Unonzwawo sekuti makumbo ako ari kupera simba here? Izvi zvinogona kunge zviri Lambert-Eaton Myasthenic Syndrome (LEMS)!

Dzimwe nguva unonzwa sekuti makumbo ako haana simba here, unonetseka kusimuka pachigaro, unonzwa kuneta paunokwira masitepisi here? Kana kuti unonzwa kuneta nezviratidzo zvakaita semaziso akaremara, muromo wakaoma, uye kuona kaviri here? Izvi zvingasava kuneta chete. Nhasi tichataura nezvechirwere chaungangodaro usina kunzwa nezvacho, asi chakakosha kuziva nezvacho, Lambert-Eaton Myasthenic Syndrome, kana kuti ``(LEMS)`` sezvatinochidana muchidimbu.

Chii chinonzi Lambert-Eaton Myasthenic Syndrome (LEMS)?

Zvichitaurwa zviri nyore, Lambert-Eaton Myasthenic Syndrome (LEMS) chirwere chinokonzerwa ne autoimmune disorder. Iye zvino ungave uchishamisika kuti chirwere chinokonzerwa ne autoimmune disorder chii . Miviri yedu ine immune system. Izvi ndizvo zvinotidzivirira nekurwisa utachiona hunokonzera zvirwere kubva kunze. Zvakafanana neuto riri munyika medu. Asi dzimwe nguva immune system iyi inotadza kushanda zvakanaka. Zvadaro inotanga kurwisa masero edu ane hutano. Ndizvo zvinoitika muLEMS.

Mu "LEMS" iyi, masoja edu ekudzivirira muviri anorwisa kubatana kuripo pakati petsinga dzedu dzemuviri netsandanyama . Kutaura chokwadi, kubatana uku kunonzi "neuromuscular junction". Kana kubatana uku kukanganisika, kutumira mameseji kubva mutsinga kuenda kutsandanyama kunokanganisika. Apa ndipo panoitika kushaya simba kwetsandanyama.

Kushaya simba uku kunowanzotanga muzvidya zvako zvepamusoro , asi kunogona kupararira zvishoma nezvishoma kusvika mumaoko ako, kufema, kumedza, uye kutaura tsandanyama.

Ndiani ane mukana mukuru wekugadzira `(LEMS)` iyi?

Kune mapoka maviri makuru anoumba `(LEMS)`.

1. Zvinobatanidzwa negomarara: Vanhu vanosvika 60% vane LEMS vane imwe mhando yegomarara. Rudzi rwakajairika igomarara remapapu remasero madiki . Zvinoshamisa kuti dzimwe nguva zviratidzo zveLEMS zvinogona kuoneka mwedzi kana kunyange makore gomarara risati ratanga. Vazhinji vevanhu ava vane makore anopfuura 60, varume, uye vakamboshandisa fodya.

2. Hapana kenza: Vamwe vanhu 40% vane LEMS havana kenza yakaonekwa. Vanhu ava vanowanzoonekwa vaine chirwere ichi pazera remakore angangoita 35 uye zvakare pazera remakore angangoita 60.

LEMS chirwere chisingawanzoitiki pakati pevana vadiki.

Ko kenza yemapapu yemasero madiki (LEMS) chete ndiyo ine chekuita neizvi here?

Kwete. Kenza yemapapu ine masero madiki ndiyo mhando yekenza yakajairika, asi mamwe marudzi ekenza anogonawo kusanganiswa neLEMS. Mienzaniso inosanganisira kenza yeprostate, thymoma, uye lymphoproliferative disorders. Nekuti zviratidzo zveLEMS zvinogona kuoneka zviratidzo zvekenza zvisati zvaonekwa, zvakakosha kuve nemiedzo yakakodzera yekutarisa kenza kana chiremba wako akakuongorora kuti une LEMS.

Ko LEMS inokonzerwa nekuchinja kwemajini here?

Masayendisiti anotenda kuti zvirwere zve autoimmune zvinogona kunge zvine chekuita nemajini. Sezvo LEMS iriwo chirwere che autoimmune, zvinokwanisika kuti inewo simba remajini, asi izvi hazvisati zvanyatsonzwisiswa.

`(LEMS)` yakajairika zvakadii?

LEMS chirwere chisingawanzoitiki . Zvinofungidzirwa kuti chirwere ichi chinobata vanhu vanosvika mamiriyoni 2.8 pasi rose. MuUnited States, kune vanhu vanosvika mazana mana vakashumwa vane LEMS. Kunyangwe pasina nhamba chaiyo yekuti vanhu vangani munyika medu, zviri pachena kuti ichi chirwere chisingawanzoitiki.

Ndezvipi zviratidzo zve `(LEMS)`?

Ngatitarisei zviratidzo zve `(LEMS)` chimwe nechimwe. Hazvisi zvese zviratidzo izvi zvinoitika panguva imwe chete mumunhu wese, zvimwe zviratidzo zvinogona kuoneka nekukurumidza, zvimwe zvinogona kuoneka gare gare.

• Kushaya simba kwetsandanyama uye kuneta: kunyanya mumakumbo ekumusoro.
• Kuoma kwekufamba: Kuoma kukwira masitepisi kana kusimuka pachigaro.
• Kurwadziwa kana kuomarara kwetsandanyama.
• Kunzwa kusanzwa zvakanaka mumakumbo.
• Maziso akarembera (ptosis): Izvi zvinogona kutaridzika sehope.
• Kuona zvinhu zviviri panguva imwe chete (diplopia).
• Muromo wakaoma nemaziso (xerostomia): kuderera kwemate, kuderera kwemisodzi mumaziso.
• Kuzvimba.
• Kudzikira kwedikita.
• Kuderedza huremu.
• Kuomerwa nekuita weti.
• Kusashanda zvakanaka kwenhengo dzemurume muvanhurume.
• Kuoma kutaura (dysarthria) uye kuoma kumedza (dysphagia): Izvi zvinowanzova zviratidzo zvinozobuda mushure mechirwere.
• Kuoma kufema (kushaya simba rekufema) uye kutadza kufema: Ichi chiratidzo chisingawanzoitiki uye chakakomba chinogona kuitika padanho rekupedzisira rechirwere.

Zvakakosha: Kana uine chimwe chezviratidzo izvi kana kupfuura, usafunge kuti iLEMS. Zvisinei, kana zviratidzo izvi zvikaramba zviripo, kuchenjera kutsvaga rubatsiro rwechiremba.

LEMS inowanzoonekwa sei uye inofambira mberi sei?

LEMS inowanzo tanga yakanganisa tsandanyama dzemuhudyu . Inogona kukanganisa tsandanyama dzepafudzi, tsandanyama dzemaoko nemakumbo, tsandanyama dzinobatsira pakutaura nekumedza, pamwe netsandanyama dzemaziso.

Pakutanga kwechirwere, ungaomerwa nekusimuka pachigaro, kukwira masitepisi kana zvikomo, kana kuita basa rinorema rinoda kushandisa makumbo ako.

Zviratidzo zvinowanzo buda zvishoma nezvishoma, kwemavhiki mashoma kusvika kumwedzi . Zvisinei, kana uine LEMS nekenza, zviratidzo zvinogona kubuda nekukurumidza.

Chii chinokonzera LEMS?

Sezvatakambokurukura, LEMS chirwere chinokonzerwa ne autoimmune . Izvi zvinoreva kuti dziviriro yemuviri wako (antibodies) inorwisa matishu ako ane hutano. MuLEMS, ma antibodies aya anorwisa nzvimbo inosangana masero etsinga netsandanyama, inonzi neuromuscular junction. Zvikuru, anorwisa nzira dzecalcium dziripo. Idzi nzira dzecalcium dzakakosha kuti tsinga dzipfuure nepakati.

Funga nezvazvo semutsara wemadhomino. Kurwiswa uku kunotanga kutevedzana kwezviitiko:

1. Ma antibodies anosunga kunzira dzecalcium dziri kumagumo emasero etsinga odzivhara.

2. Kana nzira dzecalcium dzikaderedzwa, huwandu hweacetylcholine, neurotransmitter, hunoburitswa hunoderera. Acetylcholine iyi ikemikari inokosha pakushanda kwetsandanyama.

3. Kana acetylcholine isina kubudiswa yakakwana, "mashoko" anoenda kumhasuru haasviki zvakanaka. Zvadaro tsandanyama dzako hadzigoni kushanda zvizere.

4. Nekuda kweizvozvo, tsandanyama hadzishande zvakanaka, zvichikonzera kushaya simba.

Heino maitiro anoita vanhu vane kenza yemapapu yemasero madiki: Kana uine kenza mumuviri wako, ma antibodies anowana chiratidzo chekuti "uende kuhondo." Asi pachinzvimbo chekurwisa masero ekenza (kunyanya, nzira dzecalcium dziri pamusoro pemasero ekenza), ma antibodies aya anorwisa nzira dzecalcium dziri kumagumo emasero etsinga zvisirizvo. Zvadaro zviitiko zvakafanana zvinoitika.

Masayendisiti anotenda kuti chikonzero cheLEMS muvanhu vasina kenza yemapapu kungori kusashanda zvakanaka kwemasoja emuviri. Asi hazvisati zvanyatsojeka kuti sei izvi zvichiitika.

Nzira yekuziva `(LEMS)` sei?

Danho rekutanga pakuongorora chirwere cheLEMS nderekuti chiremba wako akuongorore zvakanaka. Izvi zvinowanzosanganisira chiremba wetsinga, kana chiremba anonyanya kubata zvirwere zvetsinga netsandanyama. Chikamu cheizvi chinosanganisira kubvunza nezvezviratidzo zvako, nhoroondo yako yezvekurapa, uye chero mishonga yaunotora. Ongororo yakadzama yetsinga inoitwa kuti ione maitiro ekushaya simba nezviratidzo zvinoenderana neLEMS.

Danho rinotevera kuyedza. Miedzo ye `(LEMS)` inosanganisira:

• Kuongororwa kweropa: Izvi zvinogona kuona kana une ma antibodies ekurwisa calcium channel muropa rako. Vanhu vanosvika 85% vane LEMS vane ma antibodies aya muropa ravo.
• Electromyography (EMG): Bvunzo iyi inotarisa kuti tsandanyama dzako netsinga zviri kushanda pamwe chete zvakanaka sei. MuLEMS, bvunzo yeEMG ine zvakawanikwa zvakananga, saka inogona kubatsira kusimbisa chirwere chacho.
• X-ray yemapapu `(X-ray)` kana CT scan `(CT scan)` kana MRI scan `(MRI)` (yechipfuva): Nekuti chirwere chinonzi `(LEMS)` chinogona kuitika muvanhu vane kenza yemapapu yemasero madiki, bvunzo idzi dzinotsvaga zviratidzo zvekenza yemapapu.

Zviratidzo zveLEMS zvinogona kutanga kwemwedzi kusvika makore matanhatu kenza isati yatanga. Saka, mushure mekunge LEMS yaonekwa, unofanira kuongororwa kenza mwedzi mitatu kusvika mitanhatu yega yega kwemakore maviri. Chiremba wako achakurudzira purogiramu yekuongorora yakakunakira.

LEMS inorapwa sei?

Kune nzira dzakasiyana-siyana dzekurapa LEMS.

• Kurapa kenza iri pasi: Kana LEMS iine chekuita nekenza, senge kenza yemapapu yemasero madiki, kurapa kenza iyoyo kunogonawo kuderedza zviratidzo zveLEMS. Kurapa kenza kunogona kusanganisira chemotherapy, radiation therapy, uye/kana kuvhiyiwa. Izvi zvinoenderana nerudzi rwekenza uye mazano achiremba wako.
• Mishonga chaiyo ye (LEMS): Izvi zvinosanganisira mishonga inoderedza zviratidzo uye mishonga inodzora masoja emuviri.

Kurapa kwezviratidzo

• Amifampridine (Firdapse®), Guanidine: Mishonga iyi inowedzera simba remhasuru. Inowedzera kuburitswa kweacetylcholine neurotransmitter panosangana tsinga dzemhasuru, zvichivandudza masaini emhasuru.
• Pyridostigmine (Pyridostigmine - Mestinon®): Pyridostigmine inoshanda nekudzivirira kuparara kweacetylcholine panosangana tsinga dzemuviri. Izvi zvinowedzerawo acetylcholine signaling uye zvinovandudza simba retsandanyama. Pyridostigmine dzimwe nguva inopihwa pamwe chete neamifampridine kuvandudza mashandiro etsandanyama nekurapa zviratidzo zveLEMS zvakaita semuromo wakaoma, maziso akaoma, kuvimbiwa, kusagadzikana pabonde, uye kudzikira kwekudikitira. Zvisinei, kushandiswa kwepyridostigmine yeLEMS kushandiswa kusina kurongwa, zvichireva kuti U.S. Food and Drug Administration (FDA) haina kubvumidza mushonga wechirwere ichi.

Kurapa kunogadzirisa immune system

• Mishonga inodzvinyirira immune system:Mishonga iyi inobatsira kumisa kana kudzora mashandiro e "kudzivirira" kwemuviri wako. Chiremba wako anogona kukupa mishonga iyi kana mimwe mishonga isingadzore zviratidzo zvako. Semuenzaniso, mushonga unonzi prednisone unogona kupihwa pamwe chete neazathioprine kana cyclosporine kana kuti pasina.
• Kuchinjana kweplasma: Mukuita uku, muchina unoparadzanisa chikamu chemvura cheropa rako (plasma) kubva kumasero eropa. Ma antibodies ako ari muplasma iyi. Mushure mekuparadzaniswa, ropa rinodzoserwa mumuviri wako. Chiremba wako anogona kufunga nezvekurapa uku kana uine zviratidzo zvakakomba uye uchida kurapwa nekukurumidza.
• MaImmunoglobulins: Kune vamwe vanhu, kurapwa ne immunoglobulins yakawanda kunogona kubatsira kudzivirira chirwere ichi kuti chisanyanya kuipa.

LEMS inogona kudzivirirwa here?

Chikonzero chaicho cheLEMS hachisati chanyatsonzwisiswa, saka hazvizivikanwe kana paine nzira yekudzivirira chirwere ichi.

Zvisinei, sezvo LEMS ichigona kukura muvanhu vane kenza yemapapu, unogona kubatsira kudzivirira kenza yemapapu ne:

• Usasvuta. Kana uchishandisa fodya, kumbira chiremba wako kuti akubatsire kurega.
• Dzivisa kusvuta fodya inosvuta vanhu vanosvuta.
• Tarisa kana gasi reRadon riri mumba mako. Kugara uchisangana negasi reRadon kwenguva refu kunogona kuwedzera njodzi yako yekubatwa nekenza yemapapu.

Unogonawo kutora matanho ekuchengetedza mararamiro ane hutano:

• Idya michero nemiriwo yakawanda. Tevedzera kudya kweMediterranean kana DASH.
• Ramba uine huremu hwakanaka.
• Edza kurovedza muviri sezvawakarayirwa nachiremba wako.
• Wana hope dzakakwana - kazhinji maawa manomwe kusvika mapfumbamwe.
• Deredza huwandu hwekuzvidya mwoyo kwako. Kushushikana kunogona kuwedzera zviratidzo.
• Dzivisa kugeza mumvura inopisa. Kupisa kunogona kuwedzera zviratidzo zveLEMS.
• Ona chiremba wako nguva dzose, uye muzivise nekukasika kana hutano hwako hwachinja kana kuti ukava nezviratidzo zvitsva.

Ko LEMS inogona kurapwa zvizere here?

Zvinosuwisa kuti hapana mushonga weLEMS. Kurapa kenza iri pasi peLEMS kwakaratidzwa kuti kunovandudza zviratidzo zveLEMS. Mishonga inodzvinyirira muviri inogona kubatsira kudzora kurwisa kwe autoimmune, uye kunewo marapirwo anogona kubatsira kuderedza zviratidzo nekuwedzera huwandu hweacetylcholine mumitsetse yetsinga uye kuvandudza simba retsandanyama.

Kana uine LEMS usina kenza, unogona kutarisira kurarama hupenyu hwakajairika. Kana uine LEMS nekenza, zviratidzo zvako zvinogona kuvandudzika nekufamba kwenguva (kunyange zvisiri zvachose) kana kenza ikaonekwa uye ikatarisirwa nekukurumidza.

Hupenyu hwako hunoenderana zvakanyanya nekuoma kwezviratidzo zvako.

Ndinofanira kutaura nachiremba wangu rini kana ndine `(LEMS)`?

Kana paine shanduko pahutano hwako, kana zviratidzo zvako zvikawedzera, taura nachiremba wako chero nguva . Usatya, usavhunduka. Vanachiremba varipo kuti vakubatsire.

Ndeupi musiyano uripo pakati pe `(LEMS)` ne `(Myasthenia Gravis - MG)`?

Lambert-Eaton myasthenic syndrome (LEMS) uye myasthenia gravis (MG) dzine zviratidzo zvakafanana. Zvose zvinokonzera kushaya simba kwetsandanyama uye kuneta.

Zvisinei, myasthenia gravis (MG) inowanzo tanga yakanganisa tsandanyama dzakatenderedza maziso ako netsandanyama dzinoshandiswa kumedza. Gare gare, inogona kukanganisa tsandanyama dzakawanda, kusanganisira tsandanyama dzekufema netsandanyama dzemakumbo.

LEMS inowanzotanga nekusasimba kwemuhudyu mako ekumusoro, yozobva yaenda mumaoko ako. LEMS inogonawo kukonzera kusasimba kwemhasuru dzemaziso ako, asi kazhinji inenge isina kusimba kupfuura myasthenia gravis (MG).

Muchirwere chemyasthenia gravis (MG), kushaya simba kwakanyanya kwetsandanyama dzekufema (tsandanyama dzinokubatsira kufema) kunogona kuuraya. Izvi hazviwanzoitika muLEMS.

Vanhu vane myasthenia gravis (MG) havawanzo ratidza zviratidzo zvakawanda zvekusashanda zvakanaka kwetsinga dzemuviri (semuenzaniso, maziso akaoma, muromo wakaoma, kuzvimba, kudzikira kwekudikitira, kusagadzikana pabonde). Kusiyana neizvi, vanhu vane LEMS vanowanzova nezviratidzo izvi, uye zviratidzo izvi dzimwe nguva zvinogona kubatsira kusiyanisa zvirwere zviviri izvi.

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Pari zvino pamwe wava kunzwisisa kuti Lambert-Eaton Myasthenic Syndrome (LEMS) chirwere chinokonzerwa nekushanda zvakanyanya kwemasoja emuviri. Masero ako etsinga haatumiri mameseji akakwana kumhasuru dzako kuti "dzipwanye." Mugumisiro wacho unova kusasimba kwemhasuru nezvimwe zviratidzo.

Yeuka, vanhu vanosvika 60% vane LEMS vane kenza yemapapu yemasero madiki. Kana uine LEMS asi usina kenza, iva nechokwadi chekuti iwe nachiremba wako munoongororwa kenza mwedzi mishoma yega yega kwemakore maviri mushure mekuonekwa kuti une chirwere ichi.

Kune mishonga. Izvi zvinosanganisira kurapa kenza kana iripo, pamwe nekushandisa mishonga inovandudza kutaurirana kwemasero etsinga netsandanyama. Kuvandudza kutaurirana kunogona kuvandudza simba retsandanyama nezvimwe zviratidzo.

Ndinovimba ruzivo urwu ruchakubatsira.Gara uchiyeuka kuti kana uine chero dambudziko rehutano, zvakanaka kutaura nachiremba ane ruzivo nezvazvo.

Lambert -Eaton myasthenic syndrome, LEMS, zvirwere zve autoimmune, kusasimba kwetsandanyama, kenza yemapapu yemasero madiki, neuromuscular junction, acetylcholine