Kodi mukumvanso ngati miyendo yanu yayamba dzanzi? Izi zitha kukhala Lambert-Eaton Myasthenic Syndrome (LEMS)!

Kodi nthawi zina mumamva ngati miyendo yanu yafooka, mumavutika kudzuka pampando, mumamva kutopa mukakwera masitepe? Kapena mumamva kutopa ndi zizindikiro monga zikope zolemera, pakamwa pouma, komanso kuona zinthu ziwiri? Izi sizingakhale kutopa kokha. Lero tikambirana za vuto lomwe simunamvepo, koma lofunika kwambiri kudziwa, Lambert-Eaton Myasthenic Syndrome, kapena ``(LEMS)`` monga momwe timalitchulira mwachidule.

Kodi Lambert-Eaton Myasthenic Syndrome (LEMS) ndi chiyani?

Mwachidule, Lambert-Eaton Myasthenic Syndrome (LEMS) ndi matenda a autoimmune. Tsopano mwina mukudabwa kuti matenda a autoimmune ndi chiyani . Matupi athu ali ndi chitetezo chamthupi. Ichi ndi chomwe chimatiteteza polimbana ndi majeremusi omwe amayambitsa matenda ochokera kunja. Zili ngati gulu lankhondo mdziko lathu. Koma nthawi zina chitetezo chamthupi ichi chimasokonekera. Kenako chimayamba kuukira maselo athu athanzi. Ndicho chomwe chimachitika mu LEMS.

Mu `(LEMS)` iyi, chitetezo chathu chamthupi chimaukira kulumikizana pakati pa mitsempha yathu yamagetsi ndi minofu . Kunena zoona, kulumikizana kumeneku kumatchedwa `neuromuscular junction`. Pamene kulumikizana uku kwawonongeka, kutumiza mauthenga kuchokera ku mitsempha kupita ku minofu kumachepa. Apa ndi pamene kufooka kwa minofu kumachitika.

Kufooka kumeneku nthawi zambiri kumayambira m'chiuno mwanu chapamwamba , koma pang'onopang'ono kumatha kufalikira m'manja mwanu, kupuma, kumeza, ndi minofu yolankhula.

Ndani amene ali ndi mwayi waukulu wopeza `(LEMS)`?

Pali magulu awiri akuluakulu omwe amapanga `(LEMS)`.

1. Zokhudzana ndi khansa: Pafupifupi 60% ya anthu omwe ali ndi LEMS ali ndi khansa yamtundu wina. Mtundu wofala kwambiri ndi khansa ya m'mapapo ya maselo ang'onoang'ono . Chodabwitsa n'chakuti nthawi zina zizindikiro za LEMS zimatha kuonekera miyezi ingapo kapena zaka khansa isanayambe. Ambiri mwa anthuwa ali ndi zaka zoposa 60, ndi amuna, ndipo adagwiritsa ntchito fodya.

2. Palibe khansa: Anthu ena 40% omwe ali ndi LEMS sanapezeke ndi khansa. Anthuwa nthawi zambiri amapezeka ali ndi zaka pafupifupi 35 ndipo amapezekanso ali ndi zaka pafupifupi 60.

LEMS ndi vuto losowa kwambiri pakati pa ana aang'ono.

Kodi khansa ya m'mapapo ya maselo ang'onoang'ono (LEMS) yokha ndi yomwe imagwirizana ndi izi?

Ayi. Khansa ya m'mapapo ya maselo ang'onoang'ono ndiyo mtundu wofala kwambiri wa khansa, koma mitundu ina ya khansa ingathenso kugwirizanitsidwa ndi LEMS. Zitsanzo zake ndi khansa ya prostate, thymoma, ndi matenda a lymphoproliferative. Chifukwa zizindikiro za LEMS zimatha kuwonekera zizindikiro za khansa zisanayambe, ndikofunikira kukhala ndi mayeso oyenera kuti muwone ngati muli ndi khansa ngati dokotala wanu akukudziwitsani kuti muli ndi LEMS.

Kodi LEMS imayamba chifukwa cha kusintha kwa majini?

Asayansi amakhulupirira kuti matenda a autoimmune nthawi zambiri amakhala ndi ubale wina ndi majini. Popeza LEMS ndi matenda a autoimmune, n'zotheka kuti ilinso ndi mphamvu ya majini, koma izi sizikumveka bwino.

Kodi `(LEMS)` ndi yofala bwanji?

LEMS ndi matenda osowa kwambiri . Akuti matendawa amakhudza anthu pafupifupi 2.8 miliyoni padziko lonse lapansi. Ku United States, pali milandu pafupifupi 400 ya LEMS yomwe yanenedwa. Ngakhale kuti palibe ziwerengero zenizeni za kuchuluka kwa anthu m'dziko lathu, n'zoonekeratu kuti vutoli ndi losowa kwambiri.

Kodi zizindikiro za `(LEMS)` ndi ziti?

Tiyeni tiwone zizindikiro za `(LEMS)` chimodzi ndi chimodzi. Sizizindikiro zonsezi zomwe zimachitika nthawi imodzi mwa aliyense, zizindikiro zina zingawonekere msanga, zina zingawonekere mochedwa.

• Kufooka kwa minofu ndi kutopa: makamaka m'miyendo yakumtunda.
• Kuvuta kuyenda: Kuvuta kukwera masitepe kapena kudzuka pampando.
• Kupweteka kapena kuuma kwa minofu.
• Kumva dzanzi m'miyendo.
• Zikope zopindika (ptosis): Izi zitha kuwoneka ngati kugona.
• Kuona zinthu ziwiri nthawi imodzi (diplopia).
• Pakamwa ndi maso ouma (xerostomia): kuchepa kwa malovu, kuchepa kwa misozi m'maso.
• Kudzimbidwa.
• Kuchepa kwa thukuta.
• Kuchepetsa thupi.
• Kuvuta kukodza.
• Kulephera kwa erectile mwa amuna.
• Kuvuta kulankhula (dysarthria) ndi kuvutika kumeza (dysphagia): Izi nthawi zambiri zimakhala zizindikiro zomwe zimayamba pambuyo pa matendawa.
• Kuvuta kupuma (kupuma movutikira) ndi kuvutika kupuma: Ichi ndi chizindikiro chosowa komanso choopsa chomwe chingachitike kumapeto kwa matendawa.

Chofunika: Ngati muli ndi chimodzi kapena zingapo mwa zizindikirozi, musaganize kuti ndi LEMS. Komabe, ngati zizindikirozi zikupitirira, ndi bwino kufunafuna upangiri wa dokotala.

Kodi nthawi zambiri LEMS imawonekera bwanji ndipo ikupita patsogolo bwanji?

Kawirikawiri LEMS imakhudza minofu ya ntchafu . Kenako imatha kukhudza minofu ya mapewa, minofu ya manja ndi miyendo, minofu yomwe imathandiza polankhula ndi kumeza, komanso minofu ya maso.

Kumayambiriro kwa matendawa, mungakhale ndi vuto lodzuka pampando, kukwera masitepe kapena mapiri, kapena kugwira ntchito yolemetsa yomwe imafuna kugwiritsa ntchito miyendo yanu.

Zizindikiro nthawi zambiri zimakula pang'onopang'ono, kwa milungu ingapo mpaka miyezi ingapo . Komabe, ngati muli ndi LEMS yokhala ndi khansa, zizindikiro zimatha kukula mofulumira.

Kodi n’chiyani chimayambitsa LEMS?

Monga tafotokozera kale, LEMS ndi matenda odziteteza okha . Izi zikutanthauza kuti chitetezo cha thupi lanu (ma antibodies) chimaukira minofu yanu yathanzi. Mu LEMS, ma antibodies amenewa amaukira malo omwe maselo amitsempha ndi minofu zimakumana, otchedwa neuromuscular junction. Makamaka, amaukira njira za calcium kumeneko. Njira za calcium izi ndizofunikira kuti mauthenga amitsempha adutse.

Taganizirani izi ngati mzere wa ma domino. Kuukira kumeneku kumayambitsa zochitika zingapo:

1. Ma antibodies amamangirira ku njira za calcium kumapeto kwa maselo a mitsempha ndikuzitseka.

2. Pamene njira za calcium zachepa, kuchuluka kwa acetylcholine, neurotransmitter, komwe kumatulutsidwa kumachepa. Acetylcholine iyi ndi mankhwala ofunikira pakugwira ntchito kwa minofu.

3. Pamene acetylcholine yokwanira siitulutsidwa, "mauthenga" ku ulusi wa minofu sapita bwino. Kenako minofu yanu singathe kugwira ntchito mokwanira.

4. Zotsatira zake, minofu sigwira ntchito bwino, zomwe zimapangitsa kuti munthu afooke.

Umu ndi momwe anthu omwe ali ndi khansa ya m'mapapo ya maselo ang'onoang'ono amakhalira ndi LEMS: Mukakhala ndi khansa m'thupi lanu, ma antibodies amapeza chizindikiro choti "mupite kunkhondo." Koma m'malo moukira maselo a khansa (makamaka, njira za calcium pamwamba pa maselo a khansa), ma antibodies amenewa amaukira molakwika njira za calcium kumapeto kwa maselo a mitsempha. Kenako zochitika zomwezo zimachitika.

Asayansi amakhulupirira kuti chomwe chimayambitsa matenda a LEMS mwa anthu omwe alibe khansa ya m'mapapo ndi kulephera kwa chitetezo chamthupi. Koma sichinadziwikebe chifukwa chake izi zimachitika.

Kodi mungazindikire bwanji `(LEMS)`?

Gawo loyamba pozindikira matenda a LEMS ndi dokotala wanu kuti akufufuzeni mosamala. Izi nthawi zambiri zimaphatikizapo katswiri wa mitsempha, kapena dokotala yemwe ndi katswiri wa matenda a mitsempha ndi minofu. Gawo la njirayi limaphatikizapo kufunsa za zizindikiro zanu, mbiri yanu yachipatala, ndi mankhwala aliwonse omwe mumamwa. Kuwunika mwatsatanetsatane kwa mitsempha kumachitika kuti muwone mawonekedwe a kufooka ndi zizindikiro zomwe zikugwirizana ndi LEMS.

Gawo lotsatira ndi kuyesa. Mayeso a `(LEMS)` akuphatikizapo:

• Kuyezetsa magazi: Izi zitha kuwona ngati muli ndi ma antibodies oletsa calcium m'magazi mwanu. Pafupifupi 85% ya anthu omwe ali ndi LEMS ali ndi ma antibodies amenewa m'magazi mwawo.
• Electromyography (EMG): Mayesowa amayang'ana momwe minofu ndi mitsempha yanu zimagwirira ntchito limodzi. Mu LEMS, mayeso a EMG ali ndi zotsatira zenizeni, kotero angathandize kutsimikizira matendawa.
• Kujambula X-ray ya m'mapapo `(X-ray)` kapena CT scan `(CT scan)` kapena MRI scan `(MRI)` (ya pachifuwa): Chifukwa matenda otchedwa `(LEMS)` amatha kufalikira mwa anthu omwe ali ndi khansa ya m'mapapo ya maselo ang'onoang'ono, mayesowa amafufuza zizindikiro za khansa ya m'mapapo.

Zizindikiro za LEMS zingayambe miyezi ingapo mpaka zaka zisanu ndi chimodzi khansa isanayambe. Chifukwa chake, mukapeza LEMS, muyenera kuyezetsa khansa miyezi itatu kapena isanu ndi umodzi iliyonse kwa zaka zosachepera ziwiri. Dokotala wanu adzakulangizani nthawi yoyezetsa khansa yomwe ili yoyenera kwa inu.

Kodi LEMS imachiritsidwa bwanji?

Pali njira zingapo zochizira matenda a LEMS.

• Kuchiza khansa yomwe ili mkati mwake: Ngati LEMS ikugwirizana ndi khansa, monga khansa ya m'mapapo ya maselo ang'onoang'ono, kuchiza khansa imeneyo kungachepetsenso zizindikiro za LEMS. Mankhwala a khansa angaphatikizepo chemotherapy, radiation therapy, ndi/kapena opaleshoni. Izi zimatengera mtundu wa khansa ndi malangizo a dokotala wanu.
• Mankhwala enieni a (LEMS): Izi zikuphatikizapo mankhwala omwe amachepetsa zizindikiro ndi mankhwala omwe amawongolera chitetezo cha mthupi.

Chithandizo cha zizindikiro

• Amifampridine (Firdapse®), Guanidine: Mankhwalawa amawonjezera mphamvu ya minofu. Amawonjezera kutulutsidwa kwa acetylcholine ya neurotransmitter pamalo olumikizirana mitsempha, zomwe zimapangitsa kuti zizindikiro zifike ku minofu.
• Pyridostigmine (Pyridostigmine - Mestinon®): Pyridostigmine imagwira ntchito poletsa kusweka kwa acetylcholine pamalo olumikizirana mitsempha. Izi zimawonjezeranso mphamvu ya acetylcholine ndikuwonjezera mphamvu ya minofu. Pyridostigmine nthawi zina imaperekedwa pamodzi ndi amifampridine kuti ipangitse minofu kugwira ntchito bwino ndikuchiza zizindikiro za LEMS monga pakamwa pouma, maso ouma, kudzimbidwa, kusokonezeka kwa kugonana, komanso kuchepa kwa thukuta. Komabe, kugwiritsa ntchito pyridostigmine pa LEMS ndi ntchito yosakhala yachizolowezi, zomwe zikutanthauza kuti U.S. Food and Drug Administration (FDA) sinavomereze mankhwalawa pa vutoli.

Chithandizo chowongolera chitetezo chamthupi

• Mankhwala oletsa chitetezo chamthupi:Mankhwalawa amathandiza kuletsa kapena kuwongolera ntchito ya "chitetezo" cha chitetezo cha mthupi lanu. Dokotala wanu angakulembereni mankhwala awa ngati mankhwala ena sakuletsa zizindikiro zanu. Mwachitsanzo, mankhwala otchedwa prednisone angaperekedwe pamodzi ndi azathioprine kapena cyclosporine kapena ayi.
• Kusinthana kwa plasma: Mu njirayi, makina amalekanitsa gawo lamadzimadzi la magazi anu (plasma) ndi maselo a magazi. Ma antibodies anu ali mu plasma iyi. Pambuyo pa kupatukana, magazi amabwezeretsedwanso m'thupi lanu. Dokotala wanu angaganizire za chithandizo ichi ngati muli ndi zizindikiro zoopsa ndipo mukufuna chithandizo chachangu.
• Ma immunoglobulins: Kwa anthu ena, chithandizo cha immunoglobulins cha mlingo waukulu chingathandize kupewa matendawa kuti asakule kwambiri.

Kodi ma LEMS angapewedwe?

Chifukwa chenicheni cha LEMS sichinadziwikebe bwino, kotero sizikudziwika ngati pali njira yopewera matendawa.

Komabe, popeza kuti LEMS imatha kukula mwa anthu omwe ali ndi khansa ya m'mapapo, mutha kuthandiza kupewa khansa ya m'mapapo mwa:

• Musasute fodya. Ngati mumagwiritsa ntchito fodya, funsani dokotala wanu kuti akuthandizeni kusiya kusuta.
• Pewani utsi wa fodya womwe umagwiritsidwa ntchito ndi anthu ena.
• Yang'anani m'nyumba mwanu ngati muli ndi mpweya wa radon. Kukumana ndi mpweya wa radon kwa nthawi yayitali kungakulitse chiopsezo chanu cha khansa ya m'mapapo.

Mukhozanso kuchita zinthu zina kuti mukhale ndi moyo wathanzi:

• Idyani zipatso ndi ndiwo zamasamba zambiri. Tsatirani zakudya za ku Mediterranean kapena DASH.
• Khalani ndi kulemera koyenera.
• Chitani masewera olimbitsa thupi monga momwe dokotala wanu walangizira.
• Gonani mokwanira - nthawi zambiri maola asanu ndi awiri mpaka asanu ndi anayi.
• Chepetsani kupsinjika maganizo. Kupsinjika maganizo kungapangitse kuti zizindikirozo ziwonjezeke.
• Pewani kusamba m'madzi otentha. Kutentha kungapangitse kuti zizindikiro za LEMS ziwonjezeke kwambiri.
• Pitani kwa dokotala nthawi zonse, ndipo muwadziwitse nthawi yomweyo ngati thanzi lanu lasintha kapena ngati muyamba zizindikiro zatsopano.

Kodi ma LEMS angachiritsidwe kwathunthu?

Mwatsoka, palibe mankhwala a LEMS. Kuchiza khansa yomwe imayambitsa matenda a LEMS kwawonetsedwa kuti kumawonjezera zizindikiro za LEMS. Mankhwala oletsa chitetezo chamthupi angathandize kuwongolera kuukira kwa chitetezo chamthupi, komanso palinso mankhwala omwe angathandize kuchepetsa zizindikiro mwa kuwonjezera kuchuluka kwa acetylcholine m'mitsempha ndikuwonjezera mphamvu ya minofu.

Ngati muli ndi LEMS popanda khansa, mutha kuyembekezera kukhala ndi moyo wabwinobwino. Ngati muli ndi LEMS ndi khansa, zizindikiro zanu zimatha kusintha pakapita nthawi (ngakhale sizingasinthe kwathunthu) ngati khansayo yapezeka ndi kuthandizidwa msanga.

Moyo wanu umadalira kwambiri kuopsa kwa zizindikiro zanu.

Kodi ndiyenera kulankhula ndi dokotala wanga liti ngati ndili ndi `(LEMS)`?

Ngati thanzi lanu lasintha, kapena ngati zizindikiro zanu zikuipiraipira, funsani dokotala nthawi iliyonse . Musachite mantha, musachite mantha. Madokotala alipo kuti akuthandizeni.

Kodi kusiyana pakati pa `(LEMS)` ndi `(Myasthenia Gravis - MG)` ndi kotani?

Matenda a myasthenic a Lambert-Eaton (LEMS) ndi myasthenia gravis (MG) ali ndi zizindikiro zofanana. Zonsezi zimayambitsa kufooka kwa minofu ndi kutopa.

Komabe, myasthenia gravis (MG) nthawi zambiri imakhudza minofu yozungulira maso anu ndi minofu yomwe imagwiritsidwa ntchito pomeza. Pambuyo pake, imatha kukhudza minofu yonse, kuphatikizapo minofu yopuma ndi minofu ya miyendo.

Kawirikawiri LEMS imayamba ndi kufooka kwa ntchafu zanu zakumtunda, kenako imapita m'manja mwanu. LEMS ingayambitsenso kufooka kwa minofu ya maso anu, koma nthawi zambiri imakhala yofatsa kuposa myasthenia gravis (MG).

Mu matenda a myasthenia gravis (MG), kufooka kwakukulu kwa minofu yopumira (minofu yomwe imakuthandizani kupuma) kungakhale koopsa. Izi sizichitika kawirikawiri mu LEMS.

Anthu omwe ali ndi myasthenia gravis (MG) nthawi zambiri sasonyeza zizindikiro zambiri za kusagwira bwino ntchito kwa mitsempha ya autonomic (monga maso ouma, pakamwa pouma, kudzimbidwa, kuchepa kwa thukuta, kusagwira bwino ntchito pogonana). Mosiyana ndi zimenezi, anthu omwe ali ndi LEMS nthawi zambiri amakhala ndi madandaulo amenewa, ndipo zizindikiro zimenezi nthawi zina zimathandiza kusiyanitsa matenda awiriwa.

Pomaliza, zinthu zoti muzikumbukira (Uthenga Wopita Nawo Kunyumba)

Pofika pano mwina mukumvetsa kuti Lambert-Eaton Myasthenic Syndrome (LEMS) ndi matenda omwe amayamba chifukwa cha chitetezo chamthupi chomwe chimagwira ntchito mopitirira muyeso. Maselo anu amitsempha satumiza mauthenga okwanira ku minofu yanu kuti "achepetse." Zotsatira zake ndi kufooka kwa minofu ndi zizindikiro zina.

Kumbukirani, pafupifupi 60% ya anthu omwe ali ndi LEMS ali ndi khansa ya m'mapapo ya maselo ang'onoang'ono. Ngati muli ndi LEMS koma mulibe khansa, onetsetsani kuti inu ndi dokotala wanu mumayezetsa khansa miyezi ingapo iliyonse kwa zaka zosachepera ziwiri mutapezeka ndi khansa.

Pali mankhwala. Izi zikuphatikizapo kuchiza khansa ngati ilipo, komanso kumwa mankhwala omwe amathandiza kulumikizana kwa ulusi wa mitsempha ndi minofu. Kuwongolera kulumikizana kungathandize kulimbitsa mphamvu ya minofu ndi zizindikiro zina.

Ndikukhulupirira kuti izi zikuthandizani.Kumbukirani nthawi zonse, ngati muli ndi vuto lililonse la thanzi, ndi bwino kulankhula ndi dokotala wodziwa bwino za vutoli.

Matenda a myasthenic a Lambert -Eaton, LEMS, matenda a autoimmune, kufooka kwa minofu, khansa ya m'mapapo ya maselo ang'onoang'ono, kulumikizana kwa mitsempha, acetylcholine