I-Myasthenia Gravis, uLambert-Eaton, kunye ne-Botulism
Xa sicinga ngezizathu zobuthathaka, kunceda kakhulu ukucinga umyalezo wombane wokuthunjwa ukusuka kwikortex yengqondo ukuya kwi-muscle. Ngandlela-thile, i-impulse ihamba nge-spinal cord ibe ngophondo lwangaphambili , ngaphandle kweengcambu zomgulane, phantsi kweentsimbi ze- peripheral , kwaye ekugqibeleni uye kumbuthano we-neuromuscular.
Ikhonkco le-neuromuscular yilapho isibonakaliso sombane esibangela ukuba i-neurotransmitters ikhutshwe kwi-vesicles ekupheleni kwentsholongwane (i-terminal).
Ama-neurotransmitters awela isikhala esincinci phakathi kwesigulane se-nerve ( synapse ) kunye nomphezulu we-muscle (i-endplate). Ukulindela abathumeli ngaphesheya kweli gaba ziimpendulo ezikhethekileyo ezifanelwe umthumeli njengesikhiye kwisitshixo. Xa kukho i-fit, i-i-cascade ye-ions iholele ekuqhekekeni kwe-muscular.
I-neurotransmitter esetyenziselwa ukubonakalisa phakathi kweentsimbi kunye ne-muscle yi-acetylcholine. Kukho iindlela ezininzi zokudlula ukupheka kwe-acetylcholine neurotransmitter phakathi kwe-nerve ne-muscle cTan. Imizekelo emithathu emihle kakhulu ye- myasthenia gravis , isifo seLambert-Eaton, kunye ne-botulinum toxicity.
Myasthenia Gravis
Ngenxa yokuxhaphaka phakathi kwabantu abayi-150 ukuya kuma-200 kwisigidi, i-myasthenia gravis yinto eqhelekileyo yeentsholongwane ze-neuromuscular, kunye nenye yezona zinto ziqondwa kakuhle kuzo zonke izifo ze-neurological. Esi sifo kubangela ubuthathaka obuthakathaka ngenxa yokuvalelwa kwe-neurotransmitter receptors kwimisipha.
Ama-antibodies ngokuqhelekileyo afuna ukuhlasela izifo ezihlaselayo ziphosakele i-acetylcholine receptors ye-pathogen kunye nokuhlaselwa. Ukuzivocavoca kwenza ukuba ubuthathaka bubi nakakhulu. Phakathi kwama-60 ukuya kuma-70 ekhulwini abantu abane-myasthenia gravis baneengxaki kwi- thymus , kwaye i- thymoma ibe yi-10 ukuya kwi-12 ekhulwini. Uninzi lwezinye iindlela zokwelapha ziyafumaneka.
ULambert-Eaton Myasthenic Syndrome (LEMS)
ULambert-Eaton udla ngokuba yi- paraneoplastic syndrome , oku kuthetha ukuba izifo ezinqamana nomdlavuza nazo zihlasela inxalenye yesimiso se-nervous. Ngokungafani ne-myasthenia gravis, apho izakhiwo ezihlaseleyo zihlaselwa kwi-muscle, ingxaki kwi-LEMS iphela ekupheleni kwe-motor nerves. Iziteshi zikaCalcium zivulwa kwaye zibonakalise ukukhululwa kwee-neurotransmitters, kodwa azikwazi ukwenza njalo kwi-LEMS kuba izixhobo zamathambo zihlasele ishaneli. Ngenxa yoko, akukho ntambo ye-neurotransmitter ekhishwayo, kwaye isigulane sibhekene nobuthathaka kuba i-muscle ayikwazi ukufumana uphawu kumvumelwano. Ngokuzivocavoca ngokuphindaphindiweyo, ukusilela kunganqotshwa; ngoko nge-LEMS, ngamanye amaxesha iimpawu ziphucula ngokufutshane ngentsingiselo ephindaphindiweyo.
Botulism
Ngesinye isikhathi i-toxin ye-botulinum isebenzisa ngamabango oogqirha ukunyanzelisa izihlunu ukuphumla kwiimeko ze- dystonia . Kwifomu yayo engapheliyo, i-toxin iveliswa yi-bhakteria kwaye ingabangela ukukhubazeka okuqala ngeemisipha zobuso kunye nomqala kunye nokuhla phezu komzimba wonke. Njengazo ezinye iintlungu zesiganeko se-neuromuscular, le nto ingabangela uxinzelelo lwezonyango olufuna ukuthungulwa . Iiprotheni zokuhlaselwa kwe-toxin ezivumela i-vesicle ezele i-neurotransmitters ngaphakathi kwe-pre-synaptic neuron ukuba idibanise ekupheleni kwentsholongwane ngaphambi kokungena kwisithuba phakathi kwentsholongwane nemisipha.
Unyango luyi-antidote kwi-toxin ye-botulin, ekufuneka yenziwe ngokukhawuleza.
Ezinye iingxaki ze-Neuromuscular Junction
Ezinye iziyobisi, ezifana ne-penicillamine kunye neminye i-statins, akunakwenzeka ukuba i-reaction engaphendulanga ngokuzenzekelayo eyenza i-myathenia gravis. Ezinye ezininzi iimichiza zingaphaya okanye zilethe ingxaki kumntu osuvele ene-myasthenia gravis.
Ukuvavanywa kwezifo ze-Neuromuscular Junction
Ngaphandle kovavanyo lwangaphakathi, isinyathelo sokuqala ekufumaneni isifo se-neuromuscular junction yi- electromyogram kunye nesifundo sokuqhutyelwa kwentsholongwane. Oku akunakunceda kuphela ukwahlula phakathi kwe-myasthenia gravis, ubuthi be-botulin, kunye neLambert-Eaton kodwa kunokukunceda ukulawula ezinye izifo ezifana ne-motor neuron, kuquka i-amyotrophic lateral sclerosis.
Ukuphazamiseka kwemibutho ye-neuromuscular inokuba yinto enzulu kakhulu, efuna intubation kunye nokuphuphuma umoya ukunceda ukuphefumula ukuba ubuthathaka buba nzima kakhulu. Iinkqubo zeengxaki zihluke kakhulu, zifuna unyango oluhlukeneyo. Ukuxilongwa ngokufanelekileyo kuyisinyathelo sokuqala sokufumana amandla kunye nokukhuseleko.
Imithombo:
I-Ropper AH, i-Samuels MA. I-Adams kunye neMigaqo yeVictor ye-Neurology, 9th: I-McGraw-Hill Companies, Inc., 2009.
I-Hal Blumenfeld, i-Neuroanatomy ngokusebenzisa iiKliniki zeeKliniki. I-Sunderland: Abapapashi be-Sinauer Associates 2002.