ibibazo mu gusoma no kwandika, ariko bashobora gusubiramo ibyo abandi bavuga muri ubwo buryo.

Tekereza, so wawe ahinduka mu buryo butunguranye nk'umuntu wahoze ari we, agatuka abantu bose, agakoresha amafaranga nabi, cyangwa agacuranga indirimbo imwe umunsi wose. Cyangwa, iyo uganiriye na we, amagambo ye arahindagurika, maze akumva asa n'aho atumva ibyo uvuga. Izi ni ingero z'ibintu twaganiriyeho.

Ni iki gitera iyi ndwara? Ese yandura?

Indwara ya Pick ni ubwoko bwa FTD (indwara yo kwibagirwa imbere n'imbere mu gihe cy'ihungabana) ifite impamvu yihariye. Ubwonko bwacu n'uturemangingo tw'imitsi birimo poroteyine yihariye yitwa tau . Izi poroteyine zigomba kugira imiterere yihariye, bitabaye ibyo ntizikora neza.

Iyo poroteyine za tau zidakora neza, zigafatana, kandi zikirundanya imbere mu mitsi, utwo turemangingo turangirika kandi tugasenyuka. Utwo turemangingo twangiritse twitwa "Pick cells." Turabyimba kandi tugasa n'ibumba. Uduce twa poroteyine za tau twivanze imbere mu mitsi twitwa "Pick bodies." Ibi bishobora kubonwa hifashishijwe mikorosikopi. Icy'ingenzi ni uko utwo turemangingo twa Pick na Pick bodies bitagaragara mu zindi ndwara iyo ari yo yose.

Impamvu izi poroteyine za tau zidakora neza iracyari amayobera. Nubwo abashakashatsi bahuje impinduka zimwe na zimwe mu ngirabuzimafatizo, akenshi iyo ndwara ntabwo ari umurage.

Iyi ndwara ntabwo yandura. Ntiyandurira mu muntu umwe ijya mu wundi. Nubwo hari amahirwe yo kuba yakwirakwira mu bisekuruza bitandukanye, akenshi ntabwo ibaho.

Indwara ya Pick ipimwa ite? Ni ibihe bipimo bikorwa?

Abaganga bashobora gusuzuma indwara yo kwiheba imbere n’imbere (FTD) binyuze mu gusuzuma umubiri n’imitsi, gusuzuma no gufata amashusho, kandi ibisubizo bishobora kugaragaza niba ari indwara yo kwiheba mu buryo bw’imyitwarire cyangwa ubwoko bwa PPA.

Ariko, uburyo bwonyine bwo kumenya neza niba indwara yo kwiheba imbere n'imbere (FTD) y'umuntu iterwa n'indwara ya Pick ni ugushaka imibiri ya Pick n'uturemangingo twa Pick mu bwonko bwe. Ibi bishobora kugaragara gusa usuzumye icyitegererezo cy'uturemangingo tw'ubwonko ukoresheje mikorosikopi. Ibi bivuze ko indwara ya Pick ishobora gusuzumwa neza gusa nyuma y'uko umuntu apfuye, mu gihe cyo gusuzuma umurambo.

Ibizamini nk'ibi bishobora gukorwa kugira ngo hamenyekane FTD:

• Ibipimo by'amaraso: Ibi bishakisha ibimenyetso by'imiti byitwa "biomarkers" bishobora gufasha mu gusuzuma indwara zimwe na zimwe.
• Ibipimo by'amazi yo mu bwonko:Ibi bikorwa hakoreshejwe amazi ava mu ruti rw'umugongo (gupfundika umugongo / gupfundika uruhu rw'umugongo).
• Gupima hakoreshejwe ikoranabuhanga (CT scan) (CT scan)
• EEG (Electroencephalogram)
• Gupima imiterere y'uturemangingo
• MRI scan (Ishusho ya Magnetic resonance - MRI)
• Gupima PET (Positron emission tomography - Gupima PET)

Ese hari uburyo bwo kuvura indwara ya Pick? Ese ishobora kuvurwa?

Ikibabaje ni uko nta muti, ubuvuzi cyangwa uburyo bwo kwirinda indwara ya Pick buhari. Abaganga bashobora kuguha imiti ifasha mu kugabanya ibimenyetso nko kwiheba, kutita ku bandi, n'imyitwarire mibi. Ariko, kubera ko ubu buryo bwo kuvura butandukanye bitewe n'umuntu ku wundi, umuganga wawe ni we muntu mwiza wo kuganira na we.

Ese ibyago byo kurwara iyi ndwara bishobora kugabanuka?

Indwara ya Pick ibaho mu buryo butazwi kandi butunguranye. Kubwibyo, nta buryo bwo kuyirinda cyangwa kugabanya ibyago byo kuyirwara.

Ni ubuhe buryo bw'ejo hazaza umuntu urwaye iyi ndwara ashobora kwitega?

Mu bwoko bwose bwa FTD, harimo n'indwara ya Pick, ubwonko bugenda burushaho kwangirika. Uko bigenda, ibice by'ubwonko byagizweho ingaruka bitakaza ubushobozi bwo gukora. Muri indwara ya Pick, ibi bikunze kugira ingaruka ku bice by'ubwonko bigenzura imyitwarire cyangwa ibice by'ubwonko bihuza imvugo n'ubushobozi bwo kumva ibyo abandi bavuga.

Ushobora kandi gutakaza ubushobozi bwo gusobanukirwa indwara yawe. Ni ukuvuga ko ushobora gutakaza ubushobozi bwo gusobanukirwa ibimenyetso n'ibiri kukubaho bitewe n'iyi ndwara.

Abantu bafite ikibazo cyo kwiheba imbere n'imbere (FTD) bagenda batakaza ubushobozi bwo kubaho mu buryo bwihariye. Uko indwara ikomeza kwiyongera, bashobora gukenera kwitabwaho amasaha 24 n'abakunzi babo cyangwa inzobere zabihuguriwe. Ibi bishobora no gusaba kwitabwaho igihe kirekire.

Abantu bafite FTD bashobora kugira ikibazo cyo kumira (dysphagia) , ibyo bikaba byagorana kurya, kunywa no kuvuga. Ibi byongera ibyago byo kurwara indwara nk'umusonga cyangwa kunanirwa guhumeka.

Ubwoko bwose bwa FTD, harimo n'indwara ya Pick, ni indwara y'ubuzima bwose. Ingaruka z'iyi ndwara zirakomeye, zigafata ibice bikomeye by'ubwonko buhoro buhoro. Ingorane zishobora kuba zikomeye ndetse zikanahitana. Kubera ko igihe umuntu azamara gitandukanye bitewe n'umuntu, biragoye kuvuga neza uko iyi ndwara izagufata n'igihe izamara. Muganga wawe (cyangwa umuganga uvura uwo ukunda) ashobora kukubwira byinshi kuri ibi.

Wakoresha ubushake bwawe ute niba udashobora kwifatira ibyemezo?

Niba usanzwe urwaye indwara yo kwibagirwa imbere mu gihe cy'ububabare (FTD) mu ntangiriro, ni ngombwa kuvugana na muganga wawe, umuryango wawe cyangwa abakunzi bawe, ndetse n'abo wizeye bashobora kugufatira ibyemezo by'ingenzi vuba bishoboka. Iki kiganiro gishobora kugorana, ariko kuganira kuri ibi bintu hakiri kare aho kubiganiraho nyuma bizafasha abakunzi bawe kumenya ibyo wifuza niba udashobora kuvuga ku bibazo byawe cyangwa gufata ibyemezo.

Uretse icyo kiganiro, ni ngombwa kandi kwandika ibyifuzo byawe n'ibyemezo byawe. Niba udashobora kwiyitaho, cyangwa niba udashobora gufata ibyemezo ku bijyanye n'ubuvuzi bwawe cyangwa imibereho myiza yawe, tekereza gutegura inyandiko z'amategeko. Ushobora kubona ubufasha bw'umunyamategeko kugira ngo utegure izi nyandiko, ariko ushobora no kuzitegura ubwawe (bitewe n'amategeko yo mu gace utuyemo, zishobora gusaba uruhushya rwa noteri cyangwa undi mukozi).

Wakora iki iyo umuntu wawe wa hafi agaragaje ibi bimenyetso?

Abantu barwaye FTD bakunze kubura ubumenyi ku bimenyetso cyangwa imiterere yabo. Kubera ko batabona ko bafite ikibazo, ntibemera ko bakeneye ubufasha bwa muganga. Uku kutumvikana bishobora kubabaza no gutera ubwoba umuntu ufite ibimenyetso ndetse n'ababa hafi ye.

Niba umuntu wawe wa hafi agaragaza ibimenyetso bya FTD cyangwa indi ndwara isa nayo, ushobora kugerageza kumufasha muri ubu buryo:

• Baza uko wafasha: Nubwo abantu barwaye FTD bagaragaza ibimenyetso, bashobora kutamenya ko ibi ari ibimenyetso by'ikibazo gikomeye cy'ubwonko. Mu kubatega amatwi, mu kubaha ubufasha, bashobora kumva bafitanye isano n'abantu bizera, ndetse bashobora no kubona inkunga bakeneye yo kujya kwa muganga.
• Bashishikarize gushaka ubufasha: Nubwo indwara yo kwiheba imbere mu gihe cyo kurwara indwara ya FTD (frontotemporal dementia - FTD) ari indwara idafite umuti cyangwa uburyo bwo kuyivura, hari uburyo bwo kuvura no gucunga bimwe mu bimenyetso. Iyi mihati ishobora gufasha cyane mu kunoza ubuzima bw'umuntu uyirwaye. Kubonana na muganga bishobora gufasha umuntu urwaye FTD kwisuzumisha. Ubuvuzi bwihariye bushobora kugabanya bimwe mu bimenyetso bibabaza abantu bafite iyo ndwara n'imiryango yabo.
• Humura kandi ntugafate ibintu nk'ibyawe: Abantu barwaye FTD akenshi ntibagira ububasha ku byo bavuga cyangwa bakora. Ibi bishobora gusa nkaho bashaka kurakaza, gukoza isoni, cyangwa gukomeretsa abandi nkana, ariko mu by'ukuri ni ikibazo cy'uburwayi.
• Ntutinye gusaba ubufasha: Kwita ku muntu urwaye FTD bishobora kugorana cyane, cyane cyane ko iyo ndwara irushaho gukomera uko igihe kigenda gihita. Ntutinye gusaba ubufasha cyangwa amikoro. Ibigo byinshi bya leta n'iby'abikorera ku giti cyabo bitanga uburyo na serivisi zo gushyigikira, nko kwita ku bantu bakuru ku manywa, kwita ku kiruhuko, no kwita ku baforomo bo mu rugo bafite ubumenyi.
• Kwita ku barwaye indwara y'igihe kirekire bishobora kuba amahitamo meza: Kuri benshi, kwita ku muntu ukunda ufite FTD bishobora kumva ari akazi k'igihe cyose. Si buri wese ushobora gutanga igihe kinini cyangwa imbaraga nyinshi mu kwita ku muntu ukunda. Kubwibyo, ni ngombwa gusuzuma niba umuntu ukunda akeneye kwitabwaho mu kigo cyita ku barwaye indwara y'igihe kirekire gifite ibigo by'ubuvuzi by'abahanga. Nubwo iki gishobora kuba icyemezo kigoye gufata, ubwo buryo bwo kwita ku barwaye bushobora kuba uburyo bwiza bwo kwemeza ko bafite umutekano, bamerewe neza, kandi bafite ababitaho babishoboye kandi babihuguriwe.

Amaherezo, ubutumwa bwo kujyana mu rugo

Indwara ya Pick ni indwara yitwa frontotemporal dementia (FTD), ikaba ari ubwoko bw'indwara yo kwibagirwa igenda yangiza ubwonko buhoro buhoro ugereranyije n'izindi ndwara ziterwa n'izabukuru. FTD kandi igira ingaruka ku bice by'ubwonko bifasha umuntu gusobanukirwa uburyo imyitwarire ye ikwiye kandi idakwiye. Ibi bishobora gutera kwiheba, kutumvikana, n'ibindi bibazo bikomeye ku buzima bw'abantu bose bayirwaye. Nubwo ibimenyetso bimwe na bimwe bishobora kuvurwa, nta muti ushobora gusubiza iyo ndwara ubwayo, kandi abantu bayirwaye bakunze gusaba kwitabwaho buri gihe amasaha 24.

Niba wowe cyangwa umuntu wawe wa hafi ufite ibi bimenyetso, ikintu cyiza wakora ni ukujya kwa muganga. Wibuke ko utari wenyine, kandi hari aho wabona ubufasha.

---

Indwara ya Pick, Dementia yo mu gihe cyo kuruhuka imbere, Dementia, Indwara y'ubwonko, Indwara yo kwangirika k'ubwonko, Poroteyine ya Tau, Impinduka mu myitwarire