118 , ~ , " ' .. I ',0" fIl C)dlficari ~·uplh,.renl'ar ," • ' e .,:j ,sinte.zA intrateca]:L de g .J... It.

facuUotLV phm;H::ILo~hIP~raJbU~]!norah~S ., te verba de a ce S"I,lcrnle, In spcelnl si:nlezll. [~G tncep~ c~ 6 (?)Iu~u In utm" 'lUoare es in SM; deoare u se rernarcA
1

DldRllQfitle
j

, .,.

..'

. _,

UlM[385]: se~slbd,dar m:sp~lfic, rnoci~oariaserni1n 'rb substanto de rotltrast) ~I n evoluuc ll1onoJbzlCA SC.e~jd~fi~ ~eZl'i11l (pInel) f1~n~Il~:est sens) dsocrcrl de plbci Inill,nol $1 mar vechl eo In SM (Ii,p.Se5Cstlldule In '~.

(:~ft

:s

DilI81iO.~iic . nici un !lOU puseUSDUO noun agmvare Ten'lpia

• I.

infecdc ~n IlIHeccdente,Eil~lou~Ii?ic ,~iexomenL€R. 6 luni dupaeveniment dlDgnosuoul ponte fi consldernf .slgur dacn pdnElfn eca .

nO. mill apare

s:tudH contrelate f • , . ,.' ", .•.... ' .' tirr de 5 zil,e terupleeu deze mart dccortJzon: 5.00 mg me:tJlprednfsolonJZl, mp hnu.noglobuUne.5 x Sglzi oinci zfle.consecutlv [310] .. " puis -(erap'ic cu Endoxan 1 g In intervale de 3 pdnQ fa 4 s~pta~a~1 . . 'I ~. • eontinu~rea terDp'i~j im1Jnosupresiv~ (de ~x. pl~s~~ere.zA) ~IDfuncJle~~,evo Eva/Uric: monefaztea, mOl18.htate pend Is 30%, In cazut deptts.lfll pnmei saptmninl progn -

" •

.,

IIU c"dstA

:!:CUI va fi bun

Diagnostic sU'Plimentar

~.,' ~ .,.. ..' . de derivati boola rara, mal freeventa in Japoma. se pare m reJaue cu admmlstrarea . Dxichinoleinici (anddi.oreie) si pare aJ ,avea legatura. eu tin factor ,constilUtion.ai ..' .' ." AnaTomic pa.l%gica: demielinizare sirnetrica a: eailor piramidele, a co.rdoanelor medulare dorsale $1 a nervului optic Tabloul cilnto: inilialtulburarl prcgresive de sensibilitat.c, apo.i det1citel.llotorH. ~itulburari de vedere
Genera ifttJ tt:
• elcc1trl).neu.rografie: .potcntIBlcc;vocatelSMT:

2.4.0.6. Mi(!lQ,optico.l1ellrap~tia subacuta (MO!yS) ,

latente prelungite
eonducere intftrziata. prin caileaferente

R. Kais~r(versiune

2.5. nen patancoplazfce

si eferente

2.S.0.1. GeneralUati

medicala de Vlorica Mihailo'vici)

Deflnitie: sindreerne neurolegiee, care aper In ssoeiere eu 0 formatiunetumarala.;acestesindroame

sunt real~zale p.rin e~ecl'~ledirC?te ale masei tumcrale, nu sunt detenninate de rnefastazeleaceBteia s~,u de npaJritul une: memng~teecrcmcmatcase ClrLiJjicQre~ pana .~c1lJmnu existao c]asi.fi~a~e unanimacce~tatAj ..se ru,liIizeaza. Unele Y8riante de elasfficore in fundl1! de aspectele anatomo-clinlce sau dupa. nnttoorpu prezenti II daslficarlilB .ciinico·ano.tomlctJ, [14,262,6391:
t

nu

}.> ,o~eier ~i nervi.. ~rah.ieni.: degenere.scenfA par.al?eoplazic!i a. eerebclului s'ndr opsoclonus-rnlO. clonus, encefaht.a: hmb1ca~encefahta de ttunchl cerebral .. nevrita op tic~ ,.t", ... o~ , paraneoplDzic~ . ' ,""t re Inopane ,.. mAduva spinarii: mielita, mielopa.ne necrozanta" bonIa neuromllui Illotor sind ,. - S . . ,> sistern .~ervos perife~ic $j gangllon] ~pin.ali:, neurbnopatie SUb~CUtABerom. tiffinan pohn"uropatlesc~z'tlva, ne?rOpatle '8,l,ltonoma, pohrBd~cul~U1acufa) monQnevrit~ mUI~,zonal~(NSS)! pJexalA, neuropaH .. p,llIncreatice (tumorl ale celulelor insulere ,active 1endocrl ) ,Iple~ $1 ~evnta .. Inpa.raprotcmemh - n, neur;opatil penferice ,., [onctiune neuromuscoI ara 51i mU$ch:f: neuro-miotonie s· d :" r'fftIIl'n'IAftlm.n7.iIUI ml" ZI~t'lI.,. ,'. ·ta ' t~· " '. ., II] fOOl I.alll·b· ert Ea ton, o ~ uecrozant atm~1 mrepatte'jn cazul sindrom'lduicarclnoid '. ," (1•• mcafC dupa ,pr:ezcnta anticorpUor [533,535,639]

•-bronsie eueelule . mic~ deadt rnrA

'c:eluie miel, Ga.nllmllr ~i do endoml.!~ru

m SI1ffn;nn

Colorsren eclul'elor ncurnnnle

~"8. kUn. I oint &1 din 'C 11II~ 11"'lUnnnh~

t.es tu~

Co'omr~n
. Innp!l~I.ar

(. Innpl0·
1""l11iI1)

531\0"

\'ClICulolor Ilnnpli~ pl""tain~ d h:J.I.~tul1\ :prc:'ll1nplh:~ f' 2

l)ynumt1' cndtlt"n07~ ule

en

lIM.~

--*

Yo: inl\inlcle primitol' PDCIe:n,i I clue 5- u deserts ncesleshtdIORfue fUJd-ne:urmr /-nud (Jr-anIJbod)J (Dnl~rorplnucleftr.onl1-nt!uFOnnJIl, • GlrIi-Pu,.k1nj JJ-anribody ( ntl't"Orpl.",unli ..celule Pllrkinj~) • Azrl:lJtj~'r-'mlibod (llnlirorpi cchdc Purk Il1JIJ) 'Wlltage gatl:d calcium ehnrtnels (m1ticorpl ecntm cnonlolo:r de ,onia[u voUnj d~pel1dcntc) fR\'l.ienta.1l,.5-2% dinlJe PJcend L eu CUmoril!~1 mn~ tkovn11ln ee ttl ,eottlinoumc bronsh: Utai ~logi~ mentor I fi'ea entn pi! ienlilor au nf«liwU l1It'Urologu:'CI' primnro nu ICldm. d impIOnla,lologle.i neurole Ice ~d 0 lifer&' ,tU'mOruUI peciOci ln ser ~i/so:u LCR. unton sindrom Intlnmntof in Lea ell u.son-m ~Jqluoc:ttl.r! hlperaJbumlnorohlc u'Soruil ~i eventual sinte ISO in· tt\'" -lit allure.uze, in speciaJ D metssluelor( ~ p. t~B) !In'~ unci l11®.in_gite (~p. 140)

]20

-.

TiraJ!!!'P2.51t.i7J] . rii~ V.··· .ent tuJIlO tu1eatA rreC' d ut;MdI flIlDlJi. " e se pas· late) io e ." ill deQamee..•in PJtogem .. ~e (sau .rn.uSCU ' . • , . • • rin dclofosfi. amid!, ll. " ... za.t1 "'-~:teva 'zil<e pan.a.la. in1i1 ~~. tum.0UI,L .. an.tige.I'iH~lor.&D. po.l'_' r 'ie;!) ·e'.~n~nlaz:ice. .... . ~J")' cortizo deeuT's, d a mtbe;enelor IOglce I- • . le!,;4 "d ceni asupnl sjndr08IDcl0.f'DtllW," " .~. dulD[QI'I. ·c. (.'-.P·d Ina debut. (i.Itl_ . oomitetl1:A a lOmanl a.u • -Udl!1ia imJln05Dpre5l. v IDdmo te ira~a.t inistra cureztCpa con

ra primLID

_; '\fi elite .

stl! de.

er.

1Il1cnWu,-.. , nu are 0 Je8.cued partaltD nolIO"1 ' ..

.'

• ~ l.ca dn. ro f~ lC :.....

eat se descopera
.l!!"
Q

·..,n.tiCOtpl lmpotri. '·V.B

, 11 Inucnta

rna,

~ eupaD_panp.B s;unP':i uneOri efec~ nu este area dupl I~ s~u a.ruC11·'lunAPinlla 7 :emIrate fmrrte proml~e. ',I '.anmeoplazIce pot ap . 'este de ,CirC3 2 :anI '
Em. JuP..'"6 ~ prognosm::. "

~gJob!illnl

_. r

i v, ~

p' ._ .' .. el'or nelliro~oglce'.

!~e.a:eza.,

a .',

.. ' ..-) $~ unpl,........·

ofU

~~;:;a
.

'. '~igUl'

"

_. de-ta deseOpentea

"

ani)

.', rli tumo

~ ... medlJ suplllVl .. ' im~c.ifaJilo limbiclJ ". . __ ftnl. celul,e mici,Ca de onar .... .Ger.Ufl1i1:41i p41.6911 .' ..' . cu c:elule mid:> Ca-pu!m • mmori primare: Ga-bron$]C ~ RDJinom, nemublasfom ... ' . • autoli.Dtioorpi~ H'!'ANNA-I. tJSlJ!en.t in combinalie ~ neuro. lO.gI. '-ceasoclate ..Hu apar tree .1 "'emporale aspeetul - . t .cd • carre coame e II. ~ '.A.!:rnOtml!patOogI ..__ a~li~e a hip.ocampului eu extlDdere ' - ,-'
s: ." .

sm~,~lini ~!p.·adesroperirea wm

:roac:ro.scopic poate fi ~ineconcludem,( .'. gli . za reactiva: 5i noduli IDlcrog ~a l, In ...a.e • microscopic pierdere regiona'l:l de neurom cu ._ 10 ' .. ' P=l "iSculare de limfoeite $1 macr~fag;~ . ". _.. ' , ". -exWa. rupocampul $i ~gdala); modifican • dirtributir: sunt afecfalllo~u tem~rali (partea, ~ . ponii ba.zaH Si In dlellcefal • ~ in g1ruruI cingWar_ in omtexW in5uJar ~ fro~~azal, ~dwa~, anxietate si tulburm de s~nm, Ta.blou didi&: debut SUbllcut tU ar~tarea m~mo~el d~ ~co • "'. evoluria ulterioara eeazional ataxte la agilape si oonfuzie. psihou pamnou1a. ha[ucma1ll, depres1e •. In .. ,. ' !CIS, ~ erize foca1e complexle~ tuJburm ale tennoreglb1l • lSociere Crecventacu. Deufoparia senziuv.a. subacutJ. ' Diagnostic supli.mentar ,. .• con.firmue de.anticf)'rpi Hru~/A A-I in ser silsau LeR " Lea facuItativ pleiocitozl u~oara, hiperalbumiootahie. si sintezl !gGmtratecala • EEG: in faza. mcipi·entaadesea necencludenta, in evolutla uiterioata modificari generate, oeazional manifestari de. focu, wtusi fara cDmplexe perlodice cain encetalita eu herpes simplex • WI: modificari au semnalehiperintense temporale sao cu loealizare frontall in imaginile poniieraie in 1'2; leziLmilepot fi unUatera~el$i mai Jar bilatemle; uneori lezjuni.le sunt sub forma unor pIOr:.§C iDlocuitoare de spaliu care DU preiau. SlIbsWlla de. contrast
,.0 .•

IIII.CrDs.COpiCuu

Ii li · filtr- t

IJWgrrastic dIjerenlia/

(~can ~

?IC1"r.~lA~ etJpreeadereencefalitA
~a.to~
In

IR.M:, eu preluare de .rubstanp. de contrast' dar (OJ alte en.. ,£. lit _ ... ' . de YtJUSun lente (Slmv-vinu) 'J ~. sncera ne acute sau

d~terminata. de virusul Herpes-simplex(7

p. 81.)

_iCc. de ~ prin me[ottexa~carm~sli8, ,cito;in~ar:bino~~ ~ll$tie~fei~ tobl$i ram motiificari IlUd; , ._ encefal~patle hepatica ' - paraglllaZ6"preCliJrn SI metabolice prin ~w...'-EncefolztQ de lrunch, cerehrallence/; I"t b I . _ ~ ~ eneefalirei Jimbice- ,.:~~ . lea. . • 'tillli,.... ,uudl8n. UI p1rm.seu1 ven1Jij '1, .. .' ~1I1uutle vanablia: a (.' . ate implicata dar se citeaza: i. ~ Uhn IV SI in olwa inti, ~ , , . ~eznm.dor m trunchru! _ric: vertij! greala.. ataxie, nlsla:rn~ 10 ~.'SUnI loeahzate cele~~ multe 'C3ZUfl .lfiIam:af,C-e • pamh'll'! bulb~ I . 1I1Uarnaroru

demente degenerative(~p., [52) leziun1 ischemi,ce hilateralc v~ulite cerebrlle (-) p. (1) encefalopatie de iradiere cer.eb~: in IR.M in stadiuHnci ient hj-. .' _. .diD lI'IC"!;dlla limbICl,~.evoluUe potaparea proeese e. p .••ve-~ de semna~ asemanatoare celor • enceCalopatii:tulburari de calacterrul:= _ . carepremu substanta,dealDtrast • • •
• ~~~ A

,.,.lnu

_C1IOR~C4;' • • ....1.... In bulemmbu' , ~"~d·~~

tumori p-rimare 6i auto.anticorpi

'.

,-~.nencefaUta

,..:t~

IQ

bora

bi

I.

~:~n"

tu buran de ()culomotricittltel

-1 in seli si/sau, LCR

Dlaglloslic difi,rcflt'lal

. omin rl II tro l lelo ce: potentiute de acuune sernnificativ scszere viteza de conducer senzitfvh (V - din inu t • particip r obI p rceptibila a fibrelor motorii; in potentialele senzitive vocate, ampl tudini diminu to cresterea lotenlei

Gelleralitdl/: turneri primnr ~iauto- nncorprca m nc milia hmbica Anatomle palologlcd~ fn e zuri indi idual confirmare unei degen rari a plexulm mezen eric preeum si n unci infiltrt\r pic .ulut ell hmfo uc ,)ma ro ge Tab/ou clinic: debut subneu c motilitete redUSB II stomacului si intestinelcr (eonstipatte), tulburari circulatorii au hipotenaiune ortost tica, ulterior tulburan neurogene de golire a vezidi Ifl1poten~A si perturbareo reoc,mor pupilore $1 de neomedare
t

2.S.0.S. Neuropatia «utonom«

• pelin surep UI d It· etiologic cu preeadere prin vincristin, cispiatin sau 'UQ(ol (-7 p" 249)1 !ipso. do vitaminn B ..12, tabes dorsal sindrom-Sj6gren • meta rD~C ep'i. urn I c, menigita C8IClnomaloasd (~ Pl. 140)

Diagnostic Jupir'lIIentar

eonflrm reide ntico pi~ u-l -1 in set d.agno tic n uro II tiv ~ p. 303 ~.uj~ICJ.f~'1c dij'erentia'l: insuficienr. autonoma In polineuropaeiile de alta. etiologie, de ex. diabet p. 247), tn atrofiUe multislsternlce (-7 p. 172) mlliutt,,si prognostic:' olftlturi de suferintele tumorale propriu-zise neuropatia CRUZ4 de stop cardiae la pocientii eu sindrom peraneoplazic
ButODOmAeste cea mai

Sindromul opsaclcnus-mioalonus
mort primare ,....." ...... RU

[482'J

A..2 tip IIb aunt mal expuse decit barbntii ll9JJt12 /318). macroscopic aspeele neconcludente! mi~C?P1C: pruti~alm~ifesta ~egenerare ..a K6mer cu prccadcre in SCOQIln cerebe!oasrt GI vemus; mfillmle llmfbcitare pemmscu1a.re m
":-"-t

medalcblaarom

le adultilor Ca-mamer, mai mr Ca-bronsie eu celule mici; la eopii

_Jjllftjr",.

nucleii dinIDti $i in gangU nil b h' g1ioZ4 B subston'l,el rube . lupe rapldl subacut! limp tome or. ~psoclonus= mi$c~ yupide sCurt~ ate globilor CU ti'ecvtnte $1 omplit dini difi ~. 111J~lonus~con 'lI •.m~~~ fulg t d n u$Chi u rtiuna emu cltilor. ' 0 81vel1lJ 1dlzartrie

a climpulul

vizual