CONVULSIILE FEBRILE Definitie crize epileptice produse de febra in absenta infectiei SNC, pe un creier predispus Tratament in criza msurile suportive: ABC; aport O2; acces iv tratament: !iazepam ",# m$%&$corp rectal scderea febrei '(),#*C combaterea cauzei febrei lini+tirea familiei Tratament profilactic !e obicei nu,, -ntremitent: !./ "# m$%&$%zi in per febrile Continuu: 0/A, /B Inicatii profila!ia continu" Copii cu crize frecvente 1ntr2o perioad scurt de timp 34 (C5 in 6 luni, 47 in 8an9, durata C5:8# min, C5 necesitand interven;ie farmacolo$ic pt a fi 1ntrerupte dezvoltare psi<omotorie anormala parin;i 1nspim=nta;i, an>iosi CRI#$ EPILEPTIC$ UNIC$ Criza epileptic" % efini&ie' eveniment clinic tranzitor determinat de o descrcare paro>istic anormal, e>cesiv, sincron a unei popula;ii neuronale Crize epileptice Pro(ocate ? crize ocazionale % acute simptomatice ? rspuns tranzitor al creierului la a$resiune 3dro$uri, alcool, febr, <ipo@, infec;ii cerebrale, ACC, etc9 Nepro(ocate ? epilepsie daca crize recurente $tituine terapeutica' Stabilizare pacient Anamneza, e> clinic, neurolo$ic -nvesti$are prima criza o Bzuale lab o /C o DD@ 8 o -ma$istica CA%-EF Prima criza % can tratam) Atitudine individualizata dup prima criz cu modificri DD@ concordante dac pacientul prezint deficit neurolo$ic dac ima$istica cerebral indic anomalie structural dac pacientul +i familia consider inacceptabil riscul unei alte crize Prima criza %ar$umente contra tratarii: Dvaluare raport risc%beneficiu Ea ale FAD timp 1ndelun$at 3ani9 raportate la riscul de recuren; FAD scad riscul recurentei, nu scad riscul de epilepsie, 8"G din popula;ie 8 criz 1n via; (Berg etShinnar 1991), #"G 1n copilrie +i adolescen; crize scurte nu leziuni cerebrale Studiile: nu diferente pro$nostic daca trat 2 1nceput la -2 a criza vs a --2a (D. Hirtz and al. 2006). Eemisia depinde de etiolo$ia crizei TR$T$*ENTUL IN EPILEPSII Cine are competenta e a +, si trata epilepsia la copil) meici specialisti in NP- meici cu u.la specialitate % fosti NPI !ia$nostic -nitiere tratament Fonitorizare tratament orice nivel N/ Dvaluarea efectelor epilepsiei asupra comportamentului, co$nitiei sau dezvoltarii mentale a copilului 2 in centru tertiar N/ *eicii e familie sau meicii e alte specialitati nu au competenta e a ia+nostica epilepsia- e a initia- conuce sau opri tratamentul cu meicamente antiepileptice la copiii cu epilepsie, Principii tratament' !ia$nostic cert de epilepsie,, -nformare pacientHfamilie: epilepsie, tratament FAD, alternative, conditii de viata, etc !ecizii impreuna cu pacientulHfamilia -ndividualizare tratament 3tip criza, sindrom, comedicatie, comorbiditati9 Fonoterapie -ntroducere treptata Nu oprire Nu sc<imbare cu alte FAD daca e>ista control 2 Nu sc<imbare diverse preparate ale aceleiasi substante,,, Cipsa efect FAD 2 Cipsa efect bi%politerapie Sc/im.area *$E se face aca' /acientul prezinta crize /acientul prezinta reactii adverse /acientul prezinta deteriorare co$nitiva cu modificari DD@ specifice pe DD@ de ve$<e si de somn, $le+erea meicamentului in functie e tipul crizei' Tipul crizei *eicament e prima linie 0in orine alfa.etica1 *eicament e a 23a linie 0in orine alfa.etica1 $lte meicamente 0in orine alfa.etica1 *eicamente ce ar tre.ui e(itate 0accentueaza crizele1 0in orine alfa.etica1 Crize tonico2 clonice $eneralizate Carbamazepina Camotri$ina 0alproat de sodiu Aopiramat Clobazam Cevetiracetam O>carbazepina Acetazolamida Clonazepam 5enobarbital 5enitoina /rimidona Aia$abina 0i$abatrin Absente Dtosuccinimida Camotri$ina 0alproat de sodiu Clobazam Clonazepam Aopiramat Carbamazepina @abapentin O>carbazepina Aia$abina 0i$abatrin 5enitoina Fioclonic 0alprat de sodiu Aopiramat Clobazam Clonazepam Camotri$inaI Cevetiracetam /iracetam Aopiramat Carbamazepina @abapentin O>carbazepina Aia$abina 0i$abatrin 5enitoina Aonic Camotri$ina 0alproat de sodiu Clobazam Clonazepam Cevetiracetam Aopiramat Acetazolamida 5enobarbital 5enitoina /rimidona Carbamazepina O>carbazepina Atonic Camotri$ina 0alproat de sodiu Clobazam Clonazepam Cevetiracetam Aopiramat Acetazolamida 5enobarbital /rimidona Carbamazepina O>carbazepina 5enitoina 5ocal H%2 $eneralizare secundara Carbamazepina Camotri$ina O>carbazepina Clobazam @abapentin Cevetiracetam Acetazolamida Clonazepam 5enobarbital ( 0alproat de sodiu Aopiramat 5enitoina Aia$abina /rimidona Icu e>ceptia epilepsiilor mioclonice pro$resive, epilepsia mioclonica severa; $le+erea meicamentului in functie e sinromul epileptic' Sinroame epileptice *eicamente e prima linie *eicamente e a323a linie $lte meicamente *eicamente ce a+ra(eza crizele Dpilepsie absenta a copilului Dtosucinimida Camictal 0alproat Cevetiracetam Aopiramat Carbamazepina 5enitoin O>carbazepina Aia$abina 0i$abatrin Dpilepsie absenta a copilului Camictal 0alproat Cevetiracetam Aopiramat Carbamazepina 5enitoin O>carbazepina Aia$abina 0i$abatrin Dpilepsie mioclonica Juvenila Camictal 0alproat Clobazam Clonazepam Cevetiracetam Aopiramat Acetazolamida Carbamazepina 5enitoin O>carbazepina Aia$abina 0i$abatrin Dpilepsie $eneralizata numai cu crize $eneralizate Carbamazepina Camotri$ina 0alproat de sodiu Aopiramat Cevetiracetam Acetazolamida Clobazam Clonazepam O>carbazepina 5enitoin /rimidona
Aia$abina 0i$abatrin Dpilepsie focala cripto$enica, simptomatica Carbamazepina Camotri$ina O>carbazepina 0alproat de sodiu Aopiramat Clobazam @abapentin Cevetiracetam 5enitoina Aia$abina Acetazolamida Clonazepam 5enobarbital /rimidona Spasme infantile Steroizi 0i$abatrin Clobazam Clonazepam 0alproat de sodiu Aopiramat Nitrazepam Carbamazepina O>carbazepina Dpilepsie Carbamazepina Cevetiracetam Sultiam 7 partiala beni$na cu varfuri centro2 temporale Camotri$ina O>carbazepina 0alproat de sodiu Aopiramat Dpilepsie partiala beni$na cu varfuri occipitale Carbamazepina Camotri$ina O>carbazepina 0alproat de sodiu Cevetiracetam Aopiramat Dpilepsia mioclonica severa a copilului 3 sindromul !rave 9 Clobazam Clonazepam 0alproat de sodiu Aopiramat Cevetiracetam Stiripentol 5enobarbital Carbamazepina Camictal O>carbazepina 0i$abatrin Sindromul Cenno>2@astaut Camictal 0alproat de sodiu Aopiramat Clobazam Clonazepam Dtosuccinimda Cevetiracetam Carbamazepina O>carbazepina Dpilepsia mioclono2 astatica 3sindromul !oose9 Clobazam Clonazepam 0alproat de sodiu Aopiramat Cevetiracetam Camictal Carbamazepina O>carbazepina Sindromul Candau2 Kleffner Camictal 0alproat de sodiu Steroizi Cevetiracetam Aopiramat Sultiam Carbamazepina O>carbazepina !escarcare continua de C0B in somnul lent Clobazam Clonazepam Dtosuccinimda Camictal 0alproat de sodiu Steroizi Cevetiracetam Aopiramat Carbamazepina O>carbazepina Sindroame epileptice Fedicamente de prima linie Fedicamente de a222a linie Alte medicamente Fedicamente ce a$raveza crizele *ONITORI#$RE TR$T$*ENT !uratele de timp intre controale Fedicul N/ monitorizare tratament # /rimul control dupa initierea FAD 2 la 6 saptamani Blterior 2 la ( luni 3ma> an92 c<iar daca pacientul nu face crize !aca crizele persista sau apare re$res psi<ic 2 reevaluare la intervale mai scurte, in functie de particularitatile cazului CE SE UR*$RESTE L$ CONTROL dozele de antiepileptic frecventa si severitatea crizelor e>istenta reactiilor adverse suplimente nutritive asociate functia co$nitiva 3normala, alterata9 asocierea altor afectiuni NB: bilant seric re$ulat NB: nivele serice ale FAD INDIC$TIILE *ONITORI#$RII NIVELELOR *$E SUNT' rezistenta la tratamentul corect administrat politerapia in cazuri cu reactii adverse SD la pacienti cu tratament corect administrat alte situatii apreciate de medicul specialist SC4I*B$RE$ *EDIC$TIEI $NTIEPILEPTICE se face numai de medicul N/ sau dubla specialitate, fost N/- -n centru care asi$ura resursele te<nice pentru un control de calitate al pacientului *eicatia nu se sc/im.a la pacientii sta.ili Nota' nu se sc/im.a meicamentele ori+inale si +enerice intre ele Sc<imbarea medicatiei se ia in calcul daca: o Crizel necontrolate o Eeactii adverse o Ee$res co$nitiv 3necesita evaluare comple>a inaintea modificarii tratamentului9 o Fotive practice financiare sau de discontinuitate a aprovizionarii Sc<imbarea FAD ? treptat 3nu brusc9 si nu se fac mai multe sc<imbari o data -nitial se introduce noul medicament si numai dupa ce s2a aJuns la doza dorita se va incepe scoaterea treptat a primului medicament OPRIRE$ TR$T$*ENTULUI Se face de catre medicul N/, fost N/-, nu alte spec Se face intr2un centru cu dotari corespunzatoare Fotivatii: o Necesare cunostinte adecvate privind proprietatile farmacocinetice si farmacodinamice ale AD 6 o Necesare resurse materiale speciale: DD@, DD@ somn, video2DD@, masurarea nivelelor san$uine ale antiepilepticelor /rin conventie dupa 2 ani de tratament fara crize 3adaptat la caz9 Oprire pro$resiva 3rata particulara fiecarui FAD si caz9 Necesara monitorizare clinica si paraclinica 3DD@9 la intervale re$ulate 382( luni sau adaptat cazului9 C4IRUR5I$ EPILEPSIEI Caniati posi.ili 3 copiii cu' epilepsie rezistenta la tratament 3esecul a 2 sau ( antiepileptice corect alese9 crize ce afecteaza calitatea vietii, inclusiv reactii adverse la antiepileptice Ee$res psi<ic rapid Dpilepsii focale de preferat Etiolo+ii si sinroame frec(ente la pacientii peiatrici caniati la c/irur+ia epilepsiei Displazia corticala3 cel mai frec(ent o rezectia completa asi$ura cel mai bun control al crizelor postc<irur$ical o important de evaluat : eventuala imbunatatire co$nitive a pacientului versus riscul de deficite neurolo$ice postc<irur$icale Scleroza tu.eroasa o daca e>ista o sin$ura re$iune epilepto$ena ? rezectie c<iar daca pacientul are tuberi multipli sau modificari DD@ multifocale%difuze intercritic Polimicro+iria 4amartom /ipotalamic o bordari c<irur$icale diferite: proceduri stereotactice, endoscopice, radioc<irur$icale Sinroame /emisferice3 /emime+alencefalia- isplazii /emisferice o <emisferectomie sau <emisferotomie Sinrom Stur+e36e.er o evaluare ur$enta daca apar crize H%2 intarziere in dezvoltare sau <emipareza pro$resiva Sinrom Rasmussen o <emisferectomie sau deconectare <emisferica Sinrom Lanau37leffner o transectiuni multiple subpiale Sinrom Lenno!35astaut' o :8" ani 2 calosotomia 32%( anterioare a corpului calos9; E(aluarea prec/irur+icala' EE5 o DD@ interictal Hsomn natural o 0ideo2DD@ ictal 3se fac inre$istrari de lun$a durata zile9 L o inre$istrare cu electrozi invazivi Ima+istica cere.rala structurala o EFN cu protocol pt epilepsie o uneori e necesar CA cerebral pentru evaluarea eventualelor calcificari Ima+istica cere.rala functionala o S/DCA, /DA ictal si interictal E(aluare neuropsi/olo+ica DIET$ CETO5ENIC$ Inicatii' e prima intentie 2 deficitul de @CBA28 2 deficitul de /!M; epilepsii rezistente la tratament' 2 Spasme infantile 2 Sd !oose 3 epilepsia mooclono2astatica 9 2 Sd !ravet 3 epilepsia mioclonica severa9 2 Sd Cenno>2@astaut 2 Scleroza tuberoasa 2 Sd Eett 2 Sd Candau2Kleffner Unele .oli meta.olice 3deficit de defosfo2fructo2&inaza, $lico$enoza tip 0, boala mitocondriala de lant respirator9 PESS Contrainicatii' absolute: o deficit primar de carnitina o deficit de carnitin2palmitoil2transferaza -%-- o deficit de carnitin2translocaza o deficit de piruvat carbo>ilaza o defecte de beta o>idare 3deficit de acilde<idro$enaza cu lant lun$, mediu, scurt; deficit de (2<idro>iacil2CoA cu lant lun$, mediu 9, o porfiria; relati(e' o inabilitatea de a mentine nutritia adecvata, o /osibilitatea trat c<irur$ical o necomplianta parintilor%tutorilor VNS % INDIC$TII adulti si copii peste 82 ani pacienti cu crize cu debut focal, rezistente la medicatia antiepileptica ) pacienti la care se e>clude o cauza tratabila sau o cauza nonepileptica pacienti care nu sunt candidati la c<irur$ia epilepsiei -ntoleranta sau rezistenta la FAD reducerea frecventei crizelor va imbunatati semnificativ calitatea vietii pac ST$TUS EPILEPTICUS Faza premonitorie % crize seriate 2 !./ rectal ",#m$%&$%adm, Intraspitalicesc' o ABC 3la fiecare pas9 o Fasuri de aJutor: O2, mas @2mie, certificare ori$ine epi 3la fiecare pas9 Tratament % pas 8 !aca abord iv: B8, B6, cor acidoza !./ ",(m$%&$%doza sau Corazepam ",8 m$%&$%doza !aca nu abord iv: !./ rectal ",# m$%&$%doza sau Fidazolam bucal%nazal Tratament 3 pas 2 % monitoriz resp o Eepetare !./, Coraz iv Tratament 3 pas 9 % monitoriza cariaca o 5enitoin incarcare 8)m$%&$%doza o 5enobarbital uz iv 2" m$%&$%doza Tratament 3 pas : % $TI 3 intu.are o Aiopental (2# m$%&$%doza apoi (2#m$%&$%< o /ropofol 822 m$%&$%bolus apoi 228" m$%&$%< o Fidazolam ",2 m$%&$%bolus apoi ","#22 m$%&$%< o /entobarbital 8"28# m$%&$%bolus apoi ",#28 m$%&$%< *$TERI$LE BIBLIO5R$FICE FOLOSITE' Con(ulsii Fe.rile' Fe.rile seizures' clinical practice +uieline for t/e lon+3term mana+ement of t/e c/il ;it/ simple fe.rile seizures, Steerin$ Committee on NualitO -mprovement and Fana$ement, Subcommittee on 5ebrile Seizures American AcademO of /ediatrics, 2"") Recommenations for t/e mana+ement of <fe.rile seizures<' $ 4oc Tas= Force of LICE 5uielines Commission- Capovilla @- Fastran$elo F- Eomeo A- 0i$evano 5,- Dpilepsia 2""P Prima criza' D, 4irtz- S, $s/;al- $, Ber+- et al, Practice parameter' E(aluatin+ a first nonfe.rile seizure in c/ilren ' Report of t/e >ualit? Stanars P Su.committee of t/e $merican $caem? of Neurolo+?- t/e C/il Neurolo+? Societ?- an t/e $merican Epileps? Societ? 2@@@ABBAC8C Neurology <ttp:%%QQQneurolo$Oor$%content%##%#%686full<tml National Institute for Clinical E!cellence , Epileps?, T/e ia+nosis an mana+ement of t/e epilepsies in aults an c/ilren in primar? an seconar? care, Clinical 5uieline 2@,Octo.er 2@@:, <ttp:%%QQQniceor$u&%C@"2"adultsRuic&ref$uide , <ttp:%%QQQniceor$u&%C@"2"c<ildrenRuic&ref$uide Tratament antiepileptic' Trac? 5lauser- Elinor Ben3*enac/em- Blaise Bour+eois- $(ital Cnaan- Da(i C/a;ic=- Carlos 5uerreiro- Reetta 7al(iainen- Ric/ar *attson- Emilio Perucca- Tor.Dorn Tomson- IL$E Treatment 5uielines' E(ience3.ase $nal?sis of $ntiepileptic Dru+ Efficac? an Effecti(eness as Initial *onot/erap? for Epileptic Seizures an S?nromes- Epilepsia, :E0E1'8@F:% 882@- 2@@C SA 5renc<, F!I; AF Kanner, F!T; S Bautista, F!; B Abou2K<alil, F!; A BroQne, F!; CC Marden, F!; UM A<eodore, F!; C Bazil, F!, /<!; S Stern, F!; SC Sc<ac<ter, F!; ! Ber$en, F!; ! Mirtz, F!; @! Fontouris, F!; F Nespeca, F!; B @idal, /<arm!; US Far&s, Sr, F!; UE Aur&, F!; SM 5isc<er, F!; B Bour$eois, F!; A Uilner, F!; ED 5au$<t, Sr, F!; EC Sac<deo, F!; A BeOdoun, F!; and AA @lauser, F!, Efficac? an tolera.ilit? of t/e ne; antiepileptic ru+s I' Treatment of ne; onset epileps?A Report of t/e T/erapeutics an Tec/nolo+? $ssessment Su.committee an >ualit? Stanars Su.committee of t/e $merican $caem? of Neurolo+? an t/e $merican Epileps? Societ?- Neurology 2""7;62;82#2 SA 5renc<, F!I; AF Kanner, F!T; S Bautista, F!; B Abou2K<alil, F!; A BroQne, F!; CC Marden, F!; UM A<eodore, F!; C Bazil, F!, /<!; S Stern, F!; SC Sc<ac<ter, F!; ! Ber$en, F!; ! Mirtz, F!; @! Fontouris, F!; F Nespeca, F!; B @idal, /<arm!; US Far&s, Sr, F!; UE Aur&, F!; SM 5isc<er, F!; B Bour$eois, F!; A Uilner, F!; ED 5au$<t, Sr, F!; EC Sac<deo, F!; A BeOdoun, F!; and AA @lauser, F!, Efficac? an tolera.ilit? of t/e ne; antiepileptic ru+s II' Treatment of refractor? epileps?, Report of t/e T/erapeutics an Tec/nolo+? $ssessment Su.committee an >ualit? Stanars Su.committee of t/e $merican $caem? of Neurolo+? an t/e $merican Epileps? Societ?- Neurology 2""7;62;8268 *onitorizarea tratmentului' P/ilip N, Patsalos- Da(i G, Berr?- Blaise F, D, Bour+eois- Games C, Clo?- Trac? $, 5lauser- S(ein I, Go/annessen- Ilo E, Leppi=- Tor.D Horn Tomson- Emilio Perucca, $ntiepileptic ru+sI.est practice +uielines for t/erapeutic ru+ monitorin+' $ position paper .? t/e su.commission on t/erapeutic ru+ monitorin+- IL$E Commission on T/erapeutic Strate+ies, Epilepia, 7P3L9:82(P82L6, 2"") C/irur+ia epilepsiei la copii' S Melen Cross, /rasanna SaOa&ar, !ou$ Nordli, Olivier !elalande, Fic<ael !uc<oQnO, Meinz @ Uieser, Eenzo @uerrini, and @arO U Fat<ern /roposed 8" Criteria for Eeferral and Dvaluation of C<ildren for DpilepsO Sur$erO: Eecommendations of t<e Subcommission for /ediatric DpilepsO Sur$erO Epilepia, :E369:P#2P#P, 2""6 Stimularea (a+ala' Eobert S 5is<er, F!, /<!; and Adrian Mandfort<, F!, RE$SSESS*ENT' V$5US NERVE STI*UL$TION FOR EPILEPSJ, $ Report of t/e T/erapeutics an Tec/nolo+? $ssessment Su.committee of t/e $merican $caem? of Neurolo+? , 8PPP;#(;666 Neurology! <ttp:%%QQQneurolo$Oor$%content%#(%7%666full<tml Status epilepticus la copii' Eiviello Sr SS, As<Qal S, Mirtz !, @lauser A, Ballaban2@il K, KelleO K, Forton C!, /<illips S, Sloan S, S<innar S, /ractice /arameter: !ia$nostic assessment of t<e c<ild Qit< status epilepticus 3an evidence2based revieQ9 Eeport of t<e NualitO Standards Subcommittee of t<e American AcademO of Neurolo$O and t<e /ractice Committee of t<e C<ild Neurolo$O SocietO, NDBEOCO@V 2""6;6L:8#728##" Appleton E, C<oonara -, Fartland A, /<illips B, Scott E, U<ite<ouse U A<e Status Dpilepticus Uor&in$ /artO A<e treatment of convulsive status epilepticus in c<ildren Arc< !is C<ild 2""";)(:78#78P Appleton E, Fartland A, /<illips B !ru$ mana$ement for acute tonicclonic convulsions includin$ convulsive status epilepticus in c<ildren Coc<rane !atabase SOst Eev 2""2:C!""8P"# 88