Ingabe nawe uzizwa sengathi imilenze yakho iyaphela amandla? Lokhu kungaba yiLambert-Eaton Myasthenic Syndrome (LEMS)!

Ingabe ngezinye izikhathi uzizwa sengathi imilenze yakho ibuthakathaka, unenkinga yokuvuka esihlalweni, uzizwa ukhathele lapho ukhuphuka izitebhisi? Noma uzizwa ukhathele ngezimpawu ezifana namehlo aqinile, umlomo owomile, kanye nokubona okubili? Lokhu kungase kungabi ukukhathala nje kuphela. Namuhla sizokhuluma ngesimo okungenzeka ukuthi awukaze uzwe ngaso, kodwa okufanele ukwazi ngaso, i-Lambert-Eaton Myasthenic Syndrome, noma ``(LEMS)`` njengoba sikubiza kanjalo ngamafuphi.

Iyini i-Lambert-Eaton Myasthenic Syndrome (LEMS)?

Kalula nje, i-Lambert-Eaton Myasthenic Syndrome (LEMS) iyisifo sokuzivikela komzimba. Manje ungase uzibuze ukuthi siyini isifo sokuzivikela komzimba . Imizimba yethu inesimiso sokuzivikela komzimba. Yilokhu okusivikela ngokulwa namagciwane abangela izifo avela ngaphandle. Kufana nebutho ezweni lakithi. Kodwa ngezinye izikhathi lesi simiso sokuzivikela komzimba siyaphambuka. Bese siqala ukuhlasela amangqamuzana ethu aphilile. Yilokho okwenzekayo ku-LEMS.

Kule `(LEMS)`, amasosha omzimba wethu ahlasela ukuxhumana phakathi kwezinzwa zethu zomzimba nemisipha . Ngokuqondile, lokhu kuxhumana kubizwa ngokuthi `i-neuromuscular junction`. Uma lokhu kuhlangana konakele, ukudluliselwa kwemiyalezo kusuka ezinzwa kuya emisipheni kuyaphazamiseka. Yilapho kwenzeka khona ubuthakathaka bemisipha.

Lobu buthakathaka buvame ukuqala emathangeni akho angaphezulu , kodwa kancane kancane bungasakazekela ezingalweni zakho, ekuphefumuleni, ekugwinyeni, nasemisipheni yokukhuluma.

Ubani onamathuba amaningi okuthuthukisa lokhu `(i-LEMS)`?

Kunamaqembu amabili amakhulu akha i-`(LEMS)`.

1. Okuhlobene nomdlavuza: Cishe abantu abangu-60% abane-LEMS banohlobo oluthile lomdlavuza. Uhlobo oluvame kakhulu umdlavuza wamaphaphu wamangqamuzana amancane . Ngokumangalisayo, ngezinye izikhathi izimpawu ze-LEMS zingavela ezinyangeni noma ngisho neminyaka ngaphambi kokuba umdlavuza uqale. Iningi lalaba bantu lingaphezu kweminyaka engu-60, ngamadoda, futhi basebenzise imikhiqizo kagwayi.

2. Akukho mdlavuza: Abanye abantu abangu-40% abane-LEMS abakatholakali umdlavuza. Laba bantu bavame ukutholakala beneminyaka engaba ngu-35 ubudala futhi batholakale beneminyaka engaba ngu-60 ubudala.

I-LEMS iyisimo esingavamile kakhulu ezinganeni ezincane.

Ingabe umdlavuza wamaphaphu wamaseli amancane kuphela (i-LEMS) uhlotshaniswa nalokhu?

Cha. Umdlavuza wamaphaphu wamangqamuzana amancane uhlobo lomdlavuza oluvame kakhulu, kodwa ezinye izinhlobo zomdlavuza nazo zingahlotshaniswa ne-LEMS. Izibonelo zifaka phakathi umdlavuza we-prostate, i-thymoma, kanye nezinkinga ze-lymphoproliferative. Ngenxa yokuthi izimpawu ze-LEMS zingavela ngaphambi kokuba kuvele izimpawu zomdlavuza, kubalulekile ukuba nezivivinyo ezifanele zokuhlola umdlavuza uma udokotela wakho ekuxilonga ukuthi une-LEMS.

Ingabe i-LEMS ibangelwa izinguquko zofuzo?

Ososayensi bakholelwa ukuthi izifo zokuzivikela komzimba ngokuvamile zingase zibe nokuxhumana okuthile kwezakhi zofuzo. Njengoba i-LEMS nayo iyisifo sokuzivikela komzimba, kungenzeka ukuthi nayo inethonya lezakhi zofuzo, kodwa lokhu akukaqondakali ngokugcwele.

Kuvame kangakanani `(LEMS)`?

I-LEMS yisifo esingavamile kakhulu . Kulinganiselwa ukuthi lesi simo sithinta abantu ababalelwa ezigidini ezingu-2.8 emhlabeni jikelele. E-United States, kunezimo ezingaba ngu-400 ezibikiwe ze-LEMS. Nakuba kungekho zibalo eziqondile zokuthi bangaki abantu ezweni lethu, kusobala ukuthi lesi yisimo esingavamile.

Ziyini izimpawu ze-`(LEMS)`?

Ake sibheke izimpawu ze-`(LEMS)` ngayinye ngayinye. Akuzona zonke lezi zimpawu ezenzeka ngesikhathi esifanayo kuwo wonke umuntu, ezinye izimpawu zingase zivele kusenesikhathi, ezinye zingase zivele kamuva.

• Ubuthakathaka bemisipha kanye nokukhathala: ikakhulukazi emilenzeni engenhla.
• Ubunzima bokuhamba: Ubunzima bokukhuphuka izitebhisi noma ukuvuka esihlalweni.
• Ubuhlungu noma ukuqina kwemisipha.
• Ukuzwa sengathi umzimba uphelelwe amandla.
• Amajwabu amehlo alengayo (i-ptosis): Lokhu kungabonakala njengokulala.
• Ukubona izinto ezimbili ngesikhathi esisodwa (i-diplopia).
• Umlomo namehlo omile (xerostomia): ukwehla kwamathe, ukwehla kokukhiqizwa kwezinyembezi emehlweni.
• Ukuqunjelwa.
• Ukujuluka okunciphile.
• Ukwehlisa isisindo.
• Ubunzima bokuchama.
• Ukungasebenzi kahle kwe-Erectile kwabesilisa.
• Ubunzima bokukhuluma (i-dysarthria) kanye nobunzima bokugwinya (i-dysphagia): Lezi ngokuvamile izimpawu ezikhula kamuva kulesi sifo.
• Ubunzima bokuphefumula (ukuphelelwa umoya) kanye nokucindezeleka kokuphefumula: Lokhu futhi kuyisibonakaliso esingavamile nesinzima esingase sivele ezigabeni zokugcina zesifo.

Okubalulekile: Uma unesinye noma ngaphezulu salezi zimpawu, ungacabangi ukuthi yi-LEMS. Kodwa-ke, uma lezi zimpawu ziqhubeka, kuwukuhlakanipha ukufuna iseluleko sezokwelapha.

I-LEMS ivame ukubonakala kanjani futhi ithuthuka kanjani?

I-LEMS ivame ukuthinta imisipha yamathanga kuqala. Ingathinta imisipha yamahlombe, imisipha yezingalo nemilenze, imisipha esiza ekukhulumeni nasekugwinyeni, kanye nemisipha yamehlo.

Ekuqaleni kwalesi sifo, ungase ube nobunzima bokuvuka esihlalweni, ukukhuphuka izitebhisi noma amagquma, noma ukwenza umsebenzi onzima odinga ukusebenzisa imilenze yakho.

Izimpawu zivame ukukhula kancane, phakathi kwamasonto ambalwa kuya ezinyangeni . Kodwa-ke, uma une-LEMS enomdlavuza, izimpawu zingakhula ngokushesha.

Yini ebangela i-LEMS?

Njengoba sesixoxile ngaphambili, i-LEMS iyisifo sokuzivikela komzimba . Lokhu kusho ukuthi izivikelo zomzimba wakho (ama-antibodies) zihlasela izicubu zakho ezinempilo. Ku-LEMS, la ma-antibodies ahlasela indawo lapho kuhlangana khona amaseli ezinzwa kanye nemicu yemisipha, indawo yokuhlangana kwemisipha. Ngokukhethekile, ahlasela iziteshi ze-calcium lapho. Lezi ziteshi ze-calcium zibalulekile ukuze imiyalezo yezinzwa idluliselwe.

Cabanga ngakho njengohlu lwama-domino. Lokhu kuhlasela kuqala uchungechunge lwezehlakalo:

1. Ama-antibodies abopha iziteshi ze-calcium emaphethelweni amaseli ezinzwa bese ewavimba.

2. Uma iziteshi ze-calcium zincishisiwe, inani le- acetylcholine, i-neurotransmitter, elikhishwayo liyancipha. Le acetylcholine iyikhemikhali ebalulekile ekusebenzeni kwemisipha.

3. Uma i-acetylcholine ingakhishwa ngokwanele, "imiyalezo" eya emicu yemisipha ayifiki kahle. Khona-ke imisipha yakho ayikwazi ukusebenza ngokugcwele.

4. Ngenxa yalokho, imisipha ayisebenzi kahle, okubangela ubuthakathaka.

Nansi indlela abantu abanomdlavuza wamaphaphu wamangqamuzana amancane aba ngayo ama-LEMS: Uma unomdlavuza emzimbeni wakho, ama-antibodies athola isignali yokuthi "uye empini." Kodwa esikhundleni sokuhlasela amaseli omdlavuza (ikakhulukazi, iziteshi ze-calcium ebusweni bamaseli omdlavuza), la ma-antibodies ahlasela ngephutha iziteshi ze-calcium emaphethelweni amaseli ezinzwa. Bese kwenzeka uchungechunge olufanayo lwezenzakalo.

Ososayensi bakholelwa ukuthi imbangela ye-LEMS kubantu abangenawo umdlavuza wamaphaphu iwukungasebenzi kahle kwesimiso somzimba sokuzivikela. Kodwa akukacaci ukuthi kungani lokhu kwenzeka.

Ungayibona kanjani i-`(LEMS)`?

Isinyathelo sokuqala ekuxilongeni i-LEMS ukuthi udokotela wakho akuhlole ngokucophelela. Lokhu kuvame ukuhilela udokotela wezinzwa, noma udokotela ochwepheshe ezifweni zezinzwa nemisipha. Ingxenye yale nqubo ihilela ukubuza ngezimpawu zakho, umlando wakho wezokwelapha, kanye nanoma yimiphi imithi oyithathayo. Ukuhlolwa okuningiliziwe kwezinzwa kwenziwa ukuze kubhekwe amaphethini obuthakathaka nezimpawu ezihambisana ne-LEMS.

Isinyathelo esilandelayo ukuhlola. Ukuhlolwa kwe-`(LEMS)` kufaka phakathi:

• Ukuhlolwa kwegazi: Lokhu kungahlola ukuthi une-anti-calcium channel antibodies egazini lakho. Cishe abantu abangu-85% abane-LEMS banala ma-antibodies egazini labo.
• I-Electromyography (EMG): Lokhu kuhlolwa kubheka ukuthi imisipha yakho nezinzwa zakho zisebenza kahle kangakanani ndawonye. Ku-LEMS, ukuhlolwa kwe-EMG kunemiphumela eqondile kakhulu, ngakho-ke kungasiza ekuqinisekiseni ukuxilongwa.
• I-X-ray yamaphaphu `(X-ray)` noma i-CT scan `(CT scan)` noma i-MRI scan `(MRI)` (yesifuba): Ngenxa yokuthi isimo esibizwa ngokuthi `(LEMS)` singavela kubantu abanomdlavuza wamaphaphu wamangqamuzana amancane, lezi zivivinyo zifuna izimpawu zomdlavuza wamaphaphu.

Izimpawu ze-LEMS zingaqala ezinyangeni kuya eminyakeni eyisithupha ngaphambi kokuba umdlavuza uvele. Ngakho-ke, ngemva kokuba i-LEMS isitholakale, kufanele uhlolwe umdlavuza njalo ezinyangeni ezintathu kuya kweziyisithupha okungenani iminyaka emibili. Udokotela wakho uzoncoma isimiso sokuhlolwa esifanele wena.

Iphathwa kanjani i-LEMS?

Kunezindlela eziningana zokwelapha i-LEMS.

• Ukwelapha umdlavuza oyisisekelo: Uma i-LEMS ihlobene nomdlavuza, njengomdlavuza wamaphaphu wamangqamuzana amancane, ukwelapha lowo mdlavuza kunganciphisa nezimpawu ze-LEMS. Ukwelashwa komdlavuza kungafaka phakathi i-chemotherapy, ukwelashwa ngemisebe, kanye/noma ukuhlinzwa. Lokhu kuncike ohlotsheni lomdlavuza kanye nezincomo zikadokotela wakho.
• Ukwelashwa okuqondile kwe-(LEMS): Lokhu kufaka phakathi imithi eqeda izimpawu kanye nemithi elawula amasosha omzimba.

Ukwelashwa kwezimpawu

• I-Amifampridine (i-Firdapse®), i-Guanidine: Le mithi yandisa amandla emisipha. Yandisa ukukhululwa kwe-neurotransmitter acetylcholine endaweni yokuhlangana kwemisipha, ithuthukisa izimpawu emisipheni.
• I-Pyridostigmine (i-Pyridostigmine - i-Mestinon®): I-Pyridostigmine isebenza ngokuvimbela ukuwohloka kwe-acetylcholine endaweni yokuhlangana kwemisipha. Lokhu kwandisa nokubonakaliswa kwe-acetylcholine futhi kuthuthukisa amandla emisipha. I-Pyridostigmine ngezinye izikhathi inikezwa kanye ne-amifampridine ukuthuthukisa ukusebenza kwemisipha nokwelapha izimpawu ze-LEMS ezifana nomlomo owomile, amehlo omile, ukuqunjelwa, ukungasebenzi kahle kocansi, kanye nokujuluka okunciphile. Kodwa-ke, ukusetshenziswa kwe-pyridostigmine kwe-LEMS kuwukusetshenziswa okungekho emthethweni, okusho ukuthi i-U.S. Food and Drug Administration (FDA) ayizange ivume umuthi walesi simo.

Ukwelashwa okuguqula amasosha omzimba

• Imithi yokucindezela amasosha omzimba:Le mithi isiza ekumiseni noma ekulawuleni umsebenzi "wokuzivikela" wesimiso sakho somzimba sokuzivikela. Udokotela wakho angase akunike le mithi uma eminye imithi ingazilawuli izimpawu zakho. Isibonelo, umuthi obizwa ngokuthi i-prednisone unganikezwa kanye noma ngaphandle kwe-azathioprine noma i-cyclosporine.
• Ukushintshaniswa kwe-plasma: Kule nqubo, umshini uhlukanisa ingxenye ewuketshezi yegazi lakho (i-plasma) kumaseli egazi. Ama-antibodies akho akule plasma. Ngemva kokuhlukaniswa, igazi libuyiselwa emzimbeni wakho. Udokotela wakho angase acabangele lokhu kwelashwa uma unezimpawu ezinzima futhi udinga ukwelashwa okuphuthumayo.
• Ama-immunoglobulins: Kwabanye abantu, ukwelashwa nge-immunoglobulin eningi kungasiza ekuvimbeleni lesi sifo ukuthi singabi sibi kakhulu.

Ingabe ama-LEMS angavinjelwa?

Imbangela eqondile ye-LEMS ayikaqondakali ngokugcwele, ngakho-ke akwaziwa ukuthi ikhona yini indlela yokuvimbela lesi sifo.

Kodwa-ke, njengoba i-LEMS ingakhula kubantu abanomdlavuza wamaphaphu, ungasiza ekuvimbeleni umdlavuza wamaphaphu ngokuthi:

• Ungabhemi. Uma usebenzisa imikhiqizo kagwayi, cela usizo kudokotela wakho lokuyeka ukubhema.
• Gwema intuthu ebhemayo.
• Hlola ikhaya lakho ukuthi alinawo yini ugesi we-radon. Ukuchayeka isikhathi eside kugesi we-radon kungandisa ingozi yakho yomdlavuza wamaphaphu.

Ungathatha nezinyathelo zokugcina indlela yokuphila enempilo:

• Yidla izithelo nemifino eminingi. Landela ukudla kwaseMedithera noma kwe-DASH.
• Gcina isisindo esinempilo.
• Vivinya umzimba njengoba utshelwe udokotela wakho.
• Lala ngokwanele - ngokuvamile amahora ayisikhombisa kuya kwayisishiyagalolunye.
• Nciphisa ukucindezeleka kwakho. Ukucindezeleka kungenza izimpawu zibe zimbi kakhulu.
• Gwema ukugeza ngamanzi ashisayo. Ukushisa kungenza izimpawu ze-LEMS zibe zimbi kakhulu.
• Bona udokotela wakho njalo, futhi ubazise ngokushesha uma impilo yakho ishintsha noma uma uba nezimpawu ezintsha.

Ingabe ama-LEMS angelapheka ngokuphelele?

Ngeshwa, ayikho ikhambi le-LEMS. Ukwelapha umdlavuza oyisisekelo ohlotshaniswa ne-LEMS kuye kwabonakala kuthuthukisa izimpawu ze-LEMS. Imithi yokucindezela amasosha omzimba ingasiza ekulawuleni ukuhlasela kwe-autoimmune, futhi kukhona nokwelashwa okungasiza ekunciphiseni izimpawu ngokwandisa inani le-acetylcholine eziphethweni zemizwa nokuthuthukisa amandla emisipha.

Uma une-LEMS engenawo umdlavuza, ungalindela isikhathi sokuphila esijwayelekile. Uma une-LEMS nomdlavuza, izimpawu zakho zingase zithuthuke ngokuhamba kwesikhathi (nakuba kungenjalo ngokuphelele) uma umdlavuza utholakala futhi uphathwa kusenesikhathi.

Izinga lempilo yakho lincike kakhulu ebunzimeni bezimpawu zakho.

Kufanele ngixhumane nini nodokotela wami uma ngine-`(LEMS)`?

Uma kukhona ushintsho empilweni yakho, noma uma izimpawu zakho ziba zimbi kakhulu, xhumana nodokotela wakho nganoma yisiphi isikhathi . Ungesabi, ungesabi. Odokotela bakhona ukukusiza.

Uyini umehluko phakathi kwe-`(LEMS)` kanye ne-`(Myasthenia Gravis - MG)`?

I-Lambert-Eaton myasthenic syndrome (LEMS) kanye ne-myasthenia gravis (MG) zinezimpawu ezifanayo. Zombili zibangela ubuthakathaka bemisipha kanye nokukhathala.

Kodwa-ke, i-myasthenia gravis (MG) ivame ukuthinta imisipha ezungeze amehlo akho kanye nemisipha esetshenziselwa ukugwinya. Kamuva, ingathinta imisipha evamile, okuhlanganisa nemisipha yokuphefumula kanye nemisipha yamalungu.

I-LEMS ivame ukuqala ngobuthakathaka emathangeni akho angaphezulu, bese iya ezingalweni zakho. I-LEMS ingabangela nobuthakathaka emisipheni yamehlo akho, kodwa ngokuvamile ithambile kune-myasthenia gravis (MG).

Ku-myasthenia gravis (MG), ubuthakathaka obukhulu bemisipha yokuphefumula (imisipha ekusiza ukuphefumula) bungaba yingozi. Lokhu akuvamile ku-LEMS.

Abantu abane-myasthenia gravis (MG) ngokuvamile ababonisi izimpawu eziningi zokungasebenzi kahle kwesistimu yezinzwa ezizimele (isib. amehlo omile, umlomo owomile, ukuqunjelwa, ukuncipha kokujuluka, ukungasebenzi kahle kocansi). Ngokuphambene nalokho, abantu abane-LEMS bavame ukuba nalezi zikhalazo, futhi lezi zimpawu ngezinye izikhathi zingasiza ekuhlukaniseni lezi zifo ezimbili.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Cishe manje usuyaqonda ukuthi i-Lambert-Eaton Myasthenic Syndrome (LEMS) iyisifo esibangelwa isimiso somzimba sokuzivikela esisebenza ngokweqile. Amaseli akho ezinzwa awathumeli imiyalezo eyanele emisipheni yakho ukuze "inciphe." Umphumela uba ubuthakathaka bemisipha nezinye izimpawu.

Khumbula, cishe abantu abangu-60% abane-LEMS banomdlavuza wamaphaphu wamangqamuzana amancane. Uma une-LEMS kodwa ungenawo umdlavuza, qiniseka ukuthi wena nodokotela wakho nihlolwa umdlavuza njalo ezinyangeni ezimbalwa okungenani iminyaka emibili ngemva kokuxilongwa.

Kunezindlela zokwelapha. Lokhu kuhlanganisa ukwelapha umdlavuza uma ukhona, kanye nokusebenzisa imithi ethuthukisa ukuxhumana kwe-nerve cell nemisipha. Ukuthuthukisa ukuxhumana kungathuthukisa amandla emisipha kanye nezinye izimpawu.

Ngiyethemba ukuthi lolu lwazi luwusizo kuwe.Khumbula njalo, uma unezinkinga zempilo, kungcono ukukhuluma nodokotela onolwazi ngakho.

I- Lambert-Eaton myasthenic syndrome, i-LEMS, izifo zokuzivikela komzimba, ubuthakathaka bemisipha, umdlavuza wamaphaphu wamangqamuzana amancane, i-neuromuscular junction, i-acetylcholine