mwvave anTebiTi leikoencefalopaTiebis

diagnostikuri koliziebi

q. belTaZe, m. kiziria, al. ciskariZe, r. SaqariSvili

iv. javxiSvilis saxelobis Tbilisis saxelmwifo universiteti,

medicinis fakulteti, nevrologiisa da neiroqirurgiis kaTedra

centraluri nervuli sistemis paTologiaTa diagnostikis saqmeSi udavod didi roli Seasrula neirovizualizaciuri kvlevebis _ birTvul-mag­nitur-rezonansuli gamosaxvisa da kompiuteruli tomografiis danergvam. aRniSnuli meTodebis gamudmebiT ganviTarebisa da srulyofis pirobebSi farTovdeba neirodiagnostikis CarCoebi, muSavdeba sadiagnozo kriteriumebi, izrdeba daavadebaTa meqanizmebSi wvdomis SesaZleblobebi da aqedan gamomdinare ixveweba maTi sworad marTvis saSualebebi. yalibdeba axali diagnositikuri azrovneba; calkeuli konkretuli SemTxveva ganixileba specialistebis _ nevrologisa da radiologis erToblivi mWidro TanamSromlobis safuZvelze. Tavis mxriv neirovizualizaciur korelatebis variabelobis sazRvrebis gafarToeba arc Tu iSviaTad warmoSobs diagnostikur sirTuleebs, vinaidan arRvevs histomorfologiur verifikaciaze damyarebul tipiur klinikoanatomiuri kompleqsis harmonias da yvela konkretul SemTxvevaSi SeiZleba Sematos diagnostikur algoriTms manamde ararsebuli niuansi. aseT SemTxvevaSi Znelia daavadebisaTvis damaxasiaTebeli klinikuri niSnebis da neiorovizualizaciur monacemTa urTierTmisadageba. mocemul Sexedulebaze aqcentiT warmodgenil naSromSi ganixi­leba paTogenezurad homogenuri jgufis mwvave leikoencefalopaTiebis nairsaxeobis _ mwvave gafantuli encefalomielitis klinikuri SemT­xveva, romlis diagnostikuri algoriTmi awydeba rig diskusiur dabrkolebas.

mwvave gafantuli encefalomieliti (mgem) (post-, parainfeqciuri, postvaq­ci­naluri encefalomieliti) centraluri nervuli sistemis mwvave autoimu­nuri mademielinizebeli daavadebaa. igi aRmocendeba gadatanili viru­suli (iSviaTad mikoplazmuri an baqteriuli) infeqiis an imunizaciis Semdeg (12, 15). xSirad viTardeba virusuli infeqciebis (wiTela, yivanaxvela, Cutyva­vila, gripi, parotiti, an cofis, difteriis, wiTuras) sawinaaRmwego acris Semdeg (12, 5, 7); zogjer manifestirebuli winamorbedi daavadebis gareSe. rogorc wesi mimdinareoba monofazuria, iSviaTad progresirebadi. zogjer aRwerilia elvisebri, mZime mimdinareoba, letaluri gamosavliT.

morfologiurad mwvave fazaSi aRiniSneba tvinis SeSupeba, an makroskopuli cvlilebebi saerTod ar aris. mikroskopulad aRiniSneba perivaskuluri an periventrikuluri limfocituri infiltracia. demielinizacia ga­mo­xatulia wvrili da saSualo kalibris venebis irgvliv (13, 18). gamoxatulia agreTve oligodendrogliis degeneraciac. neirotropuli virusebiT gamow­veuli paTologiebisagan gansxvavebiT neironebi intaqturia. rogorc wesi gamomwvevis gamoyofa dazianebuli qsovilidan ar xerxdeba, umetes parainfeq­ciur nevrologiur garTulebas ganapirobebs imunuri meqanizmi.

mgem-is klinikuri suraTi mravalferovania. 90-96%-Si dominirebs encefa­litis suraTi, iSviaTad es ukanaskneli SeuRlebulia ganiv mielitTan, po­liradikulonevritTan, iSviaTad paTologia vlindeba optikomielitis sa­xiT (18, 9, 3).

Cveulebriv iwyeba cxelebiT, gamoxatuli zogadtvinovani niSnebiT (Tavis tkivili, Rebineba, cnobierebis Secvla, komatozuri mdgomareoba), meningialuri simptomebiT, arc Tu iSviaTad parcialuri an generalizebuli gul­y­riT. winamorbedi daavadebidan 3-15 dRis Semdeg viTardeba gaSlili klinikuri suraTi (12, 5, 20). diagnozi SedarebiT martivia e.w. naTeli periodis arsebobisas infeqciis gamovlenasa da nevrologiuri simptomatikis ganviTarebas Soris. kerovani simptomatika warmodgenilia pareziT, hipo-paresTeziiT, ataqsiiT da kranialur nervTa dazianebiT. Tavzurg tvinis siTxeSi Cve­uleb­­riv limfocituri pleocitozia cilis umniSvnelo momatebiT (20, 5, 7). aRdgena iwyeba ramdenime dReSi da grZeldeba kvireebis da Tveebis manZilze. 10-30%-Si aRwerilia letaluri gamosavali, 30-50%-Si sruli aRdgena, sxva SemTxvevaSi yalibdeba myari rezidualuri defeqti kognituri da moZ­ra­obiTi darRvevebi.

kompiuteruli tomografiiTa da birTvul-magnitur-rezonansuli gamo­saxviT vlindeba Tavis tvinis hemisferoebis TeTr nivTierebaSi, naTxemSi, xid­Si multifokaluri dazianebis kerebi, romelTa mxolod 25% kompiute­ruli tomografiisas irTavs sakon­t­rasto nivTierebas. kerebi ufro didi zomisaa da simetriuli, vidre gafantuli sklerozis dros (7, 1, 8, 19).

 

SemTxvevis aRwera

pacienti 25 wlis qali, pedagogi, 2002 wlis 20 marts moTavsda akad. p. sarajiSvilis sax. nevrologiisa da neiroqirurgiis institutSi CivilebiT: iol difuzur Tavis tkivilze, dabuJebasa da sisusteze marcxena xel-fexSi, barbacisa da wonasworobis darRvevis gamo siarulis gaZnelebaze, ylap­visa da metyvelebis gaZnelebaze.

daavadeba iwyeba 2002 wlis Tebervlis meore naxevarSi mwvave respiratoruli infeqciiT, romelic Tanxlebulia subfebrilitetiT, aRniSnulis gamo pacients daeniSna mkurnaloba antituberkulozuri preparatebiT: izoni­azidi, etambutoli, rifampicini. aTi dRis Semdeg mwvaved uviTardeba ioli kognituri darRvevebi, sisuste da dabuJeba marcxena xel-fexSi, wo­nas­worobis darRveva. ramdenime dReSi Seecvala metyveleba, gauWirda ylapva, metad myar sakvebze, gamoCnda Sardvaze imperatiuli moTxovnebi.

anamnezSi aRsaniSnavia ormxrivi qronikuli adneqsiti qlamidiur-triqo­mo­naduri infeqciis fonze.

obieqturi monacemebi: P _ 86, ritmuli, T/A 110/70 mmHg sv., sxeulis t _ 36,6C0. nevrologiuri statusi: cnobiereba naTeli, kontaqturia, orientirebu­lia drosa da sivrceSi, mcired akritikulia Tavisi mdgomareobis mimarT, Senelebulia, male ifiteba. mxedvelobiTi gnozisi darRveulia, dinamiur praqsisSi gaZnelebulia xelisaTvis Sesatyvisi sivrciTi mdebareobis moZebna, pozis praqsisi moSlilia, xanmokle verbalur-smeniTi mexsiere­bis mo­cu­loba Seviwroebulia, daswavlis procesSi aRiniSneba fsiqiuri aq­ti­vo­bis Zlieri meryeoba. azrovnebaSi gaZnelebulia mimarTebebis damyareba, mov­lenaTa Tanmimdevrobis dadgena.

gugebi mrgvali, Tanabari, fotoreaqciebi Senaxulia, Tvalis kaklebis SeuRlebuli moZraoba zemoT mciredSezRuduli, marjvniv cxvir-tuCis naoWis ioli gasadaveba, ylapvis da xaxis refleqsebi dabali, metyveleba dizarTriuli, swor xazze siaruli uWirs gamoxatul stato-dinamiuri ataqsiis gamo, gamoxatulia marcxenamxrivi hemiparezi, Zval-myes­Ta refleqsebi gacxovelebulia oTxive kidurSi, iwveva siWarbiT marcxniv, ormxriv babinskis niSani. marjvniv Tvalis kakalze zewola mtkivneulia, meningialuri niSnebi ar aris, sasis marcxena naxevarSi da marcxena kidurebSi zerele mgrZnobelobis ioli hipesTezia, marcxena zemo kidurSi vibraciuli mgrZnobeloba daqveiTebulia. mcire menjis disfunqcia, Sardvaze imperatiuli moTxovnis saxiT.

periferiul sisxlSi leikocitaruli formulis marcxniv gadaxra, hemoglobini  _ 136 g/l, eriTrocitebi _ 4,2.1012, feradobis maCvenebeli 0,9; Trombocitebi 77%, leikocitebi 5,2.103, metamielociti 1, CxirbirTviani 5, segmentbirTviani 66, eozinofili 1, limfociti 29, monociti 7, eds 6 mm/sT. sisxlis serologia epStein-baris virusisaTvis, citomegalovirusisTvis, toqsoplazmozisaTvis, boreliasaTvis, aiv antisxeulebisaTvis negatiuri iyo. Tav-zurg-tvinis siTxis analizi: ufero, gamWvirvale, citozi _ 0, cila _ 0,033‰, glukoza _ 2,6 mmol/l. zemoaRniSnuli virusebisaTvis virusnegatiuri. ofTalmoskopiurad – norma.

eeg-fia: gamovlinda neltalRovani dizritmia marjvena hemisferoSi, me­tad wina areebSi paroqsizmuli gantvirTvebiT maxvili talRebis da wa­max­vilebuli q potencialebis saxiT.

magnitur-rezonansuli kvleva (18.03.02): vlindeba Tavis tvinis didi hemisferoebis TeTri nivTierebis mravlobiTi kerovani da­zi­aneba. periventrikulur areSi sxvadasxva zomis kistozuri kerebi, araswori konturebiT, SigTavsi liqvorTan SedarebiT ramdenadme blantia. ga­mo­xa­tulia perifokaluri SeSupeba. 9 mm zomis kistozuri kera vlindeba mar­jvena SigniTa kafsulis ukana fexSi. infratentoriuli ubnebi intaqturia.

avadmyofs Cautarda mkurnaloba intravenurad 0,5 meTipredis infuzia 5 dRis ganmavlobaSi. mkurnalobis dawyebidan me-3 dRes mdgomareoba gaumjobesda, hemiparezi Semcirda, ataqsia naklebad iyo gamoxatuli, gaweris momentisaTvis 7 dRis Tavze ylapvisa da metyvelebis moSla srulad ukuganviTarda, dizuriuli movlenebi ar aris, rCeba ioli marcxenamxrivi hemiparezi, ioli ataqsia. avadmyofs ambulatoriul pirobebSi daeniSna 60 mg pred­nizoloni klebadi doziT per os. dinamikaSi ori Tvis Semdeg Catarebuli dakvirvebiT rCeba piramiduli simptomatika gacxovelebuli Zval-myes­Ta refleqsebis, ormxriv paTologiuri babinskis niSnebis saxiT. sxva ro­me­lime nevrologiuri darRveva ar dafiqsirda; xolo magnitur-rezonan­suli klveviT arsebuli kerebis raodenoba da zoma mniSvnelovnad Semcirda.

 

ganxilva

zemoaRwerili SemTxvevis klinikuri suraTi warmodgenilia mwvave respiratoruli infeqciis fonze ganviTarebuli mwvave nevrologiuri darR­veviT, rodesac zogadtvinovani simptomatologia minimaluria da vlindeba epizoduri xasiaTis difuzuri Tavis tkiviliT, meningialuri niSnebis da cnobierebis Secvlis gareSe. kerovani nevrologiuri darRvevebi multifokaluria da warmodgenilia: ioli kognituri disfunqciiT, marcxenamxrivi hemipareziT, ifsilateraluri hemihipesTeziiT, zomieri stato-dinamiuri ataq­siiT, sustad gamoxatuli bulbaruli simptomatikiT, ormxriv piramiduli niSnebiT, dizuriuli movlenebiT.

daavadebis mwvave periodSi Tavis tvinis neirovizualizaciiT gamovlinda uxeSi dazianebis suraTi (sur. 1). paTologiis mwvaved aRmocenebam da multifokaluri leikoencefalopaTiis suraTis arsebobam pirvel rigSi dabada eWvi iseTi mwvave anTebiTi leikoencefalopaTiebis arsebobaze, rogoricaa: mwvave gafantuli sklerozi, balos koncentruli sklerozi, mwvave gafantuli encefalomieliti, mwvave difuzuri hemoragiuli leikoencefaliti (veston-herstis daavadeba), agreTve sistemuri wiTeli mgluras, centraluri nervuli sistemis izolirebuli vaskulitis, kvanZovani poliarteritis, Segrenis sindromis, bexCetis davadebis, paraneoplastiuri procesiT ganpirobebuli leikoencefalopaTiis arsebobaze.

SemaerTebeli qsovilis sistemuri paTologiebisa da paraneoplastiuri encefalomielopaTiis gamoricxva SesaZlebeli gaxda TiToeuli maTganisaTvis specifikuri simptomokompleqsis, poliorganuli dazianebis, periferiul sisxlSi paTologiuri cvlilebebis ar arsebobis pirobebSi.

rac Seexeba balos koncentrul sklerozs da mwvave gafantuli sklerozis sxva variants _ marburgis daavadebas, isini mwvave, ganuwyvetliv prog­resirebad, fatalur mademielinizebel daavadebebs warmoadgenen, iSviaT SemTxvevebSi Zlieri imunosupresiuli Terapia iZleva xanmokle remisias (10).

mwvave hemoragiuli nekrozuli leikoencefaliti iSviaTi, mZime made­mi­elinizebeli daavadebaa, romelsac xSirad win uZRvis respiratoruli infeqcia (4,16). igi xSirad diagnozirdeba virusuli infeqciis sa­xiT. prognozi umTavresad arakeTilsaimedoa, magram aRwerilia, ramdenime SemTxveva sruli gamojanmrTelebiT, droulad Catarebuli kortikosteroidebis ga­m­o­­ye­nebiT, imunoglobulinebis, imunosupresiis da plazmaferezis gamo­ye­nebis fonze (2).

daavadebis debiutSi klinikurad Zalzed Znelia mwvave gafantuli encefalomielitis da gafantuli sklerozis gansxvaveba. mgem postinfeqciuri, an postvaqcinuri imunuri daavadebaa, swrafi monofazuri mimdinareobiT. neirovizualizaciiT gamovlenili demielinizaciis kerebi SedarebiT didi zomisaa, aqtiurad irTaven kontrasts. ganlagdebian uxSiresad simetriulad, ganmeorebiTi kvlevebisas ar aRiniSneba axali ubnebi, piriqiT Zveli ubnebi qreba an zomaSi mcirdeba (14, 7, 1).

warmodgenil SemTxvevaSi neirovizualizaciiT gamovlenil gavrcelebuli multifokaluri demielinizaciis kerebis arsebobam ganapiroba diferencialuri diagnostikis gatareba sxva mademielinizirebul daavadebebTan, rogoricaa: neiroboreliozi, adamianis T _ ujredovani limfotropuli virusi tipi 1 (HTLV-1)-iT gamowveuli centraluri nervulis sistemis paTo­lo­gia, adamianis imunodeficituri virusiT (HIV) ganpirobebuli leikoence­fa­lopaTia, neirosarkoidozi, vegeneris granulomatozi, iseTi daavadebebi, rogoricaa adrenoleikodistrofia (ald), metaq­ro­muli leikodistofia (mld), centraluri nervuli sistemis pirveladi limfomebi (11). progresirebadi multifokaluri leikoencefalopaTia, rome­lic ujreduli imunitetis depresiis gamomwvevi nebismieri daavadebis (kola­genozebi, Sidsi, limfoproliferacia, simsivneebi) dros SeiZleba ganviTardes.

im SemTxvevaSi rodesac saxezea monofazuri mimdinareobis mwvave nevrologiuri darRvevebi, Tanxlebuli multifokaluri leikoencefalopa­Tiis suraTiT, pirvel rigSi eWvi unda iqnes mitanili mgem-is arsebobaze. Semdgomi diagnostikuri Zieba unda warimarTos msgavsi neirovizualuri suraTis mqone daavadebaTa gamoricxvis gziT. swored amgvari diagnistikuri algoriTmis gamoyeneba gaxda safuZveli moyvanili SemTxvevis diagno­zirebisa. aRniSnuli SemTxvevis dRemde arsebuli ADEM-is samTviani katamnezi (Follow-up) jerjerobiT amyarebs amgvari midgomis siswores.

 

 

 

 

 

Bbibliografia

 

1. Caldemeyer KS, Smith RR, Harris TM, Edwards MK. MRI in acute disseminated encephalomyelitis. Neuroradiology 1994, 36: 216-220

2. Christopher J Klein, Eelco F.M. Wijdicks, Frank Earnest IV. Full recovery after acute hemorrhagic leukoencephalitis (Hurst’s disease). J Neurol. (2000) 247: 977-979

3. Hamaguchi k: Guillain – Barré syndrome and acute disseminates encephalomyelits. Rinsho Shinkeigaku 1966; 36: 1301-1307

4. Hurst AE (1941) Acute hemorrhagic leukoencephalitis: a previously undefined entity. Med J Aust 1:6

5. Johnson RT, Griffin DE, Gendelman HE. Postinfectious encephalomyelitis. Semin Neurol 1985; 5: 180-190

6. Johnson RT. The pathogenesis of acute viral encephalitis and postinfectious encephalomyelitis. J Infect Dis 1987; 155: 359-3664.

7. Kesselring I, Miller DH, Robb SA, Kendall BE, Mosely If, Kingslay D, Du GH, Mc Donald WI. Acute disseminated encephalomyelitis. MRI findings and distinction from multiple sclerosis Brain 1990; 113: 291-302

8. Kimura S, Nezu A, Ohtsuki N, Kobayashi T, Osaka H, Ueharas. Serial magnetic resonance imaging in children with postinfectious encephalitis. Brain Dev 1996; 18: 461-465

9. Kinoshita A, Hayashi M, Miyamoto K: Inflammatory demyelinating polyradiculitis in a patient with acute disseminated encephalomyelitis. J. Neurol Neurosurg Psychiatry 1996; 60: 87-90

10. Kuroiwa Y. Concentric sclerosis. In: Vinken PJ. Bruyn GW. Klawans Hl (eds). Handbook of Clinical Neurology, Amsterdam, 1985; 47: 409-417

11. Little N: Special neurologic problems. In: Emergency Medicine: Clinical Concepts and Practice, edt. 4. St Louis: Mosby Year Book; 1998: 2212-26

12. Murphy J, Austin J. Spontaneous infection or vaccination as a cause of acute disseminated encephalomyelitis. Neuroepidemiology 1985; 4: 138-145

13. Poser CM: Notes on the pathogenesis of subacute sclerosing panencephalitis. J. Neurol Sci 1990; 95: 219-224

14. Rolak LA: The diagnosis of multiple scelerosis. Neurol Clin. 1996 Febr. 14(1): 27-43

15. Saito H, Endo M, Takase S, Itahara K. Acute disseminated encephalomyelitis after influenza immunization. Arch Neurol 1980; 37: 564-566

16. Scully RE, Mark EI, Mc Neely WF, Ebeling Sh (1999) Weekly clinicopathological exercises. N  Engl J Med 340: 127-135

17. Shoji H, Kusuhara T, Honda Y, Hino H, Kojima K, Abe T, Watanabe M. Relapsing acute disseminated encephalomyelitis associated with chronic Epstein-Barre virus infection. MRI findings. Neuroradiology 1992; 34: 34-342

18. Tachi N, Watanabe T, Eakai S, Sato T, Shunzo C. Acute disseminated encephalomyelitis following HTLV-1 associated myelopathy. J neurol Sci 1992; 110: 234-235

19. Thajeb P, Chen St: Cranial computed tomography in acute disseminated encephalomyelitis. Neuroradiology 1989; 31: 8-12

20. Weinshenker BG, Miller D. Multiple sclerosis: one disease or many? In: Siva A, Kesselring J, Thompson AJ eds. Frontiers in multiple sclerosis. Martin Dunitz ltd, London, 1999; 37-46

 

 

Summary

Recent advances in neuroimaging have markedly expanded the spectrum of diseases, known as rarities in the nearest past among them, to be considered by the clinician in the process of diagnostic workup. The presented case warns practicing neurologist about potential diagnostic collisions appearing in case of either underestimation, overestivation or proper correlation of clinico-anamnestic and neuroimaging data.

suraTi 1. magnitur-rezonansuli kvleva 18.03.02
 

suraTi 2. magnitur-rezonansuli kvleva 23.05.02