Qhov sib txawv tseem ceeb ntawm cov kab mob neuron lub cev muaj zog thiab cov leeg nqaij dystrophy yog cov kab mob neuron lub cev muaj zog yog ib pawg ntawm cov teeb meem tsis tshua muaj tshwm sim tshwj xeeb vim muaj teeb meem hauv nruab nrab lossis lub paj hlwb peripheral, thaum cov leeg nqaij dystrophy yog ib pawg ntawm cov kab mob tsis tshua muaj. tshwm sim tshwj xeeb vim muaj teeb meem hauv cov leeg.
Tus kab mob neuromuscular yog ib yam kab mob uas cuam tshuam rau lub paj hlwb peripheral, neuromuscular junction, lossis cov leeg pob txha. Tag nrho cov khoom no yog ib feem ntawm lub cev muaj zog. Cov kab mob neuron lub cev muaj zog thiab cov leeg nqaij dystrophy yog ob hom kab mob neuromuscular.
Tus Kab Mob Neuron yog dab tsi?
motor neuron kab mob yog ib pawg ntawm cov kab mob tsis tshua muaj neurodegenerative uas tshwj xeeb cuam tshuam rau lub cev muaj zog neurons uas tswj cov leeg leeg ntawm lub cev. Cov pab pawg no suav nrog ntau yam kab mob xws li amyotrophic lateral sclerosis (ALS), kev mob bulbar palsy (PBP), pseudobulbar palsy, cov leeg nqaij atrophy (PMA), thawj lateral sclerosis (PLS), spinal muscular atrophy (SMA), monomelic amyotrophy (MMA) thiab qee qhov txawv txav tsis tshua zoo ib yam li ALS. Cov kab mob neuron lub cev muaj zog feem ntau cuam tshuam rau cov menyuam yaus thiab cov neeg laus. Feem ntau ntawm cov kab mob neuron lub cev muaj zog yog qhov tsis sib xws, thiab lawv cov laj thawj feem ntau tsis paub. Nws ntseeg tau hais tias ib puag ncig, tshuaj lom, kab mob, lossis cov caj ces muaj feem cuam tshuam nrog cov xwm txheej no. Qee hom kab mob hauv lub cev muaj zog tau txais cov hauv paus caj ces (xws li SODI gene).
Daim duab 01: Amyotrophic Lateral Sclerosis (ALS)
Cov cim qhia thiab cov tsos mob tuaj yeem suav nrog qhov tsis muaj zog ntawm kev tuav, qaug zog, cov leeg pob txha, spasms, twitches, hais lus slurred, tsis muaj zog ntawm caj npab los yog txhais ceg, clumsiness thiab stumbling, nyuaj nqos, teeb meem ua pa los yog ua tsis taus pa, lub siab tsis zoo. cov lus teb, poob phaus, cov nqaij ntshiv, tsis yooj yim txav, mob pob qij txha, drooling, tsis muaj zog yawning, hloov ntawm tus cwj pwm, thiab lub siab lub ntsws. Cov mob no tuaj yeem kuaj tau los ntawm kev kuaj ntshav thiab zis, MRI lub hlwb scans, electromyography (EMG) thiab kev tshawb fawb paj hlwb (NCS), lumbar puncture, thiab nqaij leeg nqaij. Tsis tas li ntawd, kev kho mob rau cov kab mob hauv lub cev muaj zog tuaj yeem suav nrog kev kho mob ua haujlwm, kho lub cev, kev hais lus thiab kev kho lus, cov lus qhia txog kev noj zaub mov thiab noj, tshuaj xws li riluzole, edaravone, nusinersen, onasemnogene, abeparvovec uas ua rau qeeb zuj zus ntawm tus mob no, tshuaj los txo. Cov leeg nqaij tawv (Botox) thiab pab nrog cov teeb meem qaub ncaug, tshuaj tua kab mob (ibuprofen), thiab txhawb kev xav.
Dab tsi yog Muscular Dystrophy?
Muscular dystrophy yog ib hom kab mob thiab kho mob heterogeneous pab pawg ntawm cov kab mob neuromuscular uas ua rau muaj zog zuj zus thiab ua rau cov leeg pob txha tawg. Muscular dystrophy kuj yog ib pab pawg ntawm cov kab mob uas tau tshwm sim los ntawm kev nkim ntawm cov leeg nqaij nrog lossis tsis muaj kev puas tsuaj ntawm cov paj hlwb. Ntau tshaj 30 qhov sib txawv muaj nyob rau hauv cov leeg nqaij dystrophy. Ntawm cov ntawd, Duchene muscular dystrophy (DMD) suav txog 50% ntawm cov neeg mob, uas cuam tshuam rau cov txiv neej pib thaum muaj hnub nyoog plaub xyoos. Lwm cov leeg nqaij dystrophies muaj xws li Becker nqaij dystrophy, facioscapulohumeral muscular dystrophy, myotonic dystrophy, limb-girdle muscular dystrophy, thiab congenital muscular dystrophy.
Daim duab 02: Muscular Dystrophy
Cov cim qhia thiab cov tsos mob ntawm cov leeg nqaij dystrophies muaj xws li cov nqaij ntshiv loj zuj zus, tsis muaj kev sib npaug, scoliosis, tsis muaj peev xwm taug kev, taug kev, calf deformation, txwv tsis pub txav mus los, ua pa nyuaj, cardiomyopathy, leeg nqaij, thiab Gowers' kos npe.. Ntxiv mus, feem ntau ntawm cov leeg nqaij dystrophies tau txais txiaj ntsig (DMD gene, DYSF gene), uas ua raws cov qauv sib txawv (X-txuas, autosomal recessive, autosomal dominant). Qee qhov me me, tej zaum lawv yuav tshwm sim los ntawm kev hloov pauv ntawm tus kheej de novo.
Cov leeg nqaij dystrophies tuaj yeem kuaj tau los ntawm kev kuaj ntshav thiab kuaj caj ces. Tsis tas li ntawd, kev kho mob rau cov leeg nqaij dystrophies yog cov tshuaj uas hais txog cov hauv paus hauv paus, suav nrog kev kho noob caj noob ces (Microdystrophin), tshuaj antisense (Ataluren, Eteplirsen thiab lwm yam), siv corticosteroids (Deflazacort), thiab calcium channel blockers (Diltiazem) kom qeeb pob txha thiab mob plawv. cov leeg nqaij degeneration, siv cov tshuaj tiv thaiv kab mob los tswj kev qaug dab peg, siv cov tshuaj tiv thaiv kab mob (Vamorolone) kom ncua kev puas tsuaj rau cov leeg nqaij tuag, kev kho lub cev, kho kev phais, thiab pab ua pa.
Dab tsi zoo sib xws ntawm Motor Neuron Disease thiab Muscular Dystrophy?
- motor neuron kab mob thiab muscular dystrophy yog ob hom kab mob neuromuscular.
- Ob qho kev kho mob muaj xws li pab pawg neeg mob.
- Lawv tuaj yeem cuam tshuam rau cov leeg mob siab.
- Qhov ua rau ntawm ob qho kev mob nkeeg yuav tshwm sim los yog muaj keeb kwm.
- Cov menyuam yaus thiab cov neeg laus raug cuam tshuam los ntawm ob qho kev kho mob.
- Lawv tsis tshua muaj kev kho mob.
- Lawv tau kho los ntawm cov tshuaj tshwj xeeb thiab kev txhawb nqa kev kho mob.
Qhov txawv ntawm Motor Neuron Disease thiab Muscular Dystrophy yog dab tsi?
motor neuron kab mob yog ib pawg ntawm cov teeb meem tsis tshua muaj tshwm sim tshwj xeeb vim muaj teeb meem hauv nruab nrab lossis peripheral paj hlwb, thaum cov leeg nqaij dystrophy yog ib pawg ntawm cov kab mob tsis tshua muaj tshwm sim tshwj xeeb vim muaj teeb meem hauv cov leeg. Yog li, qhov no yog qhov sib txawv tseem ceeb ntawm lub cev muaj zog neuron kab mob thiab cov leeg nqaij dystrophy. Tsis tas li ntawd, cov kab mob neuron lub cev muaj zog yog ib pab pawg uas muaj xya yam kab mob sib txawv, thaum cov leeg nqaij dystrophy yog ib pab pawg uas muaj peb caug qhov sib txawv.
Cov hauv qab no qhia txog qhov sib txawv ntawm cov kab mob neuron lub cev muaj zog thiab cov leeg nqaij dystrophy hauv daim ntawv tabular rau ib sab ntawm kev sib piv.
Summary – Motor Neuron Disease vs Muscular Dystrophy
motor neuron kab mob thiab cov leeg nqaij dystrophy yog ob hom kab mob neuromuscular. Mob neuron lub cev muaj zog tshwm sim vim muaj teeb meem hauv nruab nrab lossis peripheral paj hlwb. Muscular dystrophy tshwm sim tshwj xeeb vim muaj teeb meem hauv cov leeg. Yog li, qhov no qhia txog qhov sib txawv ntawm lub cev muaj zog neuron kab mob thiab cov leeg nqaij dystrophy.
