Xanuunka Spectrum Person Stiff
Dulmarka Caafimaad
Stiff Person Syndrome (SPS) waa cudur naadir ah oo ku dhaca dhaqdhaqaaqa difaaca jirka kaas oo saameeya habdhiska dhexe ee neerfayaasha waxaana caafimaad ahaan lagu gartaa qallafsanaanta murqaha, bararka iyo jawaab celinta bilawga oo kordhay (jawaab difaac oo miyir la'aan ah) ee kicinta lama filaan ah.
SPS waxay ka tirsan tahay kooxda Stiff person syndrome spectrum disorders (SPSD) kuwaas oo wadaaga sifooyin bukaan-socod isku mid ah iyo unugyada difaaca jirka ee ka soo horjeeda borotiinada neerfaha, kuwaas oo ka qaybqaata xannibaadda caadiga ah ee hyperexcitability dhexe taasoo sharxaysa sifooyinkeeda caafimaad. Habka saxda ah ee pathophysiological weli lama yaqaan.
Qallafsanaanta murqaha waxay caadi ahaan si qarsoodi ah uga bilaabataa dhabarka hoose iyo lugaha waxayna la jaanqaadi kartaa xanuunka dhabarka hoose, laakiin waqti ka dib waxay u janjeertaa inay u gudubto qaybaha kale ee jirka, badiyaa inay kala fogaato qaybaha addimada iyo murqaha caloosha. Natiijo ahaan, bukaannadu waxay soo bandhigaan socodka caadiga ah ee "adag" ama "robotic" oo leh hyperlordosis lumbar oo la buunbuuniyey. Qallafsanaanta caadi ahaan waxay ku xalisaa hurdo ama suuxdin.
Eeg sharaxaad dhamaystiran
Muruqyada murqaha ee SPS waa kuwo xanuun badan oo kala go'a. Waxay caadi ahaan ku dhacaan natiijada kicinta dibadda ee lama filaanka ah, badiyaa inay taabato, dhawaaq ama kicin shucuureed waxayna badiyaa socdaan dhowr ilbiriqsi ama daqiiqado. Kala-baxyada aadka u daran waxay u horseedi karaan jabka iskiis ah iyo subluxations wadajirka ah ama dhicis aan la xakameynin ee qofka leh "log-like" caadiga ah oo hoos u dhacaya.
Bukaanka qaba SPS sidoo kale waxay badanaa muujiyaan astaamo aan dhaqdhaqaaq ahayn sida walaac, niyad-jab iyo cabsi shaqo oo gaar ah. Midda ugu badan waa agoraphobia, oo leh cabsi laga qabo inuu hoos u dhaco haddii aan la socon. Sidoo kale, waxaa jiri kara calaamado ku-meel-gaar ah oo ku-meel-gaar ah oo ku-meel-gaar ah oo ku-meel-gaar ah oo keenaya firfircooni-xumo-dareen leh oo leh diaphoresis, hyperpyrexia ama cillado galmo.
Marka laga reebo SPS qadiimiga ah waxaa jira noocyo kala duwan oo cudur ah marka astaamuhu ay go'doomiyaan hal qayb oo jidhka ah, tusaale ahaan Stiff limb syndrome, ama qaabab la xidhiidha astaamo dheeraad ah oo neerfaha ah (tusaale suuxdin, ataxia, polyneuropathy) iyo sidoo kale noocyo badan oo mararka qaarkood dilaa ah sida encephalomyelopathy Progressive encephalomyelopathy oo leh qallafsanaan iyo myoclonus (PERM).
Cilad-sheegidda SPS waxay ku salaysan tahay soo bandhigiddeeda caafimaad. Natiijooyinka unugyada difaaca jirka ee sarreeya ee ka soo horjeeda GAD, glycine, DPPX, GABARAP, gephyrin iyo ampiphysine ee dareeraha cerebrospinal iyo dhiigga ayaa laga yaabaa inay ku caawiyaan laakiin maaha kuwo muhiim u ah ogaanshaha. Daraasadaha EMG waxay muujinayaan awoodaha dhaqdhaqaaqa dhaqdhaqaaqa dhaqdhaqaaqa joogtada ah ee joogtada ah (CMAP) ee muruqyada ay saameeyeen. Daraasadaha elektrophysiological ee falcelinta exteroceptive oo leh falcelin aan caadi ahayn oo masseter inhibitory ah oo leh luminta qaybta S2, iyo daahitaanka soo kabashada R2 ee Blink reflex ayaa aad u gaar ah.
Maskaxda iyo laf dhabarta MRI waa caadi waxaana loo isticmaalaa oo kaliya in laga saaro cudurrada kale ee suurtogalka ah.
SPS waxay caadi ahaan la xiriirtaa cudurrada kale ee difaaca jirka, oo ay ku jiraan vitiligo, sonkorowga mellitus iyo thyroiditis. Foomamka qaarkood (badanaa la xidhiidha unugyada anti-ampiphysin) ayaa laga yaabaa inay la xiriiraan neoplasms.
Daawaynta SPSD waxay ku salaysan tahay saddex-geesoodka daaweynta calaamadaha, daawaynta difaaca jirka, iyo ka saarista burada/daawaynta kansarka marka ay ku habboon tahay.
Daawaynta calaamaduhu waxay ku salaysan tahay daroogooyinka leh ficilka GABA-ergic. Si kastaba ha ahaatee, dabiicadda difaaca jirka awgeed, daawaynta udub-dhexaadka ah waa immunotherapy iyadoo la adeegsanayo corticosteroids, xididada immunoglobulins (IVIG) iyo/ama isweydaarsiga balasmaha sida daawaynta safka kowaad, waxay u korodhay laynka labaad ee immunotherapy iyadoo la adeegsanayo tusaale ahaan cyklosporin, cyklofosfamid ama mycofenolat mofetil xaaladaha aan la jawaabin. Daawaynta habka oncological ee hoose ayaa muhiim u ah kiisaska leh ururka paraneoplastic.
Saadaasha cudurku waa doorsooma.
Waxaa wax ku darsaday Kailash Bhatia, MD
Machadka Neerfaha
Waaxda Sobell
London, UK
Cusbooneysiinta 2019 waxaa ku darsaday:
Petra Dosekova, MD
Unugga Dhibaatooyinka Dhaqdhaqaaqa iyo Xarunta Xanuunada Dhaqdhaqaaqa Naadirka ah,
Waaxda Neerfaha
Jaamacadda PJ Safarik
Kosice, Slovakia
Resources
Maqaalladii ugu dambeeyay ee Wargeysyada
Maktabadda Kiiska Fiidiyowga
Nuxurkii ugu dambeeyay
Mowduucyada kulul iyo khilaafyada ku saabsan Xanuunada Spectrum Person Stiff Person (SPSD)
- Maqaal shabakad
- Xanuunada Dhaqdhaqaaqa Dhaqdhaqaaqa
- Xanuunka Qofka Adag (SPS)
Koorsooyinka la xiriira
Qalabka Xogta
Jadwalka Taariikhda SPS
Eeg tixraacyada
tixraacyada
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Sawirada Warbaahinta Bulshada SPS
Ku saabsan SPS
