ILambert-Eaton Myasthenic Syndrome

Umxholo

• Ziintoni Iimpawu zeLambert-Eaton Myasthenic Syndrome?
• Yintoni ebangela iLambert-Eaton Myasthenic Syndrome?
• Ukuchonga iLambert-Eaton Myasthenic Syndrome
• Ukunyanga iLambert-Eaton Myasthenic Syndrome
• Yiyiphi imbono yexesha elide?

Yintoni iLambert-Eaton Myasthenic Syndrome?

I-Lambert-Eaton myasthenic syndrome (i-LEMS) isifo esingaqhelekanga sokuzimela komzimba esichaphazela amandla akho okuhamba. Amajoni akho omzimba ahlasela izicwili zemisipha ezikhokelela kubunzima bokuhamba kunye nezinye iingxaki zemisipha.

Esi sifo asinakunyangeka, kodwa iimpawu zingancipha okwethutyana ukuba uyazimisela. Unokuyilawula imeko ngamayeza.

Ziintoni Iimpawu zeLambert-Eaton Myasthenic Syndrome?

Iimpawu eziphambili ze-LEMS bubuthathaka emlenzeni kunye nobunzima bokuhamba. Njengoko esi sifo siqhubeka, uya kuba namava:

• ubuthathaka kwimisipha yobuso
• Iimpawu zemisipha ezingazibandakanyiyo
• ukuqhina
• umlomo owomileyo
• ukungabi namandla
• iingxaki zesinyi

Ubuthathaka emlenzeni buhlala buphucula okwethutyana xa usenza umthambo. Njengoko usenza umthambo, iacetylcholine yakha ngesixa esaneleyo ukuvumela amandla okuphucula ixesha elifutshane.

Kukho iingxaki ezininzi ezinxulumene ne-LEMS. Oku kubandakanya:

• ingxaki yokuphefumla nokuginya
• usulelo
• ukwenzakala ngenxa yokuwa okanye iingxaki ngokulungelelaniswa

Yintoni ebangela iLambert-Eaton Myasthenic Syndrome?

Kwisifo esizenzekelayo, amajoni omzimba wakho enza iimpazamo emzimbeni wakho ngento yasemzini. Amajoni akho omzimba avelisa izilwa-buhlungu ezihlasela umzimba wakho.

Kwi-LEMS, umzimba wakho uhlasela iziphelo zemithambo-luvo ezilawula inani le-acetylcholineyakho ukukhutshwa komzimba. I-Acetylcholine yi-neurotransmitter ebangela ukuphambana kwemisipha. Ukuqhawulwa kwemisipha kukuvumela ukuba wenze intshukumo yokuzithandela njengokuhamba, ukujiwuza iminwe, nokunyusa amagxa.

Ngokukodwa, umzimba wakho uhlasela iprotein ebizwa ngokuba yi-voltage gated calcium channel (VGCC). I-VGCC iyafuneka ukuze kukhutshwe i-acetylcholine. Awuvelisi i-acetylcholine eyaneleyo xa i-VGCC ihlaselwa, ke izihlunu zakho azikwazi ukusebenza kakuhle.

Iimeko ezininzi ze-LEMS zidibaniswa nomhlaza wemiphunga. Abaphandi bakholelwa ukuba iiseli zomhlaza zivelisa iprotein yeVGCC. Oku kubangela ukuba amajoni akho omzimba enze ii-antibodies ngokuchasene ne-VGCC. Ezi antibodies ke zihlasela iiseli zomhlaza kunye neeseli zemisipha. Nabani na onokuphuhlisa i-LEMS ebomini babo, kodwa umhlaza wemiphunga unokunyusa umngcipheko wokuphuhlisa imeko. Ukuba kukho imbali yezifo ezizimeleyo kusapho lwakho, unokuba semngciphekweni omkhulu wokuphuhlisa i-LEMS.

Ukuchonga iLambert-Eaton Myasthenic Syndrome

Ukufumanisa i-LEMS, ugqirha wakho uya kuthatha imbali eneenkcukacha kwaye enze uvavanyo lomzimba. Ugqirha wakho uya kujonga:

• ukuncipha kwengqondo
• ilahleko yemisipha
• ubuthathaka okanye ingxaki yokuhamba ebangcono ngomsebenzi

Ugqirha wakho angayalela iimvavanyo ezininzi ukuqinisekisa imeko. Uvavanyo lwegazi luya kujonga izilwa-buhlungu ngokuchasene ne-VGCC (anti-VGCC antibodies). I-electromyography (EMG) ivavanya imicu yakho yemisipha ngokubona indlela abasabela ngayo xa bevuselelekile. Inaliti encinci ifakwe kwimisipha kwaye iqhagamshelwe kwimitha. Uya kucelwa ukuba wenze isivumelwano nalo msipha, kwaye imitha iya kufunda ukuba imisipha yakho iphendula njani.

Olunye uvavanyo olunokwenzeka luvavanyo lokuqhuba kwe-velocity test (NCV). Kolu vavanyo, ugqirha wakho uya kubeka ii-electrode kumphezulu wolusu lwakho egubungele umsipha omkhulu. Iipatches zikhupha umqondiso wombane ovuselela imithambo-luvo kunye nezihlunu. Umsebenzi ophumela kwimithambo-luvo urekhodwe zezinye ii-electrode kwaye usetyenziselwa ukufumanisa ukuba iinzwa zisabela njani ngokukhawuleza kukhuthazo.

Ukunyanga iLambert-Eaton Myasthenic Syndrome

Le meko ayinakunyangeka. Uya kusebenza nogqirha wakho ukulawula naziphi na ezinye iimeko, njengomhlaza wemiphunga.

Ugqirha wakho unokucebisa unyango lwe-immunoglobulin (IVIG) efakwa ngaphakathi. Kolu nyango, ugqirha wakho uza kufaka intsholongwane engacacanga ethomalalisa amajoni omzimba. Olunye unyango olunokubakho yi-plasmapheresis. Igazi liyasuswa emzimbeni, kwaye iplasma yahlulwe ngaphandle. Amajoni omzimba ayasuswa, ize iplasma ibuyiselwe emzimbeni.

Iziyobisi ezisebenza kunye nenkqubo yakho yemisipha ngamanye amaxesha zinokukhulula iimpawu. Oku kubandakanya i-mestinon (pyridostigmine) kunye ne-3, 4 diaminopyridine (3, 4-DAP).

La mayeza kunzima ukuwafumana, kwaye kuya kufuneka uthethe nogqirha wakho ukuze ufumane ulwazi oluthe kratya.

Yiyiphi imbono yexesha elide?

Iimpawu zinokuphucula ngokunyanga ezinye iimeko ezisisiseko, ukucinezela amajoni omzimba, okanye ukususa ii-antibodies egazini. Asinguye wonke umntu olwamkelayo unyango. Sebenza nogqirha wakho ukuza nesicwangciso sonyango esifanelekileyo.