Mhando dze Motor Neuron Zvirwere Zvinosanganisira ALS nezvimwe
Kana vanhu vazhinji vachifunga nezvechinhu chero chipi zvacho pavanonzwa mazwi "motokari yeurus neuron," vanofunga nezve amyotrophic lateral sclerosis (ALS). Iko kune, zvisinei, zvimwe zvimwewo mhando dzemagetsi e-neuron pamwe chete. Nenzira yakanaka, zvirwere zvose zvemotokari zvinowanzojairika.
Overview
Patinenge tichifamba, zviratidzo zvemagetsi zvinotumirwa kubva muuropi zvichienda kumutsipa wepelinha pamwe nepamusoro pemotokari yeururoni.
Masero emagetsi anokonzera murunyanga rwepinja yepinini uye anobva atumirwa pamwe nemakumbo emakumbo ekudzivirira mukati mehutachiona. Zviratidzo zvemagetsi zvinofamba pamwe nemishumo ye-neurons ye musimba kuti iite chibvumirano, zvichiguma nekufamba. Zvichienderana nezvimwe zviitiko zvekuongorora zvinowanikwa, nyanzvi dzezvehutano dzinogona kuziva kuti dambudziko riri kupi mune hurongwa hwemagetsi, uye kubva pane iyo, inogona kuongororwa.
Mamiriro ezvinhu anogadzirisa zviratidzo izvi zvinowanzozivikanwa sezvirwere zvemotokari. Runyanga rwekupedzisira rwemutsara unotakura mashoko anotaura nezvekunzwa, asi runyanga rwekare runotakura mashoko ane chokuita nekufamba. Motokari neuron zvirwere, nekuda kwechikonzero ichi, zvinonyanya kuchinja kufamba.
Zviratidzo Zvizhinji uye Zviratidzo
Motokari ye-neuron zvirwere zvinogona kuparadzaniswa mumapoka maviri makuru, zvichienderana nekuti inobata mumusoro wepamusoro neuron kana kudzikisa motokari ineuroni. Zvimwe zvirwere zvinokonzerwa neuron neuron zvinokanganisa chete motokari yepamusoro neuroni, asi vamwe inonyanya kunetseka pamakumbo emakumbo neuroni.
Zvimwe, zvakadai seAAL, zvinosanganisira zvose.
Zviratidzo zvepamusoro yemapuraneti neuron zvirwere zvinosanganisira:
- Kunyanya kushamwaridzana - Kusanganiswa kwekuoma kwemasumbu, kusimba, kugadzikana, uye kusagadzikana. Nekunyanyisa kwakanyanya, mishonga yako inogona kunzwa "yakanamatira." Nekunyatsopisa, iwe unogona kukwanisa kutamisa misungo yako, asi inopindura nenzira isingatarisiri kana yakajeka.
- Kubwinya - Kuzvimirira "kuoma" kwemisungo.
- Kuwedzera kwakadzika tendon reflexes - Somuenzaniso, bheji rako rinogona kunge rakakurumbira kudarika nguva yakawanda.
Zviratidzo zvepasi yemapuraneti neuron zvinosanganisira:
- Atrophy - Kurasikirwa kwesimba uye kuwanda kwemisungo.
- Fasciculations - Kupindira kwepachivande uye kusingabatsiri kwemisumbu iyo inogona kuonekwa sechinyangarika pasi peganda.
Aina dze Motor Neuron Diseases
Kune zvirwere zvakasiyana-siyana zvakasiyana-siyana neuron zvinopesana nekuremekedza kana kuti zvinokanganisa kumusoro kana kumadekari emakumbo emakumbo, zviratidzo zvekutanga, boka rezera ravanoshandisa, uye kufungidzira. Zvimwe zvezvi zvinosanganisira:
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), inozivikanwawo sehosha yaLou Gehrig, inofambira mberi ine motokari yeurus neuron inokanganisa vanhu vanenge 6 000 gore negore muUnited States. Inotanga nemukoma simba, kazhinji kune rumwe rutivi rwemuviri. Mamiriro acho anotanga mavoko kakawanda kupfuura tsoka. Pakutanga, chiratidzo chikuru chinogona kuva fasciculations, asi pakupedzisira, chinopfuurira nezvose zviri kumusoro uye motor neuron zviratidzo nezviratidzo. Apo iyo diaphragm inokanganiswa, mechanical ventilation inogona kudiwa. Chirwere ichi kazhinji hachiwanzoiti kukanganisa, uye vanhu vakawanda vakangwarira (vasina dambudziko rekushaya mungozi) kunyange kana chirwere ichi chasvika.
Ivhareji yekutarisira upenyu neAls inenge makore maviri kusvika mashanu asi inogona kusiyana-siyana, ne5 muzana yevanhu vari vapenyu mushure memakore makumi maviri. Zvinofadza, vanhu vakadai sasayendisiti Stephen Hawking vari kubatsira kubatsira kukurudzira zvose zveAIDS nezvimwe zvirwere zvemoto neuron.
Primary Lateral Sclerosis
Primary primary latal sclerosis (PLS) chirwere chepamusoro chemidhi yeuroni, kunokanganisa zviratidzo kubva muuropi kusvika kumutsipa wepelinha. Masero ari mu cerebral cortex anokonzera kufamba zvishoma nezvishoma anofa. Mugumisiro utera hunononoka zvishoma nezvishoma hunobatanidzwa nepamusoro yemotokari neuron zviratidzo, zvakadai sokuperesa, kusagadzikana, uye kuwedzera kwakadzika tendon reflexes.
Kusiyana neamyotrophic lateral sclerosis, kupera kwemajini neuron kuwanikwa, zvakadai seatrophy uye fasciculations, haisi yakakurumbira. Hazvinyatsozivi kuti PLS yakawanda sei, asi tinotenda kuti haina kufanana neAIDS.
Pakutanga kwechirwere chechirwere, primary primary lateral sclerosis inogona kuvhiringidzwa neAIDS. Sezvo ALS inogona kutanga nepamusoro pemiti yepamusoro neuron zviratidzo, zvinogona kunge makore asati aonekwa kuti PLS inowoneka. Kunyange panguva iyoyo, zvingave zvakaoma kutaura kuti ndezvipi zviripo zvinokonzera zviratidzo, sezvo vamwe vanhu vanofungidzirwa kuti PLS dzichakudziridza motokari yepasi neuron kuwana, zvichiratidza kuti chirwere chacho chave chiri ALS. Zvose izvi inzira inonyanya kuvhiringidza kutaura kuti zvingave zvisingaiti kuziva kana mamiriro acho ari ALS kana PLS kwemakore anoverengeka mushure mokutanga kwezviratidzo.
Mamwe mamiriro ezvinhu, akadai semhaka yepastic paraparesis, inofanirawo kutongwa kunze. PLS inotanga kufambira mberi zvishoma nezvishoma kupfuura ALS, nevarwere vanowanzogara makore makumi maviri nemararamiro avo.
Progressive Muscular Atrophy
Mune dzimwe nzira, kufambira mberi kwemuscular atrophy (PMA) inopesana nepakutanga lateral sclerosis. Mumakwikwi eAppa, mhepo chete yepasi ndiyo inotambudzwa, asi, mu PLS, chete iyo yemakumbo yepamusoro neuroni yakakuvara. Kubvira pakaderera motokari neuroni zvakakanganiswa, kushaya simba kunofambira mberi chiratidzo chinowanzoonekwa. Sezvo motokari yepamusoro ye-neuroni isingatambudziki, zviratidzo zvepamusoro zvemhepo neuron zvakadai sokugadzikana hakuiti. Progressive muscular atrophy isinganzwisisiki kupfuura ALS asi ine tsananguro iri nani.
Iyo inogona kuva nzira inoshungurudza yekuita kuti kuongororwa kwekufambira mberi kwemascular atrophy sezvo zviratidzo zvakafanana nezvimwe mamiriro. Kunyanya, zvirwere zvakadai seAAL, multifocal motor neuropathy (chimiro chepachiviri chetachiona neuropathy ) uye musipinha muscular atrophy inoda kutanga kutariswa kunze kwekutanga kusati kwarongororwa kunogona kuitwa.
Progressive Bulbar Palsy
Progressive bulbar palsy inosanganisira kuderera kunononoka kweuropiyo, iyo ine mitsipa ( zvinokonzera mitsipa ) iyo inodzora chiso, rurimi, uye nehuro. Somugumisiro, mumwe munhu ane chinhambwe chinopfurikidza bulbar palsy achatanga kuva nekutambudzika kutaura, kumedza nekudzora. Kuoma kwechiremera kunogonawo kuoneka zvakajeka sezvo chirwere chacho chinopfuurira, uye zvose zviri kumusoro uye pasi pedhi motokari zviratidzo. Vanhu vane progressive bulbar palsy vanogonawo kutadza kudzora uye dzimwe nguva kutadza kusina kukodzera kwekuseka kana kuchema. Hazvishamisi kune vanhu vane progressive bulbar palsy kuti varambe vachiwedzera ALS. Myasthenia gravis inoratidzira neuromuscular disorder inogonawo kuisa nenzira yakafanana.
Post-Polio Syndrome
Polio ndiyo hutachiona hunopikisa motokari yeururoni muhwamanda yepenja yepinini, zvichikonzera kuora mitezo. Ndinofara, nekuda kwemishonga inopisa, chirwere ichi chakanyanya kupedzwa. Vamwe veavo vane chirwere ichi, zvisinei, vanogona kunyunyuta nezvehutera hunonzi post-polio syndrome. Izvi zvinogona kunge zvakakonzerwa nokukwegura kana kukuvadzwa zvichiita kuti vashoma vasara motori neuroni vachidzora kufamba kwechimwe chikwata chakabatwa kuti chife. Dambudziko racho rinongobatsira vanhu vakwegura vane chirwere cheporiya munguva yakapfuura. Kazhinji haisi kutyisa upenyu.
Matambudziko aKennedy
Chirwere cheKennedy chinokonzerwa ne -X-inoshandiswa genetic mutation iyo inokanganisa receptor ye androgen. Chirwere ichi chinokonzera kupera zvishoma nezvishoma kuderera uye kurwadziwa kwemisungo iri pedyo nemhuto. Chiso, chirabwe, uye rurimi zvinobatanidzwawo. Nemhaka yekuti X-yakabatanidzwa, chirwere cheKennedy chinowanzobata vanhu. Vakadzi vane genetic mutation ndivo vatakuri, vane 50 mukana wekutapudza jeni kune vana vavo. Vakadzi vane mutsika vanogonawo kutambura nezviratidzo zviduku, zvakadai semakumbo emunwe, panzvimbo yekushaya simba kwakanyanya.
Nemhaka yokuti chirwere ichi chinokanganisa iyo androgen receptor (iyo inogamuchirwa nesrogen uye testosterone), varume vane chirwere vanogonawo kutambura nezviratidzo zvakadai segynecomastia (kuwedzerwa kwezamu), testicular atrophy, uye erectile dysfunction. Upenyu hwevanhu vane chirwere chaKennedy hunowanzoshandiswa, asi sezvo utera hwavo huchifambira mberi vangada chikwata chevasimba.
Spinal Muscular Atrophy
Mushonga wemisipiresi atrophy chirwere chakagara nhaka chinowanzokonzera vana. Inokonzerwa nezviremera mujeni re SMN1 uye inotora nhaka mumutambo wokuzvidzivirira. Nokuda kweiyo jeni risina kukwana, haikwanisi mapuroteni emu SMN, uye izvi zvinotungamirira pakuora kwemashure emakumbo neuroni. Izvi zvinokonzera kushaya simba uye kusvibiswa kwemisimba.
Iko kune mitatu mikuru mikuru yeSMA, imwe neimwe inosanganisira vana pane imwe nguva yakasiyana.
- SMA-type 1, iyo inonziwo "Werdnig-Hoffman", inova pachena panguva iyo mwana ane mwedzi mitanhatu. Mwana wacho achava ne hypotonia (floppy muscle) uye haazofambi achifamba pakarepo. Havazokwanisi kugara vari voga panguva yakatarwa. Pamusana pekuoma nemhepo uye nekuchengetedza simba rakakwana rokufema, vazhinji vevana vanofa nezera remakore maviri.
- SMA inotanga II inotanga zvishoma gare gare, ichioneka pakati pemakore 6 kusvika ku18. Idzi vana havazokwanisi kumira kana kufamba pasina rubatsiro, uyewo vachava nezvinetso nekufuridzira. Zvisinei, vana vane SMA-type II vanorarama kwenguva yakareba kupfuura avo vane Werdnig-Hoffman, dzimwe nguva vachigara muhukuru.
- SMA-type IIII inonziwo Kugelberg-Welander chirwere, inooneka pakati pemakore maviri nemakore gumi nemaviri. Vana vane chirwere ichi vangave nechinetso chekufamba kana kukwira makwande. Vanogonawo kunge vane zvinetso zvakare, zvakadai sa scoliosis . Zvisinei, vana vane chirwere ichi vanogona kunge vane hupenyu hwemazuva ose.
Kuongorora nekurapa
Hakuchina kurapa kwakanyanya kune chero ipi zvayo yemotokari ye-neuron. Kurapa kurapwa kunotarisa pakuraira zviratidzo zvechirwere zvakanakisisa sezvinobvira. Zvisinei, kuitira kuti uzive zviratidzo zvinotarisirwa, uyewo kutonga kune dzimwe zvirwere zvinoporesa, zvinokosha kuti uwane hutano hwakanaka.
Kushandisa kuongorora kwepanyama uye dzimwe nzira dzakadai se electromyography , dzidzo dzenzara dzekudzidzira, uye maitiro ekudzivirira maitiro kana zvichifanira, maurologist anokwanisa kubatsira kutsanangura kunyatsoongorora. Kuva nechirwere chakakodzera kunobvumira nyanzvi yako yezveuropiti kuti iite zviratidzo zvako zvakanyanya sezvinobvira uye kutarisira uye kugadzirira chero zvinetso zvinotarisirwa.
Kutsungirira
Pakutanga, takataura kuti "nerufaro" motokari yeeuron neuron isingawanzoitiki. Izvi zvingava zvakanaka kunze kwekuti iwe kana mudiwa waunokurudzira imwe yemamiriro aya. Zvadaro, kunze kwekutambura kwezviratidzo zvezvirwere izvi, unogona kuona kuti pane zvishoma zvitsvakurudzo uye zvishoma kudyirwa pane zvaunotarisira. Kunyange zvazvo zvirwere izvi zvisingawanzoitiki, zviyero zvakadai seOrphan Drug Act zviri kutungamirira zvakanyanya kune izvozvi zvisiri izvo asi zvisina kukosha.
Iwe unganzwa uchinge uri woga kana wakamboonekwa kuti une chirwere chinonzi neuron disease. Kusiyana nemapoka makuru e "vatsigiri vekenza yebonde" kunze uko, hationi maqembu makuru, semuenzaniso, kufambira mberi kwebarbar palsy vatsigiri. Asi kuziva kuri kuwedzera, uye pamwe neAIDS, kutsigira.
Vanhu vane zvirwere zvemoto neuron vanoda rubatsiro sevaya vane zvinhu zvakafanana. Kunyange iwe usingave neboka rinotsigira munharaunda yako, kune mitsigiritsi inotsigirwa paIndaneti apo vanhu vane mimwe mitemo ye neuron vanogona "kusangana" uye vanotaurirana nevamwe vari kutarisana nemamwe matambudziko akafanana. Kunyange zvazvo tisina "piritsi" kana chirwere chokurapa chirwere ichi, pane zvakawanda zvingaitwa kubatsira vanhu kurarama zvakanaka nechirwere, uye kutsvakurudza ikozvino kunopa tariro kuti kufambira mberi kuchaitwa mune izvo zvisati zviri kure kubva munguva yemberi.
Sources
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