Umntu oStiff Person Disorder
Isishwankathelo soNyango
I-Stiff Person Syndrome (i-SPS) yintlupheko enqabileyo yokunyakaza kwe-autoimmune echaphazela inkqubo ye-nervous central kwaye ibonakaliswe ngeklinikhi ngokuqina kwemisipha, i-spasms kunye nokunyuka kwe-startle response (impendulo yokuzikhusela engazi kakuhle) kwi-stimuli engalindelekanga.
I-SPS yeyeqela le-Stiff person syndrome spectrum disorders (SPSD) elabelana ngeempawu zeklinikhi ezifanayo kunye ne-spectrum ye-antibodies echasene neeprotheni ze-neuronal, ezithatha inxaxheba ekuthinteleni okuqhelekileyo kwe-hyperexcitability ephakathi echaza iimpawu zayo zekliniki. Eyona ndlela ichanekileyo ye-pathophysiology ayikaziwa.
Ukuqina kwezihlunu kudla ngokuqala ngokufihlakeleyo kumqolo osezantsi nasemilenzeni kwaye kunokulinganisa iintlungu ezisezantsi zomqolo, kodwa ekuhambeni kwexesha kudla ngokuqhubekela phambili ukuya kwamanye amalungu omzimba, ngakumbi kwiindawo ezikude kunye nezihlunu zesisu. Ngenxa yoko izigulana zibonakalisa "ukuqina" okanye "i-robotic" ye-gait ene-lumbar hyperlordosis exaggerated. Ukuqina ngokuqhelekileyo kusombulula ebuthongweni okanye phantsi kwe-anesthesia.
Jonga inkcazo epheleleyo
I-Muscle spasms kwi-SPS ibuhlungu kwaye iphakathi. Zenzeka ngokwesiqhelo ngenxa yovuselelo lwangaphandle olungalindelekanga, ikakhulu kwi-tactile, i-acoustic okanye i-emotional stimulus kwaye ihlala imizuzwana embalwa okanye imizuzu. I-spasms egqithisileyo inokukhokelela ekuqhekekeni okuzenzekelayo kunye nokuxhamla ngokubambisana okanye ukuwa okungalawulekiyo komntu ono "log-like" eqhelekileyo ewa phantsi.
Izigulana ezine-SPS zihlala zibonakalisa iimpawu ezingezizo iimoto ezifana nokuxhalaba, ukudakumba kunye nee-phobias ezikhethekileyo. Eyona nto ixhaphakileyo yi-agoraphobia, ngoloyiko lokuwa phantsi xa uhamba ungancediswanga. Kwakhona, kunokubakho iimpawu zexeshana ze-autonomic dysfunction ekhokelela ekubeni novelwano olune-diaphoresis, i-hyperpyrexia okanye ukungasebenzi kakuhle ngokwesondo.
Ngaphandle kwe-SPS yakudala kukho iindlela ezigxininise zesifo xa iimpawu zibekwe zodwa kumalungu omzimba omnye, umz. I-Stiff limb syndrome, okanye iifom ezinxulumene neempawu ezongezelelweyo zemithambo-luvo (umzekelo, ukuxhuzula, ataxia, polyneuropathy) kunye neentlobo ezigqibeleleyo kwaye ngamanye amaxesha ezibulalayo ezifana ne-Progressive encephalomyelopathy enokuqina kunye ne-myoclonus (PERM).
Ukuxilongwa kwe-SPS kusekelwe kwinkcazo yeklinikhi yayo. Ukufunyaniswa komgangatho ophakamileyo we-antibodies titres ngokuchasene ne-GAD, i-glycine, i-DPPX, i-GABARAP, i-gephyrin kunye ne-ampiphysine kwi-cerebrospinal fluid kunye negazi inokuba luncedo kodwa ayibalulekanga ekuxilongweni. Uphononongo lwe-EMG lubonisa amandla aqhubekayo eyunithi yemoto eqhubekayo (CMAP) kwimisipha echaphazelekayo. Izifundo ze-Electrophysiological ze-reflexes exteroceptive kunye ne-masseter inhibitory reflex engaqhelekanga kunye nokulahlekelwa kwecandelo le-S2, kunye ne-latency efinyeziweyo ye-R2 yokubuyisela kwi-Blink reflex icacisiwe ngakumbi.
I-MRI yobuchopho kunye nomqolo ziqhelekileyo kwaye zisetyenziselwa kuphela ukungabandakanyi ezinye izifo ezinokuthi zixilongwe.
I-SPS idla ngokudityaniswa nezinye izifo ezizimelayo, kuquka i-vitiligo, isifo seswekile kunye ne-thyroiditis. Ezinye iifom (ingakumbi ezinxulumene ne-anti-ampiphysin antibodies) zinokudityaniswa neoplasms.
Unyango lwe-SPSD lusekelwe kwi-triad yonyango lweempawu, unyango lwe-immunotherapy, kunye nokususwa kwethumba / unyango lwe-oncological apho kufanelekileyo.
Unyango olubonakalisa iimpawu lusekwe kumachiza anesenzo se-GABA-ergic. Nangona kunjalo, ngenxa yobume bayo obuzisebenzelayo, unyango olungundoqo lunyango lwe-immunotherapy olusebenzisa i-corticosteroids, i-intravenous immunoglobulins (IVIG) kunye/okanye ukutshintshiselana ngeplasma njengonyango lomgca wokuqala, iye yanyukela kunyango lwesibini lonyango lokugonyela umzimba kusetyenziswa umz. Unyango lwenkqubo ye-oncological esisiseko lubalulekile kwiimeko ezinombutho we-paraneoplastic.
I-prognosis yesi sifo iyahluka.
Ixhaswe nguKailash Bhatia, MD
Iziko leNeurology
iSebe leSobell
London, UK
Uhlaziyo luka-2019 oluxhaswe ngu:
Petra Dosekova, MD
IYunithi yeZiphazamiso zeMovement kunye neZiko leZiphazamiso zeMovement eziRare,
Isebe leNeurology
IYunivesithi yasePJ Safarik
eKosice, eSlovakia
Resources
Amanqaku ejenali yakutsha nje
Ithala leencwadi leevidiyo
Umxholo wamva nje
Izihloko ezishushu kunye neengxabano kwi-Stiff Person Spectrum Disorders (SPSD)
- Inqaku lewebhu
- Iziphazamiso zeMovement eziSebenzayo
- I-Stiff Person Syndrome (SPS)
Iikhosi ezinxulumene noko
Izixhobo zoLwazi
Ixesha leMbali ye-SPS
Jonga ubungakanani obugcweleyo
Jonga iimbekiselo
IZALATHISO
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SPS Social Media Graphics
Malunga ne-SPS
