Isifo seLambert-Eaton

ILambert-Eaton syndrome (i-LES) yisifo esingajwayelekile lapho ukuxhumana okuyiphutha phakathi kwezinzwa nemisipha kuholela ebuthakathakeni bemisipha.

I-LES yinkinga yokuzimela. Lokhu kusho ukuthi amasosha akho omzimba aqondise ngephutha amaseli nezicubu zomzimba. Nge-LES, amasosha omzimba akhiqizwa amasosha omzimba ahlasela amangqamuzana ezinzwa. Lokhu kwenza amangqamuzana ezinzwa angakwazi ukukhipha ngokwanele ikhemikhali elibizwa ngokuthi i-acetylcholine. Leli khemikhali lidlulisa imizwa phakathi kwezinzwa nemisipha. Umphumela uba ubuthakathaka bemisipha.

I-LES ingahle yenzeke ngomdlavuza onjengomdlavuza omncane wamaphaphu wamangqamuzana noma ukuphazamiseka kokuzimela komzimba okufana ne-vitiligo, okuholela ekulahlekelweni kombala wesikhumba.

I-LES ithinta abesilisa kaningi kunabesifazane. Iminyaka ejwayelekile yokwenzeka isondele eminyakeni engama-60. I-LES ayivamile ezinganeni.

Ubuthakathaka noma ukulahleka kokunyakaza okungaba nzima kakhulu, kufaka phakathi:

• Kunzima ukukhuphuka izitebhisi, ukuhamba, noma ukuphakamisa izinto
• Ubuhlungu bemisipha
• Ukudonsa ikhanda
• Isidingo sokusebenzisa izandla ukuvuka endaweni yokuhlala noma yokulala
• Izinkinga ukukhuluma
• Izinkinga zokuhlafuna noma ukugwinya, okungabandakanya ukuklinya noma ukuklinya
• Izinguquko zombono, njengokufiphala kwamehlo, umbono ophindwe kabili, kanye nenkinga yokugcina ukubuka okungaguquki

Ubuthakathaka ngokuvamile bumnene ku-LES. Imisipha yomlenze ithinteka kakhulu. Ubuthakathaka bungathuthuka ngemuva kokuzivocavoca, kepha ukuzikhandla okuqhubekayo kubangela ukukhathala kwezinye izimo.

Izimpawu ezihlobene nezinye izingxenye zesistimu yezinzwa zivame ukuvela, futhi zifaka:

• Umfutho wegazi uyashintsha
• Isiyezi lapho umile
• Umlomo owomile
• Ukungasebenzi kahle kwe-Erectile
• Amehlo omile
• Ukuqunjelwa
• Kwehle ukujuluka

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa komzimba abuze ngezimpawu. Ukuhlolwa kungakhombisa:

• Ukucabanga okunciphile
• Ukulahleka okungenzeka kwezicubu zemisipha
• Ubuthakathaka noma ukukhubazeka okuba ngcono kancane ngomsebenzi

Ukuhlolwa okusiza ukuxilonga nokuqinisekisa i-LES kungafaka:

• Ukuhlolwa kwegazi ukubheka amasosha omzimba ahlasela izinzwa
• I-Electromyography (EMG) yokuhlola impilo yemicu yemisipha
• I-Navigation conduction velocity (NCV) yokuhlola ijubane lomsebenzi kagesi ngezinzwa

I-CT scan neMRI yesifuba nesisu, kulandele i-bronchoscopy yababhemayo kungenziwa ngaphandle komdlavuza. Ukuskena i-PET kungenziwa futhi uma kusolwa isimila samaphaphu.

Izinhloso eziyinhloko zokwelashwa uku:

• Khomba futhi welaphe noma yiziphi izinkinga eziyimbangela, njengomdlavuza wamaphaphu
• Nikeza ukwelashwa ukusiza ngobuthakathaka

Ukushintshaniswa kweplasma, noma i-plasmapheresis, ukwelashwa okusiza ukususa emzimbeni noma yimaphi amaprotheni ayingozi (ama-antibodies) aphazamisa ukusebenza kwezinzwa. Lokhu kuhilela ukususa i-plasma yegazi equkethe amasosha omzimba. Amanye amaprotheni (afana ne-albumin) noma i-plasma enikelwe afakwa emzimbeni.

Enye inqubo ifaka phakathi ukusebenzisa i-immunoglobulin (IVIg) efakwa emthanjeni ukufaka inani elikhulu lama-antibodies awusizo ngqo egazini.

Imithi engazama futhi ifaka:

• Izidakamizwa ezicindezela impendulo yamasosha omzimba
• Izidakamizwa ze-Anticholinesterase zokuthuthukisa ithoni yemisipha (yize lokhu kungasebenzi kahle uma unikezwa wedwa)
• Izidakamizwa ezandisa ukukhishwa kwe-acetylcholine kumaseli ezinzwa

Izimpawu ze-LES zingathuthuka ngokwelapha isifo esingaphansi, ukucindezela amasosha omzimba, noma ukususa amasosha omzimba. Kodwa-ke, i-paraneoplastic LES kungenzeka ingaphenduli futhi ekwelashweni. (Izimpawu ze-Paraneoplastic LES kungenxa yempendulo yesistimu yomzimba yokuguqula isimila). Ukufa kungenxa yokulimala okuyisisekelo.

Izinkinga ze-LES zingafaka:

• Ubunzima bokuphefumula, kufaka phakathi ukwehluleka ukuphefumula (okungajwayelekile kakhulu)
• Kunzima ukugwinya
• Izifo, njenge-pneumonia
• Ukulimala kokuwa kanye nezinkinga ngokusebenzisana

Shayela umhlinzeki wakho uma izimpawu ze-LES zikhula.

Isifo se-Myasthenic; Isifo se-Eaton-Lambert; I-Lambert-Eaton myasthenic syndrome; AMAKHAMBI; LES

• Imisipha yangaphandle engaphezulu

U-Evoli A, uVincent A. Ukuphazamiseka kokudluliswa kwe-neuromuscular. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 394.

Moss HE. Iziphazamiso zemizwa yezinkophe kanye nobuso. Ku: Liu GT, Volpe NJ, Galetta SL, ama-eds. Liu, Volpe, kanye Neuro-Ophthalmology kaGaletta. 3rd ed. IPhiladelphia, PA: Elsevier; 2019: isahluko 14.

ISanders DB, iGuptill JT. Ukuphazamiseka kokudluliswa kwe-neuromuscular. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ama-eds. I-Neurology kaBradley ekwenziweni kwemitholampilo. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 109.