Isifo seLambert-Eaton

I-Lambert-Eaton syndrome (i-LES) sisifo esinqabileyo apho unxibelelwano oluphosakeleyo phakathi kwemithambo kunye nezihlunu lukhokelela kubuthathaka bemisipha.

I-LES kukuphazamiseka komzimba. Oku kuthetha ukuba amajoni akho omzimba ajonge ngempazamo iiseli kunye nezicubu emzimbeni. Nge-LES, ii-antibodies eziveliswa ngamajoni omzimba ahlasela iiseli zemithambo-luvo. Oku kwenza iiseli zemithambo-luvo zingakwazi ukukhupha ngokwaneleyo imichiza ebizwa ngokuba yi-acetylcholine. Le khemikhali ihambisa iimvakalelo phakathi kwemithambo-luvo kunye nezihlunu. Isiphumo kukuba buthathaka kwezihlunu.

I-LES inokuthi ivele ngomhlaza njengomhlaza omncinci wesifo somhlaza okanye ukuphazamiseka komzimba njenge-vitiligo, ekhokelela ekulahlekelweni ngumbala wesikhumba.

I-LES ichaphazela amadoda rhoqo kunabafazi. Ubudala obuqhelekileyo bokwenzeka kweminyaka engama-60. I-LES inqabile ebantwaneni.

Ubuthathaka okanye ukuphulukana nentshukumo enokuba ngaphezulu okanye ngaphantsi kakhulu, kubandakanya:

• Kunzima ukunyuka izinyuko, ukuhamba, okanye ukuphakamisa izinto
• Intlungu yezihlunu
• Ukujongwa kwentloko
• Isidingo sokusebenzisa izandla ukuvuka kwindawo ehleliyo okanye eleleyo
• Iingxaki ukuthetha
• Iingxaki zokuhlafuna okanye ukugwinya, ezinokubandakanya ukugxothwa okanye ukukrwitshwa
• Umbono utshintsha, njengombono ongacacanga, umbono ophindwe kabini, kunye nengxaki yokugcina ujongano oluzinzileyo

Ubuthathaka ngokubanzi buthathaka kwi-LES. Imisipha yomlenze ichaphazeleka kakhulu. Ubuthathaka bunokuphucuka emva kokuzilolonga, kodwa ukuzingisa okuqhubekayo kubangela ukudinwa kwezinye iimeko.

Iimpawu ezinxulumene namanye amalungu enkqubo ye-neva zihlala zenzeka, kwaye zibandakanya:

• Uxinzelelo lwegazi luyatshintsha
• Ukuba nesiyezi xa umi
• Umlomo owomileyo
• Ukungasebenzi kakuhle kwe-Erectile
• Amehlo omileyo
• Ukuqunjelwa
• Ukwehla kokubila

Umboneleli wezempilo uya kwenza uvavanyo lomzimba kwaye abuze malunga neempawu. Uviwo lunokubonisa:

• Ukuhla kwengqondo
• Ukuphulukana okunokwenzeka kwezihlunu zemisipha
• Ubuthathaka okanye ukukhubazeka okuba ngcono kancinci kunye nomsebenzi

Uvavanyo lokunceda ukufumanisa isifo kunye nokuqinisekisa i-LES inokubandakanya:

• Uvavanyo lwegazi ukukhangela izilwa-buhlungu ezihlasela imithambo-luvo
• I-Electromyography (EMG) yokuvavanya impilo yemicu yemisipha
• Ukuhamba kwesantya (NCV) ukuvavanya isantya sombane kunye ne-nerves

Ukuvavanywa kwe-CT kunye neMRI yesifuba kunye nesisu, kulandele i-bronchoscopy yabantu abatshayayo kunokwenziwa ngaphandle komhlaza. Ukuskena i-PET kungenziwa kwakhona ukuba kurhanelwa ithumba lemiphunga.

Iinjongo eziphambili zonyango kukuba:

• Chonga kwaye uphathe naziphi na iingxaki ezinje, njengomhlaza wemiphunga
• Nika unyango ukunceda ngobuthathaka

Ukutshintshiselana ngePlasma, okanye iplasmapheresis, lunyango olunceda ukususa emzimbeni naziphi na iiproteni ezinobungozi (ii-antibodies) eziphazamisa ukusebenza kwentliziyo. Oku kubandakanya ukususa i-plasma yegazi enee-antibodies. Ezinye iiproteni (ezinjengealbumin) okanye iplasma enikelweyo emva koko ifakwe emzimbeni.

Enye inkqubo ibandakanya ukusebenzisa i-intravenous immunoglobulin (IVIg) ukufaka isixa esikhulu sezinto zokulwa ngqo egazini.

Amayeza anokuzama kwakhona aquka:

• Iziyobisi ezicinezela impendulo yenkqubo yomzimba yokuzikhusela
• Amachiza e-anticholinesterase ukuphucula ithoni yemisipha (nangona ezi zingasebenzi kakuhle xa zinikwa zodwa)
• Iziyobisi ezonyusa ukukhutshwa kwe-acetylcholine kwiiseli zemithambo-luvo

Iimpawu ze-LES zinokuphucula ngokunyanga isifo esingaphantsi, ukucinezela amajoni omzimba, okanye ukususa ii-antibodies. Nangona kunjalo, i-paraneoplastic LES ayinakuphendula ngokunjalo kunyango. (Iimpawu ze-Paraneoplastic LES zibangelwa kukutshintsha kwenkqubo yomzimba yokuzikhusela kwisisu). Ukufa kubangelwa sisifo esibi.

Iingxaki ze-LES zingabandakanya:

• Ubunzima bokuphefumla, kubandakanya ukusilela kokuphefumla (okuqhelekileyo)
• Kunzima ukugwinya
• Usulelo, njenge-pneumonia
• Ukulimala kokuwa kunye neengxaki ngokulungelelanisa

Shayela umnikezeli wakho ukuba iimpawu ze-LES zikhula.

Isifo seMyasthenic; Isifo se-Eaton-Lambert; Isifo seLambert-Eaton myasthenic; IIMPAWU; LES

• Imisipha yangaphandle yangaphandle

U-Evoli A, uVincent A. Ukuphazamiseka kokuhanjiswa kwe-neuromuscular. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 394.

Moss HE. Ukuphazamiseka kwamehlo kunye nobuso. Ku: Liu GT, Volpe NJ, Galetta SL, ii-eds. Liu, Volpe, kunye neGaletta's Neuro-Ophthalmology. Ngomhla wesi-3. IPhiladelphia, PA: Elsevier; I-2019: isahluko 14.

ISanders DB, iGuptill JT. Ukuphazamiseka kokuhanjiswa kwe-neuromuscular. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; Ngo-2016: isahluko 109.