Ngaba nawe uvakalelwa kukuba imilenze yakho iyaphelelwa ngamandla? Oku kusenokuba yiLambert-Eaton Myasthenic Syndrome (LEMS)!

Ngaba ngamanye amaxesha uziva ngathi imilenze yakho ibuthathaka, unengxaki yokuvuka esitulweni, uziva udiniwe xa unyuka izinyuko? Okanye uziva udiniwe uneempawu ezifana neenkophe ezinzima, umlomo owomileyo, kunye nokubona kabini? Ezi zisenokungabi kukudinwa nje kuphela. Namhlanje siza kuthetha ngemeko onokuthi ungazange uve ngayo, kodwa ekufanele ukwazi ngayo, iLambert-Eaton Myasthenic Syndrome, okanye ``(LEMS)`` njengoko siyibiza ngokufutshane.

Yintoni iLambert-Eaton Myasthenic Syndrome (LEMS)?

Ngamafutshane, iLambert-Eaton Myasthenic Syndrome (LEMS) sisifo esibangelwa kukuzikhusela komzimba. Ngoku usenokuba uzibuza ukuba yintoni isifo esibangelwa kukuzimela komzimba . Imizimba yethu inenkqubo yokuzikhusela komzimba. Oku kukusikhusela ngokulwa neentsholongwane ezibangela izifo ezivela ngaphandle. Kufana nomkhosi kwilizwe lethu. Kodwa ngamanye amaxesha le nkqubo yokuzikhusela komzimba iyaphazama. Emva koko iqala ukuhlasela iiseli zethu eziphilileyo. Yiloo nto yenzekayo kwiLEMS.

Kule `(LEMS)`, inkqubo yethu yomzimba yokuzikhusela ihlasela unxibelelwano phakathi kwemithambo-luvo yethu kunye nemisipha . Ngokuchanekileyo, olu nxibelelwano lubizwa ngokuba yi`neuromuscular junction`. Xa olu nxibelelwano lonakele, ukudluliselwa kwemiyalezo esuka kwimithambo-luvo ukuya kwimisipha kuyaphazamiseka. Kulapho ke kwenzeka ubuthathaka bemisipha.

Olu buthathaka ludla ngokuqala emathangeni akho angaphezulu , kodwa lunokusasaza kancinci kancinci kwiingalo zakho, ekuphefumleni, ekuginyeni, nasekuthetheni.

Ngubani onokuthi aphuhlise le `(LEMS)`?

Kukho amaqela amabini aphambili enza i-`(LEMS)`.

1. Okunxulunyaniswa nomhlaza: Malunga nama-60% abantu abane-LEMS banohlobo oluthile lomhlaza. Uhlobo oluqhelekileyo ngumhlaza wemiphunga weeseli ezincinci . Okumangalisayo kukuba, ngamanye amaxesha iimpawu ze-LEMS zinokubonakala kwiinyanga okanye iminyaka ngaphambi kokuba umhlaza uvele. Uninzi lwaba bantu lungaphezulu kweminyaka engama-60 ubudala, ngamadoda, kwaye basebenzise iimveliso zecuba.

2. Akukho mhlaza: Amanye ama-40% abantu abane-LEMS abakafumani mhlaza. Aba bantu badla ngokufunyaniswa ukuba banawo xa bemalunga neminyaka engama-35 ubudala baze baphinde bafunyanwe xa bemalunga neminyaka engama-60 ubudala.

I-LEMS yimeko engaqhelekanga kakhulu phakathi kwabantwana abancinci.

Ingaba umhlaza wemiphunga weeseli ezincinci (i-LEMS) kuphela onxulumene noku?

Hayi. Umhlaza wemiphunga weeseli ezincinci lolona hlobo luqhelekileyo lomhlaza, kodwa ezinye iintlobo zomhlaza zinokunxulunyaniswa ne-LEMS. Imizekelo ibandakanya umhlaza weprostate, i-thymoma, kunye neengxaki ze-lymphoproliferative. Ngenxa yokuba iimpawu ze-LEMS zinokuvela ngaphambi kokuba kuvele iimpawu zomhlaza, kubalulekile ukuba novavanyo olufanelekileyo lokujonga umhlaza ukuba ugqirha wakho ukuxilongile ukuba une-LEMS.

Ngaba i-LEMS ibangelwa lutshintsho lwemfuza?

Izazinzulu zikholelwa ukuba izifo ezizikhuselayo ngokubanzi zinokuba nonxibelelwano oluthile lwemfuza. Ekubeni i-LEMS ikwasisifo esizikhuselayo, kusenokwenzeka ukuba nayo inefuthe kwimfuza, kodwa oku akukaqondwa ngokupheleleyo.

Ixhaphake kangakanani `(LEMS)`?

I-LEMS sisifo esingaqhelekanga kakhulu . Kuqikelelwa ukuba esi sifo sichaphazela abantu abamalunga nezigidi ezi-2.8 kwihlabathi liphela. E-United States, kukho malunga neemeko ezingama-400 ezixeliweyo ze-LEMS. Nangona kungekho zibalo zichanekileyo zokuba bangaphi abantu kweli lizwe lethu, kuyacaca ukuba esi sisifo esingaqhelekanga.

Zithini iimpawu ze-`(LEMS)`?

Makhe sijonge iimpawu ze-`(LEMS)` nganye nganye. Ayizizo zonke ezi mpawu ezenzeka ngaxeshanye kubo bonke abantu, ezinye iimpawu zinokuvela kwangethuba, ezinye zinokuvela kamva.

• Ubuthathaka bemisipha kunye nokudinwa: ingakumbi kwimilenze ephezulu.
• Ubunzima bokuhamba: Ubunzima bokunyuka izinyuko okanye ukuphakama esitulweni.
• Intlungu okanye ukuqina kwemisipha.
• Ukuziva ngathi umzimba awusebenzi kakuhle.
• Iinkophe ezigobileyo (i-ptosis): Oku kunokubonakala ngathi kukozela.
• Ukubona izinto ezimbini ngaxeshanye (diplopia).
• Umlomo owomileyo namehlo (xerostomia): ukuncipha kokuvuza kwamathe, ukuncipha kweenyembezi emehlweni.
• Ukuqhinwa.
• Ukuncipha kokubila.
• Ukuhla ukusinda.
• Ubunzima bokuchama.
• Ukungasebenzi kakuhle kwe-erectile kumadoda.
• Ubunzima bokuthetha (i-dysarthria) kunye nobunzima bokuginya (i-dysphagia): Ezi zihlala ziimpawu ezikhula kamva kwesi sifo.
• Ubunzima bokuphefumla (ukuphelelwa ngamandla) kunye nobunzima bokuphefumla: Olu luphawu olungaqhelekanga nolunzima olunokwenzeka kwisigaba sokugqibela sesi sifo.

Kubalulekile: Ukuba unenye okanye ngaphezulu kwezi mpawu, musa ukucinga ukuba yi-LEMS. Nangona kunjalo, ukuba ezi mpawu ziyaqhubeka, kububulumko ukufuna ingcebiso kagqirha.

I-LEMS idla ngokubonakala njani kwaye iqhubeka njani?

I-LEMS idla ngokuchaphazela izihlunu zamathanga kuqala. Emva koko inokuchaphazela izihlunu zamagxa, izihlunu zeengalo nemilenze, izihlunu ezinceda ekuthetheni nasekuginyeni, kunye nezihlunu zamehlo.

Ekuqaleni kwesi sifo, unokuba nobunzima bokuvuka esitulweni, ukunyuka izinyuko okanye iinduli, okanye ukwenza umsebenzi onzima ofuna imilenze yakho isebenze.

Iimpawu zihlala zikhula kancinci, kwiiveki ezimbalwa ukuya kwiinyanga . Nangona kunjalo, ukuba une-LEMS enomhlaza, iimpawu zinokukhula ngokukhawuleza.

Yintoni ebangela i-LEMS?

Njengoko besikhe sathetha ngaphambili, i-LEMS sisifo esibangelwa kukuzikhusela komzimba . Oku kuthetha ukuba ukhuselo lomzimba wakho (ii-antibodies) luhlasela izicubu zakho eziphilileyo. Kwi-LEMS, ezi antibodies zihlasela indawo apho iiseli zemithambo-luvo kunye neefayibha zemisipha zidibana khona, ebizwa ngokuba yi-neuromuscular junction. Ngokukodwa, zihlasela imisele yecalcium apho. Ezi misele yecalcium zibalulekile ukuze imiyalezo yemithambo-luvo idlule.

Cinga ngayo njengomqolo weedomino. Olu hlaselo luqala uthotho lweziganeko:

1. Ii-antibodies zibopha kwimijelo ye-calcium eziphelweni zeeseli zemithambo-luvo zize ziyithintele.

2. Xa imijelo yecalcium inciphile, ubungakanani be -acetylcholine, i-neurotransmitter, ekhutshwayo buyancipha. Le acetylcholine yikhemikhali ebalulekileyo ekusebenzeni kwezihlunu.

3. Xa i-acetylcholine ingakhutshelwanga ngokwaneleyo, "imiyalezo" eya kwimicu yemisipha ayifiki kakuhle. Emva koko izihlunu zakho azikwazi ukusebenza ngokupheleleyo.

4. Ngenxa yoko, izihlunu azisebenzi kakuhle, nto leyo ebangela ubuthathaka.

Nantsi indlela abantu abanomhlaza wemiphunga weeseli ezincinci abakhula ngayo i-LEMS: Xa unomhlaza emzimbeni wakho, ii-antibodies zifumana isibonakaliso sokuba "uye emfazweni." Kodwa endaweni yokuhlasela iiseli zomhlaza (ngokukodwa, ii-calcium channels ezikumphezulu weeseli zomhlaza), ezi antibodies zihlasela ngempazamo ii-calcium channels eziphelweni zeeseli zemithambo-luvo. Emva koko kwenzeka uthotho olufanayo lweziganeko.

Izazinzulu zikholelwa ukuba unobangela we-LEMS kubantu abangenayo umhlaza wemiphunga kukungasebenzi kakuhle kwenkqubo yomzimba yokuzikhusela. Kodwa akukacaci ukuba kutheni oku kusenzeka.

Ungazichonga njani `(i-LEMS)`?

Inyathelo lokuqala ekuxilongeni i-LEMS kukuba ugqirha wakho akuhlole ngononophelo. Oku kudla ngokubandakanya ingcali yezifo zemithambo-luvo, okanye ugqirha ogxile kwizifo zemithambo-luvo kunye nemisipha. Inxalenye yale nkqubo ibandakanya ukubuza ngeempawu zakho, imbali yakho yezonyango, kunye nayo nayiphi na imithi oyithathayo. Uvavanyo oluneenkcukacha lwemithambo-luvo lwenziwa ukujonga iipateni zobuthathaka kunye neempawu ezihambelana ne-LEMS.

Inyathelo elilandelayo kukuvavanya. Uvavanyo lwe-`(LEMS)` luquka:

• Uvavanyo lwegazi: Oku kunokujonga ukuba unazo na ii-antibodies ze-anti-calcium channel egazini lakho. Malunga nama-85% abantu abane-LEMS banazo ezi antibodies egazini labo.
• I-Electromyography (EMG): Olu vavanyo lujonga indlela izihlunu zakho kunye nemithambo-luvo ezisebenzisana ngayo. Kwi-LEMS, uvavanyo lwe-EMG luneziphumo ezithile, ngoko ke lunokunceda ukuqinisekisa ukuxilongwa.
• I-Lung X-ray `(X-ray)` okanye i-CT scan `(CT scan)` okanye i-MRI scan `(MRI)` (yesifuba): Ngenxa yokuba imeko ebizwa ngokuba yi-`(LEMS)` inokukhula kubantu abanomhlaza wemiphunga weseli encinci, olu vavanyo lukhangela iimpawu zomhlaza wemiphunga.

Iimpawu ze-LEMS ziqala kwiinyanga ukuya kwiminyaka emithandathu ngaphambi kokuba umhlaza uvele. Ke ngoko, emva kokuba i-LEMS ifunyenwe, kufuneka uhlolwe umhlaza rhoqo emva kweenyanga ezintathu ukuya kwezintandathu ubuncinane iminyaka emibini. Ugqirha wakho uya kukucebisa ishedyuli yokuhlolwa efanelekileyo kuwe.

Iphathwa njani i-LEMS?

Kukho iindlela ezahlukeneyo zonyango lwe-LEMS.

• Ukunyanga umhlaza osisiseko: Ukuba i-LEMS inxulumene nomhlaza, njengomhlaza wemiphunga weeseli ezincinci, ukunyanga loo mhlaza kunokunciphisa iimpawu ze-LEMS. Unyango lomhlaza lungabandakanya i-chemotherapy, unyango lwe-radiation, kunye/okanye utyando. Oku kuxhomekeke kuhlobo lomhlaza kunye neengcebiso zikagqirha wakho.
• Unyango oluthile lwe-(LEMS): Olu luquka amayeza athomalalisa iimpawu kunye namayeza alawula inkqubo yomzimba yokuzikhusela.

Unyango lweempawu

• I-Amifampridine (Firdapse®), iGuanidine: La mayeza andisa amandla ezihlunu. Andisa ukukhululwa kwe-neurotransmitter acetylcholine kwindawo yokudibana kwemisipha, nto leyo ephucula imiqondiso kwiihlunu.
• I-Pyridostigmine (i-Pyridostigmine - iMestinon®): I-Pyridostigmine isebenza ngokuthintela ukuqhekeka kwe-acetylcholine kwindawo yokudibana kwemisipha. Oku kwandisa imiqondiso ye-acetylcholine kwaye kuphucula amandla emisipha. I-Pyridostigmine ngamanye amaxesha inikwa kunye ne-amifampridine ukuphucula ukusebenza kwemisipha kunye nokunyanga iimpawu ze-LEMS ezifana nomlomo owomileyo, amehlo omileyo, ukuqhina, ukungasebenzi kakuhle ngokwesondo, kunye nokuncipha kokubila. Nangona kunjalo, ukusetyenziswa kwe-pyridostigmine kwi-LEMS kukusetyenziswa okungekho mthethweni, oko kuthetha ukuba i-U.S. Food and Drug Administration (FDA) ayilivumanga eli yeza kule meko.

Unyango lokuguqula amajoni omzimba

• Izinto ezithintela amajoni omzimba:La mayeza anceda ekumiseni okanye ekulawuleni umsebenzi "wokuzikhusela" wenkqubo yakho yokuzikhusela. Ugqirha wakho angakunika la mayeza ukuba amanye amayeza awakulawuleli iimpawu zakho. Umzekelo, iyeza elibizwa ngokuba yi-prednisone linokunikelwa kunye okanye ngaphandle kwe-azathioprine okanye i-cyclosporine.
• Utshintsho lweplasma: Kule nkqubo, umatshini wahlula inxalenye yolwelo yegazi lakho (iplasma) kwiiseli zegazi. Ii-antibodies zakho zikwiplasma. Emva kokwahlukana, igazi libuyiselwa emzimbeni wakho. Ugqirha wakho angacinga ngolu nyango ukuba uneempawu eziqatha kwaye ufuna unyango olukhawulezileyo.
• Ii-Immunoglobulins: Kwabanye abantu, unyango lwe-immunoglobulins olunezinga eliphezulu lunokunceda ekuthinteleni ukuba isifo singabi mandundu.

Ngaba ii-LEMS zingathintelwa?

Isizathu esichanekileyo se-LEMS asikaqondwa ngokupheleleyo, ngoko ke akwaziwa ukuba ikho na indlela yokuthintela esi sifo.

Nangona kunjalo, ekubeni i-LEMS inokukhula kubantu abanomhlaza wemiphunga, unokunceda ekuthinteleni umhlaza wemiphunga ngokuthi:

• Musa ukutshaya. Ukuba usebenzisa iimveliso zecuba, cela ugqirha wakho akuncede uyeke.
• Kuphephe ukutshaya umsi osetyenziswa ngabanye abantu.
• Jonga ikhaya lakho ukuba akukho gesi ye-radon na. Ukuvezwa yigesi ye-radon ixesha elide kunokunyusa umngcipheko wakho wokuba nomhlaza wemiphunga.

Ungathatha amanyathelo okugcina indlela yokuphila esempilweni:

• Yitya iziqhamo nemifuno emininzi. Landela ukutya kweMeditera okanye kweDASH.
• Gcina ubunzima bakho busempilweni.
• Zilolonge ngokwemiyalelo kagqirha wakho.
• Lala ngokwaneleyo - ngesiqhelo iiyure ezisixhenxe ukuya kwezisithoba.
• Yehlisa uxinzelelo lwakho. Uxinzelelo lunokwenza iimpawu zibe zimbi ngakumbi.
• Kuphephe ukuhlamba ngamanzi ashushu. Ubushushu bunokwenza iimpawu ze-LEMS zibe zimbi ngakumbi.
• Bona ugqirha wakho rhoqo, kwaye ubazise ngoko nangoko ukuba impilo yakho iyatshintsha okanye ukuba ubonakalisa iimpawu ezintsha.

Ngaba iiLEMS zinganyangeka ngokupheleleyo?

Ngelishwa, akukho nyango lwe-LEMS. Ukunyanga umhlaza osisiseko onxulunyaniswa ne-LEMS kubonisiwe ukuba kuyayiphucula iimpawu ze-LEMS. Amayeza okunciphisa umzimba anokunceda ukulawula uhlaselo lomzimba oluzenzekelayo, kwaye kukwakho nonyango olunokunceda ekunciphiseni iimpawu ngokunyusa inani le-acetylcholine kwiziphelo zemithambo-luvo kunye nokuphucula amandla emisipha.

Ukuba une-LEMS engenamhlaza, ungalindela ubomi obuqhelekileyo. Ukuba une-LEMS kunye nomhlaza, iimpawu zakho zinokuphucuka ngokuhamba kwexesha (nangona zingenjalo ngokupheleleyo) ukuba umhlaza ufunyaniswa kwaye ulawulwa kwangethuba.

Umgangatho wobomi bakho uxhomekeke kakhulu kubukhali beempawu zakho.

Ndingaqhagamshelana nini nogqirha wam ukuba ndine `(LEMS)`?

Ukuba kukho utshintsho kwimpilo yakho, okanye ukuba iimpawu zakho ziya zisiba mandundu, qhagamshelana nogqirha wakho nangaliphi na ixesha . Ungoyiki, ungoyiki. Oogqirha bakhona ukukunceda.

Yintoni umahluko phakathi kwe-`(LEMS)` kunye ne-`(Myasthenia Gravis - MG)`?

I-Lambert-Eaton myasthenic syndrome (LEMS) kunye ne-myasthenia gravis (MG) zineempawu ezifanayo. Zombini zibangela ubuthathaka bemisipha kunye nokudinwa.

Nangona kunjalo, i-myasthenia gravis (MG) idla ngokuchaphazela izihlunu ezijikeleze amehlo akho kunye nezihlunu ezisetyenziselwa ukuginya. Kamva, inokuchaphazela izihlunu ngokubanzi, kuquka izihlunu zokuphefumla kunye nezihlunu zamalungu omzimba.

I-LEMS idla ngokuqala ngobuthathaka emathangeni akho angasentla, ize iye ezingalweni zakho. I-LEMS inokubangela ubuthathaka kwimisipha yamehlo akho, kodwa idla ngokuba buthathaka kune-myasthenia gravis (MG).

Kwi-myasthenia gravis (MG), ubuthathaka obukhulu bemisipha yokuphefumla (imisipha ekuncedayo ukuphefumla) bunokuba yingozi. Oku kunqabile kwi-LEMS.

Abantu abane-myasthenia gravis (MG) badla ngokungabonisi zimpawu zininzi zokungasebenzi kakuhle kwenkqubo yemithambo-luvo (umz., amehlo omileyo, umlomo owomileyo, ukuqhina, ukuncipha kokubila, ukungasebenzi kakuhle ngokwesondo). Ngokwahlukileyo koko, abantu abane-LEMS badla ngokuba nezi zikhalazo, kwaye ezi mpawu ngamanye amaxesha zinokunceda ukwahlula ezi zifo zimbini.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Okwangoku mhlawumbi uyaqonda ukuba iLambert-Eaton Myasthenic Syndrome (LEMS) sisifo esibangelwa yinkqubo yomzimba yokuzikhusela esebenza kakhulu. Iiseli zakho zemithambo-luvo azithumeli imiyalezo eyaneleyo kwimisipha yakho ukuze "zinciphe." Isiphumo kukuba buthathaka kwemisipha kunye nezinye iimpawu.

Khumbula, malunga nama-60% abantu abane-LEMS banomhlaza wemiphunga weeseli ezincinci. Ukuba une-LEMS kodwa awunayo umhlaza, qiniseka ukuba wena nogqirha wakho nihlolwa umhlaza rhoqo emva kweenyanga ezimbalwa ubuncinane iminyaka emibini emva kokuba nixilongiwe.

Kukho unyango. Olu luquka ukunyanga umhlaza ukuba ukhona, kunye nokusebenzisa amayeza aphucula unxibelelwano phakathi kwe-nerve cells kunye ne-muscle fiber. Ukuphucula unxibelelwano kunokuphucula amandla emisipha kunye nezinye iimpawu.

Ndiyathemba ukuba olu lwazi luluncedo kuwe.Hlala ukhumbula, ukuba uneengxaki zempilo, kungcono ukuthetha nogqirha onolwazi ngayo.

Isifo se - Lambert-Eaton myasthenic, i-LEMS, izifo ze-autoimmune, ubuthathaka bemisipha, umhlaza wemiphunga weeseli ezincinci, i-neuromuscular junction, i-acetylcholine