Sivame ukumangala lapho sibona izinguquko ezingalindelekile ekuziphatheni nasekukhulumeni kwabanye babathandekayo bethu, akunjalo? Namuhla sizokhuluma ngesimo sezokwelapha esingase sibe imbangela yalezi zinguquko. Nakuba lesi kuyisihloko esiyinkimbinkimbi, ake sizame ukusiqonda kalula.

Iyini Isifo SikaPick? Sihluke kanjani kwisifo sikaNiemann-Pick?

Kalula nje, i-Pick's Disease yisimo esithinta ubuchopho, sibulala kancane kancane amangqamuzana. Luhlobo lwe-dementia olubizwa ngokuthi i-frontotemporal dementia (FTD) . Luvame ukubonakala kubantu abangaphansi kweminyaka engu-65. Esikhathini esidlule, i-frontotemporal dementia ngokwayo yayibizwa nangokuthi isifo sikaPick. Kodwa manje, odokotela basebenzisa leli gama kuphela ezimweni lapho kuhlangatshezwana nezindinganiso ezithile.

Manje ungase ucabange, "Oh... ingabe isifo sikaPick sifana nesifo sikaNiemann-Pick?" Cha, ziyizifo ezimbili ezihlukene ngokuphelele. Ngisho noma ingxenye yegama elithi "Pick" ifana ngengozi, lezi zifo ezimbili azihlobene.

• Isifo SikaPick: Siqanjwe ngo-Arnold Pick, isazi sezinzwa saseCzech kanye nodokotela wezifo zengqondo owaqala ukuhlonza lesi sifo ngo-1892. Luhlobo lwe-frontotemporal dementia (FTD), okusho ukuthi luthinta ubuchopho bakho kuphela.
• Isifo sikaNiemann-Pick: Siqanjwe ngodokotela ababili baseJalimane, u-Albert Niemann noLudwig Pick, lesi sifo sibonakala ngokuqongelela kwamafutha emzimbeni kanye nokuphazamiseka endleleni alawulwa ngayo. Lapho amafutha eqongelela, angathinta izitho eziningi zomzimba, okuhlanganisa ubuchopho, isibindi, ubende, umnkantsha wamathambo kanye namaphaphu.

Uyawuqonda umehluko? Enye into elinganiselwe ebuchosheni, kanti enye into ethinta izingxenye ezahlukene zomzimba.

Ubani othola lesi sifo? Sivame kangakanani?

Isifo sikaPick sivame ukutholakala sisebancane kunezinye izimo ezifana ne-dementia. Sivame ukutholakala kubantu abaneminyaka engu-50 no-60. Kodwa-ke, ngezinye izikhathi singatholakala kubantu abaneminyaka engu-20 noma engu-80.

Kunobufakazi bokuthi lesi sifo singangena emindenini. Abacwaningi bathole okungenani izinguquko ezintathu zezakhi zofuzo ezihlotshaniswa naso. Kodwa-ke, ezimweni eziningi, lesi sifo sivela ngokungahleliwe. Lokhu kusho ukuthi othile angasithola ngaphandle komlando womndeni.

Kunzima ukusho ukuthi lesi sifo sivame kangakanani. Ochwepheshe balinganisela ukuthi kunezimo eziphakathi kuka-15 no-22 kubantu abayi-100,000. Kodwa-ke, kukhona ukungabaza ngokunemba kwalezi zibalo, ngoba lesi sifo kunzima kakhulu ukusithola ngesikhathi sokuphila, futhi ngezinye izikhathi ngisho nangemva kokufa, kuyinselele ukusithola. Ngakho-ke inani langempela leziguli lingase libe phezulu.

Isifo sikaPick siwuthinta kanjani umzimba?

Isifo sikaPick siyisifo esiwohloka ngenxa yemizwa, uhlobo lwe-dementia olubizwa ngokuthi i-frontotemporal dementia (FTD). Kalula nje, amangqamuzana ezinzwa (ama-neurons) ebuchosheni ayayeka ukusebenza kancane kancane. Izingxenye zobuchopho ezithintekile ziqala ukuncipha (ukuwohloka). Bese, ulahlekelwa amakhono lawo malungu ayewasebenzisa ukulawula. Nakuba sinokufana okuthile nesifo sika-Alzheimer, isifo sikaPick sivame ukuqala usemncane, futhi kunezinhlobo eziningana zomehluko obalulekile.

Ngenxa yokuthi iSifo sikaPick sithinta izingxenye ezithile zobuchopho kuphela, izimpawu eziyinhloko izinguquko ekuziphatheni noma emakhonweni olimi . Ngokuvamile, abantu abanesifo sikaPick abakwazi ukubona ukuthi banenkinga noma isimo esithile. Lokhu kungenxa yokuthi ubuchopho babo abukwazi ukucubungula ulwazi mayelana nesimo sabo. Lokhu kubizwa ngokuthi "ukuntuleka kokuqonda."

Ziyini izimpawu zesifo sikaPick?

Isifo sikaPick senzeka ngezindlela ezimbili eziyinhloko: esinye yi -behavioral variant frontotemporal dementia (bvFTD) , kanti esinye yi- primary progressive aphasia (PPA), ethinta izingxenye zobuchopho ezihlobene nolimi.

I-Behavioral variant frontotemporal dementia (bvFTD)

Izimpawu zalesi simo se-bvFTD zingahlukaniswa ngezigaba eziyisithupha eziyinhloko:

• Ukulahlekelwa ukuzithiba: "Ukuzithiba" yingxenye yobuchopho esitshela ukuthi "ungakwenzi lokhu." Uma lezi zingxenye zonakele, asikwazi ukuzibamba ekusho noma ekwenzeni izinto ezingafanele.
• Ukuntuleka "kokuhlunga" lokho okushiwoyo: Ukusho okusengqondweni yakho, ngezinye izikhathi ngendlela elimaza abanye, kuwukuhlambalaza, noma akufanele. Isibonelo, ukuthuka umuntu ngokuzumayo embuthanweni womndeni.
• Ukuntuleka kwenhlonipho ngabanye: Bangase bangazihloniphi izimfihlo zabanye, bangase babathinte ngokungathandi, bangase bazame ukuziphatha ngendlela engafanele ngokocansi, futhi bangase bathukuthele noma bacasuke ngokuzumayo.
• Izenzo nokuziphatha okunganaki: Lokhu kungafaka phakathi ukusebenzisa imali ngokunganaki, mhlawumbe ngisho nokweba ezitolo.

• Ukunganaki: Lokhu kungase kubonakale njengokucindezeleka, kodwa kuhlukile kancane.
• Ukulahlekelwa yisithakazelo kunoma yini: Ukulahlekelwa yisithakazelo ezintweni owawuzijabulela (izinto zokuzilibazisa, ukuchitha isikhathi nabangani).
• Ukuzihlukanisa nabantu: Ukugwema ukuba nabangani nomndeni.
• Ukuncipha kokuzinakekela: Ukulahlekelwa isithakazelo ekugezeni, ekushintsheni izingubo, nasekuhlaleni uhlanzekile.

• Ukulahlekelwa uzwela:Lokhu kubizwa nangokuthi "ukufiphaza ngokomzwelo." Kuba nzima ukuqonda imizwa yabanye. Ababonisi ukwesekwa ezinhlungwini noma enjabulweni zabanye abantu njengoba babevame ukwenza.

• Ukuziphatha okuphoqelekile: Abantu abanalesi sifo babonisa izindlela zokuziphatha ezihluke kakhulu kwabanye.
• Ukunyakaza okuphindaphindayo: Ukwenza ukunyakaza okuncane okuphindaphindiwe, njengokushaya izandla, ukunyathela izinyawo, nokuhamba uye phambili naphambili.
• Ukuziphatha okuyinkimbinkimbi noma okungokwesiko: ukubukela ama-movie afanayo ngokuphindaphindiwe, ukufunda izincwadi ezifanayo, ukuqoqa izinhlobo ezifanayo zezinto. Lokhu kuhlanganisa nokugcina imali.
• Ukuphindaphinda: Ukuphindaphinda imisindo efanayo, amagama, nemisho ngokuphindaphindiwe.

• Izinguquko ekudleni noma ekuziphatheni okugxile emlonyeni: Abantu abanalesi sifo bangase babe nesimo esibizwa ngokuthi "i-hyperorality," okuyisithakazelo esingavamile ezintweni ezihlobene nomlomo.
• Izinguquko emikhubeni yokudla: Abantu bavame ukuzitika ngokweqile ngokudla abakuthandayo, ikakhulukazi ukudla okulula, amaswidi, notshwala. Lokhu kungaholela ekukhuluphaleni.
• Ukuziphatha komlomo okuphoqelekile: Ukubhema okuphoqelekile, ukudla ngokweqile, njll. Futhi, ukuhlola ngomlomo. Nakuba lokhu kuvamile ukuba izingane ezincane zikwenze njengoba zihlola umhlaba, akuvamile ngabantu abadala ukukwenza.
• I-Pica: Lokhu ukudla okuphoqelekile kwezinto ezingadliwayo, ezingezona ezondlayo (izinhlamvu zemali, uthuli, amatshe amancane).

• Ukulahlekelwa umsebenzi wokuphatha kodwa amanye amakhono aphelele: "Umsebenzi wokuphatha" ubhekisela ekhonweni lokuhlela usuku, ukuxazulula izinkinga, nokwenza izinto. Nakuba la makhono ephazamiseka kubantu abanalesi sifo, amanye amakhono, njengenkumbulo kanye nokucubungula okubonakalayo, awathinteki kangako ezigabeni zokuqala. Lesi yisici esibalulekile esihlukanisa i-frontotemporal dementia nesifo i-Alzheimer's.

I-Aphasia Eqhubekayo Eyinhloko (i-PPA)

I-PPA ingabangelwa yisifo sikaPick. Lokhu kubangela ukulahlekelwa kancane kancane kwamakhono olimi, njengokukwazi ukukhuluma nokuqonda lokho abanye abakushoyo. Nakuba kunezinhlobo ezintathu eziyinhloko ze-PPA, izinhlobo ezimbili eziyinhloko ezingahlotshaniswa nesifo sikaPick yilezi:

• I-NonfvPPA ehlukile (i-nfvPPA): Kulolu hlobo, kunzima ukufanisa amagama nokusebenzisa uhlelo lolimi olufanele. Nakuba amagama angawodwa nemisho elula kungaqondakala, imisho eyinkimbinkimbi inobunzima bokuyiqonda.
• I-PPA ehlukile ye-Semantic (svPPA):Lolu hlobo lunenkinga yokukhetha igama elifanele noma ukuqonda incazelo yamagama. Ezinye izinto ezishiwoyo zingase zingabi nangqondo, futhi kunzima ukuqonda ukuthi abanye bathini. Kungase kube nezinkinga zokufunda nokubhala, kodwa zingaphinda lokho abanye abakushoyo ngendlela efanayo.

Cabanga nje, ubaba wakho uvele ahluke kumuntu ayenguye ngaphambili, athethise wonke umuntu, asebenzise imali ngokunganaki, noma ahube ingoma efanayo usuku lonke. Noma, uma ukhuluma naye, amazwi akhe ayaphambana, futhi azizwe sengathi akaqondi ukuthi uthini. Lezi yizibonelo zezinto ebesikhuluma ngazo.

Yini ebangela lesi simo? Ingabe siyathelelana?

Isifo sikaPick uhlobo lwe-frontotemporal dementia (FTD) olunembangela ethile. Ubuchopho bethu namaseli ezinzwa aqukethe iphrotheni ekhethekile ebizwa ngokuthi i-tau . Lawa maprotheni kudingeka abe nesimo esithile, kungenjalo ngeke asebenze kahle.

Uma amaprotheni e-tau engasebenzi kahle, ehlangana ndawonye, ​​​​futhi eqoqana ngaphakathi kwama-neurons, lawo maseli ayalimala futhi abhujiswe. Ama-neurons alimele abizwa ngokuthi "amaseli e-Pick." Ayavuvukala futhi abe njengebhaluni. Amaqoqo amaprotheni e-tau ahlangene ngaphakathi kwamaseli abizwa ngokuthi "imizimba ye-Pick." Lawa angabonakala ngaphansi kwe-microscope. Okubalulekile, la maseli e-Pick kanye nemizimba ye-Pick awabonakali kunoma yisiphi esinye isifo.

Ukuthi kungani lawa maprotheni e-tau engasebenzi kahle kuseyimfihlakalo. Nakuba abacwaningi bexhumanise izinguquko ezithile zofuzo nalokhu, ezimweni eziningi lesi sifo asizuzwa njengefa.

Lesi sifo asithelelani. Asidluliselwa kusuka komunye umuntu kuya komunye. Nakuba kungenzeka ukuthi sidluliselwe ezizukulwaneni ngezizukulwane, akwenzeki ezimweni eziningi.

Isifo sikaPick sitholakala kanjani? Yiziphi izivivinyo ezenziwayo?

Odokotela bangakwazi ukuxilonga i-frontotemporal dementia (FTD) ngokuhlolwa ngokomzimba nangokwezinzwa, ukuhlolwa kokuxilonga kanye nokuhlola izithombe, futhi imiphumela inganquma ukuthi ngabe i-FTD yokuziphatha noma uhlobo oluthile lwe-PPA.

Kodwa-ke, okuwukuphela kwendlela yokwazi ngokuqinisekile ukuthi i-frontotemporal dementia (FTD) yomuntu ibangelwa yisifo sikaPick ukubheka imizimba kaPick namaseli kaPick ezicutshini zobuchopho bakhe. Lokhu kungabonakala kuphela ngokuhlola isampula yezicubu zobuchopho ngaphansi kwe-microscope. Lokhu kusho ukuthi isifo sikaPick singatholakala kuphela ngemva kokushona komuntu, ekuhlolweni kwesidumbu.

Ukuhlolwa okunjengalokhu kungenziwa ukuze kutholakale i-FTD:

• Ukuhlolwa kwegazi: Lokhu kubheka izimpawu zamakhemikhali ezibizwa ngokuthi “ama-biomarker” angasiza ekuxilongeni izifo ezithile.
• Ukuhlolwa koketshezi lwe-cerebrospinal:Lokhu kwenziwa ngokuthatha uketshezi oluvela emgogodleni (umpompi womgogodla / ukubhobozwa kwe-lumbar).
• I-CT scan (i-computerized tomography - i-CT scan)
• I-EEG (i-Electroencephalogram)
• Ukuhlolwa kofuzo
• Ukuskena kwe-MRI (Umfanekiso we-Magnetic resonance - i-MRI)
• Ukuskena kwe-PET (i-Positron emission tomography - ukuskena kwe-PET)

Ingabe ikhona indlela yokwelapha isifo sikaPick? Ingabe singelapheka?

Ngeshwa, akukho ukwelashwa, ukwelashwa, noma ukuvimbela isifo sikaPick. Odokotela bangakunikeza imithi yokusiza ukulawula izimpawu ezifana nokucindezeleka, ukunganaki, kanye nokuziphatha okunolaka. Kodwa-ke, njengoba lokhu kwelashwa kuhluka kumuntu nomuntu, udokotela wakho ungumuntu ongcono kakhulu ongakhuluma naye.

Ingabe ingozi yokuthola lesi sifo ingancishiswa?

Isifo sikaPick senzeka ngezindlela ezingalindelekile nezingaziwa. Ngakho-ke, okwamanje ayikho indlela yokusivimbela noma yokunciphisa ingozi yokusithola.

Hlobo luni lwekusasa umuntu onalesi sifo angalulindela?

Kuzo zonke izinhlobo ze-FTD, okuhlanganisa ne-Pick's Disease, ubuchopho buya buwohloka kancane kancane. Njengoba kunjalo, izingxenye zobuchopho ezithintekile zilahlekelwa amandla azo okusebenza. Ku-Pick's Disease, lokhu kuvame ukuthinta izingxenye zobuchopho ezilawula ukuziphatha noma izingxenye zobuchopho ezihlanganisa inkulumo kanye nekhono lokuqonda lokho abanye abakushoyo.

Ungase futhi ulahlekelwe ukuqonda ngesimo sakho. Okusho ukuthi, ungase ulahlekelwe ikhono lokuqonda izimpawu nokuthi kwenzekani kuwe ngenxa yalesi sifo.

Abantu abane-frontotemporal dementia (FTD) kancane kancane balahlekelwa ikhono lokuphila ngokuzimela. Njengoba lesi sifo siqhubeka, bangadinga ukunakekelwa amahora angama-24 kwabathandekayo noma ochwepheshe abaqeqeshiwe. Lokhu kungadinga ngisho nokunakekelwa kwesikhathi eside.

Abantu abane-FTD bangase babe nobunzima bokugwinya (i-dysphagia) , okungenza kube nzima ukudla, ukuphuza, nokukhuluma. Lokhu kwandisa ingozi yokuthuthukisa izimo ezifana ne-pneumonia noma ukwehluleka kokuphefumula.

Zonke izinhlobo ze-FTD, okuhlanganisa nesifo sikaPick, ziyizimo zokuphila konke. Lesi sifo sibi kakhulu, sibhubhisa kancane kancane izingxenye ezinkulu zobuchopho. Izinkinga zingaba zimbi kakhulu futhi zibulale. Ngenxa yokuthi isikhathi sokuphila siyahlukahluka kumuntu nomuntu, kunzima ukusho ngqo ukuthi lesi sifo sizokuthinta kanjani nokuthi sizohlala isikhathi esingakanani. Udokotela wakho (noma udokotela owelapha othandekayo wakho) angakutshela okwengeziwe ngalokhu.

Ungayisebenzisa kanjani intando yakho uma ungakwazi ukuzenzela izinqumo?

Uma kutholakala ukuthi une-frontotemporal dementia (FTD) isesigabeni sayo sokuqala, kubalulekile ukukhuluma nodokotela wakho, umndeni wakho noma abathandekayo bakho, kanye nalabo obathembayo abangakwenzela izinqumo ezibalulekile ngokushesha okukhulu. Le ngxoxo ingaba nzima, kodwa ukukhuluma ngalezi zinto kusenesikhathi kuzosiza abathandekayo bakho bazi ukuthi yini oyifisayo uma ungakwazi ukukhuluma ngezindaba zakho noma ukwenza izinqumo.

Ngaphezu kwaleyo ngxoxo, kubalulekile futhi ukubhala phansi izifiso zakho nezinqumo zakho. Uma ungakwazi ukuzinakekela, noma uma ungakwazi ukwenza izinqumo mayelana nokunakekelwa noma inhlalakahle yakho, cabanga ngokulungiselela amadokhumenti asemthethweni. Ungathola usizo kummeli ukuze ulungiselele la madokhumenti, kodwa ungazilungiselela nawe amanye awo (kuye ngokuthi umthetho wendawo yakini, angadinga imvume yommeli noma esinye isikhulu).

Wenzani uma umuntu oseduze nawe ebonisa lezi zimpawu?

Abantu abane-FTD bavame ukungaziqondi izimpawu noma isimo sabo. Ngenxa yokuthi ababoni ukuthi banenkinga, abakholelwa ukuthi badinga usizo lwezokwelapha. Lokhu kungaqondi kungaba yinto ekhungathekisayo neyesabisayo kumuntu onezimpawu kanye nalabo abaseduze nabo.

Uma umuntu oseduze nawe ekhombisa izimpawu ze-FTD noma isimo esifanayo, ungazama ukusiza ngalezi zindlela:

• Buza ukuthi ungasiza kanjani: Ngisho noma abantu abane-FTD bebonisa izimpawu, bangase bangaqapheli ukuthi lezi yizimpawu zenkinga enkulu yobuchopho. Ngokubalalela, ukunikeza ukwesekwa kwakho, bangazizwa bexhumene nabantu ababathembayo, futhi bangathola nesikhuthazo abasidingayo sokubona udokotela.
• Bakhuthaze ukuba bafune usizo: Nakuba i-frontotemporal dementia (FTD) iyisimo esingenalo ikhambi noma ukwelashwa, kunezindlela zokwelapha nokuphatha ezinye zezimpawu. Le mizamo ingasiza kakhulu ekuthuthukiseni ikhwalithi yokuphila komuntu onalesi simo. Ukubona udokotela kungasiza umuntu one-FTD ukuthi axilongwe. Ukunakekelwa okukhethekile kunganikeza impumuzo kwezinye zezimpawu ezingakhululekile kubantu abanalesi simo kanye nemindeni yabo.
• Hlala uzolile futhi ungathathi izinto ngendlela efanele: Abantu abane-FTD bavame ukungabi namandla okulawula lokho abakushoyo noma abakwenzayo. Lokhu kungase kubonakale sengathi bazama ngamabomu ukucasula, ukuhlazisa, noma ukulimaza abanye, kodwa empeleni kuyinkinga yezokwelapha.
• Ungesabi ukucela usizo: Ukunakekela umuntu one-FTD kungaba nzima kakhulu, ikakhulukazi njengoba isimo siba sibi ngokuhamba kwesikhathi. Ungesabi ukucela usizo noma izinsiza. Izinhlangano eziningi zomphakathi nezizimele zinikeza izinhlelo zokusekela kanye nezinsizakalo, njengokunakekelwa kwabantu abadala emini, ukunakekelwa kwesikhashana, kanye nokunakekelwa kwabahlengikazi abanekhono emakhaya.
• Ukunakekelwa kwesikhathi eside kungaba yindlela engcono kakhulu: Kwabaningi, ukunakekela othandekayo one-FTD kungaba umsebenzi wesikhathi esigcwele. Akuwona wonke umuntu onganikela isikhathi esiningi noma umzamo ekunakekeleni othandekayo wakhe. Ngakho-ke, kubalulekile ukucabangela ukuthi othandekayo wakho udinga ukunakekelwa yini esikhungweni sokunakekelwa kwesikhathi eside esinezikhungo zokunakekela abahlengikazi abanekhono. Nakuba lokhu kungaba yisinqumo esinzima ukusenza, ukunakekelwa okunjalo kungaba yindlela engcono kakhulu yokuqinisekisa ukuthi baphephile, bakhululekile, futhi banabanakekeli abaqeqeshiwe nabaqeqeshiwe.

Ekugcineni, umyalezo okufanele uwuthathe uye nawo ekhaya

Isifo sikaPick yisimo esibizwa ngokuthi i-frontotemporal dementia (FTD), okuwuhlobo lwe-dementia olulimaza kancane kancane ubuchopho busencane kunezinye izifo zobuchopho ezihlobene nokuguga. I-FTD ithinta nezingxenye zobuchopho ezisiza umuntu ukuqonda ukufaneleka nokungafaneleki kokuziphatha kwakhe. Lokhu kungabangela ukukhungatheka, ukungaqondani, kanye nokunye ukuphazamiseka okukhulu empilweni yabo bonke abathintekayo. Nakuba ezinye izimpawu zingelashwa, ayikho ikhambi elingaguqula lesi sifo ngokwaso, futhi abantu abanalesi simo bavame ukudinga ukunakekelwa okuqhubekayo, amahora angama-24.

Uma wena noma othile oseduze nawe ebhekene nalezi zimpawu, into engcono kakhulu ongayenza ukufuna iseluleko sezokwelapha. Khumbula, awuwedwa, futhi kunezindawo zokuthola usizo.

Isifo sikaPick , i-Frontotemporal Dementia, i-Dementia, isifo sobuchopho, i-Neurodegenerative, i-Tau Protein, izinguquko zokuziphatha