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    Conf. Dr. Ioan Buraga - Curs Neurologie

    CURSCURS

    DEDE

    NEUROLOGIENEUROLOGIE

    UNIVERSITATEA DE MEDICINA SI FARMACIEUNIVERSITATEA DE MEDICINA SI FARMACIE

    CAROL DAVILACAROL DAVILA

    BUCURESTIBUCURESTI

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    TUMORILE CEREBRALETUMORILE CEREBRALE

    = procese expansive intracraniene= procese expansive intracraniene

    -determina HIC prin modificri cantitative aledetermina HIC prin modificri cantitative aleparenchimului, sngelui, LCR ntr-un continator inextensibilparenchimului, sngelui, LCR ntr-un continator inextensibil

    HIC se manifestaHIC se manifesta clinicclinic ::

    - cefalee- cefalee- greata uneori si vrsturi (in jet)- greata uneori si vrsturi (in jet)- bradicardie- bradicardie- semne neurologice de focar- semne neurologice de focar- redoare de ceafa si alte semne de iritaie meningeala- redoare de ceafa si alte semne de iritaie meningeala-- alterarea strii de contienta mai ales la instalarea bruscaalterarea strii de contienta mai ales la instalarea bruscaExamenul fundului de ochi evidentiaza - edem +/- staza papilaraExamenul fundului de ochi evidentiaza - edem +/- staza papilarasi leziuni hemoragicesi leziuni hemoragice

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    MRI of pituitary adenoma

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    Large intraventricular mass in neurocytoma

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    Conf. Dr. Ioan Buraga - Curs NeurologieCT scan of craniopharyngioma

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    MRI of dysembryoplastic neuroepithelial tumor

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    FiziopatologicFiziopatologic distructia tesutului cerebraldistructia tesutului cerebral deplasarea spaiului vascular si LCRdeplasarea spaiului vascular si LCR depasirea limitei de volum = > HIC insotita de edemdepasirea limitei de volum = > HIC insotita de edem

    papilarpapilar

    edem vasogenedem vasogen fuga lichidului din vas in parenchim fuga lichidului din vas in parenchim- evident mai ales in substana alba, aspect- evident mai ales in substana alba, aspect

    digitiform apare tardiv in substanadigitiform apare tardiv in substana cenuiecenuie! edem citotoxic in AVC prin distru! edem citotoxic in AVC prin distruccie neuronala =>ie neuronala =>

    acumularea apei intracelularacumularea apei intracelular

    Cele 2 tipuri coexista, dar predomina cel vasogenCele 2 tipuri coexista, dar predomina cel vasogen semne de suferina a parenchimului, ce nu suntsemne de suferina a parenchimului, ce nu sunt

    concordante cu localizarea tumorii => fenomen deconcordante cu localizarea tumorii => fenomen deangajare =angajare = false semne de localizarefalse semne de localizare (hernii subfaciale,(hernii subfaciale,subtentoriale de lob temporal -> modificri pupilare,subtentoriale de lob temporal -> modificri pupilare,

    amigdale cerebeloase dau aspect de redoare de ceafa)amigdale cerebeloase dau aspect de redoare de ceafa)ex. paralizia N oculomotor extern izolata => tumoraex. paralizia N oculomotor extern izolata => tumoracerebrala NU afectare pontincerebrala NU afectare pontin

    paralizia uni/bilaterala de N IVparalizia uni/bilaterala de N IV

    Pot apare manifestri de tip Parkinsonian sau semnePot apare manifestri de tip Parkinsonian sau semne

    uni/bilaterale de suferinta a cailor piramidaleuni/bilaterale de suferinta a cailor piramidale

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    DEBUT CLINICDEBUT CLINIC greu de identificat mai ales cele benigne ex. meningiomgreu de identificat mai ales cele benigne ex. meningiom

    - discrete tulburri cognitive, comp- discrete tulburri cognitive, comport.ort.,,obosobos. i. intelectuala, fara semne focalentelectuala, fara semne focale

    - hemipareza progresiva- hemipareza progresiva

    - crize epileptice cu debut tardiv- crize epileptice cu debut tardiv- HIC +/- semne de localizare- HIC +/- semne de localizare- sindroame definite- sindroame definite

    - ttumorile primitive se localizeaz frecvumorile primitive se localizeaz frecv.. inin llobul frontal siobul frontal sitemporaltemporal

    VARIANTEVARIANTE alterarea generala a funciilor cerebrale cu alterarea generala a funciilor cerebrale cua) astenia mentalaa) astenia mentala (psihomotor(psihomotoriie) -> nu e depresie !e) -> nu e depresie !- se accentueaz in saptamani / luni- se accentueaz in saptamani / luni;; evolevol.. spre stupor -> comaspre stupor -> coma-- fara valfara val.. localizatorilocalizatoriee, in general, in general -- tumori ale structurilor centraletumori ale structurilor centraleb) cefaleeb) cefalee din tumori din tumori,, profunda, nepulsatila, difuza, nocturnaprofunda, nepulsatila, difuza, nocturna

    si dimsi dim

    .

    .la trezire, se disipeaz dup cteva orela trezire, se disipeaz dup cteva ore

    - poate mima migrena sau cluster headache- poate mima migrena sau cluster headache- determinata de edemul local, distorsiuni de vase, ulterior de HIC- determinata de edemul local, distorsiuni de vase, ulterior de HIC- uneori are valoare localizatorie- uneori are valoare localizatoriec)c) varsaturivarsaturi acomp acomp.. cefaleea (frecvent in tumori de fosa postcefaleea (frecvent in tumori de fosa post..))d). crize epilepticed). crize epileptice focale/general focale/general.;.; 20-30%20-30%;; nu se trateaz cunu se trateaz cu

    antiepileptice, raspund la anticonvulsivanteantiepileptice, raspund la anticonvulsivante;; ff.. discrete la debutdiscrete la debut

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    Glioblastom multiform (GM) si astrocitom anaplazic (AA)Glioblastom multiform (GM) si astrocitom anaplazic (AA)

    -- 11 /5 din tumori /5 din tumori;; debut 30-40 ani AA; peste 40 (50-60) GMdebut 30-40 ani AA; peste 40 (50-60) GM;;localizare cortex, trunchi cerebral, cerebel, mduva spinriilocalizare cortex, trunchi cerebral, cerebel, mduva spinrii- raport M/F = 2/1- raport M/F = 2/1;; ocazional pot complica sindroame geneticeocazional pot complica sindroame genetice(neurofibromatoza)(neurofibromatoza)- se pot extinde -> la nivelul meningelui (-> proteinorahie- se pot extinde -> la nivelul meningelui (-> proteinorahiecrescuta -100mg, pleiocitoza moderata in LCR 10-100/min)crescuta -100mg, pleiocitoza moderata in LCR 10-100/min)sau catre peretele ventricularsau catre peretele ventricular

    ->-> la nivel spinal => focare radiculare,la nivel spinal => focare radiculare,

    gliomatoza meningealagliomatoza meningealaTumorile cerebrale rar metastazeaza, ele se extind! Cnd totuiTumorile cerebrale rar metastazeaza, ele se extind! Cnd totuimetastazeaza o fac doar inmetastazeaza o fac doar in SNCSNC, extranevraxial insamantare, extranevraxial insamantaredoar iatrogena.doar iatrogena.

    CLINICCLINIC

    EEvolutie rapida (saptvolutie rapida (sapt.. 1-2 luni) 1-2 luni);; simptome de sufsimptome de sufererinta difuzainta difuza;;ccrize epilepticerize epilepticeRMNRMN -- efect de masa important => hernieriefect de masa important => hernieri

    -- in substana alba, posibila extensie prin corpul calos =>in substana alba, posibila extensie prin corpul calos =>ambele emisfereambele emisfere;; infiltrare masivainfiltrare masiva (un emisfer, tot creierul) =>(un emisfer, tot creierul) =>gliomatoza cerebrala => tulbgliomatoza cerebrala => tulb..mentale, crize epilmentale, crize epil.., edem papilar, edem papilar

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    CT scan of glioblastoma multiforme arising from splenium (1)

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    CT scan ofglioblastomamultiforme arisingfrom splenium (2)

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    MRI (T2-weighted) of an anaplastic astrocytoma

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    Brainstem glioma (11)

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    CT scan of gangliogliomaThere was no response to contrast. A tumor cyst and calcification are evident.

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    ASTROCITOMULASTROCITOMUL

    cea mai frecventacea mai frecventa

    la oameni tineri, copii 20-30 anila oameni tineri, copii 20-30 ani grad 1-2 de malignitategrad 1-2 de malignitate;; evoluie lenta, puin vascularizata =>evoluie lenta, puin vascularizata =>au tendina sa formeze chiste (se necrozeaz)!au tendina sa formeze chiste (se necrozeaz)!Se pot ntlni calcificri parialeSe pot ntlni calcificri pariale

    aspectul LCR - normal / uoara proteinorahieaspectul LCR - normal / uoara proteinorahie

    CLINICCLINIC -- crize epileptice -> morfeice (in somn)crize epileptice -> morfeice (in somn)- semne focale tardive (discrete)- semne focale tardive (discrete);; HIC si cefalee (tardiv)HIC si cefalee (tardiv)- modif- modif.. comportcomport.., tulb, tulb.. de personalitate de personalitate loblobul temporalul temporal- semne de piramidalitate deficit motor lobul frontal- semne de piramidalitate deficit motor lobul frontal

    - tulb- tulb.. de limbaj si sensibilitate de limbaj si sensibilitate lobul temporal si parietallobul temporal si parietal- ataxie la copii local- ataxie la copii local.. in cerebel, va detin cerebel, va det.. HIC precoceHIC precoce

    RMNRMN masa difuza cu grad mic de edem, efect de masa localizat masa difuza cu grad mic de edem, efect de masa localizat

    PETPET - hipometabolism pentru glucoza- hipometabolism pentru glucoza

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    MR scansfrom 4patientswith grade2astrocytomaT2-

    weighted

    images (Aand B) and

    FLAIR

    images (C

    and D).

    None of

    thesetumors

    showed

    gadolinium

    contrast

    enhanceme

    nt.

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    Low-grade glioma with edema

    On this MRI scan, the tumor (red arrow) is surrounded by edema (blue arrows), trackinginto the white matter.

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    MRI of childhood supratentorial malignant glioma

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    OLIGODENDROGLIOMULOLIGODENDROGLIOMUL

    localizare in lobii frontal si temporal (40-70%), adesealocalizare in lobii frontal si temporal (40-70%), adeseaprofund in substana albaprofund in substana alba

    1/3 degenereaz malign = oligodendroblastom1/3 degenereaz malign = oligodendroblastom la debut dezvoltare lentala debut dezvoltare lenta sngerare spontana => pseudo AVC (!dg diferenial cu AVC)sngerare spontana => pseudo AVC (!dg diferenial cu AVC) crize epileptice focale / generalizatecrize epileptice focale / generalizate semne de lateralizaresemne de lateralizare

    poate calcificapoate calcifica;; e sensibe sensibilil la chimioterapiela chimioterapie

    EPENDINOMEPENDINOM in ventricul / esutul cerebral adiacentin ventricul / esutul cerebral adiacent frecvent in ventriculul IV la copil => semne de suferina defrecvent in ventriculul IV la copil => semne de suferina de

    fosa posterioara (ataxie), sindrom vestibular, vrsturifosa posterioara (ataxie), sindrom vestibular, vrsturi clinic ~ gliomulclinic ~ gliomul localizarea periventricular trebuie difereniata de limfomullocalizarea periventricular trebuie difereniata de limfomul

    primar !primar ! poate debuta ca sindrom de coada de calpoate debuta ca sindrom de coada de cal

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    CT(noncontrast-enhanced)showing leftfrontaloligodendrogliomaCT of left frontal

    oligodendroglio

    ma showing

    calcification and

    minimal mass

    effect.

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    MRI of cerebellar ependymoma(A) Horizontal view shows the enhancing tumor (red arrows), here with surrounding

    edema (yellow arrows). (B) Sagittal view with the tumor (red arrows) and the occludedfourth ventricle (green arrow).

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    MENINGIOMMENINGIOM-- 20 %20 %;; raport M/F = 2/3 sauraport M/F = 2/3 sau 1/2;1/2; pot insoti alte neoplaziipot insoti alte neoplazii- sporadice sau multiple ca in boala von Recklinhausen- sporadice sau multiple ca in boala von Recklinhausen- localizat pe convexitate sau la baza creierului- localizat pe convexitate sau la baza creierului

    - dezvoltare lenta, nu determina edem -> tumora benigna- dezvoltare lenta, nu determina edem -> tumora benigna-- !!dup operaie progndup operaie progn. f.. f. bun cu condiia ca sngerarea sa fiebun cu condiia ca sngerarea sa fiemica, iar ingrijirea postoperatorie sa fie fmica, iar ingrijirea postoperatorie sa fie f.. BunaBuna-- invadeaza sau erodeaza osul sau detinvadeaza sau erodeaza osul sau det.. reactie osteoblasticareactie osteoblastica

    CLINICCLINIC ((DIFERIT IN FUNCTIE DE LOCALIZARE:DIFERIT IN FUNCTIE DE LOCALIZARE:- sant olfactic => anosmie, compresie NII => atrofie, edem papil- sant olfactic => anosmie, compresie NII => atrofie, edem papil..pe NII contralateral (cnd e mare)pe NII contralateral (cnd e mare)- aripa mica a sfenoidului => paralizie N III, hipoestezie si- aripa mica a sfenoidului => paralizie N III, hipoestezie siparestezie in teritoriul oftalmic al N V ~ sindrom de tromboflebitaparestezie in teritoriul oftalmic al N V ~ sindrom de tromboflebitaa sinusului cavernosa sinusului cavernos-- sindrom apex orbital = N III, IV, Va + IIsindrom apex orbital = N III, IV, Va + II- tuberculum sala- tuberculum salarr=> hemianopsie heteronima temporala=> hemianopsie heteronima temporala- coasa creier => parapareza spastica cu evoluie progresiva- coasa creier => parapareza spastica cu evoluie progresivaRMNRMN coada durala coada durala

    - capteaz omogen substanta de contrast- capteaz omogen substanta de contrast

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    Meningioma cerebellopontine angleAxial MR imaging shows a well circumscribed mass in the right cerebellopontine angle

    that is slightly hyperintense on T2 and isointense to brain on the T1 weighted images.

    Post-contrast images show homogenous and strong enhancement throughout the mass

    and a prominent dural attachment at the base. The mass only deforms the adjacent

    parenchyma without altering the signal and the mass does not extend through the

    internal auditory canal.

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    Olfactory groove meningiomaCoronal T1 post-contrast images (A, B) show a diffusely enhancing mass along the floor

    of the interior cranial fossa with a dural attachment consistent with a meningioma. Axial

    image (C) also shows this mass along the left parasagittal olfactory groove. This mass

    also has resulted in cystic encephalomalacia along the side the mass.

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    Parasagittal meningiomaThis axial MRI shows a diffusely enhancing mass along the left parasaggital vertex with

    a prominent dural attachment along the interhemispheric fissure consistent with a

    meningioma.

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    LIMFOM PRIMARLIMFOM PRIMAR- apare in imunodepresii - SIDA, metastaze- apare in imunodepresii - SIDA, metastaze;; rar congenitalrar congenital- localiz- localiz.. supratentoriala corticalsupratentoriala cortical sau periventricular (sau periventricular (LLCRCR??))

    CARCINOAME METASTATICECARCINOAME METASTATICE1.1. craniene si duralecraniene si durale-- sn, prostata, mielom multiplu (MM), melanom malignsn, prostata, mielom multiplu (MM), melanom malign

    (metastaze cu caracter hemoragic)(metastaze cu caracter hemoragic)- asimptomatice / nu- asimptomatice / nu;; localizare la baza craniului => semne delocalizare la baza craniului => semne de

    nervi cranieni IX, X, XI sau sindrom de hemibaza de craniunervi cranieni IX, X, XI sau sindrom de hemibaza de craniuGarcin (toi nervii cranieni homolateral)Garcin (toi nervii cranieni homolateral)

    2.2. cerebralecerebrale- plmn, sn, MM, tract gastro-intestinal, rinichi, vezica biliara,- plmn, sn, MM, tract gastro-intestinal, rinichi, vezica biliara,

    ficat, tiroida, testicul, ovar (frecvent), prostata, esofag (rar)ficat, tiroida, testicul, ovar (frecvent), prostata, esofag (rar)- clinic => HIC cu evoluie rapidaclinic => HIC cu evoluie rapida;; semne focale discretesemne focale discrete;;

    edem vasogen mareedem vasogen mare;; pot mima dementepot mima demente;; ataxie, crizeataxie, crize

    epilepticeepilepticeTRATAMENTTRATAMENT unica intervenie chirurgicala unica intervenie chirurgicala

    - chimioterapie- chimioterapie- excizia tumorii si a metastazelor- excizia tumorii si a metastazelor

    - doua apropiate => excizie- doua apropiate => excizie

    - mulitple chimioterapie paleativa- mulitple chimioterapie paleativa

    MRI of patient with

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    MRI of patient withsmall cell lung cancermetastatic to theparietal duraAxial T1-weighted CE

    MRI section near thevertex. The enhancing

    right parietal mass elicits

    vasogenic edema into

    the brain, displacing it.

    The mass is flattened

    against the inner table ofthe calvarium. This

    behavior is typical for a

    dural-based mass. The

    calvarium is normal here,

    but there is a dural tail

    (red arrow) associatedwith the lesion. Although

    a dural tail was once

    thought to be specific for

    meningioma, it is now

    known to occur with any

    lesion that involves thedura.

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    MRI showing large brain metastasis

    Gadolinium-enhanced MR scan showing a large brain metastasis with considerablesurrounding edema, and two smaller nearly lesions.

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    MRI showingmultiple small

    brain metastasesGadolinium-enhanced MR

    scan showing

    multiple small

    brain metastases

    from melanoma.

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    MRI (gadolinium-enhanced) of multifocal primary central nervous system

    lymphoma

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    CARCINOMATOZA MENINGEECARCINOMATOZA MENINGEE insamantarea cu celule neoplazice a meningelui si ainsamantarea cu celule neoplazice a meningelui si a

    ventriculilorventriculilor;; adenocarcadenocarc.. mamar, pulmonar, tract gastro-mamar, pulmonar, tract gastro-intestinalintestinal

    clinic cefaleeclinic cefalee;; dureri rahidienedureri rahidiene;; poliradiculoneuropatiipoliradiculoneuropatii-- paralizii multiple de nervi cranieniparalizii multiple de nervi cranieni,, stari confuzionalestari confuzionale LCR proteinorahie crescutaLCR proteinorahie crescuta;; pleiocitoza limfocitarapleiocitoza limfocitara glicorahie scazutaglicorahie scazuta; +/-; +/- metastaze parenchimatoasemetastaze parenchimatoase

    SINDROMUL PARANEOPLAZIC NEUROLOGICSINDROMUL PARANEOPLAZIC NEUROLOGIC

    frecvent in neoplasmele pulmonare, dar si ovariene, de tractfrecvent in neoplasmele pulmonare, dar si ovariene, de tractgastro-intestinalgastro-intestinal

    apare prin Ac anti Ag tumorale ce determina reacii incrucisateapare prin Ac anti Ag tumorale ce determina reacii incrucisatecu componente neuronale in SNC si SNPcu componente neuronale in SNC si SNP

    ex. encefalita hipocampica -> tulbex. encefalita hipocampica -> tulb.. de comportde comport.. si de memoriesi de memorie

    degenerescenta cerebeloasa (sindrdegenerescenta cerebeloasa (sindr.. cerebelos paraneoplazic)cerebelos paraneoplazic) sindrom opsoclonus mioclonussindrom opsoclonus mioclonus leucoencefalita multifocala progresivaleucoencefalita multifocala progresiva mielopatii paraneoplazice necrozantemielopatii paraneoplazice necrozante stiff person syndrom afecteaza structurile CAstiff person syndrom afecteaza structurile CA sindrom senzitivo motorsindrom senzitivo motor