ნარკოლეფსია, როგორც აფექტური სპექტრის დაავადება (საქართველოს ფსიქიატრიის მოამბე #7)

narkolefsia, rogorc afeqturi speqtris daavadeba

irakli giorgobiani, elene kavsaZe, merab arvelaZe, narkologiis s/k instituti, Tbilisi, saqarTvelo

   narkolefsiuri sindromis, rogorc Zilis aSlilobis erT erTi formis klinikuri ganmarteba ekuTvnis gamoCenil germanel fsiqiatrs da nevrologs vestfals (1977), magram termini narkolefsia da misi sruli klinikuri suraTi aRwera frangma fsiqiatrma Jelinom 1880 wels, ris gamoc adreul periodSi mas “Jelinos daavadebasac” uwodebdnen.

   narkolefsiisaTvis damaxasiaTeblad iTvleba simptomuri tetrada: 1. Zilis xanmokle Setevebi; 2. katalefsia; 3. Zilis dambla da 4. hipnogogiuri halucinaciebi. aRniSnuli oTxi simptomidan yvela maTganis erTdrouli arseboba aucilebeli ar aris, klinikur suraTSi xan erTi simptomia wamyvani da xan meore.

   daavadebis manifestacia xdeba swrafad da uecrad, umetes SemTxvevaSi dRis  saaTebSi Zilis SetevebiT. garkveuli periodis Semdeg Zilis Setevas emateba sxeulis kunTovani sistemis tonusis nawilobrivi an mTliani daknineba (katalefsia). mTel rig SemTxvevebSi Zilis Setevis dawyebas win uswrebs ramdenime saaTiT mTel organizmSi sisustis, moqancvis da Tavis tkivilis SegrZneba. amave periodSi SesaZlebelia adgili hqondes garkveul winaswarSegrZnebas, e.w. auras, rac gamoixateba sxvadasxva paresTeziebSi Sexurebis, Tavbrusxvevis da tkivilis SegrZnebaSi epigastralur areSi. Zilis Seteva ufro xSirad manifestirdeba mosvenebul mdgomareobaSi an monotonuri moqmedebis procesSi dRis garkveul monakveTSi, zogjer erTsa da imave saaTebSi.

    Zilis Seteva SeiZleba daiwyos ara marto mSvid, aramed  sxvadasxva situaciaSi, zogjer sicocxlisaTvis saSiS momentSic, magram rogorc wesi ar arRvevs motoruli aqtis Sesrulebis SesaZleblobas. mag.: avadmyofma SeiZleba CaiZinos fexze mdgomma da Zilis dros ar waiqces; Tu Seteva daiwyo siarulis dros - gaagrZelos siaruli;  Tu xelSi uWiravs raime sagani - ar dauvardeba da a.S. Tu Setevas win uswrebs e.w. aura, avadmyofi aswrebs sagnis dadebas, dajdomas an dawolas. Setevis procesSi avadmyofis saerTo mdgomareoba bevrad ar gansxvavdeba janmrTeli adamianis Zilis mdgomareobisgan: kunTovani sistema moSvebulia, sunTqva wynaria da riTmuli. Seteva grZeldeba 1 an ramdenime wuTs da Zlier iSviaTad ufro xangrZlivad. dRis ganmavlobaSi Setevebi SeiZleba ganmeordes 2-100-mde da metjer, saualod 3-5-jer. Zilis Setevis Sewyveta SesaZlebelia xelovnurad gaRviZebiT, ris Semdegac avadmyofi agrZelebs Cveul saqmianobas. Zogjer Setevis xelovnur Sewyvetas Tan sdevs avadmyofis ukmayofileba, gaRizianeba, gabrazeba da gonebis gafantuloba.

   Setevidan spontanurad gamosvlis Semdeg, ara iSviaTad viTardeba e.w. “Zilis dambla”, ra drosac avadmyofs ar ZaluZs xelis ganZreva, gadabruneba loginSi da sxva umartivesi moZraobis Sesruleba. mimika daZabuli aqvs, SiSisa da SfoTvis gamomxatveli, rasac erTvis koSmaruli da mtanjveli gancdebi, eufleba sikvdilis SiSi, romlis gadmocemac ar SeuZlia. xSirad am mdgomareobas Tan erTvis feradi, xatovani, usiamovno Sinaarsis koSmaruli da Semzaravi halucinaciebi. Setevis gareSec, Ramis saaTebSi, imisda mixedviT, Tu ra xarisxiT aqvs darRveuli fiziologiuri Zili, pacientebs aReniSnebaT hipnogogiuri halucinaciebi.

   Ddadgenilia, rom Ramis Zili ar warmoadgens erTian da myar mdgomareobas. fiziologiur ZilSi gamoyofen e.w. “nel” da swraf” (paradoqsul) Zilis fazebs. aRniSnuli fazebi Cveulebrivi Zilis drosac erTmaneTs enacvlebian cikluri TanmimdevrobiT, rac sakmaod zustad aris gamoTvlili da regulirebadi Tavis tvinis erT erTi urTulesi funqciebis matarebeli – hipoTalamusis Sesaferis struqturebis mier. gansakuTrebuli TaviseburebiT xasiaTdeba narkolefsiuri Zili, romlis ganviTarebaSic, avtorTa umravlesobis azriT (45, 46), gansakuTrebuli mniSvneloba eniWeba e.w. “swrafi” Zilis fazis ganviTarebas. aRmoCnda, rom narkolefsiis dros, rogorc RamiT, ise dRisiT, CaZinebis procesi metad xanmoklea (ramdenime wuTi), rac SeiZleba SevatyoT pacients Tvalis kaklebis moZraobiT, kunTovani tonusis daqveiTebiT da eleqtroencefalografiuli gamokvlevis Sedegad - desinqronizaciiT. Aam monacemebma misca biZgi koncefcias, rom narkolefsia aris daavadebis forma, romelic xasiaTdeba “swrafi” Zilis araadeqvaturi, paroqsizmuli CarTvebiT. SemdgomSi es koncefcia mravalma avtorma ar gaiziara, radgan Tvlidnen rom gacilebiT ufro rTul meqanizmebTan unda gvqondes saqme.

   Nnarkolefsiis Setevebi iwyeba upiratesad mamakacebSi, axalgazrda asakSi 20-dan 40 wlamde, xolo qalebSi 20 wlis asakamde. 40 wlis Semdeg dawyebuli narkolefsia kazuisturad CaiTvleba. Setevebs Tan axlavs sxvadasxvagvari neirovegetatiuri aSlilobani vagotonuri simptomebis upiratesobiT. xSirad adgili aqvs nivTierebaTa cvlis aSlilobas, romelic vlindeba Warb simsuqneSi. araiSviaTia am dros endokrinuli jirkvlebis funqciis moSlac, rac gamoixateba: fariseburi jirkvlis gadidebiT, sasqeso organoebis da maTi funqciebis ganuviTareblobiT, akromegaliiT, Tirkmelzeda jirkvlis da hipofizis funqciebis gaZlierebiT, Zlieri wyurviliT, bulemiiT, amenoreiT, da a.S. rentgenologiurad dadgenilia Turquli kexis SefardebiTi simcire. zogi avtoris monacemebiT (10), narkolefsias axasiaTebs: Tvalis mamoZravebeli nervebis dazianebis niSnebiT, parkinsonizmi da piramiduli aSlilobani. bolo wlebis eleqtroencefalografiuli gamokvlevebis mixedviT dadgenilia, rom narkolefsiisaTvis damaxasiaTebelia maRaltalRovani neli talRebi (1 ryeva wamSi).

    narkolefsiis dros Ramis Zilis ciklis aSla ganapirobebs pirovnebis struqturuli maxasiaTeblebis iseT Secvlas, romelic mogvagonebs endokrinul fsiqosindroms. narkolefsiis dros pirovnuli cvlilebebi vlindeba fsiqikuri aqtivobis da miswrafebebis daqveiTebaSi, interesebis wris SeviwrovebaSi, advilad daRlilobaSi, gamRizianeblobasa da afeqturi aSlis naTlad gamoxatul niSnebSi rogoricaa: depresia, cremlianoba, ukmayofileba, sevdianoba, xSiria suiciduri azrebi da moqmedebani.

    esenciuri narkolefsiis garda, arsebobs e.w. simtomuri narkolefsiac anu narkolefsiuri sindromi, romelic SeiZleba ganviTardes centraluri nervuli sistemis organuli an zogierTi funqciuri dazianebis SemTxvevaSi, rogoricaa: Tavis tvinis simsivne, Tavis qalas travma, epidemiuri encefaliti, intoqsikaciebi, epilefsia, Sizofrenia da sxva. am SemTxvevebSi narkolefsiur da katalefsiur sindromebTan erTad gamovlenili pirovnebis Secvlis niSnebi garkveulad Seesabamebian ZiriTadi daavadebisaTvis damaxasiaTebel gamovlinebebs. narkolefsiuri Setevebi, pirvel rigSi saWiroeben diferencirebas epilefsiis mcire gulyrebisgan, e.w. piknolefsiisgan da isTeriuli xasiaTis Zilis darRvevebisgan. gansxvaveba imaSia, rom katalefsiuri Setevebi ufro xSirad motorul sferoSi gamovlindeba da Tan ar axlavs cnobierebis epilefsiiseburi Secvlis niSnebi, rac Seexeba diferencirebas isTeriasTan, isTeriuli paroqsizmi yovelTvis fsiqogenuria da droSi gacilebiT gaxangrZlivebulia.

    narkolefsiis pirdapiri eTiologia dRemde dauzustebelia. varaudoben, rom arsebobs misi gamomwvevi sxvadasxva mizezebi: infeqciebi (tifi, malaria, wiTela da sxva), endokrinuli aSlilobani. saTanado mniSvneloba aqvs genetikur faqtorsac, anu narkolefsia viTardeba iq sadac arsebobs genetikuri, an egzogenurad ganpirobebuli winaswarganwyoba (33,38). zogi avtoris azriT mis ganviTarebaSi wamyvani adgili eniWeba Tavis tvinis biologiurad aqtiur nivTierebas oreqsins (hipokretins), romelic aregulirebs Zilisa da gamoRviZebis procesebs.

   Nnarkolefsiis klinikur tetradasTan erTad xSiria mniSvnelovani fsiqikuri da fsiqosocialuri aSlilobani, dawyebuli socialuri adaptaciis calkeuli darRvevebidan (mouwyobloba samsaxurSi, ojaxSi, socialur garemoSi) da damTavrebuli fsiqikuri aSlilobis manifestur niSnebamde (afeqturi aSlilobis sxvadasxva formebi: depresiis Setevebi, zogjer suiciduri tendenciebiT, alkoholizmi, pirovnuli aSlilobani da a.S.). amfetaminebiT mkurnalobis procesSi xSiria maniakaluri sindromis da iseTi fsiqopaTologiuri simptomebis ganviTarebac, romlebic Sizofrenias mogvagonebs. zogjer adgili aqvs stereotipiisa da qcevis avtomatizmebiT ganpirobebul kriminalur qmedebebs (22). narkolefsiuri sindromis dros sxvadasxva somatur da fsiqopaTologiur daavadebaTa arseboba miuTiTebs Tavis tvinis mezodiencefaluri warmonaqmnebis dazianebaze.

   narkolefsiis paTofiziologiuri safuZvlebis dadgenaSi Znelia ar gaviTvaliswinoT i. pavlovis moZRvreba umaRlesi nervuli moqmedebis Sesaxeb, romlis Tanaxmadac narkolefsiis safuZveli unda iyos Tavis tvinis qerquli funqciebis ganlevadoba da masSi ganviTarebuli Sekavebis procesiT ganpirobebuli parcialuri Zilis Setevebi. narkolefsiuri Zilis Setevis dros Tavis tvinis qerqis Sekaveba difuzuria da mis mTel zedapirze vrceldeba, magram am procesSi ar iTrevs motoruli analozatoris qerqul ubnebs. xolo im SemTxvevebSi, rodesac narkolefsias Tan axlavs katalefsia, maSin Sekavebis procesi exeba Tavis tvinis im struqturebsac, sadac ganlagebulia motoruli analizatori.

N  narkolefsiis mkurnaloba simptomuria da gamiznulia ZiriTadad dRis saaTebSi Zilis Setevebis salikvidaciod. Aam mizniT gamoiyeneba: gamafxizlebeli aminebi (fenamini, deqstramfetamini, levamfetamini da sxva), meTilfenidati, efedrini, piridroli, pipradroli da a.S. narkolefsiis, katalefsiis da sxva Tanmxlebi simptomebis erToblivi mkurnalobisaTvis gamoiyeneba Timoanalefsiuri preparatebi: imipramini da pertofrani (8). orive am medikamentma mniSvnelovnad Seamcira katalefsiis Setevebi, “Zilis dambla” da hipnogogiuri halucinaciebi, magram ZiriTadi narkolefsiuri sindromis – depresiis mimarT nakleb efeqturi aRmoCndnen, ris gamoc saWiro gaxda amfetaminebis da meTilfenidatis gamoyeneba. aRniSnuli preparatebi rCebian arCeviT saSualebebad, radgan mTel rig SemTxvevebSi, Terapiuli efeqtis misaRebad saWiro xdeba amfetaminebis dozebis mateba, ris Sedegadac adgili aqvs am preparatebis mimarT damokidebulebis ganviTarebas.

   narkolefsiis dros arsebul depresiaze dadebiT zegavlenas axdenen  antidepresantebi. gasaTvaliswinebelia, rom tricikluri antidepresantebis gamoyeneba naklebefeqturia, radganac TviTon iwveven sedaciuri movlenebs (32). ukanasknel xans gamoiyeneba am jgufis ara sedaciuri aqtivobis mqone antidepresanti protiptilini, romelic mcire dozebSi (dReSi 10-20 mgr) sruliad xsnis narkolefsiur da katalefsiur Setevebs da ar iwvevs tolerantobas, romelic riskisfaqtors warmoadgens fsiqostimulatorebis gamoyenebis dros.

   Zilis ciklis sadReRamiso fazebis normalizebisaTvis gamoiyeneba, agreTve amitriftilini, fenobarbitali da tizercini, rac iwvevs Ramis Zilis gaxangrZlivebas. polisimptomuri narkolefsiis samkurnalod zogi avtoris mier gamoiyeneba medikamenti “l-dofa”, romelic ar avlens gamaaqtivebel Tvisebebs da ar amcirebs Zilis Setevebs, magram garkveul wilad awonasworebs emociur sferos. SemdgomSi imave avtorma gamoiyena natriumis oqsibutirati, riTic miaRwia SesamCnev gaumjobesebas polisimtomuri narkolefsiis dros.

   gansakuTrebiT Znelia im avadmyofTa mkurnaloba, romlebic erTdroulad daavadebulni arian SizofreniiT da narkolefsiiT. am SemTxvevebSi narkolefsiis sawinaaRmdego stimulatorebis gamoyenebam SesaZloa gaamwvavos Sizofreniis ZiriTadi simptomebi, xolo Sizofreniis samkurnalo neirolefsiuri medikamentebi aZliereben narkolefsiur sindroms. zogi avtori am SemTxvevebSi gvirCevs gamoviyenoT deqstramfetaminis kombinacia flufenazinTan. narkolefsiis stimulatorebiT mkurnalobis procesSi SesaZlebelia ganviTardes Sizofreniismagvari bodviTi sindromi. aRwerilia narkolefsiis da toqsikomaniis erTdrouli miqstis SemTxveva, rodesac pacienti xangrZlivi drois ganmavlobaSi iRebda samkurnalod bromis Semcvel sirofs da mas intoqsikaciis Sedegad ganuviTarda epilefsiis magvari cnobierebis bindiseburi Secvla, hipnogogiuri halucinaciebi, narkolefsiuri Setevebi da e.w. “nevrologiuri krizebi”.

   ukanasknel xans, narkolefsiis samkurnalod farTod gamoiyeneba sxvadasxva narkotikuli jgufis fsiqostimulatorebi: meTilfenidati, deqstroamfetamini, metamfetamini an modafinili (nuvigili), tricikluri antidepresantebi: qlomipramini, imipramini, protriftilini an serotoninerguli antidepresanti venlafaqsini.

   narkolefsiis klinikur struqturaSi afeqturi aSlilobis sindromTa manifesturi an SeniRbuli saxiT arseboba da misi ZiriTadi niSnebis ukuganviTarebaSi Timoanalefsiuri, Timoeretiuli saSualebebis da fsiqostimulatorebis efeqturoba, warmoadgens afeqturi speqtris aSlilobebis jgufSi am daavadebis gaerTianebis mizezs.

Нарколепсия, как заболевание афективного спектра.

И.Гиоргобиани, Е.Кавсадзе, М.Арвеладзе, НИИ наркологии, Тбилиси, Грузия

Нарколепсия - заболевание нервной системы, относящееся к гиперсомниям, характеризуется дневными приступами непреодолимой сонливости и приступами внезапного засыпания, приступами катаплексии, то есть полной или частичной утраты мышечного тонуса, нарушениями ночного сна, появлениями гипнагогических (при засыпании) галлюцинаций. Обычно нарколепсия развивается у молодых людей, чаще у мужчин. Есть мнения, что болезнь носит наследственный характер. Для лечения нарколепсии широко применяются различные психостимуляторы: амфетамины, декстроамфетамины и др., трициклические  или серотонинергные антидепресанты: кломипрамин, имипрамин  венлафаксин и др.  Таким образом, применение данных препаратов является одной из причин обединения нарколепсии  с заболеваниями афективного спектра.

Narcolepsy, as ander affeqtive spectr disease.

I.giorgobiani, E.Kavsadze, M.Arveladze, Research institute on addictaion,Tbilisi, Georgia

The main characteristic of narcolepsy is excessive daytime sleepiness (EDS), even after adequate nighttime sleep. Four of the other classic symptoms of the disorder, often referred to as the "tetrad of narcolepsy," are cataplexy, sleep paralysis, hypnagogic hallucinations, and automatic behavior. The main treatment of excessive daytime sleepiness in narcolepsy is with central nervous system stimulant drugs such as methylphenidate, racemic - amphetamine, dextroamphetamine, and methamphetamine, or modafinil (Provigil), a new stimulant with a different pharmacologic mechanism, and more recently, armodafinil (Nuvigil). Cataplexy and other REM-sleep symptoms are frequently treated with tricyclic antidepressants such as clomipramine, imipramine, or protriptyline, as well as other drugs that suppress REM sleep. Venlafaxine, an antidepressant which blocks the reuptake of serotonin and norepinephrine, has shown usefulness in managing symptoms of cataplexy, however, it has notable side-effects including sleep disruption.
 Lliteratura:

1.      Arias-Carrión O, Murillo-Rodriguez E, Xu M, Blanco-Centurion C, Drucker-Colín R, Shiromani PJ (December 2004). "Transplantation of hypocretin neurons into the pontine reticular formation: preliminary results". Sleep 27 (8): 1465–70. PMID 15683135. 

2.       Caputo, F; Zoli, G; Provini, Federica; Albani, Fiorenzo; Riva, Roberto (March 2007). "Treatment of narcolepsy with cataplexy". Lancet 369 (9567): 1080–1081.

3.      Carter, Bill (November 3, 2003). "In The Land Of The Insomniac The Narcoleptic Wants To Be King". New York Times. http://www.nytimes.com/2002/11/03/magazine/in-the-land-of-the-insomniac-the-narcoleptic-wants-to-be-king.html. Retrieved 2009-12-11. 

4.       Fry, JM; Pressman, MR; Diphillipo, MA; Forst-Paulus, M (1986). "Treatment of narcolepsy with codeine". Sleep 9 (1 Pt 2): 269–74.

5.      Ferguson A.V., Samson W.K. // Frontiers in Neuroendocrinology. 2003. V.24. № 1. P.141–150.

6.      Hallmayer J, Faraco J, Lin L, et al. (June 2009). "Narcolepsy is strongly associated with the T-cell receptor alpha locus". Nat. Genet. 41 (6): 708–11.

7.       John J. et al. // Neuron. 2004. V.42. № 5. P.619–634.

8.      Kilduff T.S., Peyron C. //TINS. 2000. V.23. № 8. P.359–365.

9.      Kandel, Eric R.; James H. Schwartz, Thomas M. Jessell. Principles of Neural Science (4th ed.). McGraw-Hill. p. 949. ISBN 0-8385-7701-6. 

10.  Klein J, Sato A (September 2000). "The HLA system. Second of two parts". N. Engl. J. Med. 343 (11): 782–6.

11.  Mignot E. // Sleep Medicine. 2000. V.1. № 1. P. 87–90.

12.   Maret, S; Tafti M (November 2005). "Genetics of narcolepsy and other major sleep disorders" (PDF). Swiss Medical Weekly (EMH Swiss Medical Publishers Ltd.) 135 (45-46): 662–5. Retrieved 2008.03.07. 

13.  Mignot, E (2001). "A commentary on the neurobiology of the hypocretin/orexin system". Neuropsychopharmacology 25 (5 Suppl): S5-13.

14.  Manni R, Politini L, Nobili L, et al. (May 2001). "Hypersomnia in the Prader Willi syndrome: clinical-electrophysiological features and underlying factors". Clin Neurophysiol 112 (5): 800–5.

15.  Mayer, G; Ewert Meier, K; Hephata, K (1995). "Selegeline hydrochloride treatment in narcolepsy. A double-blind, placebo-controlled study". Clin Neuropharmacol 18 (4): 306–19.

16.  Scammel T.E. // Current Biology. 2001. V.11. P.R769-R771.

17.  Siegel J.M. // Annual Review of Psychology. 2004. V.55. № 2. P.125–148.

18.  Siegel J.M. et al. // Neuropsychopharmacology. 2001. V.25. № S5. P.S14-S20.

19.   Vankova J, Stepanova I, Jech R, et al. (June 2003). "Sleep disturbances and hypocretin deficiency in Niemann-Pick disease type C". Sleep 26 (4): 427–30.

20.   Вейн А.М. Гиперсомнический синдром. «Нарколепсия и другие формы патологической сонливости. М, 1966, стр. 236.

21.     Ковальзон В.М. Природа сна // Природа. 1999. № 8. С.172–179.

22.   Латыш Л.П. – Гипоталамус, приспособительная активность и электроэнцефалограмма, М.1968.

23.   Латыш Л.П. – Роль гипоталамуса и некоторых функциональных с ним связанных образований головного мозга человека в организации приспособительной активности. Докт.дисс. М.1967.

24.   Ушакова И.Н. – Нарколепсия. Вопросы клиники, экспертизы, трудоспособности, реабилитации больных.//Ленинградский НИИ Экспертизы трудоспособности и организации труда инвалидов.