I-Lambert-Eaton Myasthenic Syndrome

-Delile

• Ziyini Izimpawu zeLambert-Eaton Myasthenic Syndrome?
• Yini Ebangela ILambert-Eaton Myasthenic Syndrome?
• Ukuhlonza iLambert-Eaton Myasthenic Syndrome
• Ukwelapha iLambert-Eaton Myasthenic Syndrome
• Uyini Umbono Wesikhathi Eside?

Kuyini iLambert-Eaton Myasthenic Syndrome?

ILambert-Eaton myasthenic syndrome (i-LEMS) isifo esingajwayelekile sokuzilimaza esithinta ikhono lakho lokuhamba. Amasosha akho omzimba ahlasela izicubu zemisipha okuholela ebunzimeni bokuhamba nezinye izinkinga zemisipha.

Lesi sifo asikwazi ukwelashwa, kodwa izimpawu zingancipha okwesikhashana uma uzikhandla. Ungakwazi ukuphatha lesi simo ngemithi.

Ziyini Izimpawu zeLambert-Eaton Myasthenic Syndrome?

Izimpawu eziyinhloko ze-LEMS ubuthakathaka bomlenze nobunzima bokuhamba. Njengoba lesi sifo siqhubeka, uzozwa nokuthi:

• ubuthakathaka emisipheni yobuso
• izimpawu zemisipha ezingazibandakanyi
• ukuqunjelwa
• umlomo owomile
• ukungabi namandla
• Izinkinga zesinye

Ubuthakathaka bomlenze buye buthuthuke okwesikhashana ngokuzikhandla. Njengoba uzivocavoca, i-acetylcholine yakha ngamanani amakhulu ngokwanele ukuvumela amandla okuthuthuka isikhashana.

Kunezinkinga eziningana ezihambisana ne-LEMS. Lokhu kufaka phakathi:

• inkinga yokuphefumula nokugwinya
• izifo
• ukulimala ngenxa yokuwa noma izinkinga ngokusebenzisana

Yini Ebangela ILambert-Eaton Myasthenic Syndrome?

Esifweni sokuzivikela komzimba, amasosha omzimba wakho enza amaphutha emzimbeni wakho entweni yangaphandle. Amasosha omzimba wakho akhiqiza amasosha omzimba ahlasela umzimba wakho.

Ku-LEMS, umzimba wakho uhlasela ukuphela kwezinzwa okulawula inani lokukhishwa komzimba we-acetylcholineyour. I-Acetylcholine iyi-neurotransmitter edala ukusikwa kwemisipha. Ukuncipha kwemisipha kukuvumela ukuthi wenze ukunyakaza kokuzithandela njengokuhamba, ukunyakazisa iminwe yakho, nokunyusa amahlombe akho.

Ngokuqondile, umzimba wakho uhlasela iphrotheni ebizwa nge-voltage gated calcium channel (VGCC). I-VGCC iyadingeka ukuze kukhishwe i-acetylcholine. Awukhiqizi i-acetylcholine eyanele lapho i-VGCC ihlaselwa, ngakho-ke imisipha yakho ayikwazi ukusebenza kahle.

Amacala amaningi e-LEMS ahlotshaniswa nomdlavuza wamaphaphu. Abaphenyi bakholelwa ukuthi amangqamuzana omdlavuza akhiqiza amaprotheni e-VGCC. Lokhu kubangela ukuthi amasosha akho omzimba enze amasosha omzimba alwa ne-VGCC. La ma-antibody abe esehlasela womabili amangqamuzana omdlavuza namaseli emisipha. Noma ngubani angathuthukisa i-LEMS esikhathini sakhe sokuphila, kepha umdlavuza wamaphaphu ungakhuphula ubungozi bokuba nesimo. Uma kunomlando wezifo ezizimele emndenini wakho, ungaba sengozini enkulu yokuthuthukisa i-LEMS.

Ukuhlonza iLambert-Eaton Myasthenic Syndrome

Ukuxilonga i-LEMS, udokotela wakho uzothatha umlando oningiliziwe bese enza ukuhlolwa komzimba. Udokotela wakho uzobheka:

• ukwehla kwengqondo
• ukulahleka kwezicubu zomzimba
• ubuthakathaka noma inkinga yokuhamba ebangcono ngomsebenzi

Udokotela wakho angalawula izivivinyo eziningana ukuqinisekisa isimo. Ukuhlolwa kwegazi kuzobheka amasosha omzimba alwa ne-VGCC (anti-VGCC antibodies). I-electromyography (EMG) ihlola imicu yakho yemisipha ngokubona ukuthi isabela kanjani lapho ivuselelwa. Inaliti encane ifakwa emsipheni futhi ixhunywe kwimitha. Uzocelwa ukuthi ukhonte leyo misipha, futhi imitha izofunda ukuthi imisipha yakho iphendula kahle kangakanani.

Okunye ukuhlolwa okungenzeka kube yi-nerve conduction velocity test (NCV). Kulesi sivivinyo, udokotela wakho uzobeka ama-electrode ebusweni besikhumba sakho amboze isicubu esikhulu. Amachashazi akhipha isignali kagesi evuselela izinzwa nemisipha. Umsebenzi ovela ezinzwa uqoshwa ngamanye ama-electrode futhi usetshenziselwa ukuthola ukuthi izinzwa zisabela ngokushesha kangakanani ekuvuseleleni.

Ukwelapha iLambert-Eaton Myasthenic Syndrome

Lesi simo asinakulapheka. Uzosebenza nodokotela wakho ukuphatha noma yiziphi ezinye izimo, njengomdlavuza wamaphaphu.

Udokotela wakho angancoma ukwelashwa ngemithambo ye-immunoglobulin (IVIG). Kulokhu kwelashwa, udokotela wakho uzofaka i-antibody engacacisi ethoba amasosha omzimba. Okunye ukwelashwa okungenzeka kube yi-plasmapheresis. Kususwa igazi emzimbeni, bese kuhlukaniswa ne-plasma. Ama-antibody ayasuswa, bese i-plasma ibuyiselwa emzimbeni.

Izidakamizwa ezisebenza nesistimu yakho yemisipha kwesinye isikhathi zinganciphisa izimpawu. Lokhu kufaka phakathi i-mestinon (pyridostigmine) ne-3, 4 diaminopyridine (3, 4-DAP).

Le mithi kunzima ukuyithola, futhi kufanele ukhulume nodokotela wakho ukuthola eminye imininingwane.

Uyini Umbono Wesikhathi Eside?

Izimpawu zingathuthuka ngokwelapha ezinye izimo ezingaphansi, ukucindezela amasosha omzimba, noma ukususa amasosha omzimba egazini. Akuwona wonke umuntu osabela kahle ekwelashweni. Sebenza nodokotela wakho ukuthola uhlelo lokwelashwa olufanele.