{"id":106598,"date":"2020-02-10T12:06:01","date_gmt":"2020-02-10T11:06:01","guid":{"rendered":"https:\/\/sdk.mk\/?p=106598"},"modified":"2020-02-16T08:47:38","modified_gmt":"2020-02-16T07:47:38","slug":"ju-njoftojme-me-semundjet-e-rralla-encefalomieliti-progresiv-me-ngurtesi-dhe-mioklonus","status":"publish","type":"post","link":"https:\/\/sdk.mk\/index.php\/uncategorized-sq\/ju-njoftojme-me-semundjet-e-rralla-encefalomieliti-progresiv-me-ngurtesi-dhe-mioklonus\/","title":{"rendered":"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: ENCEFALOMIELITI PROGRESIV ME NGURT\u00cbSI DHE MIOKLONUS"},"content":{"rendered":"<div class=\"f52428582a4b906b2d2e2a7d733c7d27\" data-index=\"7\" style=\"float: none; margin:10px 0 10px 0; text-align:center;\">\n<!-- MSP Ads Asynchronous JS Tag - Generated with Revive Adserver v5.4.1 -->\r\n<ins data-revive-zoneid=\"167\" data-revive-id=\"060fdb01f6430ec164d3519234aa4967\"><\/ins>\r\n<script async src=\"\/\/panel.ads.com.mk\/www\/delivery\/asyncjs.php\"><\/script>\n<\/div>\n<p><em><strong><a href=\"https:\/\/www.youtube.com\/watch?v=iUo0DHx4RU0&amp;list=PLPGcigDEy-qj0I1um2ByEqy4a1IdVRjzf&amp;index=11&amp;t=0s\" target=\"_blank\" rel=\"noopener noreferrer\">Encefalomieliti progresiv me ngurt\u00ebsi dhe mioklonus (EPNM)<\/a><\/strong><\/em><\/p>\n<p>MKB G04.8<br \/>\nProgressive encephalomyelitis with rigidity and myoconus<br \/>\nNocion<br \/>\nEncefalomieliti progresiv me ngurt\u00ebsi dhe mioklonus \u00ebsht\u00eb i rralll\u00eb dhe nj\u00eb sindrom i r\u00ebnd\u00eb neurologjik q\u00eb ve\u00e7ohet me ngurt\u00ebsi muskulore, spaz\u00ebm, ng\u00ebr\u00e7e si dhe mosfunksionim t\u00eb trungut truror dhe sistemit nervor autonom vegjetativ.<\/p>\n<p>Simptome<br \/>\nMosfunksionimi autonom vegjetativ, ng\u00ebr\u00e7e t\u00eb dhimbshme, hiperrefleksi, probleme me frym\u00ebmarrjen, amnezi, diskinezi (aktivitet i pavullnetsh\u00ebm i muskujve i cili mund t\u00eb paraqitet n\u00eb form\u00ebn e tikave t\u00eb zakonshme ose l\u00ebvizjeve stereotipe). Simptomet paraqiten p\u00ebr shkak t\u00eb d\u00ebmtimit t\u00eb trasmetimit t\u00eb glicin\u00ebs ( glicina \u00ebsht\u00eb neurotransmetues n\u00eb sistemin nervor q\u00ebndror) n\u00eb sinapsat, ve\u00e7an\u00ebrisht n\u00eb palc\u00ebn kurrizore dhe shtyll\u00ebn kurrizore.<br \/>\nEpidemiologjia<br \/>\nEPNM klasike paraqitet te grat\u00eb dy deri tre her\u00eb m\u00eb shum\u00eb se sa te meshkujt. T\u00eb propozuara jan\u00eb disa ndarje t\u00eb EPNM sipas r\u00ebnd\u00ebsis\u00eb ose shp\u00ebrndarjes s\u00eb ngurt\u00ebsis\u00eb dhe paraqitjeve neuologjike shoq\u00ebruese.<br \/>\nPatogjeneza<br \/>\nNj\u00eb hap i madh p\u00ebr kuptimin e patogjenez\u00ebs s\u00eb EPNM \u00ebsht\u00eb b\u00ebr\u00eb n\u00eb vitin 1988, kur lidhja midis anti-GAD antitrupthave dhe EPNM \u00ebsht\u00eb studjuar p\u00ebr her\u00eb t\u00eb par\u00eb. Antitrupthat kund\u00ebr GAD (glutamat dekarboksilaza) jan\u00eb pozitive n\u00eb 80% t\u00eb pacient\u00ebve me EPNM. Antitrupthat e tjer\u00eb, si N-metil-D-aspartat (NMDA) antitrupthat jan\u00eb t\u00eb lidhura me n\u00ebnnj\u00ebsi t\u00eb ndryshme t\u00eb receptorit NMDA. T\u00eb paraqitura jan\u00eb ndryshime edhe n\u00eb pjes\u00eb t\u00eb tjera t\u00eb trurit si\u00e7 \u00ebsht\u00eb korpus kalosum ose trungu truror. M\u00eb shum\u00eb se gjysma e pacient\u00ebve kan\u00eb tumor shoq\u00ebrues, zakonisht teratom t\u00eb vezor\u00ebve i cili mund t\u00eb ngat\u00ebrohet me cist\u00eb beninje. Zbulimi i tumorit \u00ebsht\u00eb i rrall\u00eb te meshkujt me EPNM. Tumor\u00eb t\u00eb tjer\u00eb n\u00eb raste t\u00eb rralla jan\u00eb teratom i mediastinumit, kancer i mushk\u00ebrive, limfoma Ho\u00e7kin, neuroblastoma, kancer i gjirit dhe tumori i testikujve. Anti-LGI1 antitrupthat te kjo s\u00ebmundje jan\u00eb shkak p\u00ebr p\u00ebrkeq\u00ebsimin e kujtes\u00ebs, sulmeve epileptike, \u00e7rregullimeve mendore dhe hiponatremis\u00eb. Hiponatremia \u00ebsht\u00eb nj\u00eb tipar karakteristik i anti-LGI1 AE, kurse 60% deri 80% t\u00eb pacient\u00ebve t\u00eb till\u00eb kan\u00eb hiponatremi rezistente. Mekanizma patogjenike, me shum\u00eb mund\u00ebsi, \u00ebsht\u00eb e lidhur me sindromin e tajitjes s\u00eb pap\u00ebrshtatshme t\u00eb hormonit antidiuretik. Kombinimi i antitrupthave t\u00eb cilat jan\u00eb t\u00eb pranish\u00ebm tek kjo s\u00ebmundje mendohet q\u00eb mund t\u00eb jet\u00eb p\u00ebrgjegj\u00ebs p\u00ebr karakterin progresiv t\u00eb saj.<\/p>\n<p>Diagnoza<br \/>\nR\u00ebnd\u00eb \u00ebsht\u00eb t\u00eb konstatohet diagnoza e EPNM. EMG, antitrupthat n\u00eb serum dhe l\u00ebngu truroshpinor\u00eb mund t\u00eb ndihmojn\u00eb ne konfirmimin e dijagnoz\u00ebs. Paraqitja klinike fillestare mund t\u00eb jet\u00eb jospecifike dhe e pazakonshme. Dallohet edhe nivel i lart\u00eb i D-dimer\u00ebve. Kjo e shpjegon edhe rezikun e mundsh\u00ebm p\u00ebr trombemboli. Diagnoza diferenciale t\u00eb mundshme t\u00eb EPNM jan\u00eb sindroma paraneoplastike dhe s\u00ebmundje t\u00eb tjera neuromuskulore.<\/p>\n<p>Terapia<br \/>\nImunoterapia, plazmafereza, imunoglobulinat intravenoz (IVIG), kortikosteroidat dhe rituksimab jan\u00eb t\u00eb paraqitur si t\u00eb suksessh\u00ebm n\u00eb raste individuale, edhe pse efikasiteti i k\u00ebtyre agjent\u00ebve nuk \u00ebsht\u00eb i konstatuar. P\u00ebrve\u00e7 k\u00ebsaj, disa raporte e paraqesin efikasitetin e trajtimit afatgjat\u00eb me azatioprin. Sidoqoft\u00eb, EPNM mbetet nje entitet klinik i cili vazhdimisht pasurohet me simptoma dhe antitruptha t\u00eb rinj. Ciklet e plazmaferez\u00ebs dhe IVIG nuk paraqesin p\u00ebrparim t\u00eb rendesish\u00ebm terapeutik.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Encefalomieliti progresiv me ngurt\u00ebsi dhe mioklonus (EPNM) MKB G04.8 Progressive encephalomyelitis with rigidity and myoconus Nocion Encefalomieliti progresiv me ngurt\u00ebsi [&hellip;]<\/p>\n","protected":false},"author":24,"featured_media":106596,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_mo_disable_npp":"","inline_featured_image":false},"categories":[1794],"tags":[],"imageURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2020\/02\/10-Progressive-encephalomyelitis-with-rigidity-and-myoclonus-PERM-650x366.png","thumbURL":"https:\/\/sdk.mk\/wp-content\/uploads\/2020\/02\/10-Progressive-encephalomyelitis-with-rigidity-and-myoclonus-PERM-250x141.png","categoryNames":["Uncategorized"],"feature_caption":"","subtitle":"","views":"1472","source":"\u0421\u043a\u043e\u043f\u0458\u0435, (\u0421\u0410\u041a\u0410\u041c\u0414\u0410\u041a\u0410\u0416\u0410\u041c.\u041c\u041a)","yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v15.0 - 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