Lambert-Eaton Myasthenic Syndrome

Zoo Siab

• Cov tsos mob ntawm Lambert-Eaton Myasthenic Syndrome zoo li cas?
• Dab tsi ua rau Lambert-Eaton Myasthenic Syndrome?
• Kev kuaj mob Lambert-Eaton Myasthenic Syndrome
• Kho Cov Lambert-Eaton Myasthenic Syndrome
• Dab Tsi Yog Qhov Long-Term Outlook?

Lambert-Eaton Myasthenic Syndrome yog dab tsi?

Lambert-Eaton myasthenic syndrome (LEMS) yog ib tus kab mob autoimmune uas tsis tshua muaj kev cuam tshuam koj lub peev xwm txav tau. Koj lub cev tiv thaiv cov leeg muaj zog ua rau cov leeg mob uas ua rau mus taug kev nyuaj thiab lwm yam teeb meem nqaij.

Tus kab mob no tsis tuaj yeem kho kom zoo, tab sis cov tsos mob tuaj yeem txo qis ib ntus yog tias koj siv zog koj tus kheej. Koj tuaj yeem tswj hwm tus mob nrog kev noj tshuaj.

Cov tsos mob ntawm Lambert-Eaton Myasthenic Syndrome zoo li cas?

Thawj qhov tsos mob ntawm LEMS yog txhais ceg tsis muaj zog thiab taug kev nyuaj. Thaum tus mob loj zuj zus, koj yuav tau muaj:

• tsis muaj zog ntawm cov leeg ntsej muag
• tsis kam cov leeg mob
• cem quav
• qhov ncauj qhuav
• impotence
• lub teeb meem zais zis

Kev tsis muaj zog ceg feem ntau txhim kho ib ntus thaum hais tawm. Raws li koj qoj ib ce, acetylcholine tsim tawm hauv ntau ntau los txhawm rau kom muaj zog los txhim kho rau lub sijhawm luv.

Muaj ntau yam teebmeem cuam tshuam nrog LEMS. Cov no suav nrog:

• teeb meem ua pa thiab nqos
• kis tau tus mob
• kev raug mob vim poob lossis teeb meem nrog kev ua haujlwm

Dab tsi ua rau Lambert-Eaton Myasthenic Syndrome?

Hauv kab mob autoimmune, koj lub cev tiv thaiv kab mob ua rau koj tus kheej lub cev tsis ncaj rau ib yam khoom txawv teb chaws. Koj lub cev tiv thaiv kab mob tsim cov tshuaj tiv thaiv uas tawm tsam koj lub cev.

Hauv LEMS, koj lub cev tawm tsam cov kev ua haujlwm kawg uas tswj cov nyiaj hauv acetylcholineyour lub cev tso tawm. Acetylcholine yog ib tug neurotransmitter uas ua rau leeg mob plab. Kev mob nqaij pob txha tso cai rau koj ua kev tawm dag zog xws li taug kev, co ntiv tes, thiab ua lub xub pwg nyom.

Tshwj xeeb, koj lub cev tawm tsam cov protein hu ua voltage gated calcium channel (VGCC). VGCC tsim nyog rau kev tso tawm ntawm acetylcholine. Koj tsis tsim cov tshuaj acetylcholine txaus thaum VGCC tawm tsam, yog li koj cov leeg ua haujlwm tsis tuaj yeem ua haujlwm.

Ntau yam mob LEMS cuam tshuam nrog mob ntsws. Cov kws tshawb nrhiav ntseeg tias cov qog nqaij hlav cancer tsim cov protein VGCC. Qhov no ua rau koj lub cev tiv thaiv kab mob ua rau cov tshuaj tiv thaiv tawm tsam VGCC. Cov tshuaj tiv thaiv no tom qab ntawd tua ob lub hlwb mob hlwb thiab cov leeg mob. Txhua tus neeg tuaj yeem tsim kho LEMS hauv lawv lub neej, tab sis kab mob ntsws yuav ua rau koj muaj feem mob tus mob. Yog tias muaj keeb kwm ntawm cov kab mob autoimmune hauv koj tsev neeg, koj yuav muaj kev pheej hmoo siab los tsim LEMS.

Kev kuaj mob Lambert-Eaton Myasthenic Syndrome

Txhawm rau txheeb xyuas LEMS, koj tus kws kho mob yuav coj cov keeb kwm ntxaws thiab kuaj lub cev. Koj tus kws kho mob yuav xyuas:

• tsis tshua mob reflexes
• cov leeg nqaij poob
• qaug zog lossis teeb meem txav uas tau zoo dua nrog kev ua si

Koj tus kws kho mob yuav xaj kuaj ntau yam kom paub tseeb tus mob. Kev kuaj ntshav yuav nrhiav cov tshuaj tiv thaiv tawm tsam VGCC (tshuaj tiv thaiv VGCC). Ib qho hluav taws xob electromyography (EMG) ntsuas koj cov leeg nqaij los ntawm qhov pom li cas lawv hnov ​​mob thaum hnov ​​mob. Ib rab koob me me tau ntxiv rau hauv cov leeg nqaij txuas thiab txuas nrog lub taub dej. Koj yuav raug hais kom ua tiav cov leeg ntawd, thiab lub cav kuaj nyeem yuav nyeem seb koj cov leeg teb tau zoo npaum li cas.

Lwm qhov tuaj kuaj tau yog cov ntsuas kev sib txuas ntawm cov leeg (NCV). Rau qhov kev ntsuas no, koj tus kws kho mob yuav tso cov electrodes rau ntawm koj cov tawv nqaij uas npog hauv cov leeg loj. Cov kab xev muab tawm hluav taws xob teeb liab uas ua rau cov leeg thiab cov leeg. Cov kev ua ub no uas tau los ntawm cov hlab ntsha tau sau tseg los ntawm lwm cov hluav taws xob thiab siv los nrhiav kom paub sai sai lub paj hlwb hnov ​​mob rau kev ua kom lub zog.

Kho Cov Lambert-Eaton Myasthenic Syndrome

Tus mob no yuav kho tsis tau. Koj yuav ua haujlwm nrog koj tus kws kho mob los tswj lwm yam mob, xws li mob ntsws.

Koj tus kws kho mob yuav qhia cov tshuaj txhaj rau immunoglobulin (IVIG) kev kho mob. Rau txoj kev kho no, koj tus kws kho mob yuav txhaj ib lub cev tsis muaj zog uas ua rau lub cev tiv thaiv tsis tau tus kabmob. Lwm txoj kev kho mob tuaj yeem yog plasmapheresis. Cov ntshav tau tawm ntawm lub cev, thiab lub ntshav ntshav cais tawm hauv lub cev. Cov tshuaj tiv thaiv ua haujlwm tawm, thiab cov ntshav ntshav rov qab rau lub cev.

Cov tshuaj uas ua haujlwm nrog koj cov leeg ua haujlwm qee zaum tuaj yeem txo cov tsos mob. Cov no suav nrog mestinon (pyridostigmine) thiab 3, 4 diaminopyridine (3, 4-DAP).

Cov tshuaj no nyuaj rau tau, thiab koj yuav tsum tham nrog koj tus kws kho mob kom paub cov ntaub ntawv ntxiv.

Dab Tsi Yog Qhov Long-Term Outlook?

Cov tsos mob tuaj yeem hloov kho los ntawm kev kho lwm yam mob hauv qab, ua kom lub cev tsis muaj zog, lossis tshem tawm cov tshuaj tiv thaiv ntawm cov ntshav. Tsis yog txhua tus teb tau zoo rau kev kho mob. Ua haujlwm nrog koj tus kws kho mob kom los nrog txoj kev npaj khomob tsim nyog.