Mitundu ya Matenda a Neuron Matenda kuphatikizapo ALS ndi zambiri
Ngati anthu ambiri amalingalira chilichonse akamva mawu akuti "motor motors," amaganiza za amyotrophic lateral sclerosis (ALS). Komabe, palinso mitundu yambiri ya magalimoto otchedwa neuron. Mwamwayi, matenda onse a mphutsi ndi achilendo.
Mwachidule
Tikasuntha, zizindikiro zamagetsi zimatumizidwa kuchokera mu ubongo kupita kumsana wa msana pamphepete mwazitali zamoto.
Maselo a mitsempha amasinthasintha m'mphuno yamtundu wa msana ndiyeno amatumizidwa kumatenda apansi a m'mphepete mwa mitsempha. Zizindikiro zamagetsi zimayenda motsatira zizindikiro za neuron kuti minofu ikhale yogwirizana, zomwe zimapangitsa kuyenda. Malingana ndi kafukufuku wina wa thupi, akatswiri a sayansi ya zamoyo amatha kudziŵa kumene vutoli liri mu dongosolo la mitsempha, ndipo amachokera kutero, matenda omwe angapezeke.
Zinthu zomwe zimakhudza chizindikirochi mwachidziwitso zimatchulidwa ngati matenda a mphutsi. Nyanga yam'mimba ya msana imatulutsa uthenga wokhudzana ndi kumva, pamene nyanga yam'mbuyo imanyamula uthenga wokhudza kuyenda. Matenda a mphutsi, chifukwa chaichi, amakhudza makamaka kayendedwe ka kayendetsedwe kake.
Zizindikiro Zambiri ndi Zisonyezo
Matenda a mphutsi akhoza kupatulidwa m'magulu akuluakulu awiri, malingana ndi momwe amakhudzidwira pamtunda wotchedwa neuron kapena m'munsi mwake. Matenda ena amtundu wa neuroni amakhudza kanyumba kamene kamene kamakhala ndipamwamba kwambiri, pamene ena amakhudzidwa makamaka ndi makina oyenda pansi.
Zina, monga ALS, zimakhudza zonse.
Zizindikiro za matenda apamwamba otchedwa neuron ndi:
- Kuthamanga - Kusakanikirana kwa minofu, kuumitsa, kukhwima, ndi kulephera. Mukamachita zinthu monyanyira, minofu yanu ingamveke "yosagwedezeka." Pang'ono ndi pang'ono, mungathe kusuntha minofu yanu, koma imayankha mwadzidzidzi kapena mwachangu.
- Kulemera - "Kuuma" kosavomerezeka kwa minofu.
- Kuwonjezeka kwakukulu tendon reflexes - Mwachitsanzo, mawondo anu amawoneka mochuluka kuposa nthawi zonse.
Zizindikiro za matenda apansi otchedwa neuron ndi:
- Atrophy - Kutayika kwa mphamvu ndi minofu yambiri.
- Kufotokozera - Kutsutsana kwadzidzidzi komanso kosasunthika kwa minofu yomwe ikhoza kuoneka ngati ikugwedeza pansi pa khungu.
Mitundu ya Matenda a Neuron Matenda
Pali matenda osiyanasiyana a neuron omwe amasiyana ndi momwe amakhudzidwira m'mwamba kapena m'munsi mwazizindikiro, zizindikiro zoyambirira, zaka zomwe zimakhudza, ndi kutsegula. Ena mwa awa ndi awa:
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), yomwe imadziwikanso kuti matenda a Lou Gehrig, ndi matenda omwe amachititsa anthu 6,000 chaka chilichonse ku United States. Zimayamba ndi kufooka kwa minofu, nthawi zambiri pambali imodzi yokha ya thupi. Chikhalidwe chimayamba m'manja mowirikiza kuposa mapazi. Poyambirira, chizindikiro chachikulu chikhoza kukhala fasciculations, koma potsiriza, chimapita ndi zizindikiro zapamwamba ndi za motor neuron. Pamene diaphragm imakhudzidwa, mpweya wabwino ungakhale wofunikira. Matendawa kawirikawiri samakhudza kuzindikira, ndipo anthu ambiri amakhala osamala (ngakhale alibe matenda) ngakhale pamene matendawa apita patsogolo.
Kawirikawiri nthawi yokhala ndi moyo ndi ALS ndi pafupifupi zaka ziwiri kapena zisanu koma zimatha kusiyana, ndi asanu mwa anthu 100 alionse atakhala ndi moyo zaka makumi awiri. Mwamwayi, anthu monga asayansi Stephen Hawking akuthandiza kuzindikira za ALS ndi matenda ena a mphuno.
Pambuyo Pambuyo Pambuyo
Matenda oyambirira a lateral sclerosis (PLS) ndi matenda a mitsempha yapamwamba yamagetsi, kusokoneza zizindikiro kuchokera mu ubongo kupita ku msana wa msana. Maselo omwe ali mu ubongo wamtunduwu omwe amayendetsa kayendetsedwe ka pang'onopang'ono amafa. Zotsatira zake ndizofooka pang'onopang'ono zomwe zimagwirizanitsidwa ndi zizindikiro zapamwamba zamagetsi, monga kusasamala, kukhwima, ndi kuwonjezeka kwa tendon reflexes.
Mosiyana ndi amyotrophic lateral sclerosis, zotsika zapansi zotchedwa neuron, monga atrophy ndi fasciculations, siziri zotchuka. Sitikudziwa kuti PLS ndi yamba bwanji, koma timakhulupirira kuti ndi yochepa kuposa ALS.
Kumayambiriro kwa matendawa, chiwerengero chachikulu cha matendawa chikhoza kusokonezeka ndi ALS. Popeza kuti ALS ingayambe ndi zizindikiro zamtundu wapamwamba zamagetsi, zikhoza kukhala zaka zambiri asanazindikire kuti PLS imaonekera. Ngakhale panthawi imeneyo, zingakhale zovuta kudziwa zomwe zimayambitsa zizindikiro, popeza anthu ena omwe ali ndi PLS omwe akuganiza kuti angapangitse kupeza zowonongeka, zimatsimikizira kuti matendawa ndi ALS. Zonsezi ndizovuta kwambiri kunena kuti sikutheka kudziwa ngati matenda ali ALS kapena PLS kwa zaka zingapo pambuyo poyambira zizindikiro.
Zinthu zina, monga cholowa cha paraparesis, zidzafunikanso kuwonetsedwa. PLS imayamba kupita pang'onopang'ono kusiyana ndi ALS, ndi odwala omwe amakhala ndi zaka 20 omwe ali ndi zizindikiro zawo.
Mapuloteni Opita Patsogolo
Mwa njira zina, kupuma kwa muscularphytic (PMA) kumakhala kosiyana ndi msinkhu woyamba wa sclerosis. M'malo mwa PMA, ndizitsulo zokhazokha zomwe zimakhudzidwa, pomwe, mu PLS, ndizowopsa kwambiri pamtunda. Popeza kuti mpweya wotsika wapansi umakhudzidwa, kufookera kwapang'onopang'ono ndi chizindikiro chofala. Popeza kuti mpweya wapamwamba wamagetsi sagwedezeka, zizindikiro zapamwamba zamagetsi zotupa monga kusagwedezeka sikuchitika. Kuthamanga kwa minofu kosavuta kumakhala kosavuta kuposa ALS koma kumakhala bwino.
Kungakhale njira yopweteketsa mtima kuti adziwe kuti matendawa amatha kupweteka chifukwa zizindikirozo zimakhala zofanana ndi zina. Makamaka, matenda monga ALS, matenda opatsirana amtundu wa multifocal (mawonekedwe a mitsempha ya m'mimba ) ndi mitsempha ya msana wamtundu wa msana amafunika kuti awonongeke poyamba asanamvetsetse bwinobwino.
Mphuno ya Mbalame Yopuma
Kuphulika kwaphalala kumaphatikizapo kuchepa kwa ubongo, komwe kumakhala ndi mitsempha yambiri yomwe imalamulira nkhope, lirime, ndi mmero. Chotsatira chake, munthu yemwe ali ndi ziphuphu zowonongeka adzayamba kuvutika kulankhula, kumeza ndi kutafuna. Kufooka kwa nyongolotsi kungakhalenso koonekera kwambiri pamene matendawa akupita, ndi zizindikiro zonse zapamwamba ndi zapansi zamagetsi. Anthu omwe ali ndi zofooketsa zapamwamba amatha kukhala osasinthasintha ndipo nthawi zina amafunika kusangalala kapena kuseka. Si zachilendo kwa anthu omwe ali ndi zofooketsa zapang'onopang'ono kuti apititse patsogolo ALS. Myasthenia gravis ndi matenda osokoneza bongo omwe amatha kufanana nawo.
Syndrome ya Post
Polio ndi kachilombo kamene kamayambitsa ma neuroni m'kati mwa nyanga ya msana, zomwe zimayambitsa kufooka. Mwamwayi, chifukwa cha katemera wochuluka, kachilombo ka HIV kakathetsedwa. Ena mwa iwo omwe adayambanso matendawa angadandaule ndi zofooka zotchedwa post-polio syndrome. Izi zikhoza kukhala chifukwa cha ukalamba kapena kuvulala zomwe zimachititsa kuti mavitamini ochepa omwe akukhalapo amatha kuyendetsa chiwalo choyambidwa kale. Matendawa amakhudza anthu okalamba omwe adakhala ndi polio m'mbuyomo. Kaŵirikaŵiri siikawopsya moyo.
Matenda a Kennedy
Matenda a Kennedy amayamba chifukwa cha kusintha kwa majeremusi okhudza X omwe amakhudza kachilombo ka androgen. Matendawa amabweretsa zofooka pang'onopang'ono komanso kupweteka kwa minofu yoyandikana kwambiri ndi nthendayi. Nkhope, msuwa, ndi lilime zimakhudzidwa. Chifukwa chakuti X-linked, matenda a Kennedy amakhudza anthu. Azimayi omwe ali ndi genetic mutation ndi othandizira, ndi 50 peresenti mwayi wopatsira jini kwa ana awo. Azimayi omwe ali ndi kusintha kwa thupi angathenso kuvutika ndi zizindikiro zazing'ono, monga zipsinjo zazing'ono, m'malo mofooka kwambiri.
Chifukwa chakuti matendawa amachititsa kuti atrogen receptor (yolandira kumene estrogen ndi testosterone), amuna omwe ali ndi vutoli akhoza kuvutika ndi zizindikiro monga gynecomastia ( kutulutsa mawere), testicular atrophy, ndi erectile dysfunction. Nthawi ya moyo ya anthu omwe ali ndi matenda a Kennedy kawirikawiri, ngakhale kuti zofooka zawo zikupita, amafunika kukhala ndi olumala.
Mitsempha ya m'mphepete mwa mitsempha
Matenda a msana ndi athanzi omwe amabadwa nawo makamaka omwe amakhudza ana. Zimayambitsa zolepheretsa mu jini la SMN1 ndipo zimayambitsidwa mwachitsanzo. Chifukwa cha jini ili lopanda mphamvu, sitingakwanitse kupanga mapuloteni a SMN, ndipo izi zimayambitsa kutsika kwa mpweya wotsika. Izi zimabweretsa kufooka ndi kupweteka kwa minofu.
Pali mitundu itatu yaikulu ya SMA, yomwe imaphatikizapo ana m'zaka zosiyana.
- Mtundu wa SMA 1, womwe umatchedwanso Werdnig-Hoffman matenda, umadziwikiratu pamene mwana ali ndi miyezi isanu ndi umodzi. Mwanayo adzakhala ndi hypotonia (floppy muscle) ndipo sadzapita nthawi yomweyo. Iwo sangathe kukhala okha pa nthawi yoyembekezeka. Chifukwa cha zovuta ndi ulendo wa pamsewu komanso kukhala ndi mphamvu zokwanira, ambiri a iwo amafa ali ndi zaka ziwiri.
- Mtundu wa SMA II umayamba patapita kanthawi, ndikuwoneka pakati pa miyezi 6 mpaka 18. Ana awa sangathe kuima kapena kuyenda popanda thandizo, komanso adzakhalanso ndi vuto ndi kupuma. Komabe, ana omwe ali ndi SMA mtundu wachiwiri amakhala moyo wautali kuposa omwe ali ndi Werdnig-Hoffman, nthawi zina amakhala akukula.
- Mtundu wa SMA IIII umatchedwanso Kugelberg-Welander matenda, umaonekera pakati pa zaka ziwiri ndi 17. Ana omwe ali ndi matendawa angakhale ndi zovuta kapena kuyenda. Angakhalenso ndi mavuto ambuyo, monga scoliosis . Komabe, ana omwe ali ndi matendawa akhoza kukhala ndi moyo wathanzi.
Kuzindikira ndi Chithandizo
Palibe mankhwala othandiza kwambiri pa matenda aliwonse a mphutsi. Thandizo lachipatala limayang'ana ku zizindikiro za matendawa momwe zingathere. Komabe, kuti mudziwe zizindikiro zomwe muyenera kuyembekezera, komanso kutulutsa matenda ena ochiritsira, ndikofunika kuti mudziwe bwinobwino.
Pogwiritsa ntchito mayeso awo komanso njira zina monga electromyography , maphunziro a mitsempha yopangitsa munthu kukhala ndi mitsempha, komanso kuyeretsa kwa majeremusi pakakhala koyenera, akatswiri a sayansi ya ubongo angathandize kufotokozera bwino matendawa. Kukhala ndi matenda abwino kumathandiza katswiri wanu wamagetsi kuti athetse matenda anu momwe angathere ndi kuyembekezera ndi kukonzekera mavuto alionse omwe akuyembekezeka.
Kulimbana
Poyambirira, tinayankha kuti "mwachisangalalo" matenda opangidwa ndi mphutsi zapachilendo sizodziwika. Izi zingakhale zabwino pokhapokha inu kapena wokondedwa wanu musakayike chimodzi mwazimenezi. Kenaka, kuwonjezera pa kuvutika ndi zizindikiro za matendawa, mungapeze kuti pali zochepa zofukufuku komanso zochepa zothandizira kuposa momwe mungayembekezere. Ngakhale kuti matendawa ndi achilendo, mayendedwe monga Orphan Drug Act akuwongolera kwambiri pazimenezi koma zosafunika kwenikweni.
Mutha kumverera nokha ngati mwapezeka kuti muli ndi matenda a neuron. Mosiyana ndi magulu akuluakulu a "otsogolera khansa ya m'mawere" kunja uko, sitimayang'ana magulu akuluakulu, mwachitsanzo, omwe amalimbikitsa odwala matendawa. Komabe kuzindikira kukukula, komanso kwa ALS, thandizo.
Anthu omwe ali ndi matenda a neuron amafunika kuthandizidwa monga omwe ali ndi zizolowezi zambiri. Ngakhale kuti simungakhale ndi gulu lothandizira m'deralo, pali anthu ogwirizana pa intaneti komwe anthu omwe ali ndi zida zina zapamwamba zimatha "kukomana" ndikuyankhulana ndi ena omwe akukumana ndi mavuto omwewo. Ngakhale kuti tilibe "mapiritsi" kapena opaleshoni kuti tipeze matendawa, pali zambiri zomwe tingachite kuti athandize anthu kukhala bwino ndi matendawa, ndipo kafukufuku wamakono akupereka chiyembekezo kuti kupita patsogolo sikudzapangidwira kuchoka mtsogolo.
Zotsatira
- Garg, N., Park, S., Vucic, S. et al. Kusiyanitsa Chinthu Chochepa Chakumapeto kwa Magetsi a Neuron Syndromes. Journal of Neurology, Neurosurgery, ndi Psychiatry . 2016 Dec 21. (Epub patsogolo pa kusindikiza).
- Kasper, Dennis L .., Anthony S. Fauci, ndi Stephen L .. Hauser. Mfundo za Harrison za Mankhwala a M'kati. New York: Maphunziro a Mc Graw-Hill, 2015. Print.
- Ng, L., Khan, F., Young, C., ndi M. Galea. Matenda a Zizindikiro za Amyotrophic Lateral Sclerosis / Motor Neuron Matenda. Dongosolo la Cochrane la Zosintha Zogwirizana . 2017. 1: CD0111776.
- Roper. Adams ndi Victor's Principles of Neurology, 10e. Np: McGraw-Hill, 2014. Print.