Koj puas xav tias koj ob txhais ceg loog thiab? Qhov no yuav yog Lambert-Eaton Myasthenic Syndrome (LEMS)!

Puas muaj tej lub sij hawm koj xav tias koj ob txhais ceg tsis muaj zog, koj nyuaj siab sawv ntawm lub rooj zaum, koj nkees thaum koj nce ntaiv? Los yog koj puas xav tias nkees nrog cov tsos mob xws li qhov muag hnyav, qhov ncauj qhuav, thiab pom ob sab? Tej zaum cov no tsis yog tsuas yog nkees xwb. Hnub no peb yuav tham txog ib qho mob uas koj yuav tsis tau hnov ​​​​dua, tab sis nws tsim nyog paub txog, Lambert-Eaton Myasthenic Syndrome, lossis "(LEMS)" raws li peb hu nws luv.

Lambert-Eaton Myasthenic Syndrome (LEMS) yog dab tsi?

Hais yooj yim xwb, Lambert-Eaton Myasthenic Syndrome (LEMS) yog ib qho kab mob autoimmune. Tam sim no koj yuav xav paub tias kab mob autoimmune yog dab tsi . Peb lub cev muaj lub cev tiv thaiv kab mob. Qhov no yog qhov tiv thaiv peb los ntawm kev tawm tsam cov kab mob ua rau muaj kab mob los ntawm sab nraud. Nws zoo li cov tub rog hauv peb lub tebchaws. Tab sis qee zaum lub cev tiv thaiv kab mob no ua tsis tau zoo. Tom qab ntawd nws pib tawm tsam peb cov hlwb noj qab haus huv. Qhov ntawd yog qhov tshwm sim hauv LEMS.

Hauv qhov '(LEMS)' no, peb lub cev tiv thaiv kab mob tawm tsam qhov kev sib txuas ntawm peb cov hlab ntsha thiab cov leeg . Yog xav kom meej, qhov kev sib txuas no hu ua 'kev sib txuas ntawm cov hlab ntsha thiab cov leeg'. Thaum qhov kev sib txuas no puas lawm, kev xa cov lus los ntawm cov hlab ntsha mus rau cov leeg yuav raug cuam tshuam. Qhov no yog thaum cov leeg tsis muaj zog .

Qhov tsis muaj zog no feem ntau pib ntawm koj ob txhais ceg sab saud , tab sis nws tuaj yeem maj mam kis mus rau koj txhais tes, ua pa, nqos, thiab hais lus.

Leej twg yuav muaj feem ntau dua los tsim qhov '(LEMS)' no?

Muaj ob pawg tseem ceeb uas tsim ua `(LEMS)`.

1. Muaj feem cuam tshuam nrog mob qog noj ntshav: Kwv yees li 60% ntawm cov neeg uas muaj LEMS muaj qee hom mob qog noj ntshav. Hom mob qog noj ntshav feem ntau yog mob qog noj ntshav me me hauv lub ntsws . Qhov xav tsis thoob yog, qee zaum cov tsos mob ntawm LEMS tuaj yeem tshwm sim ntau lub hlis lossis txawm tias ntau xyoo ua ntej mob qog noj ntshav tshwm sim. Feem ntau ntawm cov neeg no muaj hnub nyoog tshaj 60 xyoo, yog txiv neej, thiab tau siv cov khoom haus luam yeeb.

2. Tsis muaj mob qog noj ntshav: Lwm 40% ntawm cov neeg uas muaj LEMS tsis tau kuaj pom tias muaj mob qog noj ntshav. Cov neeg no feem ntau raug kuaj pom thaum muaj hnub nyoog li ntawm 35 xyoo thiab dua thaum muaj hnub nyoog li ntawm 60 xyoo.

LEMS yog ib qho mob tsis tshua muaj ntawm cov menyuam yaus.

Puas yog tsuas yog mob qog nqaij hlav me me (LEMS) cuam tshuam nrog qhov no xwb?

Tsis yog. Mob qog nqaij hlav ntsws me me yog hom mob qog nqaij hlav uas feem ntau pom, tab sis lwm hom mob qog nqaij hlav kuj tuaj yeem cuam tshuam nrog LEMS. Piv txwv li mob qog nqaij hlav prostate, thymoma, thiab lymphoproliferative disorders. Vim tias cov tsos mob ntawm LEMS tuaj yeem tshwm sim ua ntej cov tsos mob ntawm mob qog nqaij hlav tshwm sim, nws yog ib qho tseem ceeb kom muaj kev kuaj mob kom raug los kuaj xyuas mob qog nqaij hlav yog tias koj tus kws kho mob kuaj pom tias koj muaj LEMS.

Puas yog LEMS tshwm sim los ntawm kev hloov pauv caj ces?

Cov kws tshawb fawb ntseeg tias cov kab mob autoimmune feem ntau yuav muaj qee qhov kev sib txuas ntawm caj ces. Vim tias LEMS kuj yog ib qho kab mob autoimmune, nws muaj peev xwm hais tias nws kuj muaj kev cuam tshuam ntawm caj ces, tab sis qhov no tseem tsis tau nkag siab tag nrho.

"(LEMS)" muaj pes tsawg hom?

LEMS yog ib yam kab mob tsis tshua muaj . Kwv yees li ntawm 2.8 lab tus tib neeg thoob ntiaj teb muaj tus kab mob no. Hauv Tebchaws Meskas, muaj li ntawm 400 tus neeg mob LEMS. Txawm hais tias tsis muaj cov ntaub ntawv tseeb txog pes tsawg tus neeg nyob hauv peb lub tebchaws, nws yog qhov tseeb tias qhov no yog ib yam kab mob tsis tshua muaj.

Cov tsos mob ntawm `(LEMS)` yog dab tsi?

Cia peb saib cov tsos mob ntawm '(LEMS)' ib qho zuj zus. Tsis yog txhua tus tsos mob no tshwm sim tib lub sijhawm rau txhua tus, qee cov tsos mob yuav tshwm sim ua ntej, qee qhov yuav tshwm sim tom qab.

• Cov leeg tsis muaj zog thiab qaug zog: tshwj xeeb tshaj yog nyob rau sab ceg sab saud.
• Kev nyuaj siab taug kev: Nyuaj siab nce ntaiv lossis sawv ntawm lub rooj zaum.
• Mob nqaij los yog mob nqaij tawv.
• Kev xav ntawm loog hauv ob txhais ceg.
• Qhov muag poob qis (ptosis): Qhov no zoo li tsaug zog.
• Pom ob yam tib lub sijhawm (diplopia).
• Qhov ncauj qhuav thiab qhov muag (xerostomia): txo qis kev qaub ncaug, txo qis kua muag hauv qhov muag.
• Cem quav.
• Txo kev tawm hws.
• Poob phaus.
• Nyuaj siab tso zis.
• Erectile dysfunction hauv cov txiv neej.
• Nyuaj hais lus (dysarthria) thiab nyuaj nqos (dysphagia): Cov no feem ntau yog cov tsos mob uas tshwm sim tom qab hauv tus kab mob.
• Ua pa nyuaj (ua tsis taus pa) thiab ua pa nyuaj: Qhov no kuj yog ib qho tsos mob tsis tshua muaj, hnyav uas tuaj yeem tshwm sim rau theem kawg ntawm tus kab mob.

Tseem Ceeb: Yog tias koj muaj ib lossis ntau yam ntawm cov tsos mob no, tsis txhob xav tias nws yog LEMS. Txawm li cas los xij, yog tias cov tsos mob no tseem muaj, nws yog qhov zoo uas yuav tsum nrhiav kev pab kho mob.

Feem ntau LEMS tshwm sim thiab nce qib li cas?

`(LEMS)` ‍ . , , , .

Thaum pib muaj tus kab mob, tej zaum koj yuav muaj teeb meem sawv ntawm lub rooj zaum, nce ntaiv lossis roob, lossis ua haujlwm hnyav uas yuav tsum tau siv koj ob txhais ceg.

Cov tsos mob feem ntau tshwm sim qeeb qeeb, dhau ob peb lub lis piam mus rau ntau lub hlis . Txawm li cas los xij, yog tias koj muaj LEMS nrog mob qog noj ntshav, cov tsos mob yuav tshwm sim sai dua.

Dab tsi ua rau LEMS?

Raws li peb tau tham ua ntej lawm, LEMS yog ib yam kab mob autoimmune . Qhov no txhais tau tias koj lub cev tiv thaiv (antibodies) tawm tsam koj cov nqaij noj qab haus huv. Hauv LEMS, cov tshuaj tiv thaiv no tawm tsam qhov chaw uas cov hlwb hlab ntsha thiab cov leeg nqaij sib ntsib, qhov chaw sib tshuam ntawm cov hlab ntsha thiab cov leeg. Tshwj xeeb, lawv tawm tsam cov calcium channels nyob ntawd. Cov calcium channels no yog qhov tseem ceeb rau cov lus ntawm cov hlab ntsha kom xa mus.

Xav txog nws zoo li ib kab ntawm cov dominoes. Qhov kev tawm tsam no ua rau muaj ntau yam xwm txheej:

1. Cov tshuaj tua kab mob khi rau cov calcium channel ntawm qhov kawg ntawm cov hlwb paj hlwb thiab thaiv lawv.

2. Thaum cov calcium channels raug txo qis, qhov ntau ntawm acetylcholine, ib qho neurotransmitter, uas tso tawm, yuav txo qis. Cov acetylcholine no yog ib qho tshuaj tseem ceeb rau kev ua haujlwm ntawm cov leeg nqaij.

3. Thaum tsis muaj acetylcholine txaus, cov "lus" rau cov leeg nqaij yuav tsis mus txog zoo. Ces koj cov leeg yuav ua haujlwm tsis tau zoo.

4. Yog li ntawd, cov leeg nqaij tsis ua haujlwm li qub, ua rau qaug zog.

Nov yog li cas cov neeg uas muaj mob qog nqaij hlav ntsws me me tsim LEMS: Thaum koj muaj mob qog nqaij hlav hauv koj lub cev, cov tshuaj tiv thaiv kab mob tau txais lub cim qhia kom "mus ua tsov rog." Tab sis es tsis txhob tawm tsam cov hlwb qog nqaij hlav (tshwj xeeb, cov calcium channels ntawm qhov chaw ntawm cov hlwb qog nqaij hlav), cov tshuaj tiv thaiv kab mob no tawm tsam cov calcium channels ntawm qhov kawg ntawm cov hlwb hlab ntsha. Tom qab ntawd tib txoj saw ntawm cov xwm txheej tshwm sim.

Cov kws tshawb fawb ntseeg tias qhov ua rau muaj LEMS rau cov neeg uas tsis muaj mob qog noj ntshav hauv lub ntsws tsuas yog qhov ua tsis tau zoo ntawm lub cev tiv thaiv kab mob. Tab sis tseem tsis tau meej tias vim li cas qhov no thiaj tshwm sim.

Yuav ua li cas txheeb xyuas `(LEMS)`?

Kauj ruam thawj zaug hauv kev kuaj mob LEMS yog kom koj tus kws kho mob kuaj koj kom zoo. Qhov no feem ntau yuav muaj tus kws kho mob hlwb, lossis tus kws kho mob uas tshwj xeeb hauv cov kab mob ntawm cov hlab ntsha thiab cov leeg. Ib feem ntawm cov txheej txheem no suav nrog kev nug txog koj cov tsos mob, koj keeb kwm kev kho mob, thiab txhua yam tshuaj uas koj noj. Kev kuaj mob hlwb kom ntxaws yog ua los nrhiav cov qauv ntawm qhov tsis muaj zog thiab cov cim qhia sib xws nrog LEMS.

Kauj ruam tom ntej yog kev sim. Kev sim rau `(LEMS)` suav nrog:

• Kev kuaj ntshav: Qhov no tuaj yeem kuaj xyuas seb koj puas muaj cov tshuaj tiv thaiv kab mob calcium hauv koj cov ntshav. Kwv yees li 85% ntawm cov neeg uas muaj LEMS muaj cov tshuaj tiv thaiv no hauv lawv cov ntshav.
• Kev kuaj mob hluav taws xob (EMG): Qhov kev kuaj no saib seb koj cov leeg thiab cov hlab ntsha ua haujlwm ua ke zoo npaum li cas. Hauv LEMS, qhov kev kuaj EMG muaj cov ntaub ntawv tshwj xeeb heev, yog li nws tuaj yeem pab lees paub qhov kev kuaj mob.
• Duab X-ray ntawm lub ntsws (Lung X-ray) los yog CT scan (CT scan) los yog MRI scan (MRI) (ntawm lub hauv siab): Vim tias muaj ib yam mob hu ua (LEMS) uas tuaj yeem tshwm sim rau cov neeg uas muaj mob qog nqaij hlav me me hauv lub ntsws, cov kev kuaj no nrhiav cov cim qhia tias muaj mob qog nqaij hlav hauv lub ntsws.

Cov tsos mob ntawm LEMS tuaj yeem pib ntau lub hlis txog rau xyoo ua ntej mob qog noj ntshav tshwm sim. Yog li ntawd, tom qab kuaj pom tias muaj LEMS, koj yuav tsum tau kuaj mob qog noj ntshav txhua peb txog rau lub hlis rau tsawg kawg yog ob xyoos. Koj tus kws kho mob yuav pom zoo rau lub sijhawm kuaj mob uas haum rau koj.

Yuav kho LEMS li cas?

Muaj ntau txoj kev kho mob rau LEMS.

• Kev Kho Mob Qog Ntsws: Yog tias LEMS muaj feem cuam tshuam nrog qog nqaij hlav, xws li qog nqaij hlav ntsws me me, kev kho mob qog nqaij hlav ntawd kuj tseem yuav txo cov tsos mob ntawm LEMS. Kev kho mob qog nqaij hlav yuav suav nrog kev siv tshuaj kho mob qog nqaij hlav, kev kho mob hluav taws xob, thiab/lossis phais. Qhov no nyob ntawm hom qog nqaij hlav thiab koj tus kws kho mob cov lus pom zoo.
• Cov kev kho mob tshwj xeeb rau (LEMS): Cov no suav nrog cov tshuaj uas pab txo cov tsos mob thiab cov tshuaj uas tswj lub cev tiv thaiv kab mob.

Kev kho mob rau cov tsos mob

• Amifampridine (Firdapse®), Guanidine: Cov tshuaj no ua rau cov leeg muaj zog. Lawv ua rau cov neurotransmitter acetylcholine tso tawm ntau ntxiv ntawm qhov chaw sib txuas ntawm cov leeg, ua kom cov cim qhia rau cov leeg zoo dua.
• Pyridostigmine (Pyridostigmine - Mestinon®): Pyridostigmine ua haujlwm los ntawm kev tiv thaiv kev tawg ntawm acetylcholine ntawm qhov chaw sib txuas ntawm cov leeg nqaij. Qhov no kuj ua rau acetylcholine signaling nce ntxiv thiab txhim kho cov leeg nqaij lub zog. Pyridostigmine qee zaum muab ua ke nrog amifampridine los txhim kho cov leeg nqaij ua haujlwm thiab kho cov tsos mob ntawm LEMS xws li qhov ncauj qhuav, qhov muag qhuav, cem quav, kev ua haujlwm tsis zoo ntawm kev sib deev, thiab txo qis kev hws. Txawm li cas los xij, kev siv pyridostigmine rau LEMS yog kev siv tsis raws li daim ntawv lo, txhais tau tias Tebchaws Meskas Lub Chaw Haujlwm Saib Xyuas Khoom Noj thiab Tshuaj (FDA) tsis tau pom zoo cov tshuaj rau qhov mob no.

Kev kho mob uas ua rau lub cev tiv thaiv kab mob zoo dua

• Cov tshuaj tiv thaiv kab mob:Cov tshuaj no pab nres lossis tswj lub luag haujlwm "kev tiv thaiv" ntawm koj lub cev tiv thaiv kab mob. Koj tus kws kho mob yuav sau ntawv kom muab cov tshuaj no yog tias lwm cov tshuaj tsis tswj koj cov tsos mob. Piv txwv li, cov tshuaj hu ua prednisone yuav muab nrog lossis tsis muaj azathioprine lossis cyclosporine.
• Kev pauv ntshav hauv cov ntshav: Hauv cov txheej txheem no, lub tshuab cais cov kua ntawm koj cov ntshav (plasma) ntawm cov qe ntshav. Koj cov tshuaj tua kab mob nyob hauv cov ntshav no. Tom qab kev sib cais, cov ntshav raug muab rov qab rau hauv koj lub cev. Koj tus kws kho mob yuav xav txog kev kho mob no yog tias koj muaj cov tsos mob hnyav thiab xav tau kev kho mob sai.
• Immunoglobulins: Rau qee tus neeg, kev kho mob immunoglobulin ntau dhau yuav pab tiv thaiv kom tus kab mob tsis txhob hnyav zuj zus.

Puas tuaj yeem tiv thaiv LEMS tau?

Qhov ua rau muaj LEMS tseem tsis tau nkag siab tag nrho, yog li ntawd tsis paub tias puas muaj txoj hauv kev los tiv thaiv tus kab mob no.

Txawm li cas los xij, vim tias LEMS tuaj yeem tshwm sim rau cov neeg uas muaj mob qog nqaij hlav ntsws, koj tuaj yeem pab tiv thaiv mob qog nqaij hlav ntsws los ntawm:

• Tsis txhob haus luam yeeb. Yog tias koj siv cov khoom luam yeeb, nug koj tus kws kho mob kom pab koj tso tseg.
• Tsis txhob haus luam yeeb.
• Tshawb xyuas koj lub tsev seb puas muaj pa roj radon. Kev raug pa roj radon mus ntev yuav ua rau koj muaj feem mob qog noj ntshav hauv lub ntsws.

Koj tuaj yeem ua cov kauj ruam los tswj kev noj qab haus huv:

• Noj txiv hmab txiv ntoo thiab zaub ntau ntxiv. Ua raws li Mediterranean lossis DASH diet.
• Tswj koj qhov hnyav kom noj qab nyob zoo.
• Ua si nawv raws li koj tus kws kho mob qhia.
• Pw tsaug zog txaus - feem ntau yog xya txog cuaj teev.
• Txo koj txoj kev ntxhov siab. Kev ntxhov siab tuaj yeem ua rau cov tsos mob hnyav zuj zus.
• Tsis txhob da dej hauv dej kub. Kub tuaj yeem ua rau cov tsos mob ntawm LEMS hnyav dua.
• Mus ntsib koj tus kws kho mob tsis tu ncua, thiab qhia rau lawv paub tam sim ntawd yog tias koj txoj kev noj qab haus huv hloov pauv lossis yog tias koj muaj cov tsos mob tshiab.

Puas kho tau LEMS kom zoo tag nrho?

Tu siab kawg li, tsis muaj kev kho rau LEMS. Kev kho mob qog nqaij hlav uas cuam tshuam nrog LEMS tau pom tias txhim kho cov tsos mob ntawm LEMS. Cov tshuaj tiv thaiv kab mob tuaj yeem pab tswj kev tawm tsam autoimmune, thiab kuj tseem muaj kev kho mob uas tuaj yeem pab txo cov tsos mob los ntawm kev nce cov acetylcholine hauv cov hlab ntsha thiab txhim kho cov leeg nqaij lub zog.

Yog tias koj muaj LEMS yam tsis muaj mob qog noj ntshav, koj tuaj yeem cia siab tias yuav muaj lub neej ntev li qub. Yog tias koj muaj LEMS thiab mob qog noj ntshav, koj cov tsos mob yuav zoo dua qub (txawm tias tsis yog tag nrho) yog tias kuaj pom thiab tswj tau mob qog noj ntshav thaum ntxov.

Koj lub neej zoo feem ntau nyob ntawm qhov hnyav ntawm koj cov tsos mob.

Thaum twg kuv yuav tsum hu rau kuv tus kws kho mob yog tias kuv muaj '(LEMS)'?

Yog tias muaj kev hloov pauv hauv koj txoj kev noj qab haus huv, lossis yog tias koj cov tsos mob hnyav zuj zus, hu rau koj tus kws kho mob txhua lub sijhawm . Tsis txhob ntshai, tsis txhob ntshai. Cov kws kho mob nyob ntawd los pab koj.

Qhov txawv ntawm `(LEMS)` thiab `(Myasthenia Gravis - MG)` yog dab tsi?

Kab mob Lambert-Eaton myasthenic syndrome (LEMS) thiab myasthenia gravis (MG) muaj cov tsos mob zoo sib xws. Ob qho tib si ua rau cov leeg tsis muaj zog thiab qaug zog.

Txawm li cas los xij, myasthenia gravis (MG) feem ntau ua rau cov leeg nqaij nyob ib puag ncig koj lub qhov muag thiab cov leeg nqaij siv rau kev nqos. Tom qab ntawd, nws tuaj yeem cuam tshuam rau cov leeg nqaij dav dav, suav nrog cov leeg nqaij ua pa thiab cov leeg nqaij ntawm ob txhais ceg.

Feem ntau LEMS pib nrog qhov tsis muaj zog hauv koj ob txhais ceg sab saud, tom qab ntawd txav mus rau koj txhais tes. LEMS kuj tseem tuaj yeem ua rau koj cov leeg nqaij qhov muag tsis muaj zog, tab sis feem ntau nws tsis hnyav dua li myasthenia gravis (MG).

Hauv cov neeg mob myasthenia gravis (MG), cov leeg nqaij ua pa tsis muaj zog heev (cov leeg uas pab koj ua pa) tuaj yeem ua rau tuag taus. Qhov no tsis tshua muaj rau cov neeg mob LEMS.

Cov neeg uas muaj tus kab mob myasthenia gravis (MG) feem ntau tsis qhia ntau yam tsos mob ntawm kev ua haujlwm tsis zoo ntawm lub paj hlwb (piv txwv li, qhov muag qhuav, qhov ncauj qhuav, cem quav, tawm hws tsawg, kev ua haujlwm tsis zoo ntawm kev sib deev). Qhov sib txawv, cov neeg uas muaj LEMS feem ntau muaj cov teeb meem no, thiab cov tsos mob no qee zaum tuaj yeem pab cais ob yam kab mob.

Thaum kawg, tej yam yuav tsum nco ntsoov (Cov Lus Coj Mus Tsev)

Txog rau tam sim no tej zaum koj yuav nkag siab lawm tias Lambert-Eaton Myasthenic Syndrome (LEMS) yog ib yam kab mob uas tshwm sim los ntawm lub cev tiv thaiv kab mob ua haujlwm ntau dhau. Koj cov hlab ntsha tsis xa cov lus txaus rau koj cov leeg nqaij kom "qaug zog." Qhov tshwm sim yog cov leeg nqaij tsis muaj zog thiab lwm yam tsos mob.

Nco ntsoov, kwv yees li 60% ntawm cov neeg uas muaj LEMS muaj mob qog nqaij hlav ntsws me me. Yog tias koj muaj LEMS tab sis tsis muaj mob qog nqaij hlav, xyuas kom koj thiab koj tus kws kho mob kuaj mob qog nqaij hlav txhua ob peb lub hlis rau tsawg kawg yog ob xyoos tom qab kuaj pom.

Muaj kev kho mob. Cov no suav nrog kev kho mob qog noj ntshav yog tias nws muaj, nrog rau kev noj tshuaj uas txhim kho kev sib txuas lus ntawm cov hlwb thiab cov leeg nqaij. Kev txhim kho kev sib txuas lus tuaj yeem txhim kho lub zog ntawm cov leeg nqaij thiab lwm yam tsos mob.

Vam tias cov ntaub ntawv no yuav pab tau koj.Nco ntsoov tias yog koj muaj teeb meem kev noj qab haus huv, qhov zoo tshaj plaws yog nrog kws kho mob paub txog qhov ntawd tham.

Lambert -Eaton myasthenic syndrome, LEMS, cov kab mob autoimmune, cov leeg tsis muaj zog, mob qog nqaij hlav me me hauv lub ntsws, kev sib txuas ntawm cov leeg ntawm lub paj hlwb, acetylcholine