Paroxysmal myoplegia yog kab mob neuromuscular uas tshwm sim los ntawm qhov mob tshwm sim hauv cov leeg pob txha. Tus kab mob no hnyav heev, ua rau immobility.
yog vim li cas rau nws qhov tsos? Puas muaj tej yam yuav tsum tau ua ua ntej? Cov tsos mob dab tsi tuaj yeem suav tias yog ceeb? Kev kuaj mob thiab kev kho mob li cas? Cov lus nug no thiab ntau lwm cov lus nug tam sim no yuav tsum tau teb.
keeb kwm me ntsis
Cov kab mob hauv nqe lus nug tau piav qhia thawj zaug hauv xyoo 1874. Tom qab 6 xyoo, cov lus piav qhia ntxaws txog tus kab mob no tshwm sim. I. V. Shakhnovich thiab K. Westphal tau txiav txim siab txog keeb kwm ntawm pathology. Los ntawm txoj kev, thaum lub sij hawm tus kab mob no muaj npe tom qab lawv. Nyob rau hauv lub xyoo pua 19th, myoplegia hu ua kab mob Westphal-Shakhnovich.
Sijhawm dhau mus, kev tshawb fawb txuas ntxiv mus. Xyoo 1956, J. Gamstorp tau kawm txog cov kab mob hyperkalemic (nws yuav tsum tau tham kom meej tom qab). Qhov no yog kev kho mob. Tau kawg, tus kab mob tau muab lub npe "Gamsthorpe's disease".
Tsuas yog 5 xyoos tom qab ntawd, cov kws tshawb fawb Poskanzer thiab Kerr tau tshawb pom - lawv tau txheeb pom tus kab mob thib peb. Qhov no yog ib daim ntawv normokalemic, uas yog tsis tshua muaj heev. Yog li ob peb tus neeg paub txog cov tshuaj uas kev kho mob tseem tsis tau tsim los txog rau niaj hnub no. Nws tsuas yog ua tau los ua pov thawj tias Diakarb (carbonic anhydrase inhibitor) tuaj yeem pab tiv thaiv kev tawm tsam.
Tsis tas li, cov kws tshaj lij tau tswj xyuas kom pom tias myoplegia hauv qee kis yog theem nrab, cov tsos mob. Nws tuaj yeem tshwm sim raws li qhov tshwm sim ntawm xws li pathologies:
- qog nqaij hlav qog adrenal.
- YPyelonephritis.
- YThyrotoxicosis.
- YAdrenal insufficiency.
- Kab mob plab, nrog ntuav thiab raws plab.
- YHyperaldosteronism.
- Malabsorption Syndrome.
Nws kuj tau raug pov thawj tias tuag tes tuag taw tuaj yeem tshwm sim hauv cov neeg uas yav tas los muaj tshuaj lom barium, nrog rau cov neeg uas tau siv tshuaj laxatives thiab diuretics.
Pathogenesis
Hnub no, cov txheej txheem ntawm kev loj hlob ntawm paroxysmal myoplegia tseem yog qhov kev kawm los ntawm physiologists, biochemists thiab kws kho mob thoob ntiaj teb.
Yav dhau los nws tau xav tias qhov kev mob tshwm sim no tshwm sim vim muaj kev tso tawm ntau ntxiv ntawm aldosterone. Cov version zoo li plausible, txij li kev kho nrog antagonists tau txais txiaj ntsig zoo. Txawm li cas los xij, tom qab ntawd nws tau ua pov thawj tias txhua qhov kev hloov pauv hauv qhov concentration ntawm cov tshuaj no yog theem nrab.
Muaj kev kwv yees hais txog caj cesKev txiav txim siab ua txhaum cai ntawm permeability ntawm daim nyias nyias ntawm cell membranes thiab myofibrils nyob rau hauv striated leeg nqaij. Allegedly, vim li no, dej thiab sodium ions sib sau ua ke hauv lub vacuole, thiab vim muaj kev hloov pauv hauv kev sib npaug, kev sib kis ntawm cov paj hlwb raug thaiv.
Yog tias qhov no yog vim li cas, ces cov kab mob tuaj yeem suav tias zoo ib yam li Thomsen's myotonia. Txog tam sim no, cov kev xav hais txog kev ua haujlwm tsis zoo ntawm cov cell membrane channel yog qhov muaj txiaj ntsig zoo tshaj plaws thiab kev tshawb fawb zoo.
Hypokalemic myoplegia
Muaj me ntsis yuav tsum tau them rau kev faib tawm. Hypokalemic paroxysmal myoplegia yog hom kab mob ntau tshaj plaws. Nws yog ib qho autosomal dominant inherited disorder characterized by incomplete gene penetrance.
Pab pawg pheej hmoo suav nrog cov neeg muaj hnub nyoog 10 txog 18 xyoo. Pathology tsis tshua muaj tshwm sim hauv cov neeg muaj hnub nyoog 30, thiab feem ntau ntawm cov txiv neej dua li poj niam.
Tsis yog lub hom phiaj lossis cov cim qhia ntawm hypokalemic paroxysmal myoplegia raug txiav txim. Tab sis cov hauv qab no tuaj yeem ua rau muaj kev tawm tsam:
- Nyob zoo.
- Kev quav cawv.
- lub cev nce siab.
- noj ntau ntsev los yog carbohydrates.
Cov poj niam feem ntau muaj qaug dab peg thawj hnub ntawm lawv lub sijhawm. Los yog 1-2 hnub ua ntej nws pib.
Txhob Cia Siab
Cov paroxysm ntawm hom hypokalemic tuaj yeem ua rau nws tus kheej hnov thaum sawv ntxov lossis hmo ntuj. Thaum sawv ntxov, ib tug neeg paub tias nws tuag tes tuag taw. Lub caj dab, caj dab, cov leeg - txhua yam yog txwv. Yog ibqhov mob hnyav heev, tus tuag tes tuag taw tuaj yeem cuam tshuam rau lub ntsej muag lossis cov leeg ua pa.
Lwm yam tshwm sim muaj xws li cov tsos mob hauv qab no:
- Sharp nqaij hypotension.
- Poob pob txha reflexes.
- Hyperemia ntawm lub ntsej muag.
- Tachycardia.
- Ua pa yooj yim.
- Polydipsia.
Nws yog ib qho tseem ceeb uas yuav tsum nco ntsoov tias qhov kev tawm tsam kuj nrog rau cov kab mob plawv. ECG nyeem hloov pauv, systolic yws yws tshwm sim. Tab sis kev nco qab nyob twj ywm.
Kev tawm tsam tuaj yeem nyob ntawm ib teev mus rau 1-2 hnub. Tab sis cov xwm txheej zoo li no tsis tshua muaj. Feem ntau nws ploj mus hauv 2-4 teev. Thaum kawg, muaj qhov nce qeeb hauv cov leeg nqaij, uas pib nrog cov extremities.
Nws tuaj yeem rov qab ua haujlwm sai sai yog tias tus neeg mob koom nrog qhov no nrog nws txoj haujlwm nquag.
qaug dab peg npaum li cas? Hauv qhov xwm txheej hnyav, lawv tshwm sim txhua hnub. Qhov no provokes cov tsos mob ntawm cov leeg tsis muaj zog. Qhov tshwm sim no feem ntau ua rau muaj kev noj zaub mov tsis txaus.
Hyperkalemic myoplegia
daim ntawv no tsis tshua muaj dua li saum toj no. Paroxysmal myoplegia ntawm hom no yog tus cwj pwm los ntawm autosomal dominant qub txeeg qub teg (siab penetrance). Interestingly, nyob rau hauv tej rooj plaub, no pathology tsis dhau nyob rau hauv tsev neeg rau plaub tiam.
Tus kab mob no cuam tshuam rau txiv neej thiab poj niam uas muaj zaus sib npaug. Tab sis qhov no paroxysmal myoplegia nyob rau hauv cov menyuam yaus feem ntau tshwm sim, nws xyaum tsis cuam tshuam rau cov neeg laus. Cov menyuam yaus muaj kev pheej hmoohnub nyoog qis dua 10.
Ua rau muaj kev tshaib plab lossis so tom qab lub cev ua haujlwm ntev. Ua ntej, paresthesia tshwm sim hauv thaj tsam ntawm lub ntsej muag, tom qab ntawd lawv tshwm sim hauv cov ceg. Tsis muaj zog kis mus rau lub distal ob txhais ceg thiab caj npab, thiab ces mus rau lwm cov leeg nqaij. Nyob rau hauv parallel, muaj tendon areflexia thiab hypotension. Cov tsos mob yog raws li nram no:
- Nyob hauv siab.
- Nyob nqhis dej.
- Hypertension.
- Autonomic disorders.
Raws li txoj cai, qhov kev tawm tsam no tsis kav ntev tshaj li ob teev. Nws yog ib qho tseem ceeb heev uas yuav tau muab kev pab raws sij hawm, vim hais tias vim li cas tshwm sim, ib tug neeg tej zaum yuav immobilized kiag li (qhov no yog hu ua plegia).
Normokalemic myoplegia
Qhov no yog ib qho tsis tshua muaj kab mob. Tus lej ntawm cov xwm txheej tau piav qhia hauv cov ntaub ntawv tuaj yeem suav rau ntawm tus ntiv tes. Paroxysmal myoplegia ntawm hom no muaj cov kab mob autosomal dominant xwm.
Nws muaj cov tsos mob hnyav. Tus kab mob tuaj yeem tshwm sim nws tus kheej li tsis muaj zog txaus lossis ua tiav tuag tes tuag taw cuam tshuam rau lub ntsej muag cov leeg. Hypertrophy tuaj yeem ua tau, nrog rau kev tsim ntawm lub cev kis las. Paroxysmal myoplegia tshwm sim hauv cov menyuam yaus, tshwm sim thaum thawj 10 xyoo ntawm lub neej.
Triggered, raws li txoj cai, los ntawm kev ua si lub cev hnyav lossis hypothermia. Nws kuj tuaj yeem tshwm sim vim kev pw tsaug zog ntau dhau. Manifested los ntawm ib tug qeeb nce nyob rau hauv tsis muaj zog. Cov kev xav no dhau mus qeeb dua.
Daim ntawv kab mob no kuj txawv ntawm nws lub sijhawm. Kev tawm tsam tuaj yeem dhau los ntawm 1-2 hnub, lossis ob peb lub lis piam.
Diagnosis
Nws tsis nthuav tawm ib qho teeb meem tshwj xeeb. Kev kuaj mob ntawm paroxysmal myoplegia tsis txawm xav tau kev ntsuas tshwj xeeb. Tus kab mob manifests nws tus kheej raws li ib tug tshwm sim ntawm qaug dab peg thiab tsis muaj lub paj hlwb kev ntshawv siab thaum lub sij hawm lub sij hawm ntawm "ceev". Ntxiv mus, yeej muaj keeb kwm tsev neeg.
Txawm li cas los xij, qee zaum muaj teeb meem los tsim kev kuaj mob. Lawv tshwm sim nrog qhov tshwm sim thawj zaug ntawm paroxysm, qhov tshwm sim ntawm kev tawm tsam, lossis hauv qhov xwm txheej tsis sib xws.
Qhov kev kuaj mob tau txheeb xyuas los ntawm tus kws tshuaj caj ces. Nws kuj tseem xav tau kev sab laj nrog tus kws kho mob endocrinologist thiab tus kws kho mob hlwb.
Ib ntus koj yuav tsum pub ntshav rau kev tshuaj xyuas biochemical. Nyob rau lub sijhawm ntawm kev tawm tsam, kev sib txawv tsis pom, tab sis nrog paroxysms, qhov txo qis ntawm cov poov tshuaj hauv cov ntshav tau pom - txog li 2 mEq / l thiab txawm tias qis dua. Kuj tseem muaj qhov txo qis ntawm phosphorus, nce suab thaj.
Tab sis qhov no yog nrog daim ntawv hyperkalemic ntawm paroxysmal myoplegia (ICD-10 code - E87.5). Nyob rau hauv cov ntaub ntawv ntawm hypokalemia, cov suab thaj qis thiab cov ntsiab lus potassium ntxiv tau raug kaw.
Qee zaus electromyography ua tiav. Nws nyob rau hauv txhua daim ntawv tso cai rau koj los xyuas qhov tsis muaj bioelectric kev ua haujlwm.
Nws tseem ua tau los qhia txog cov tshuaj potassium chloride rau ib tus neeg. Yog hais tias tom qab 20-40 feeb paroxysm pib tsim, ces tus neeg mob muaj ib tug hyperkalemic daim ntawv ntawm tus kab mob. Tau kawg, qhov kev daws teeb meem yog muab rau kev kuaj mob.
Nrog kev tawm tsam tsis tu ncuatuaj yeem ua cov nqaij ntshiv. Nws feem ntau pab txheeb xyuas qhov vacuolar daim ntawv ntawm pathology.
Vim li cas peb thiaj li xav tau kev ntsuas ntsuas rau paroxysmal myoplegia, cov tsos mob uas twb tau qhia tias nws muaj lawm? Txhawm rau kom paub qhov txawv ntawm tus kab mob ntawm hysteria, myopathy, Conn's disease, Landry's paralysis, nrog rau cov kab mob txha caj qaum.
Kho rau tus kab mob hypokalemic
Peb kuj yuav tsum tham txog kev kho mob ntawm paroxysmal myoplegia. Kev kho mob yog nqa tawm txawv, ntau nyob ntawm tus kab mob.
Yog tias, piv txwv li, tus kab mob muaj daim ntawv hypokalemic, ces qhov kev tawm tsam raug tshem tawm los ntawm kev noj 10% tshuaj ntawm potassium chloride. Koj yuav tsum siv nws txhua teev. Kev txhaj tshuaj magnesium thiab potassium aspartate tau pom zoo.
Thaum tus neeg qaug dab peg tsis cuam tshuam, nws yuav tsum tau noj cov tshuaj diuretic Spironolactone. Tab sis nrog kev saib xyuas zoo. Hauv cov poj niam, nws tuaj yeem ua rau hirsutism, feem ntau cuam tshuam rau kev coj khaub ncaws. Cov txiv neej feem ntau raug kev txom nyem los ntawm gynecomastia thiab impotence. Yog li, "Acetazolamide" dhau los ua lwm txoj hauv kev. Ntau tus kws tshaj lij xav txog Triamteren kom ua tau zoo.
Kev noj haus
Nws yog ib qho tseem ceeb heev uas yuav tsum ua raws li kev noj zaub mov kom raug. Yog tias ib hom hypokalemic ntawm paroxysmal myoplegia raug kuaj pom, nws yuav tsum tau txo qis cov carbohydrates thiab ntsev noj. Nws yog qhov zoo dua tsis kam lees qhov kawg tag nrho.
Nws tseem yuav tsum ua kom cov zaub mov muaj txiaj ntsig zoo nrog cov khoom noj uas muaj cov poov tshuaj txaus. Rau "cov ntaub ntawv tuav"Cov ntsiab lus ntawm cov khoom no suav nrog:
- qos.
- txiv lws suav qhuav.
- Ntaus.
- Prunes.
- Avocado.
- Salmon.
- Spinach.
- Pumpkin.
- txiv kab ntxwv.
Tea, soy, cocoa, nplej bran, ceev, noob, qej qus thiab qej, nplooj zaub, txiv tsawb, nceb thiab khoom noj siv mis kuj muaj cov poov tshuaj ntau.
Kev ua neej
Vim peb tab tom tham txog cov tsos mob thiab kev kho mob ntawm paroxysmal myoplegia ntawm hom hypokalemic, peb yuav tsum tau xyuam xim rau lub ncauj lus no.
Ntxiv rau kev noj zaub mov, nws tseem ceeb heev kom ua raws li kev ua neej nyob. Nws yog ib qho tsim nyog kom tsis suav nrog kev tawm dag zog lub cev hnyav thiab ib qho overvoltage. Tab sis ntev nyob rau hauv so yog contraindicated. Nws yog ib qho tseem ceeb los ua kom muaj kev sib haum xeeb. Koj tuaj yeem mus ncig ua si ib ntus mus rau lub pas dej lossis yav tsaus ntuj taug kev hauv huab cua ntshiab hauv koj qhov kev tswj hwm.
Nws tseem ceeb heev kom tsis txhob muaj cua sov thiab ua rau lub cev qis. Qhov kub thiab txias yuav tsum xis nyob.
Hyperkalemic kab mob kho
Kev tawm tsam ntawm tus kab mob no tau txo qis los ntawm kev txhaj tshuaj 40% qabzib nrog insulin lossis calcium chloride (10%).
Qee zaum muaj txiaj ntsig zoo tom qab nqus tau "Salbutamol". Nws ntseeg tau tias txoj kev no tsis yog tsuas yog txwv tsis pub qaug dab peg, tab sis kuj tiv thaiv lawv.
Thaum lub sijhawm tso cai, tus neeg mob yuav tsum noj Acetazolamide lossis Hydrochlorothiazide.
Kev noj haus
Nyob rau hauv hyperkalemic paroxysmal myoplegia, qhov ua rau thiab cov tsos mob uas tau teev tseg ua ntej, nws yog ib qho tsim nyog los txwv kev siv tag nrho cov khoom noj uas muaj cov poov tshuaj. Kev noj zaub mov zoo nrog carbohydrates thiab ntsev yog pom zoo. Feem ntau, cov kev cai yog txawv kiag li rau cov zaub mov qhia rau tus kab mob hypokalemic.
Tsis muaj teeb meem koj yuav tsum tso cai rau qhov kev tshaib plab tshwm sim, vim nws yog qhov tseeb uas ua rau qaug dab peg. Yog li ntawd nws yog ib qho tseem ceeb uas yuav tsum tau hloov mus rau cov khoom noj khoom haus fractional, noj tsawg kawg ib zaug txhua ob peb teev. Koj tsis tuaj yeem noj ntau dhau, koj tsuas yog yuav tsum tswj hwm lub xeev tag nrho.
Huab cua
Nws tsis tuaj yeem tsis lees paub tias tus kab mob hauv nqe lus nug cuam tshuam rau lub neej zoo thiab ua rau muaj teeb meem rau tus neeg mob. Tab sis nyob rau hauv dav dav, prognosis yog haum. Cov neeg tuag thaum lub sijhawm qaug dab peg yog qhov tsawg heev. Tsis tas li ntawd, ua raws li cov lus pom zoo kho mob thiab ua lub neej zoo, koj tuaj yeem nres lawv sai sai.
Thiab tau kawg qhov kev kho mob ntawm cov tsos mob ua rau tuag tes tuag taw yog qhov tseem ceeb. Nws koom nrog kev kho mob ntawm cov kab mob hauv qab, nrog rau kev kho kom zoo ntawm electrolyte tshuav.
