! "#

Pathologie vasculaire et inflammatoire cérébrale :

quelques clés pour reconnaître les tableaux les plus fréquents

BB.. HHuussssoonn,, FF.. TToouullggooaatt,, KK.. DDeeiivvaa,, MM.. BBaalluu

PATHOLOGIE VASCULAIRE CEREBRALE !

ACCIDENTS ISCHEMIQUES CEREBRAUX !

$%&&%!'(&)*+*,-%!'%.&!/.01%2-0!&*.&!(.!+*2,!3%!+(!'40-*3%!'43-(&0-5.%!%&!676%!%2!(2&4!2(&(+8!9+!/:(,-&!

3%!+4/-*2/!'(0%2;)<6(&%./%/!-/;)46-5.%/!/%;*23(-0%/!=!.2%!(&&%-2&%!(0&40-%++%8!

>%/!(;;-3%2&/!-/;)46-5.%/!;404?0(.@!AB9$C!3%!+(!'40-*3%!24*2(&(+%!;*2/&-&.%2&!.2!,0*.'%!*0-,-2(+!

'(06-!+%/!B9$!3%!+:%2D(2&8!$%/!(;;-3%2&/!/*2&!E!=!F!D*-/!'+./!;*.0(2&/!5.%!;)%G!+%!,0(23!%2D(2&!%&!

+%.0! '041(+%2;%! %/&! %/&-64%! =! HIJ! KKK! 2(-//(2;%/! 1-1(2&%/8! 9+/! 0%'04/%2&%2&! .2%! ;(./%! -6'*0&(2&%!

3:)46-'+4,-%! ;*2,42-&(+%L! 3%! &0*.?+%/! ;*,2-&-D/! %&! 3:4'-+%'/-%8! 9+/! '%.1%2&! /%!6(2-D%/&%0! 3(2/! +%/!

'0%6-%0/! M*.0/! 3%! 1-%! '(0! 3%/! ;*21.+/-*2/! )46-;*0'*0%++%/! %&! 04;-3-1(2&%/! /.01%2(2&! ;)%G! .2!

2*.1%(.N24! /(2/! )-/&*-0%! *?/&4&0-;(+%! '(&%2&%8! O%1(2&! ;%! &(?+%(.! ;+-2-5.%L! +:%@(6%2! '0%6-%0! %/&!

/*.1%2&! +:4;)*,0(')-%! &0(2/D*2&(2%++(-0%8! P++%! 6*2&0%! .2%! )<'%04;)*,42-;-&4! &0-(2,.+(-0%! =! ?(/%!

;*0&-;(+%L!&<'-5.%6%2&!?-%2!+-6-&4%!%&!'(0D*-/!3%/!(2*6(+-%/!3%!+(!1(/;.+(0-/(&-*2!%2!O*''+%08!O(2/!

+%/! -2D(0;&./! 4&%23./L! +(! &*6*3%2/-&*64&0-%! 6*2&0%! .2%! )<'*3%2/-&4! ?-%2! +-6-&4%! +*;(+-/4%! =! .2!

&%00-&*-0%! (0&40-%+8! Q*.0! 3%/! +4/-*2/! 3%! '+./! '%&-&%! &(-++%! *.! +*0/5.%! +:%@(6%2! %/&! 3%! 04(+-/(&-*2!

'04;*;%L! +%! 3-(,2*/&-;! %/&! '+./! 34+-;(&! %&! +:9RS! (1%;! /45.%2;%/! 3%! 3-DD./-*2! %/&! (;&.%++%6%2&! +(!

64&)*3%! +(! '+./! /%2/-?+%8! 92-&-(+%6%2&L! +%/! /-,2%/! '%.1%2&! 7&0%! 3-/;0%&/! /.0! +%/! /45.%2;%/! %2!

'*2340(&-*2!TH! %&!TE! A343-DD40%2;-(&-*2! ;*0&%@I/.?/&(2;%!?+(2;)%L! &*./!3%.@! %2!)<'*/-,2(+!TH! %&!

)<'%0/-,2(+! TEC! (+*0/! 5.:-+/! /*2&! 41-3%2&/! %2! 3-DD./-*2! /*./! D*06%! 3:.2! D0(2;! )<'%0/-,2(+! 3%!

/</&46(&-/(&-*2!(0&40-%++%!(1%;!;).&%!3.!;*%DD-;-%2&!3%!3-DD./-*28!B'0U/!HKNHJ!M*.0/L!+(!3-DD./-*2!/%!

24,(&-1%! (+*0/! 5.%! +%/! /45.%2;%/! TH! %&! TE! 6*2&0%2&! 3%/! (2*6(+-%/! D0(2;)%/8! P2! ;(/! 3%! +4/-*2!

4&%23.%L! ;%/! (2*6(+-%/! '%.1%2&! 41*+.%0! 1%0/! .2%! ;(1-&(&-*2! (1%;! 3%/&0.;&-*2! 3.! '(0%2;)<6%8! 9+!

! "#

$%&'($!)*+,-&'!./!01)$+'&2/*3!45!'.+3&2/*/(!3*!3&6&($!&/($+/$!7$!3*!8-/$!&'90:6&:$!;.&!)-.++*&(!<(+$!$/!

+*))-+(!*=$9!./$!)+-3&,:+*(&-/!9*)&33*&+$!-.!3*!)+:'$/9$!7$!6*9+-)0*2$'!90*+2:'!7$!3&)&7$'>!?3!)$.(!

,*9&3&($+!3*!6&'$!$/!:=&7$/9$!7$!3*!3:'&-/!'.+!3$'!&6*2$'!&/&(&*3$'!$(!'$!+$/,-+9$!'-.=$/(!*)+@'!5#A5B!

C-.+'!7D:=-3.(&-/>!ED$%&'($/9$!7D./$!*(($&/($!7.!,*&'9$*.!9-+(&9-')&/*3!'$6F3$!*=-&+!./$!,-+($!=*3$.+!

)+:7&9(&=$! ):C-+*(&=$! )-.+! 3D:=-3.(&-/!6-(+&9$>!G33$! 'D$%)+&6$!7@'! 3$'! )+$6&$+'! C-.+'! $/! 7&,,.'&-/!

*=$9! ./! 01)$+'&2/*3! :($/7.! 7$! 3*! 9*)'.3$! &/($+/$! *.! (+-/9! 9:+:F+*3! H)*+,-&'! C.';.D*.! F.3F$I>!J$'!

'(+.9(.+$'! )$.=$/(! $/'.&($! 'D*(+-)0&$+>! ED&/($+)+:(*(&-/! 7$! 9$'! 6-7&,&9*(&-/'! $'(! +*))-+(:$! *.!

)0:/-6@/$!7$!7$'(+.9(&-/!*%-/*3$!*=$9!6-+(!9$33.3*&+$!'.+=$/*/(!K!7&'(*/9$!7$!3*!8-/$!3:'&-//$33$!

H7:2:/:+$'9$/9$!L*33:+&$//$I>!

J0$8!M#!N!7$'!$/,*/('!*=$9!O?J!/:-/*(*3P!3*!)+:'$/(*(&-/!93&/&;.$!$'(!+$(*+7:$>!E$!)3.'!'-.=$/(P!./!

(*F3$*.! 7D0:6&)3:2&$! 9:+:F+*3$! &/,*/(&3$! ,*&(! +:*3&'$+! ./$! ?QR! 9:+:F+*3$! 6-/(+*/(! ./$! 3:'&-/!

&'90:6&;.$!7D*33.+$!':;.$33*&+$!7*/'!3D0:6&')0@+$!9-/(+-3*(:+*3!*.!7:,&9&(!6-($.+>!

!

J0$8! 3D$/,*/(! )3.'! 2+*/7P! 3*! )+:'$/(*(&-/! 93&/&;.$! 0*F&(.$33$! $'(! 3*! '.+=$/.$! F+.(*3$! 7D./! 7:,&9&(!

/$.+-3-2&;.$P!3$!)3.'!'-.=$/(!./$!0:6&)*+:'&$!*=$9!*(($&/($!,*9&*3$P!*''-9&:$!7*/'!B#N!7$'!9*'!K!7$'!

9-/=.3'&-/'>!

ED&6*2$+&$! K! +:*3&'$+! $/! .+2$/9$! $'(! ./$! ?QR! *=$9! 7$'! ':;.$/9$'! 45! $(! 4SP! ./$! ':;.$/9$! 7$!

7&,,.'&-/!$(!7$'!':;.$/9$'!7D*/2&-?QR!7.!)-312-/$!7$!T&33&'!$(!7$'!=*&''$*.%!7.!9-.>!ED&/C$9(&-/!

7$!2*7-3&/&.6!/D$'(!)*'!/:9$''*&+$!$/!.+2$/9$>!J$+(*&/$'!:;.&)$'!+:*3&'$/(!./!'9*//$+!*=$9!&/C$9(&-/!

7D&-7$! $(! */2&-'9*//$+>! U-.'! /D*=-/'! )*'! 3D$%):+&$/9$! 7$! 9$! (1)$! 7D$%)3-+*(&-/! $(! /-.'! /-.'!

3&6&($+-/'!7-/9!*.%!7-//:$'! ,-.+/&$'!$/! ?QR>!ED&6*2$+&$! +$/'$&2/$!'.+! 3*!)+:'$/9$!7D./$! 3:'&-/!

&'90:6&;.$!7*/'!./!($++&(-&+$!*+(:+&$3!$(!+$90$+90$!7$'!3:'&-/'!*+(:+&$33$'>!E$'!':;.$/9$'!7$!7&,,.'&-/!

6-/(+$/(!3$'!3:'&-/'!&'90:6&;.$'!7@'!3*!)0*'$!*&2.V!H7*/'!3$'!B!)+$6&@+$'!0$.+$'!*)+@'!3D&/'(*33*(&-/!

7.!7:,&9&(I!*=$9!./$!'$/'&F&3&(:!7$!"WN!$(!./$!'):9&,&9&(:!7$!5##N>!X*/'!3$'!6<6$'!9-/7&(&-/'P!3$'!

':;.$/9$'!93*''&;.$'!7D?QR!-/(!./$!'$/'&F&3&(:!7$!BYN!$(!./$!'):9&,&9&(:!7$!5##N>!E$'!($++&(-&+$'!

7:)$/7*/(! 7.! '1'(@6$! 9*+-(&7&$/! '.+(-.(! 3$! 9:+:F+*3! 6-1$/P! '-/(! 3$'! )3.'! '-.=$/(! *(($&/('>! Z/$!

*(($&/($! 3&6&(:$! *.%!/-1*.%!9*.7:'! $(! 3$/(&9.3*&+$'! $'(! +$(+-.=:$!7*/'!)+@'!7D./!9*'! '.+! (+-&'>!E$'!

3:'&-/'! *+(:+&$33$'! '-/(! )+:'$/($'! 7*/'! *.!6-&/'! B#N!7$'! 9*'>! ED*+(:+&-2+*)0&$! +$'($! 3D$%*6$/! 7$!

+:,:+$/9$! K! +:*3&'$+! '&! 3$'! ':;.$/9$'! 7D*/2&-?QR! /$! )$+6$(($/(! )*'! ./$! */*31'$! )$+(&/$/($! 7$'!

3:'&-/'! *+(:+&$33$'! -.!;.*/7! *.9./$! 3:'&-/! *+(:+&$33$! /D$'(! =&'&F3$>!G/! &/(+*9:+:F+*3P! 3*! ($+6&/*&'-/!

7$'! *+(@+$'! 9*+-(&7$'! &/($+/$'! $(! 3$! '$26$/(! &/&(&*3! 7$'! *+(@+$'! '13=&$//$'! H'$26$/(!R5I! '-/(! 3$'!

'$26$/('! *+(:+&$3'! 3$'! )3.'! '-.=$/(! *(($&/('! HWMN! 7$'! 9*'I>! E$'! 3:'&-/'! 7.! (+-/9! F*'&3*&+$! $(! 7$'!

! "#

$%&'%()!*+%+,%$-()!./)&+%0(1%()!)/2&!%(&%/13+()!4$2)!##5!4()!*$)6!78$&&(02&(!4()!$%&'%()!*+%+,%$-()!4(!

9/:(2!(&!.(&0&!*$-0,%()!()&!%$%(!(&!)(1-(!-8$%&+%0/;%$.<0(!.(%9(&!.$%=/0)!48(2!=$0%(!-(!40$;2/)&0*6!7$!

%(*<(%*<(!):)&+9$&0>1(!4(! -+)0/2)!$%&+%0(--()!$1!203($1!*(%30*$-!()&! ?1)&0=0+(!.$%! -$!./))0,0-0&+!2/2!

(@*(.&0/22(--(!4(!40))(*&0/2!$%&+%0(--(!A(2!.$%&0*1-0(%!(2!*$)!4B$&&(02&(!4(!-$!=/))(!./)&+%0(1%(C6!

7()! *$1)()! 48$**04(2&)! 0)*<+90>1()! *+%+,%$1@! ADEFC! 4(! -8(2=$2&! )/2&! &%')! 3$%0+()! (&! .$%=/0)! G!

-8/%0;02(! 4(! %+*0403()H! ?1)&0=0$2&! 4/2*! 12! ,0-$2! .(%9(&&$2&! 4(! -()! 04(2&0=0(%! %$.04(9(2&6! 7$!

-/*$-0)$&0/2!(&! -8$).(*&!4()! -+)0/2)!$%&+%0(--()!30)1$-0)+()!(2!DIJ!(&K/1!(2!$%&+%0/;%$.<0(!)/2&!4()!

+-+9(2&)!48/%0(2&$&0/2!+&0/-/;0>1()!09./%&$2&)!L!

M!12(!/**-1)0/2!/1!12!4+=(*&!(24/-1902$-!()&!&%')!+3/*$&(1%!4812(!/%0;02(!(9,/-0>1(!(&!12(!

! *$1)(!*$%40$>1(!4/0&!N&%(!)/0;2(1)(9(2&!%(*<(%*<+(6!

! M! 12(! $2;+0&(! &%$2)0&/0%(! 48/%0;02(! 02=(*&0(1)(! (&! */9./)+(! 4(! )&+2/)()! 91-&0.-()! .(1&! )(!

4+3(-/..(%! )1%! -$! ./%&0/2! &(%902$-(! 4(! -$! *$%/&04(! 02&(%2(! $3(*! (@&(2)0/2! $1@! ./%&0/2)!

02&0$-()! 4(! -$! ):-30(22(! (&! 4(! -$! *+%+,%$-(! $2&+%0(1%(6! 7()! $%&'%()! -(2&0*1-/)&%0+()! )/2&!

+;$-(9(2&!$&&(02&()!4$2)!*(!*$)!(@.-0>1$2&!4()!-+)0/2)!0)*<+90>1()!=%+>1(2&()!$1!203($1!4()!

2/:$1@! ;%0)! *(2&%$1@6! O-1)0(1%)! $;(2&)! 02=(*&0(1@! /2&! +&+! %(&%/13+)H! -(! .-1)! =%+>1(2&! +&$2&!

l’herpes virus varicellae APQPC!30%1)!4(!-$!3$%0*(--(!(&!41!R/2$6!

! M!-()!*%0&'%()!40$;2/)&0>1()!4(!40))(*&0/2!)/2&!*-$))0>1()!L!-(!4+*/--(9(2&!4(!-802&09$H!-8$).(*&!

4(! 4/1,-(! -190'%(H! 12(! )&+2/)(! (==0-+(! /1! -(! 4+3(-/..(9(2&! 4812! .)(14/$2+3%:)9(6! S2!

$2&+*+4(2&!4(! &%$19$&0)9(!4$&$2&!.$%=/0)!4(!>1(->1()! ?/1%)!()&! )/13(2&! %(&%/13+!(2!*$)!4(!

40))(*&0/2!*(%30*$-(!$-/%)!>1812(!40))(*&0/2!02&%$*%T20(22(!)1%30(24%$0&!.-1&U&!$1!*/1%)!4812!

(==/%&6!

M! -(! ):24%/9(! 4(! J/:$VJ/:$! )(! 4+=020&! .$%! -$! */2)&0&1&0/2! .%/;%())03(! 4812(! )&+2/)(!

!,0-$&+%$-(! 3/0%(! 4812(! /**-1)0/2! 4()! &(%902$0)/2)! 4()! *$%/&04()! 02&(%2()! (&! 4()! )(;9(2&)!

!020&0$1@! 4()! ):-30(22()! (&! 4()! *+%+,%$-()! $2&+%0(1%()6! S2! %+)($1! 3$)*1-$0%(! */--$&+%$-! )(!

!4+3(-/..(!./1%!*/2&/1%2(%!-$!R/2(!.$&</-/;0>1(!$3(*!4(!2/9,%(1@!.(&0&)!3$0))($1@!30)0,-()!

!4$2)!-$!%+;0/2!4()!2/:$1@!;%0)!*(2&%$1@6!7$!):9.&/9$&/-/;0(!*-020>1(!()&!=$0&(!020&0$-(9(2&!

4(!91-&0.-()!$**04(2&)!0)*<+90>1()!&%$2)0&/0%()!.-1)!>1(!4812!4+=0*0&!2(1%/-/;0>1(!,%1&$-!2/2!

%+;%())0=6!W$2)! -$!9$?/%0&+!4()!*$)H! -8+3/-1&0/2!)(! =$0&!.%/;%())03(9(2&!3(%)! -$!*/2)&0&1&0/2!

4812!4+=0*0&!9/&(1%!)+3'%(!$3(*!$&&(02&(!*/;20&03(6!

! M!-$!4%+.$2/*:&/)(!91-&0.-0(!.$%!XXY!-(!%0)>1(!48DEF!*<(R!-8(2=$2&6!D.%')!-(!.%(90(%!+.0)/4(!

! 48DEFH!(2! -8$,)(2*(!4(! &%$0&(9(2&H! -(!%0)>1(!4(!%+*0403(!()&!4(!Z[5!4$2)! -()!#X!G!X\!9/0)!

! )103$2&)! $3(*! 4()! )+>1(--()! (2! .$%&0*1-0(%! */;20&03()6! 7$! 3$)*1-/.$&<0(! $))/*0+(! G! -$!

! 4%+.$2/*:&/)(! $! 12! $).(*&! .%/*<(! 4(! *(--(! 41! ):24%/9(! 4(!J/:$VJ/:$6! 7()! )&+2/)()! )(!

! */2)&0&1(2&! .%/;%())03(9(2&! .$%=/0)! 4')! -8T;(! 4(! X! $2)6! 7$! )1%3(0--$2*(! .$%! 4/..-(%!

! "#

! $%&'()%*'+,'! -,%.,$! /,! /0$,)$,%! 1,(! ,'2&'$(! 3! 24%$! %+(56,! /789:! ,$! /,! .,$$%,! ,'! -1&),! 6'!

! $%&+$,.,'$!-&%!$%&'(26(+4'(!%0-0$0,(!&2+'!/,!($&;+1+(,%!1&!<&()614-&$=+,>!

?'!24')$+4'!/6!)4'$,@$,!)1+'+56,!,$!/7&'4.&1+,(!;+414A+56,(!-&%$+)61+B%,(C!/7&6$%,(!)&6(,(!-16(!%&%,(!

/789:! /,! 17,'2&'$! (,%4'$! %,)=,%)=0,(! D16-6(! 0%E$=0.&$,6@! /+((0.+'0C! .&1&/+,(! .0$&;41+56,(C!

.0'+'A+$,(>>>F>!

G,! %+(56,!/,! %0)+/+<,!/,(!89:!/,! 17,'2&'$! ,($! 0$%4+$,.,'$! 1+0! 3! 170$+414A+,>! 91! ,($! 2&+;1,! ,'! )&(!/,!

<&()614-&$=+,!$%&'(+$4+%,!/74%+A+',!+'2,)$+,6(,!&14%(!567+1!,($! +.-4%$&'$!,'!)&(!/7&$$,+'$,!<&()61&+%,!

-%4A%,((+<,!)4..,!/&'(!1,!(E'/%4.,!/,!H4E&IH4E&>!

G,!%+(56,!/,!(056,11,(!,($!)4'(+/0%0!)4..,!01,<0!&<,)!,'!-&%$+)61+,%!6',!&$$,+'$,!.4$%+),!/02+'+$+<,!

/&'(!JKL!/,(!)&(>!

!

!

THROMBOSE VEINEUSE CEREBRALE

!

G,(! $=%4.;4(,(! <,+',6(,(! )0%0;%&1,(! DMN:F! 4'$! 6',! 2%056,'),! ,($+.0,! 3! OCKPQROO! OOO! )=,S!

17,'2&'$>! :,11,I)+! ,($! <%&+(,.;1&;1,.,'$! (46(! ,($+.0,! )&%! /,(! 24%.,(! )1+'+56,(! .+',6%,(! '4'!

,@-14%0,(!%&/+414A+56,.,'$!(4'$!-%4;&;1,.,'$!'4'!/+&A'4($+560,(>!86!)4'$%&+%,C!),%$&+'(!$&;1,&6@!

)1+'+56,(!(4'$!(0<B%,(!&<,)!6'!%+(56,!),%$&+'!/,!(056,11,(!',6%414A+56,(!/02+'+$+<,(>!G70)=4A%&-=+,!

)0%0;%&1,! &<,)! 0$6/,! ,'! /4--1,%! )461,6%! ,'! -0%+4/,! '04'&$&1,! ,$! 179TH! &<,)! &'A+49TH!<,+',6(,!

-,%.,$$,'$!/7,@-14%,%!1,(!,'2&'$(!/,!2&U4'!(+.-1,C!2+&;1,!,$!'4'!+'<&(+<,>!

V6,156,!(4+$!17*A,!/,!17,'2&'$C!1,(!.&'+2,($&$+4'(!)1+'+56,(!1,(!-16(!)1&((+56,(!(4'$!/,(!$%46;1,(!/,!1&!

<+A+1&'),! -46<&'$! &11,%! W6(567&6! )4.&! ,$! /,(! )4'<61(+4'(>! X,(! $&;1,&6@! /7=E-,%$,'(+4'! +'$%&!

)%*'+,'',!46!/,(!/02+)+$(!',6%414A+56,(!/Y+'($&11&$+4'!;%6$&1,!.&+(!&6((+!/,(!)0-=&10,(!-,%(+($&'$,(!

+(410,(!-,6<,'$!(,!<4+%!)=,S!1,(!-16(!A%&'/(>!!

:=,S!1,(!,'2&'$(!/,!.4+'(!/,!R!&'C!170)=4A%&-=+,!$%&'(24'$&',11&+%,!&<,)!/4--1,%!)461,6%!.4'$%,!1,!

$=%4.;6(! (46(! 24%.,! /76',! +.&A,! =E-,%0)=4AB',! ,'! -&%$+)61+,%! ;+,'! <+(+;1,! &6! '+<,&6! /6! (+'6(!

14'A+$6/+'&1!(6-0%+,6%,!(6%!/,(!)46-,(!2%4'$&1,(>!?'!/4--1,%C!17&;(,'),!/,!216@!&6!'+<,&6!/76'!(+'6(!

<,+',6@! )4'2+%.,! 1,! /+&A'4($+)>! G70$6/,! -&%,')=E.&$,6(,! -,6$!.4'$%,%! 6',! S4',! =E-,%0)=4AB',!

/7+'2&%)+((,.,'$>!G,!()&'',%!-,6$!Z$%,!)4'$%+;6$+2C!1,!$=%4.;6(!&E&'$!6',!/,'(+$0!<&%+&;1,!(,14'!(4'!

&')+,'',$0>![,'/&'$!1&!-%,.+B%,!(,.&+',C!+1!&--&%&\$!(-4'$&'0.,'$!=E-,%/,'(,>!G76$+1+(&$+4'!/76',!

2,'Z$%,! 01&%A+,! ,($! '0),((&+%,! -46%! 1,! /+220%,')+,%! /,(! ($%6)$6%,(! 4((,6(,(! 14%(! /,! 170$6/,!/6! (+'6(!

(&A+$$&1!(6-0%+,6%!46!/,(!(+'6(!1&$0%&6@>!8-%B(!+'W,)$+4'!/,!)4'$%&($,C!1,!$=%4.;6(!'4'!%,=&6((0!,($!

(+1=46,$$0!-&%!1&!-%+(,!/,!)4'$%&($,!/,(!-&%4+(!/6%&1,(!%0&1+(&'$!1,!)1&((+56,!]!(+A',!/6!/,1$&!^>!X,(!

10(+4'(! +()=0.+56,(! <4+%,! =0.4%%&A+56,(! <414'$+,%(! ;+1&$0%&1,(! (4'$! 1&! )4'(056,'),! /,! 1&!

! "#

$%&'()'*+,! -..+*! *'/$! 0&1*+/$+*! 23/*! 45! 6! 778! 2+*! 93*! 2+! :;<! 2+! .=+/>3/$,! ?+@&*! .'93.A*3$A'/*!

>&'/$'B03&A1$3.+!03&3*3CA$$3.+!0&'9%+!2@!D+&$+E!'@! $+(0'&'B'99A0A$3.+! A/>1&A+@&+!D'A&+!91&+)+..+@*+!

>'/$! &+9%+&9%+&! @/+! $%&'()'*+! 2@! *A/@*! *3CA$$3.! '@! 2=@/! *A/@*! .3$1&3.,! F/+! 3$$+A/$+! $%3.3(AG@+!

)A.3$1&3.+!2'A$!>3A&+!&+9%+&9%+&!@/+!$%&'()'*+!2+*!D+A/+*!91&1)&3.+*!A/$+&/+*H!2+!.3!D+A/+!2+!I3.A+/!

'@!2@!*A/@*!2&'A$,!?3!0&1*+/9+!2=@/!103/9%+(+/$!01&A91&1)&3.!+*$!+/!&300'&$!3D+9!.3!CJ/+!3@!&+$'@&!

D+A/+@E,! K+! /'()&+@E! >3@E! 0'*A$A>*! +$! >3@E! /1C3$A>*! *'/$! 9'//@*! +/! *93//+&! +E0.AG@3/$!

G@=39$@+..+(+/$! .=LMN! 3D+9! *1G@+/9+! 2=3/CA'LMN! D+A/+@*+! 3003&3A$! *@01&A+@&+,! ?+*! .1*A'/*!

A*9%1(AG@+*! *'/$!(A+@E!DA*@3.A*1+*!03&! .3! *1G@+/9+!2+!2A>>@*A'/!+$! .+*! .1*A'/*!%1('&&3CAG@+*!03&!

@/+!*1G@+/9+!19%'!2+!C&32A+/$!:O,!<=+*$!+/!0%3*+!3AC@P!Q7!0&+(A+&*!R'@&*SH!G@3/2!.+!$%&'()@*!+*$!

A*'A/$+/*+! +/!:T! +$! %U0'A/$+/*+! +/!:O!G@+! .3! *1G@+/9+!2=3/CA'LMN!D+A/+@*+! +*$! .3! 0.@*!@$A.+! +/!

('/$&3/$!@/+!3)*+/9+!2+!>.@E!23/*!.+!*A/@*!$%&'()'*1,!?+!V!*AC/+!2@!2+.$3!W!0+@$!J$&+!&+$&'@D1!30&X*!

A/R+9$A'/! 2+! C32'.A/A@(,! -/! 0%3*+! *@)3AC@PH! .+! $%&'()@*! +*$! *0'/$3/1(+/$! %U0+&A/$+/*+! +/! :T!

9'((+!+/!:O,!

?+*! >39$+@&*! >3D'&A*3/$*! .3! *@&D+/@+! 2=@/+! :;<! *'/$!(@.$A0.+*,! ?+*! 0.@*! 9'//@*! *'/$! .=A/>+9$A'/!

Q*A/@*A$+H!(3*$'Y2A$+*! +$!(1/A/CA$+*SH! .3! 21*%U2&3$3$A'/H! .=A/*@>>A*3/9+! &1/3.+H! .+! $&3@(3$A*(+H! @/+!

$@(+@&! +$! .+*! 3/'(3.A+*! %1(3$'.'CAG@+*! Q9'3C@.'03$%A+*H! 0'.UC.')@.A+H! 3/1(A+! 9%&'/AG@+! +/!

03&$A9@.A+&S,!

?=@$A.A*3$A'/!2=@/!$&3A$+(+/$!3/$A9'3C@.3/$!)A+/!G@+!2+!0.@*!+/!0.@*!@$A.A*1!9%+Z!.=+/>3/$!&+*$+! $&X*!

21)3$$@!9'(0$+!$+/@!2+!*'/!&A*G@+!+$!2+*!2A>>A9@.$1*!2+!*'/!1G@A.A)&3$A'/!*@&$'@$!9%+Z!.+*!0.@*!R+@/+*!

03$A+/$*,!

?+!0&'/'*$A9!2+*!:;<!+*$!9.3**AG@+(+/$!0.@$[$!(3@D3A*,!F/!\C+!2+!*@&D+/@+!0.@*!$3&2A>H!.=3$$+A/$+!

.A(A$1+!2=@/!*A/@*!.3$1&3.!'@!*AC('Y2+!+$!.=3)*+/9+!2+!.1*A'/!03&+/9%U(3$+@*+!*'/$!2+*!1.1(+/$*!2+!

)'/!0&'/'*$A9,!K+*!&19A2AD+*!*'/$!0'**A).+*!+/!>'/9$A'/!2=@/+!93@*+!9%&'/AG@+!2+!:;<,!

!

!

MALFORMATIONS VASCULAIRES

!

?+*!(3.>'&(3$A'/*! D3*9@.3A&+*! 91&1)&3.+*! 2+! .=+/>3/$! 9'/*$A$@+/$! @/! C&'@0+! 2+! 03$%'.'CA+*! &3&+*H!

D3&A1+*! +$! 3D+9! *'@D+/$! 2+*! 93&39$1&A*$AG@+*! 2+! >&1G@+/9+! +$]'@! 2=1D'.@$ADA$1! 2A>>1&+/$+*! 2+! 9+..+*!

9'//@+*! 9%+Z! .=32@.$+,! -..+*! 0+@D+/$! 9'(0'&$+&! @/! *%@/$! 3&$1&A'D+A/+@E! '@! *+! .'93.A*+&! *@&! .+!

*U*$X(+!D+A/+@EH! .=3$$+A/$+!2@! *U*$X(+!3&$1&A+.! Q3/1D&U*(+!3&$1&A+.S! 1$3/$! &3&+! 6! .=\C+!012A3$&AG@+,!

?+*! (3.>'&(3$A'/*! 3D+9! *%@/$! 3&$1&A'D+A/+@E! 0+@D+/$! *A1C+&! 23/*! .+! *+9$+@&! *'@*! 3&39%/'Y2A+/!

Q(3.>'&(3$A'/!3/1D&U*(3.+!2+!.3!D+A/+!2+!I3.A+/SH!23/*!.+!*+9$+@&!10A2@&3.!Q(3.>'&(3$A'/!2+*!*A/@*!

! "#

$%&'%()! *%! $'+,! -.! ,./0.%&! 1+0&'2'&.+/345'0.%(! 65'-7*&5'01*+! '&08&1*9.1+.%,.! 21'-.):! ;.,!

5'-7*&5'01*+,! 9.1+.%,.,! &.<&*%2.+0! -.,! /'9.&+*5.,! .0! -.! ,4+$&*5.! 9.1+.%(! 580'58&1=%.!

/8&8>&*7'/1'-! 6,4+$&*5.! $.! ?0%&<.@A.>.&):! ;.,! '+*5'-1.,! 9.1+.%,.,! $.! $89.-*22.5.+0! ,*+0! $.,!

9'&1'+0.,!$.!-'!+*&5'-.!=%B1-!7'%0!>1.+!/*++'C0&.:!

;'!<'55.!$1'<+*,01=%.!$.!5'-7*&5'01*+,!9',/%-'1&.,!9'&1.!'9./!-BD<.!$.!-B.+7'+0!'%!5*5.+0!$.!,'!

$8/*%9.&0.:!E+!'+08+'0'-F!-.,!5'-7*&5'01*+,!'+89&4,5'-.,!$.!-'!9.1+.!$.!G'-1.+!.0!$.,!,1+%,!$%&'%(!

2.%9.+0! H0&.!$1'<+*,01=%8.,:!I! -'!28&1*$.!+8*+'0'-.!.0!2.+$'+0! -.,!J!2&.51K&.,!'++8.,!$.!91.F! .+!

2-%,!$.!/.,!J!042.,!$.!5'-7*&5'01*+,F!*+!2.%0!9*1&!$.,!5'-7*&5'01*+,!'&08&1*9.1+.%,.,!21'-.,:!L-%,!

0'&$F!*%0&.!-.,!5'-7*&5'01*+,!'&08&1*9.1+.%,.,!21'-.,F!-.,!$1'<+*,01/,!$.!/'9.&+*5.!.0!$B'+89&4,5.!

'&08&1.-!,*+0!2*,,1>-.,:!;.,!,1<+.,!/-1+1=%.,!&898-'+0!-'!5'-7*&5'01*+!,*+0!8<'-.5.+0!$1778&.+0,!,.-*+!

-BD<.!$%!2'01.+0M!;'!$87'1--'+/.!/'&$1'=%.!.,0!/'&'/08&1,01=%.!$.!-'!28&1*$.!'+08!.0!+8*+'0'-.:!E+0&.!J!

5*1,! .0! J! '+,F! %+.! 5'/&*/&D+1.! 2-%,! *%! 5*1+,! ',,*/18.! N! %+.! 34$&*/823'-1.! .,0! -.! 085*1+! $.,!

$8,*&$&.,! 34$&*9.1+.%(! ,./*+$'1&.,! N! -'! 5'-7*&5'01*+:! O3.P! -B.+7'+0! 2-%,! <&'+$F! -.,! ,1<+.,!

+.%&*-*<1=%.,!$871/10'1&.,!.0Q*%!-.,!/*+9%-,1*+,!,*+0!2-%,!3'>10%.-,:!

;B8/3*<&'231.! .0! -BRST! 2.&5.00.+0! $.! 7'1&.! -.! $1'<+*,01/! $.! /.,!5'-7*&5'01*+,! .0! $B'22&8/1.&! -.!

2'&.+/345.! /8&8>&'-:! ;B'&08&1*<&'231.! 2&8/1,.! -B'&/310./0%&.! $.! -'! -8,1*+! .0! 2&8/1,.! ,.,!

/'&'/08&1,01=%.,! 385*$4+'51=%.,:! E--.! &.2&8,.+0.! '/0%.--.5.+0! <&D/.! '%(! .5>*-1,'01*+,F! -'!

2*,,1>1-108!038&'2.%01=%.!-'!2-%,!71'>-.:!!

!

1. La malformation anévrysmale de la veine de Galien (MAVG)

;'! 5'-7*&5'01*+! '+89&4,5'-.! $.! -'! 9.1+.! $.! G'-1.+! 6TIUG)! .,0! -'! 5'-7*&5'01*+! 9',/%-'1&.!

/8&8>&'-.!-'!2-%,!7&8=%.55.+0!$1'<+*,01=%8.!N!-'!28&1*$.!'+08!.0!+8*+'0'-.:!E--.!,.!$89.-*22.!$'+,!

-'! 71,,%&.! /3*&*V$1.++.! .0! ,.,! '778&.+/.,! '&08&1.--.,! ,*+0! 2&1+/12'-.5.+0! -.,! '&0K&.,! /3*&*V$1.++.,!

2*,08&1.%&.,!6>&'+/3.,!$.,!'&0K&.,!/8&8>&'-.,!2*,08&1.%&.,)!'9./!2'&7*1,!%+.!'-15.+0'01*+!N!2'&01&!$.!

-B'&0K&.!/8&8>&'-.!'+08&1.%&.:!W.%(!'+<1*'&/310./0%&.,!2.%9.+0!,.!9*1&!M!-'!7*&5.!/3*&*V$1.++.!=%1!.,0!

-'!2-%,!/*52-.(.F!.,0!,*%9.+0!&.+/*+0&8.!$'+,!-.,!7*&5.,!$.!&898-'01*+!2&8/*/.!'-*&,!=%.!-'!7*&5.!

5%&'-.!.,0!51.%(!0*-8&8.!'9./!$.,!,3%+0,!5*1+,!+*5>&.%(:!;.!$&'1+'<.!9.1+.%(!,.!7'10!2'&!-'!9.1+.!

58$1'+.!$%!2&*,.+/823'-.!=%1!.,0!-.!2&8/%&,.%&!.5>&4*-*<1=%.!$.!-'!9.1+.!$.!G'-1.+:!;.!,1+%,!$&*10!

.,0!3'>10%.--.5.+0!'>,.+0!.0!.,0!&.52-'/8!2'&!%+!,1+%,!7'-/*&1.-!=%1!&.X*1+0!-.!01.&,!2*,08&1.%&!$%!,1+%,!

,'<100'-!,%28&1.%&:!!

E+!'+08+'0'-F!-.!$1'<+*,01/!,.!7'10!-.!2-%,!,*%9.+0!'%!0&*1,1K5.!0&15.,0&.!$.!<&*,,.,,.:!;B8/3*<&'231.!

! "#

$%&'()! *&)! +$,-)! ,&./0%-1&)! 2)! 3,! 3+-&)!$.2+,&)! 4+'*.)! )&! ,((+1()! 2*! '(%+4+1$)! 5)&'(+/*3)6! 7,!

8%($)!2+')!)&!(,9*)'')!/%(()4:%&2!;!3,!2+3,','+%&!5)+&)*4)!9*+!4)!2(,+&)!:,(!*&)!4'(*/'*()!'*<*3,+()!

/%(()4:%&2,&'! ,*! 4+&*4! 8,3/%(+)36! 7)! 2%::3)(! /%*3)*(! $%&'()! 3)4! 83*=! +&'(,3.4+%&&)34! '*(<*3)&'4!

/%&8+($,&'! 3)! 2+,-&%4'+/! 2)! $,38%($,'+%&! 5,4/*3,+()6! >)4! ,&%$,3+)4! /.(.<(,3)4! 2%+5)&'! ?'()!

()/0)(/0.)4! /,(! 3)*(! )=+4')&/)! )4'! *&! .3.$)&'! $,@)*(! 2)! :(%&%4'+/! :.@%(,'+86! 7)! ()')&'+44)$)&'!

/,(2+,9*)! )4'! ,*44+! +$:%(',&'! ;! .5,3*)(6! 7ABCD! 8E',3)! :(./+4)! ,*! $+)*=! 3)4! 3.4+%&4!

:,()&/0F$,')*4)46! G&! :.(+%2)! &.%&,',3)H! 3A./0%-(,:0+)! '(,&48%&',&)33,+()! ()'(%*5)! 3)4! $?$)4!

.3.$)&'4! $,38%($,'+846! 7ABCD! )4'! 3A)=,$)&! 3)! :3*4! 4)&4+<3)! :%*(! 3,! ()/0)(/0)! 2)4! 3.4+%&4!

:,()&/0F$,')*4)4!)'!2%+'!?'()!4F4'.$,'+9*)$)&'!(.,3+4.)6!>)4!4.9*)&/)4!,=+,3)4!IJ!)'!IK!4:+&!./0%H!

*&)!4.9*)&/)!2)!2+88*4+%&H!*&)!,&-+%BCD!5)+&)*4)!:)($)'')&'!2)!(.,3+4)(!*&!<+3,&!+&8%($,'+8!2,&4!

*&! 2.3,+! 2A)=,$)&! (,+4%&&,<3)! :%*(! /)4! &%*5),*=! &.4! 2%&'! 3,! 8%&/'+%&! /,(2+,9*)! )4'! 4%*5)&'!

:(./,+()6!L&)!MCD!2*!:%3F-%&)!2)!N+33+4!)4'!.-,3)$)&'! +&'.()44,&')!:%*(!.5,3*)(! 3,!/%$:%4,&')!

,('.(+)33)!2)!3,!$,38%($,'+%&6!7,!:%/0)!5)+&)*4)!,::,(,O'!)&!0F:%4+-&,3!IJ!)'!IK6!7)4!83*=!(,:+2)4!

9*+! F! /+(/*3)&'! :)*5)&'! -.&.()(! 2)4! ,('.8,/'4! '(14! +&')&4)46! 7)! '(%&/! <,4+3,+()! ,::,(,+'! .3,(-+! )'!

'%('*)*=6! 7)! 4+&*4! 8,3/%(+)3! )&'()! 3,! :%/0)! 5)+&)*4)! )'! 3)! 4+&*4! 4,-+'',3! 4*:.(+)*(! )4'! 4%*5)&'! 2)!

4+'*,'+%&! :3*4! 0,*')! 9*)! 3)! 4+&*4! 2(%+'6! 7)! '%(/*3,(! )'! 3)4! 4+&*4! 3,'.(,*=! @*49*A,*=! -%38)4! 2)!

@*-*3,+()4!4%&'!.3,(-+46!7,!4.9*)&/)!2)!2+88*4+%&!$%&'()!3)4!:%44+<3)4!3.4+%&4!2A+4/0.$+)!/.(.<(,3)!

,+-*)!:,(8%+4!2)!2+,-&%4'+/!:3*4! 4*<'+3! 4*(! 3)4! 4.9*)&/)4! 4:+&!./0%6!M!/)'')!:.(+%2)!2)!2+,-&%4'+/!

&.%&,',3H! 3A,('.(+%-(,:0+)! &A)4'! :,4! &./)44,+()! 4+! ,*/*&! '(,+')$)&'! +$$.2+,'! 2)! 3,! $,38%($,'+%&!

&A)4'!)&5+4,-.6!M:(14!3,!:.(+%2)!&.%&,',3)H! 3A,4:)/'!2)!3,!$,38%($,'+%&!()4')!+2)&'+9*)6!P,(!/%&'()!

2)4! 4+-&)4! 2)! 4*(/0,(-)! 0F2(%5)+&)*4)H! ,<4)&'4! ;! 3,! &,+44,&/)H! 2%+5)&'! ?'()! ()/0)(/0.46!M+&4+! *&!

)&-,-)$)&'!2)4!'%&4+33)4!/.(.<)33)*4)4H!9*+!'(,2*+'!3,!4',4)!5)+&)*4)!/.(.<)33)*4)H!)4'!5+4*,3+4.!4*(!

*&)!4.9*)&/)!4,-+'',3)!IJ6!B3!&A,!,*/*&!(,::%('!,5)/!3,!',+33)!2)!3A)/',4+)!5)+&)*4)!)'!&A)4'!:,4!3+.!;!

*&)!/%$:()44+%&!2)!3A,9*)2*/!2)!QF35+*46!B3!&A)4'!:,4!3)!4+-&)!2A*&)!$,38%($,'+%&!2)!R0+,(+!'F:)!B!

,44%/+.)6!G&!4*4!')&'%(+)3H!*&!.3,(-+44)$)&'!2)4!5)&'(+/*3)4!)4'! '(14!8(.9*)&'6!QA+3!()4')!$%2.(.H!/)!

4+-&)!&A)4'!:,4!+&9*+.',&'6!P,(!/%&'()!*&)!2+3,','+%&!8(,&/0)!)'S%*!4)!$,@%(,&'!(,:+2)$)&'!,5)/!*&)!

,//.3.(,'+%&! 2)! 3,! /(%+44,&/)! 2*! :.(+$1'()! /(T&+)&! 4+-&)! 3A)=+4')&/)! 2A*&)! 4',4)! 5)+&)*4)! 4.51()!

,5)/! (+49*)! 2)! 3.4+%&4! /.(.<(,3)4! )'! 2A0F:)(')&4+%&! +&'(,! /(T&+)&&)6! 7,! &%($,3+'.! 2)! 4+-&,3! 2*!

:,()&/0F$)!/.(.<(,3!)4'!5.(+8+.)!,+&4+!9*)! 3,!:(%-()44+%&!2)! 3,!$F.3+&+4,'+%&6!7A,('.(+%-(,:0+)!)4'!

(.,3+4.)!5)(4!#UV!$%+4!2)!5+)!,*!$%$)&'!%W!2.<*')!3)!'(,+')$)&'!)&2%5,4/*3,+()!2)!3,!$,38%($,'+%&!

(.,3+4.! 2,&4! 3)! $?$)! ')$:46! RA)4'! 3A)=,$)&! 2)! (.8.()&/)! :%*(! .',<3+(! ,5)/! :(./+4+%&!

! "#

$%&'()*&+,-)./,.0+/!1/! $&!2&$3*+2&.)*'4! &55+6,)/+! $/7! 3$08!90)!:!,)+,0$/'.! /.! 6;&$0/+! $&!90&$).6!1/7!

;*)/7!1/!1+&)'&(/!;/)'/08!,6+6<+&$=! >$! /7.! )'1)75/'7&<$/!5*0+!16,)1/+!1/7!2*1&$).67!10! .+&)./2/'.!

/'1*;&7,0$&)+/=!!

!

2. Les malformations des sinus duraux (MSD)

?/7!2&$3*+2&.)*'7!1/7!7)'07!10+&08!@ABCD!+/5+67/'./'.!$&!EF2/!2&$3*+2&.)*'!;&7,0$&)+/!7/!+6;6$&'.!

/'!'6*G'&.&$=!H$$/7!7*'.!5$07!+&+/7!90/!$/7!2&$3*+2&.)*'7!&'6;+:72&$/!1/!$&!;/)'/!1/!I&$)/'=!H$$/7!

7*'.!3*+26/7!1%0'/!$&+(/!5*,-/!;/)'/07/!&77*,)6/!J!1/!20$.)5$/7!7-0'.7!&+.6+)*G;/)'/08!J!3$08!$/'.!

7%*0;+&'.! 1&'7! $&! 5&+*)! 10! 7)'07!2&$3*+26=! ?&!2&K*+).6! 1/! ,/7!2&$3*+2&.)*'7! )'.6+/77/! $/7! 7)'07!

.+&'7;/+7/74!7)(2*L1/7!/.!$/!.*+,0$&+=!?/!+)790/!/7.!$&!70+;/'0/!1%0'/!.-+*2<*7/!1/!$&!2&$3*+2&.)*'!

5*0;&'.!&55&+&M.+/!.+F7!.N.!/.!/'.+&M'&'.!0'/!)7,-62)/!;/)'/07/!6./'10/=!!

?/!1)&('*7.),!5/0.!O.+/! 3&).! /'!&'.6'&.&$! 70+! $&!;)70&$)7&.)*'!1%0'/!2&77/!-:5*6,-*(F'/!1/! 3*+2/!

(+*77)F+/2/'.!.+)&'(0$&)+/!7).06/!1&'7!$&!3*77/!5*7.6+)/0+/!/'!&++)F+/!10!;/+2)7!/.!+&..&,-6/!J!$&!10+/!

2F+/=!B*'!;*$02/!/7.!;&+)&<$/=!B*'!6,-*7.+0,.0+/!5/0.!O.+/!-6.6+*(F'/!/'! +&55*+.!&;/,!1/7!P*'/7!

,)+,0$&'./7!/.! .-+*2<*76/7=!?%>QA!3R.&$/!/7.! )25*+.&'./!J!+6&$)7/+!/'!,*25$62/'.!5*0+!5/+2/..+/!

1/!5+6,)7/+!$/!1)&('*7.),!/.!1/!+/,-/+,-/+!1%6;/'.0/$$/7!$67)*'7!,6+6<+&$/7=!?&!2&$3*+2&.)*'!&55&+&M.!

,*22/!0'/!5*,-/!;/)'/07/!(6&'./!/.!,)+,0$&'./!1/!$&!3*77/!5*7.6+)/0+/4!.:5)90/2/'.!,/'.+6/!5&+!$/!

.*+,0$&+=!S'!.-+*2<07!5/0.!O.+/!;)7)<$/!7*07!$&!3*+2/!1%0'/!P*'/!1/!7)('&$!-6.6+*(F'/!-:5/+)'./'7/!

/'!TU=!!

?&!ABC!5/0.!O.+/!1)&('*7.)906/!5$07!.&+14!J!90/$90/7!2*)7!1/!;)/!@VG#!2*)7!/'!2*:/''/D!1/;&'.!

$%/8)7./',/! 1%0'/! 2&,+*,+W')/=! S'! .&<$/&0! 1%-:5/+./'7)*'! )'.+&! ,+W')/''/! 2*16+6/! 5/0.! O.+/!

+6;6$&./0+!@)++).&<)$).64!,*';0$7)*'74!&0(2/'.&.)*'!10!XYD=!H'!,&7!1/!.-+*2<*7/!&)(0Z!1/!$&!ABC4!0'!

7:'1+*2/!1/!,*'7*22&.)*'!5/0.!&55&+&M.+/!&;/,!5&+3*)7!0'/!6;*$0.)*'!1+&2&.)90/=!?%>QA!2*'.+/!

0'!7)'07!10+&$!.+F7!6$&+()!&0!');/&0!1/!$&!3*77/!5*7.6+)/0+/!&;/,!0'/!&../)'./!3+690/'./!10!.*+,0$&+4!

,&+&,.6+)7.)90/! 1/7! 3*+2/7! (+&;/7=! ?/7! 7.+0,.0+/7! 5&+/',-:2&./07/7! /'! +/(&+1! 7*'.! +/3*0$6/7! /.!

,*25+)26/7=!S'!/'(&(/2/'.!1/7!.*'7)$$/7!,6+6</$$/07/7!/.!0'/!1)$&.&.)*'!;/'.+),0$&)+/!1*);/'.!O.+/!

+/,-/+,-67=!?/7! $67)*'7!,6+6<+&$/7!,*'7.).0/'.!0'!6$62/'.! 3*'1&2/'.&$!10!5+*'*7.),!'/0+*$*()90/=!

?%&+.6+)*(+&5-)/!/7.!)25*+.&'./!,&+!/$$/!5+6,)7/!&0!2)/08!$/!1+&)'&(/!1/!$&!2&$3*+2&.)*'!&)'7)!90/!

,/$0)!10!,/+;/&0=!H$$/!/7.!0'!5+6&$&<$/!3*'1&2/'.&$!J!.*0./!16,)7)*'!.-6+&5/0.)90/=!

Y$&77)90/2/'.4! $/! 5+*'*7.),! 1/7! ABC! 6.&).! ,*'7)16+6! ,*22/! .+F7! +67/+;6=! [,.0/$$/2/'.! 1/7!

6;*$0.)*'7! 3&;*+&<$/7! 7*'.! 1*,02/'.6/7! /.! $/! 16;/$*55/2/'.! 1/7! .+&)./2/'.7! /'1*;&7,0$&)+/7! *'.!

! "#

$%&'()!*+,'-.(/&%&!0%!*%1%2(&3!

!

!

3. Les anomalies veineuses de développement (AVD)

4%)! ,2/',.(%)!1%(2%5)%)!*%!*-1%./$$%'%26! 789:;! )/26!52%!1,&(,26%! %'<&=/./>(?5%!*5!*&,(2,>%!

1%(2%5@3!A()6/./>(?5%'%26B!%..%!%)6!0/2)6(65-%!*%!1%(2%)!*(.,6-%)!*/26!.%)!$,&/()!)/26!-$,())(%)3!4%)!

,&6C&%)B!.%)!0,$(..,(&%)!',()!,5))(!.%!$,&%20D='%!1/()(2!*%!.+,2/',.(%!)/26!2/&',5@3!4+89:!%)6!.%!

)%5.!)=)6C'%!*%!*&,(2,>%!1%(2%5@!*5!)%06%5&!0-&-<&,.!/E!%..%!)(C>%3!FD%G!.+%2H,26!(.!H,56!&%6%2(&!?5%!

.,! '()%! %2! -1(*%20%! *+52%! 89:! %)6! H/&65(6%! %6! 2%! $%56! %@$.(?5%&! ,5052! )='$6I'%! 7),5H! 0,)!

$,&6(05.(%&)!J!)D526!)%!*&,(2,26!*,2)!.K89:B!6D&/'</)%!*%!.K89:;3!!

L5&!52! )0,22%&! ,1%0! (2M%06(/2!*+N/*%B!/2!1()5,.()%! .%! &%D,5))%'%26!*%!$.5)(%5&)!$%6(6%)! )6&5065&%)!

1,)05.,(&%)!0/21%&>%,26!1%&)!52%!1%(2%!*(.,6-%B!%..%O'P'%!)%!*(&(>%,26!)/(6!1%&)!.%!)=)6C'%!1%(2%5@!

$&/H/2*!/5!,5!0/26&,(&%!1%&)! .%!0/&6%@!0-&-<&,.3!Q2!NRSB!.%)!)-?5%20%)!),2)! (2M%06(/2!'/26&%26! .,!

1%(2%! *%! *&,(2,>%! )/5)! H/&'%! *+52! D=$/)(>2,.! H&,20! .(2-,(&%! /5! 05&1(.(>2%! 6&,2)0-&-<&,.3! 8$&C)!

(2M%06(/2!*%!T,*/.(2(5'B!.%)!'5.6($.%)!1%(2%)!&%D,5))-%)B!)(65-%)!*,2)!.,!)5<)6,20%!<.,20D%!&-,.()%26!

52%!(',>%!%2!H/&'%!*+-6/(.%!?5(!)%!*&,(2%!*,2)!.,!1%(2%!0/..%06&(0%!.,&>%!%6!52(?5%3!F+%)6!,)$%06!%)6!

0,&,06-&()6(?5%!*+52%!89:!%6! .+,&6-&(/>&,$D(%! %)6! (256(.%! $/5&! 0/2H(&'%&! 0%! *(,>2/)6(03!4+,<)%20%!

*+,&6C&%! ,HH-&%26%! *(.,6-%! $%&'%6! *+-0,&6%&! .%! *(,>2/)6(0! *(HH-&%26(%.! *%! ',.H/&',6(/2! ,&6-&(/O

1%(2%5)%3!4,!./0,.(),6(/2!*%)!89:!%)6!$/5&!UVW!*%)!0,)!,5!2(1%,5!)5)6%26/&(%.3!Q..%)!2+%@()6%26!$,)!

,5!2(1%,5!*%!.,!'/%..%3!:%)!H/&'%)!'5.6($.%)!*+89:B!)5$&,!%6!(2H&,!6%26/&(%..%)!/5!<(.,6-&,.%)B!/26!

-6-!&,$$/&6-%)3!

:%! 2/'<&%5)%)! ,2/',.(%)! /26! -6-! 6&/51-%)! ,))/0(-%)! X! 52%! 89:3! 4%)! $.5)! H&-?5%26%)! )/26! .%)!

0,1%&2/'%)B! <%,50/5$! $.5)! &,&%'%26! *%)! ',.H/&',6(/2)! 1%(2%5)%)! *%! .,! 6P6%! %6! *5! 0/5! %6! *%)!

,2/',.(%)! *%! >=&,6(/23! L(! 52! >%)6%! 0D(&5&>(0,.! %)6! %21(),>-! $/5&! .+%@-&C)%! *+52! 0,1%&2/'%B! .%!

0D(&5&>(%2! */(6! P6&%! $&-1%25! *%! .,! $&-)%20%! *%! .+89:3!Q2! %HH%6! .+,<.,6(/2! *%! .+89:! %26&,Y2%! 52!

(2H,&065)!1%(2%5@!*/26!.%)!0/2)-?5%20%)!$%51%26!$,&H/()!P6&%!*&,',6(?5%)3!!

!

4. Les malformations artério-veineuses (MAV)

4%)! ',.H/&',6(/2)! ,&6-&(/O1%(2%5)%)! 7S89;! $%51%26! )%! 1/(&! X! 6/56! Z>%! ,1%0! 52! &()?5%!

D-'/&&,>(?5%! ('$/&6,26! ?5(! H,(6! .%5&! >&,1(6-3! Q..%)! )/26! X! .+/&(>(2%! *%! W[O\[!]! *%)! D-'/&&,>(%)!

(26&,0-&-<&,.%)!*%!.+%2H,263!F%)!',.H/&',6(/2)!)%!*-H(2())%26!$,&!.+%@()6%20%!*+52%!0/''52(0,6(/2!

%26&%!,&6C&%!%6!1%(2%3!L(!0%66%!0/''52(0,6(/2!)%!H,(6!*(&%06%'%26B!/2!$,&.%!*%!H()65.%!,&6-&(/1%(2%5)%!

7^89;3!L(!52!2(*5)!0/'$/)-!*%!'5.6($.%)!$%6(6)!1,())%,5@!%)6!*-1%./$$-!%26&%!.%!1%&),26!,&6-&(%.!%6!

! "#

$%! &%'()*+! &%,*%-./! $%! +%'0%! 1%! 234! %(+! %05$6789! :%(! 0)$;6'0)+,6*(! %.,(+%*+! 1)*(! $)! ;6((%!

56(+8',%-'%!<600%!=! $>8+)?%!(-(! +%*+6',%$/!<%++%!1%'*,@'%! $6<)$,()+,6*!8+)*+!0)A6',+),'%9!B%(!;6'0%(!

0-$+,5$%(!(6*+!18<',+%(!5$-(!;'8C-%00%*+!<D%E!$>%*;)*+!FGH/IJK!C-%!<D%E!$>)1-$+%!F"!JK9!B%!',(C-%!

1%!'8<,1,&%!D806'')?,C-%/! ,1%*+,C-%!=!<%$-,!1%! $>)1-$+%! FILMJ!1%!',(C-%!)**-%$K/!%(+!1>)-+)*+!5$-(!

0%*)N)*+! C-%! $>%(58')*<%! 1%! &,%! %(+! 5$-(! $6*?-%9! :%<,! %.5$,C-%! $%(! 1,(<-((,6*(! )<+-%$$%(! (-'! $)!

'%<D%'<D%!1>-*%!+D8')5%-+,C-%!')1,<)$%!%+!$)!06,*(!18$8+@'%!56((,O$%!56-'!$%!5)'%*<D70%!<8'8O')$9!

B%(!234! 1%! $>%*;)*+! 5%-&%*+! (%!0)*,;%(+%'! 1@(! $)! 58',61%! *86*)+)$%! 5)'! 1%(! (705+P0%(! &)',8(!

<600%!-*%! ,*(-;;,()*<%! <)'1,)C-%/! -*%! 85,$%5(,%/! -*%!0)<'6<'Q*,%! %+! 1%(! 18;,<,+(! *%-'6$6?,C-%(9!

R-%$C-%(!<)(!1%!1,)?*6(+,<!)*+8*)+)$!6*+!8+8! ')556'+8(9!B)!0)A6',+8!1%!<%(! $8(,6*(!(%! '8&@$%*+!5)'!

-*%! (-(5,<,6*! 1>D806'')?,%! ,*+')<'Q*,%**%9! B>,0)?%',%! 1>-'?%*<%! %(+! $%! (<)**%'! <8'8O')$! ()*(!

,*A%<+,6*! 1%! <6*+')(+%9! B>)'<D,+%<+-'%! 1%! $)! 234! %(+! 0)(C-8%! 5)'! $>D806'')?,%9! 3! <6+8! 1%!

D75%'1%*(,+8!1%! $>D80)+60%/!1%(!<)$<,;,<)+,6*(! (6*+!5)';6,(!&,(,O$%(9!S*%! ,*6*1)+,6*!&%*+',<-$),'%!

5%-+!T+'%!)((6<,8%!),*(,!C->-*%!D806'')?,%!08*,*?8%9!3!<%!(+)1%/!<>%(+!$>)'+8',6?')5D,%!C-,!56-'')!

;),'%! $%!1,)?*6(+,<9!U*!1%D6'(!1>-*!(),?*%0%*+/! $%! (<)**%'! ()*(! ,*A%<+,6*!06*+'%!-*%!5$)?%!5$-+P+!

D75%'1%*(%! =! $,0,+%(! ;$6-%(9! V%(! <)$<,;,<)+,6*(! (6-&%*+! 1%! 5%+,+%! +),$$%! (6*+! ;'8C-%*+%(9! B%(!

&),((%)-.! )*6'0)-.! 5%-&%*+! T+'%! (56*+)*80%*+! &,(,O$%(! 1-! ;),+! 1%! $%-'! <)$,O'%! )-?0%*+8/! (6-(!

;6'0%!1>D75%'1%*(,+8(!(%'5,?,*%-(%(9!B%!5)'%*<D70%!&6,(,*!5%-+!T+'%!D7561%*(%!6-!)+'65D,C-%!)&%<!

-*%! 1,$)+)+,6*! &%*+',<-$),'%! $6<)$,(8%9! S*%! 1,$)+)+,6*! ?$6O)$%! 8&6C-%! -*%! D71'6<85D)$,%! (6,+!

(%<6*1),'%!=!-*%!D806'')?,%!,*+')&%*+',<-$),'%!(6,+!1-!;),+!1%!+'6-O$%!D71'6&%,*%-.!)((6<,8(9!35'@(!

,*A%<+,6*!1%!<6*+')(+%/!$%!'%D)-((%0%*+!1%!$>%*(%0O$%!1%!$)!234!%(+!,*+%*(%9!B%(!&%,*%(!1%!1'),*)?%!

6*+!-*!<)$,O'%!(-58',%-'!=!<%$-,!1%(!)'+@'%(!*6-'',<,@'%(9!B>WX2!%(+!(7(+80)+,C-%0%*+!'8)$,(8%!56-'!

(,+-%'!$)!234!5)'!')556'+!)-.!),'%(!;6*<+,6**%$$%(!(,!%$$%!%(+!(-5%';,<,%$$%!6-!5)'!')556'+!)-.!E6*%(!

(%*(,O$%(! <600%! $)! <)5(-$%! ,*+%'*%! 1)*(! $%(! $8(,6*(! 5'6;6*1%(! F(8C-%*<%! YG! %*! )<C-,(,+,6*!

&6$-0,C-%K9! B>8+-1%! 1-! 5)'%*<D70%! )&%<! '%<D%'<D%! 1>D75%'(,?*)-.! %(+! ;),+%! 5)'! 1%(! (8C-%*<%(!

ZB3WX!6-!YI!Y[U9!S*%!(8C-%*<%!%*!YI!8<D6!1%!?')1,%*+!06*+'%!1%(!(,?*%(!D806'')?,C-%(!)*<,%*(9!

B>3X2!)'+8',%$$%!;6-'*,+!-*%!<)'+6?')5D,%!1%! $)!234!\!*60O'%!%+!6',?,*%!1%(!581,<-$%(!)'+8',%$(/!

+')A%+(! %+! <)$,O'%!1%(!&%,*%(/!&6$-0%!1-!*,1-(.!B>WX2!;6*<+,6**%$$%/! C-)*1!%$$%! %(+! '8)$,()O$%/! %(+!

5'8<,%-(%! 56-'! $6<)$,(%'! )-! 0,%-.! $%(! '8?,6*(! ;6*<+,6**%$$%(! 5)'! ')556'+! =! $)! 0)$;6'0)+,6*9!

B>)'+8',6?')5D,%! '%(+%! $>%.)0%*! 1%! '8;8'%*<%! 56-'! $>8+-1%! <605$@+%! 1%! $)!2349!U$$%! %(+! '8)$,(8%!

)&)*+!+6-+!?%(+%!+D8')5%-+,C-%9!

S*%!5',(%!%*!<D)'?%!0-$+,1,(<,5$,*),'%!'%?'6-5)*+!<D,'-'?,%*(/!*%-'6')1,6$6?-%(!,*+%'&%*+,6**%$(!%+!

! ""

#$%&'()*#$+,-(,.!,.(!.'-)$&($/0,!+'-#!%*1&2&#!-2,!.(#$(*3&,! ()*#$+,-(&4-,!$%$+(*,!5!6)$4-,!6$.7!8,!

/-(! #,6),#6)*! ,.(! 09'660-.&'2! ('($0,! %,! 0$! :;<! +'-#! +#'(*3,#! 0,! +$(&,2(! %,! ('-(! #&.4-,! %,!

.$&32,=,2(7!

!

5. Les cavernomes

8,.! 6$>,#2'=,.! .'2(! %&$32'.(&4-*.! $>$2(! 09?3,! %,! @A! $2.! %$2.! $-! ='&2.! BC! D! %,.! 6$.7! E,.!

=$01'#=$(&'2.!2,!.'2(!+$.!>&.&/0,.!,2!$#(*#&'3#$+)&,!6$#!0,!10-F!>$.6-0$&#,!G!,.(!(#H.!0,2(7!;>$2(!0,!

%*>,0'++,=,2(! %,! 09IJ:! 6,.! 0*.&'2.K! %'2(! 0$!=$L'#&(*! #,.(,! $.G=+('=$(&4-,K! *($&,2(! 6'2.&%*#*,.!

6'==,! #$#,.7! E,#($&2.! 6$>,#2'=,.! .'2(! 6'23*2&($-FK! .,! #*>*0$2(! %H.! 0$! +#,=&H#,! $22*,! %,! >&,7!

M9$-(#,.! $++$#$&..,2(! N%,! 2'>'O! 6'==,! 09'2(! +#'->*! 0,.! +#'('6'0,.! %,! .-#>,&00$26,! %,.! 1'#=,.!

1$=&0&$0,.! %,! 6,((,! =$0$%&,7! P21&2! 09,F&.(,26,! %,! 6$>,#2'=,.! #$%&'&2%-&(.! $-! %*6'-#.! %9-2,!

#$%&'()*#$+&,!$!/&,2!*(*!='2(#*,!6),Q!%,.!,21$2(.!%,!='&2.!%,!@C!$2.7!!

R2!6$>,#2'=,!,.(!6'2.(&(-*!%9-2!,2.,=/0,!%,!>$&..,$-F!.$23-&2.!%G.(#'+)&4-,.!,(!,6($.&4-,.!.$2.!

(&..-! 2,#>,-F! &2(,#+'.*! ,(! .$2.! $#(H#,! 2'-##&6&H#,7! M,.! .$&32,=,2(.K! %,.! ()#'=/'.,.! +,->,2(!

.-#>,2&#K!%,.!%*+S(.!6$06&4-,.!.'2(!1#*4-,2(.7!8,.!6$>,#2'=,.!.,!#,26'2(#,2(!%$2.!('-(!0,!TUEK!$>,6!

-2,!0'6$0&.$(&'2!+#*1*#,2(&,00,!%$2.!0,.!)*=&.+)H#,.!6*#*/#$-F7!!

T,0'2! 0,! 2'=/#,! %,! 6$>,#2'=,.! (#'->*.! 6),Q! -2! +$(&,2(K! '2! %&.(&23-,! B! (G+,.! %,! =$0$%&,!V! 0,.!

6$>,#2'=,.! =-0(&+0,.K! 4-&! .'2(! 0,.! +0-.! #$#,.K! .'2(! #,26'2(#*.! %$2.! 0,.! 1'#=,.! 1$=&0&$0,.! %$2.!

,2>&#'2!AW!D!%,.!6$.7!R2,!(#$2.=&..&'2!$-('.'=&4-,!%'=&2$2(,!$!*(*!%*='2(#*,!,(!+0-.&,-#.!3H2,.!

'2(! *(*! &%,2(&1&*.! ,2! +$#(&6-0&,#! .-#! 0,! 6)#'='.'=,! X7! 8,.! 6$>,#2'=,.! &.'0*.! .'2(! 1#*4-,==,2(!

(#'->*.!%$2.!0,.!1'#=,.!.+'#$%&4-,.7!

8,.! 6$>,#2'=,.! +,->,2(! .,! #*>*0,#! /#-($0,=,2(! +$#! -2,! )*='##$3&,! &2(#$+$#,26)G=$(,-.,! '-!

&2(#$>,2(#&6-0$&#,K!6,!='%,!%,!#*>*0$(&'2!.,=/0$2(!0,!+0-.!1#*4-,2(!6),Q!09,21$2(7!M$2.!%9$-(#,.!6$.!

-2,! *+&0,+.&,K! +0-.! #$#,=,2(! -2! .G2%#'=,! (-='#$0! $>,6! %*1&6&(! 2,-#'0'3&4-,! +#'3#,..&1! '-! %,.!

6*+)$0*,.! 6)#'2&4-,.! 6'2.(&(-,2(! 0,.! .G=+(S=,.! #*>*0$(,-#.7! P21&2! 0,.! 0*.&'2.! $.G=+('=$(&4-,.!

#,+#*.,2(,2(!,2>&#'2!@CD!%,.!6$.7!!

M$2.! 09-#3,26,!%9-2,!+#*.,2($(&'2!)*='##$3&4-,K! 0,! .6$22,#! .$2.! &2L,6(&'2!='2(#,! 09)*=$('=,! 0,!

+0-.!.'->,2(! &2(#$+$#,26)G=$(,-F!.$2.!+'->'&#!,2!+#*6&.,#! 0$!6$-.,7!P2!%,)'#.!%,!6,!6'2(,F(,K! 0$!

0*.&'2! $++$#$Y(! )G+,#%,2.,K! $..,Q! /&,2! 0&=&(*,K! 6'2(,2$2(! .'->,2(! %,.! 6$06&1&6$(&'2.! ,(! +#,2$2(!

='%*#*=,2(!0,!6'2(#$.(,7!8,!%&$32'.(&6!%&11*#,2(&,0!$>,6!-2,!(-=,-#!2*6,..&(,!0$!#*$0&.$(&'2!%9IJ:7!

M,.!.*4-,26,.!Z@!,(!ZB!TPK!-2,!.*4-,26,!ZB!*6)'!%,!3#$%&,2(!.'2(!&2%&.+,2.$/0,.7!E'==,!%$2.!0$!

+'+-0$(&'2!$%-0(,K!09&=$3,!(G+&4-,!,.(!-2,!0*.&'2!,2!N!6'6$#%,!O7!T'2!6,2(#,!,.(!%,!.&32$0!)*(*#'3H2,!

=&F(,!)G+,#!,(!)G+'.&32$0!,2!Z@!6'==,!,2!ZB![6$06&1&6$(&'2.K!=*()*='30'/&2,\!$>,6!,2!+*#&+)*#&,!

-2!$22,$-!,2!1#$26!)G+'.&32$0!ZB![%*+S(!%9)*='.&%*#&2,\7!R2,!0*.&'2!+,-(!](#,!1'#=*,!%,!+0-.&,-#.!

! "##

$%! &%'! ()*+,-'.! /)0,123'-%'4! 5-16+'1*,! )*%! 73581,+3*! 236963:-%! 8)5+7358%! $%! ,1+66%! ;15+1:6%<! =6!

*>%0+',%! 21'! $>?$@8%! 2-5+6-'+3**%6! %*! $%A35'! $>)*%! A-83551B+%! 5-&%*,%<! C1! '-D)%*&%! -&A3! $%!

B51$+%*,!EF!2%58%,!$%!;+')16+'%5!'3)'!7358%!$>A923'+B*1)0!2)*&,+7358%'!$%'!2%,+,'!&1;%5*38%'!D)+!

*%!'3*,!21'!;+'+:6%'!G!&%!',1$%!')5!6%'!1),5%'!'-D)%*&%'<!C%)5!8+'%!%*!-;+$%*&%!%',!+8235,1*,%!23)5!

$+1B*3',+D)%5!6%'!7358%'!8)6,+26%'<!H%'!7358%'!8)6,+26%'4!3),5%!6%)5!&151&,@5%!718+6+16!,5@'!75-D)%*,4!

3*,!)*!5+'D)%!A-83551B+D)%!26)'!-6%;-4!&%6)+I&+!-,1*,!-;16)-!G!#<J!KL6-'+3*L1*!23)5!6>%*'%8:6%!$%'!

&1;%5*38%'<!C%!*38:5%!$%!&1;%5*38%!%',!6%!'%)6!21518@,5%!D)+!+*76)%!')5!6%!5+'D)%!$%!'1+B*%8%*,<!

M*7+*4! +6! *>%',! 21'! 515%! $%! ,53);%5! )*%! 1*3816+%! ;%+*%)'%! $%! $-;%6322%8%*,! G! 2530+8+,-! $>)*!

&1;%5*38%<!H%,,%!1*3816+%!%',!:+%*!;+'+:6%!')5!)*%!'-D)%*&%!1;%&! +*/%&,+3*!$%!N1$36+*+)8<!=6!%',!

+8235,1*,! $%! 61! $-&5+5%! &15! %*! &1'! $%! ,51+,%8%*,! &A+5)5B+&16! $)! &1;%5*38%4! 61! 5-'%&,+3*! $%! &%,,%!

1*3816+%!1''3&+-%!2%),!'%!&3826+D)%5!$>)*!+*715&,)'!;%+*%)0!O;3+5!6%!2151B512A%!')5!6%'!1*3816+%'!

;%+*%)'%'!$%!$-;%6322%8%*,P<!Q)5!$%'!%018%*'!5-2-,-'!G!$+',1*&%4! 6%'! 6-'+3*'!2%);%*,!&A1*B%5!$%!

;36)8%!%,!$%!'+B*16!%,!$%!*3);%66%'!6-'+3*'!2%);%*,!$%;%*+5!;+'+:6%'<!C%!,51+,%8%*,!$%'!&1;%5*38%'!%',!61!5-'%&,+3*!&A+5)5B+&16%<!H%66%I&+!%',!%*;+'1B-%!%*!&1'!$>-2+6%2'+%!

'-;@5%!3)!%*!&1'!$>1BB51;1,+3*!&6+*+D)%!512+$%<!C1!63&16+'1,+3*!$%!61!6-'+3*!%',!$-,%58+*1*,%!23)5!61!

$-&+'+3*!&A+5)5B+&16%<!M*!&1'!$>1:',%*,+3*4!+6!*%!2151R,!21'!*-&%''1+5%!$%!5-2-,%5!6%'!%018%*'!=ST!%*!

215,+&)6+%5!&A%U!$%'!21,+%*,'!1'982,381,+D)%'<!C1!51$+3,A-512+%!%',!&3*,5%!+*$+D)-%4!$%'!&1;%5*38%'!

-,1*,!12215)'!125@'!51$+3,A-512+%!1$8+*+',5-%!23)5!,)8%)5'!&-5-:516%'<!

!

6 Syndrome de Sturge Weber (SSW)

C%!'9*$538%!$%!Q,)5B%!V%:%5! OQQVP!%',!)*%!2A1&381,3'%!$-&5+,%!$%2)+'!"WJ#<!H%,,%!21,A363B+%!

*3*! 718+6+16%! '%! $-7+*+,! 215! 6>1''3&+1,+3*! $>)*%! 81673581,+3*! ;%+*%)'%! &),1*-%! '+-B%1*,! $1*'! 6%!

,%55+,3+5%! $)! *%57! ,5+/)8%1)! 1;%&! )*%!81673581,+3*! ;%+*%)'%! 6%2,38-*+*B-%<! C%'! '+B*%'! &6+*+D)%'!

122151+''%*,! 1;1*,! 6>XB%! $%! F! 1*'! &38235,1*,! 3),5%! $%'! 253:6@8%'! %',A-,+D)%'! %*! 512235,! 1;%&! 61!

6-'+3*!&),1*-%4!$%'!81*+7%',1,+3*'!*%)5363B+D)%'!D)+! 73*,! 61!B51;+,-!$%! 61!8161$+%<!C>-2+6%2'+%!%',!

,5@'! 75-D)%*,%!1;%&!$%'!81*+7%',1,+3*'!23698352A%'<!C%'!1),5%'!'982,Y8%'!'3*,! 6>12215+,+3*!$>)*!

5%,15$!$%!$-;%6322%8%*,!%,!$>)*!$-7+&+,!73&16!1''3&+-!21573+'!G!)*%!A-8+1*32'+%!61,-516%!A383*98%<!

Z%'! 1*3816+%'! 35:+,1+5%'! 2%);%*,! -B16%8%*,! %0+',%5! O:)2A,168+%4! 81673581,+3*! ;%+*%)'%!

&A353[$+%**%4! &1,151&,%! %,! B61)&38%P<! C%'! 1*3816+%'! 25-'%*,%'! $1*'! 6%! QQV! '%51+%*,! $)%'! G! 61!

2%5'+',1*&%!$%'!;1+''%1)0! '+*)'3+$1)0!25+835$+1)0!25-'%*,'! %*,5%! 61!\@8%!%,! 61!W@8%! '%81+*%!$%!

! "#"

$%&''(''()!*!+(,,(!-.%/&0(1!23(+,&0(%4(!0(',/5.!6!0(7(5/%!28!-(89!0(!28!-8%,/(!'9-.%/(9%!09!7/'8$(!(,!

0(! 23:/2! %(+&97%(! 2(! ,9;(! 5(9%82! -&',.%/(9%! <9/! 0(7/(50%8! 2(! 2&;(! &++/-/,82)! =8! -(%'/',85+(! 0395!

-2(>9'!7(/5(9>!(4;%?&2&$/<9(!-&9%%8/,!(>-2/<9(%!2('!85&482/('!7(/5(9'('!%(,%&97.('!+@(A!+(%,8/5'!

0(! '('! -8,/(5,'! (>-2&%.'! -&9%! BBC1! 87(+! -(%'/',85+(! 0(! 7(/5('! %80/82('! ,%85'4.09228/%('1! 0('!

85&482/('!7(/5(9'('!0(!0.7(2&--(4(5,!&9!0('!D/',92('!09%82(')!=('!85&482/('!78'+928/%('!-%.'(5,('!

085'!2(!BBC!+&%%('-&50(5,!6!0('!,@%&4;&'('!7(/5(9'('!+&%,/+82('!87(+!95(!-%&2/D.%8,/&5!7(/5(9'(!

+8-/228/%(! (,! 95(! 0/28,8,/&5! 09! 0%8/58$(! 7(/5(9>! +&228,.%82! ,%85'4.09228/%()! =('! +&5'.<9(5+('! 0(!

238;'(5+(! 0395! '?',E4(! 7(/5(9>! '9-(%D/+/(2! D&5+,/&55(2! -&9%%8/(5,! 8--8%8F,%(! -%&$%(''/7(4(5,!

<9850! 2(! 0%8/58$(! 0(! '9--2.85+(! 0(7/(5,! /5'9DD/'85,! 87(+! @?-&>/(! (,! /'+@.4/(! -8%! @?-(%,(5'/&5!

7(/5(9'()! G('! -@.5&4E5('! (>-2/<9(%8/(5,! 28! 0.,.%/&%8,/&5! 5(9%&2&$/<9(! -%&$%(''/7(! 8/5'/! <9(!

238--8%/,/&5! 0(! 238,%&-@/(! +.%.;%82(! 8''&+/.('! 6! 0('! +82+/D/+8,/&5'! +&%,/+82(')! =3! HIJ! (,! 28! KLJ!

4&5,%(5,! ,?-/<9(4(5,! 95(! -%/'(! 0(! +&5,%8',(! $?%/D&%4(! 89! 5/7(89! 0(! 28! 482D&%48,/&5! 7(/5(9'(!

2(-,&4.5/5$.(! '&97(5,! 8''&+/.(! 6! 95! %.@89''(4(5,! /5,(5'(! 09! -2(>9'! +@&%&M0(! @&4&28,.%82)! =('!

,@%&4;&'('!7(/5(9'('! +&%,/+82('! '(!485/D(',(5,!-8%!0('! +82+/D/+8,/&5'! 87(+! 8,%&-@/(! +&%,/+82(')!N5!

-.%/&0(!5.&58,82(! (,! 085'! 2('!-%(4/E%('! '(48/5('!0(!7/(1! 2(!0/8$5&',/+! +2/5/<9(!-(9,! O,%(!0/DD/+/2(!

0(785,! 95! ,8;2(89! /5+&4-2(,)! =('! '/$5('! (5! HIJ! '&5,! '9;,/2('! 6! +(,! P$(! +8%! 238,%&-@/(! (,! 2('!

+82+/D/+8,/&5'!'&5,!%8%(4(5,!-%.'(5,(')!H2!(',!+(-(5085,!/4-&%,85,!0(!-&%,(%!2(!0/8$5&',/+!2(!-29'!,Q,!

-&''/;2(! -&9%! ,(5,(%! 0(! 2/4/,(%! 28! D%.<9(5+(! 0('! +%/'('! +&5792'/7('! (5! /5,%&09/'85,! 95! ,%8/,(4(5,!

808-,.)!=38''&+/8,/&5!0(!23@?-(%,%&-@/(!09!-2(>9'!+@&%&M0(!@&4&28,.%82!6!28!482D&%48,/&5!7(/5(9'(!

2(-,&4.5/5$.(1! ,&9'!2('!0(9>!%(@89''.'!8-%E'!+&5,%8',(1!6!0('!85&482/('!0(!'/$582!0(!28!'9;',85+(!

;285+@(! (5! %($8%0! 0(! 2385&482/(! 7(/5(9'(! '9-(%D/+/(22(! (',! 82&%'! ,%E'! .7&+8,%/+()! L9! +Q,.! 0(! 28!

482D&%48,/&5!78'+928/%(1!28!'9;',85+(!;285+@(!-%.'(5,(!95!@?-(%'/$582!K"!(,!95!@?-&'/$582!KR!<9/!

'(%8/(5,!(5! %8--&%,!'&/,!87(+!95(!8++.2.%8,/&5!0(! 28!4?.2/5/'8,/&5!'&/,!87(+!95(!@?-(%&>?$.58,/&5!

,/''928/%()! G('! -@.5&4E5('! '&5,! 2/.'! 89>! 85&482/('! 0(! -(%D9'/&5! +.%.;%82('! .,90/.('! -8%! BSNGK!

T'/5$2(! -@&,&5! (4/''/&5! +&4-9,(0! ,&4&$%8-@?U! +8%8+,.%/'.('! -8%! 95(! @?-(%-(%D9'/&5! 085'! 2('!

-%(4/(%'! 4&/'! 0(! 7/()! S29'! ,8%01! 89! ',80(! 038,%&-@/(1! 95(! @?-&-(%D9'/&5! '(+&508/%(! 89>! 2.'/&5'!

! "#$

%&'()*%+,-&! -&.! *%&-! -/! )0%1-/'-2! 3/4%/! %5! -&.! 67849%&! 69&&%:5-! 1-! 0%&,75%&-8! 1-&! 0-%/-&!

.87/&*)1,557%8-&!1%57.)-&2!

!7 Anévrysmes artériels

;-&!7/)08<&*-&!78.)8%-5&!=>>?!&9/.!878-&!@!5ABC-!6)1%7.8%+,-!70-'!,/-!48)+,-/'-!)075,)-!@!-/0%89/!

DEF!G!1-!5A-/&-*:5-!1-&!7/)08<&*-&2!H,.8-!'-..-!47%:5-!48)+,-/'-I!65,&%-,8&!'787'.)8%&.%+,-&!=.7%55-I!

59'75%&7.%9/I! ).%959C%-?! 8-/1-/.! '-&! 7/)08<&*-&! 98%C%/7,J! 678! 876698.! @! '-,J! 8-/'9/.8)&! '(-K!

5A71,5.-2! L78! -J-*65-I! '(-K! 5A-/47/.! %5! -J%&.-! ,/-! 68)19*%/7/'-! *7&',5%/-! @! 5A%/0-8&-! 1-! 57!

696,57.%9/! 71,5.-2! ;-&! .()876-,.%+,-&! -/1907&',57%8-&! -.! 1-! *%'89'(%8,8C%-! 9/.! 6-8*%&! ,/-!

7*)5%987.%9/!487/'(-!1,!689/9&.%'!1-!'-&!5)&%9/&2!

;-!*91-!1-!8)0)57.%9/!5-!65,&!48)+,-/.!-&.!57!&,80-/,-!1A,/-!()*9887C%-!&9,&!787'(/9M1%-//-!=65,&!

1-! D#! G! 1-&! >>! 1-! 5A-/47/.?2! L7849%&! 7&&9'%)-! @! ,/! ()*7.9*-! %/.87! 678-/'(<*7.-,J! 9,! @! ,/-!

%/9/17.%9/! 0-/.8%',57%8-I! -55-&! 8-&.-/.! 5-! 65,&! &9,0-/.! *9%/&! &)0N8-&! +,-! '-55-&! &,80-/7/.! '(-K!

5A71,5.-2!;-&!7,.8-&!&%C/-&!'5%/%+,-&!1)'8%.&!&9/.!,/!1)4%'%.!/-,8959C%+,-!689C8-&&%4!9,!1-&!')6(75)-&2!

;7!59'75%&7.%9/!57!65,&!48)+,-/.-!-&.!57!:%4,8'7.%9/!'789.%1%-//-!=$D!E!D#G1-&!'7&?2!;A7..-%/.-!1-!57!

:%4,8'7.%9/! &<50%-//-! -&.! )C75-*-/.! 8-*78+,7:5-! =65,&! 1-! "DG! 1-&! '7&?! =O?! '9**-! 5A7..-%/.-! 1,!

&<&.N*-!0-8.):89E:7&%57%8-!876698.)-!17/&!$P!@!P#!G!1-&!'7&!=5%08-?2!

;-&! '7,&-&! 1-! '-&! >>! &9/.! 078%)-&! -.! 4%/75-*-/.! 5A98%C%/-! '9/C)/%.75-! 6787Q.! 878-2! R-&!>>! &9/.!

'57&&%+,-*-/.! 8-/'9/.8)&! 17/&! '-8.7%/-&!*7571%-&! '9/C)/%.75-&!*7%&! %5&! 8-&.-/.! 878-&! @! 57! 6)8%91-!

6)1%7.8%+,-!-/! 8-C781!1-&!*7/%4-&.7.%9/&!9:&-80)-&!@! 5ABC-!71,5.-2!;-&!>>!1-! .<6-! &7'',57%8-!9/.!

,/-! 48)+,-/'-! 1-! O$! G2! ;-&! .87,*7.%&*-&! '8B/%-/&! &-87%-/.! @! 5A98%C%/-! 1A>>! 1%&&-'7/.&! 9,! 1-!

8,6.,8-&! 19//7/.! 1-&! 6&-,197/)08<&*-! 17/&! 68N&! 1-! D#! G! 1-&! '7&2! 3/4%/! 5-&! >>! 1A98%C%/-!

%/4-'.%-,&-! -/'98-! 766-5)&! 7/)08<&*-&!*<'9.%+,-&! 9/.! ,/-! 48)+,-/'-! 1-! 68-&+,-! "D!G! 70-'! 1-&!

498*-&!*,5.%65-&!1A>>2!;7!69&&%:%5%.)!1-!1)0-5966-8! '-! .<6-!1A>>!-&.!:%-/!19',*-/.)-!'(-K! 5-&!

67.%-/.&! 698.-,8&! 1A,/! 1)4%'%.! %**,/%.7%8-! =%/4-'.%9/! 678! STUI! %**,/9&,668-&&%9/! %7.89CN/-! 9,!

'9/C)/%.75-?2!;7!48)+,-/'-!1-&!>>!C)7/.&!-&.!1A-/0%89/!"DE$#GI!/-..-*-/.!&,6)8%-,8-!@!'-55-!1-!57!

696,57.%9/!71,5.-2!

! "#$

%&'! ()!*+,!-.'./')(0!'.)(12.0!03!&'403-0!56&'!2&251-163!789.:6'')410! 13;')-'<310330=!&30! 1:)40!

78)771;163! 9>50'70320! ?! 5'6@1:1;.! 702! A)1220)&@! 7&! 56(>4630! 70! B1((12! 761;! C)1'0! .A6D&0'! (0!

71)4362;1-E!F8GH,!761;!-6:56';0'=!6&;'0!702!2.D&03-02!)@1)(02!*"!0;!I()1'=!&30!2.D&03-0!*J!.-96!70!

4')7103;! 56&'! 7.;0-;0'! (89.:6'')410! 0;! &30! )3416GH,! 7&! 56(>4630! 70!B1((12E! F8)3416GH,! 50&;!

A12&)(120'! &3! KK! ?! 5)';1'! 70! $LM! ::! 70! 71):N;'0E! O6&'! (02! KK! 4.)3;2=! (02! ;&'/&(03-02! 70! C(&@!

50&A03;!0:5P-90'!()!A12&)(12);163!30;;0!702!(1:1;02!70!(8KKE!Q30!KH,!)A0-!13R0-;163!70!-63;')2;0!

6&!)&!:10&@!&30!KH,!7>3):1D&0!50':0;!70!28)CC')3-91'!70!-02!)';.C)-;2E!S&0(D&0!261;!(02!5'64'N2!

70!-02!;0-931D&02=!(80@):03!70!'.C.'03-0!'02;0!(8)';.'164')5910E!T0;!0@):03!50':0;!70!71)4362;1D&0'!

.4)(0:03;!&3!A)2625)2:0!D&1!50&;!P;'0!1:56';)3;E!*6&;0C612!()!;')7&-;163!-(131D&0!7&!A)2625)2:0!

02;!/0)&-6&5!:6132!C'.D&03;0!0;!2&';6&;!:6132!2.AN'0!D&0!-90U!(8)7&(;0E!!

F0!;')1;0:03;!)3;1/16;1D&0!02;!/103!2V'!(0!5'0:10'!?!'.)(120'!03!-)2!78KK!786'14130!13C0-;10&20E!O6&'!

(02!)&;'02!KK=!(0!-961@!;9.')50&;1D&0!03;'0!;')1;0:03;!0376A)2-&)()1'0!0;!-91'&'410!761;!20!712-&;0'!

)&!-)2!5)'!-)2!03!5'03)3;!03!-6:5;0!(0!;>50!70!(8KK=!263!)--0221/1(1;.!0;!(81:56';)3-0!C63-;16330((0!

7&!;0''1;61'0!)';.'10(!-63-0'3.E!F6'2!78&30!5'0:1N'0!9.:6'')410=! ()!:6';)(1;.!02;!.A)(&.0!?!""W!)&!

-6&'2! 702! MX! 5'0:1N'02! 90&'02E! K5'N2! ;')1;0:03;=! &30! .A6(&;163! C)A6')/(0! 2)32! 2.D&0((0! )! .;.!

')556';.0!7)32!Y$EYW!702!-)2E!

!

!

Références

!"E! T9)/'10'! %=! Z&2263! [=! \)&;90'63! ]=! F)37'10&! OE! G3C)'-;&2! )';.'10(2! 0;! ;9'6:/6202! A0130&202!

-.'./')(02!7&!36&A0)&L3.E!^,T!_^(20A10'!,)2263!%K%=!O)'12`=!O.71);'10=!ML##JL%La#=!J##a!

JE!\1')'7!b=!T9)&:61;'0!c=![033);9)3!,=!T9)563!IE!K--1703;!A)2-&()1'0!-.'./')(!C6-)(E!G:)40'10!

5.71);'1D&0!0;!Cd;)(0E!O)'12!e!I()::)'163!,.70-130L%-103-02!f!J##gE!T9)51;'0!M=!MJLMM!

$E!H)R&!*bc=!b0(263!c[=!I0''10'6!+=!F>3-9!hcE!G2-90:1-!50'13);)(!2;'6i0e!2&::)'>!6C!)!j6'i2965!

256326'07!/>!;90!3);163)(!132;1;&;0!6C!-91(7!90)(;9!)37!9&:)3!70A0(65:03;!)37!;90!3);163)(!132;1;&;0!

6C!30&'6(641-)(!7126'70'2!)37!2;'6i0E!O071);'1-2E!J##gf"J#eY#aLY"Y!

! "#$

$%! &'()*+! ,-! ./0/1/(! 2-! 3*+)'4*+! ,5-! 5674(/4*(! 8-! 9:(*;;! 5%! 3(/<=>/.! ?/('+6)6@! '<7:/>'7!

<)(*A/B!C6<7=@6(!7@6<<';'76)'*+!?(/.'7)<!*=)7*>/<%!,++!D/=(*@%!E##FGHIB$IHJ$$I%!

K%! L*6(.>6+! M3-! 26+/<6+! 0-! N=):/(;*(.! 5,-! 96=+./(<! 8O-! 5/(7=('! O-! P*Q6+! R%! 564+/)'7!

(/<*+6+7/! '>64/! 7*((/@6)/<! *;! :/>'?6(/<'<! 6;)/(! +/*+6)6@! 6+.! 7:'@.:**.! >'..@/! 7/(/1(6@! 6()/(S!

<)(*A/%!3/.'6)('7<!E##KG""KBIE"JIEH%!

H%!T=!UT-!5'@@/(!93-!P:'+!&-!P*@@'+<!,O-!V*>/@'!9P-!P:=6+4!D,-!/)!6@%!5=@)'?@/!('<A!;67)*(<!'+!

+/*+6)6@!<'+*C/+*=<!):(*>1*<'<%!D/=(*@*4S!E##EG!KWB$IFJ$#%!

X%! R')Y4/(6@.! &P-! T'@@'6><! V9-! 26(4! L3-! P6(C6@:*! &9-! 2*@*>1! 5N%! P/(/1(6@! 9'+*C/+*=<!

Z:(*>1*<'<!'+!):/!D/*+6)/%!,(7:!D/=(*@!E##HG!HIB$#KJW%!

F%! T=! UT-! [6>('7A! 9O-! 5'@@/(! 93-! [6Q6(.! 5R-! V6'! 5P-! P6@@/+! 3T-! /)! 6@%! \+)(6C/+)('7=@6(!

:/>*((:64/!'+!)/(>!+/*+6)/<!76=</.!1S!<'+*C/+*=<!):(*>1*<'<%!,++!D/=(*@!E##IG!K$B"EIJH%!

W%! 5*:6('(! 5-! 9:(*;;! 5-! 5672(/4*(! 8-! ,.6><! 5-! L:67:=(6! 3-! ./0/1/(! 2%! P@'+'76@! 6+.!

(6.'*4(6?:'7! ;/6)=(/<! *;! ):(*>1*<'<! ?(*?646)'*+! '+! +/*+6)6@! 6+.! 7:'@.:**.! 7/(/1(6@! <'+*C/+*=<!

):(*>1*<'<%!,++!D/=(*@!E##HG!H#B<"$"%!

"#%! [=<<*+! L-! V6<]6=+'6<! 3%! 56@;*(>6)'*+<! C6<7=@6'(/<! 7^(^1(6@/<! ./! @_/+;6+)%! O5P! `O@</C'/(!

56<<*+! 9,9-! 36('<a-! N6.'*.'64+*<)'7! b! 9c=/@/))/! +*(>6@JD/=(*(6.'*@*4'/J,??6(/'@! @*7*>*)/=(-!

I"JHEIJ,J"#!E##F!

""%![=<<*+!L-!V6<]6=+'6<!3%!36):*@*4'/<!C6<7=@6'(/<!7*+4^+')6@/<!/)!67c='</<%!\>64/('/!?^.'6)('c=/!

/)!;d)6@/%!36('<!B!R@6>>6('*+!5^./7'+/J97'/+7/<!G!E##X%!P:6?')(/!W!

"E%!V6<]6=+'6<!3-! )/(!L(=44/!&!2-!L/(/+<)/'+!,%! \+!9=(4'76@!D/=(*6+4'*4(6?:S-!Z*>/!IB!7@'+'76@!

6+.!'+)/(C/+)'*+6@!6<?/7)<!'+!7:'@.(/+%!L/(@'+-!9?('+4/(JC/(@64-!E##HB!"#KJEE$!

PATHOLOGIE INFLAMMATOIRE CEREBRALE

!

Encéphalomyélite aiguë disséminée (EMAD)

"#$$#!%&$'()(*+#!&$$#+,$!$-%+./#0#,$!)#1!#,2&,$1!3#!1#4#!0&15/)+,!67*#!083+&,!9!:!;!&,1!3&,1!

)<8$/3#! 3#!=+31#%! >??@AB@@;C! &+,1+! ./#! )#1! D#/,#1! &3/)$#1! 1&,1! &,$8583#,$! %&$'()(*+./#! #,!

%&E$+5/)+#E!,#/E()(*+./#F!G<HIJK!1/EL+#,$!3/E&,$!)&!%8E+(3#!'+L#E,&)!#$!)#!%E+,$#0%1M!#$!N>O!

3#1! #,2&,$1! &$$#+,$1! (,$! %E81#,$8! /,! 8%+1(3#! +,2#5$+#/4! #,!0(-#,,#! 3#! B! 1#0&+,#1! &L&,$! )&!

0&)&3+#F!

G#1! 5E+$PE#1! 3+&*,(1$+./#1! 1(,$! &5$/#))#0#,$! Q+#,! 382+,+1!R! /,#! &)$8E&$+(,! 3#! )&! 5(,15+#,5#!

&11(5+8#! S! &/!0(+,1! /,! 1-0%$T0#! ,#/E()(*+./#! #$! /,#! &$$#+,$#! 3#! )&! 1/Q1$&,5#! Q)&,5'#! #,!

+0&*#E+#! 1(,$! ,85#11&+E#1! %(/E! E#$#,+E! )&!0&)&3+#F! G&! *E&L+$8! 3#! )<&$$#+,$#! 3#! )&! 5(,15+#,5#!

%#/$!&))#E!3</,#!1+0%)#! +EE+$&Q+)+$8!%#/!1%85+2+./#!S!/,!5(0&!,85#11+$&,$!/,#!%E+1#!#,!5'&E*#!

E8&,+0&$(+E#F!G#1! 1-0%$T0#1!,#/E()(*+./#1! 1(,$! 1(/L#,$!0/)$+%)#1! &L#5!3#1! 58%'&)8#1M! 3#1!

1+*,#1!0($#/E1!382+5+$&+E#1M!/,#!&$$#+,$#!3#1!%&+E#1!5E7,+#,,#1M!3#1!5(,L/)1+(,1F!

G<8$/3#!3/!G"U!0(,$E#!2E8./#00#,$!/,#!5#))/)&E+$8!V!N@!&L#5!'-%#E%E($8+,(E&5'+#!V!@MN!*W)!

6X@!O!3#1!#,2&,$1!3#!,($E#!18E+#CF!

G<+0&*#E+#! S! E8&)+1#E! E&%+3#0#,$! #1$! )<YZIF! G&! $(0(3#,1+$(08$E+#! %#E0#$! 3<8)+0+,#E! #,!

/E*#,5#! )#1! 3+&*,(1$+51! 3+228E#,$+#)1! 3#1! 5(0&1! 28QE+)#1! 608,+,*+$#! 5(0%)+./8#M! &Q5P1!

58E8QE&)[C!#$!0+,+0+1#!)#!3+&*,(1$+5!3<#,58%'&)+$#!'#E%8$+./#!%(/EL(-#/1#!E&%+3#!3#!)81+(,1!

S! %E83(0+,&,5#! 5(E$+5&)#F!H,! #22#$! #,! 5&1! 3<HIJKM! )#! 15&,,#E! %E85(5#! #1$! 1(/L#,$! ,(E0&)!

69@A\@O! ]KI! ,(E0&/4CF! G#! %E($(5()#! 3<YZI! 3(+$! 5(0%(E$#E! 3#1! 18./#,5#1! ^)&+E! $EP1!

1#,1+Q)#1! &/4! )81+(,1! 3#! 1/Q1$&,5#! Q)&,5'#! 1/1$#,$(E+#))#M! )#1! 18./#,5#1! ]B! ]UH! 8$&,$! %)/1!

%#E$+,#,$#1! %(/E! )<8$/3#!3#1! %&E#,5'-0#1!3#! )&! 2(11#!%(1$8E+#/E#! 5'#_! )#! D#/,#! #,2&,$F!`,#!

18./#,5#!3#!3+22/1+(,!#$!/,#! +,D#5$+(,!3#!a&3()+,+/0!1(,$!/$+)#1!%(/E!5&E&5$8E+1#E!&/!0+#/4!

)#1! )81+(,1F!H,2+,! /,#! 8$/3#!083/))&+E#! %#E0#$! 3#! E8&)+1#E! /,! Q+)&,! #4'&/1$+2! 3#1! )81+(,1! #$!

! "#$

%&'()*+,-'.! /'.01+2'3! 12451*+'3! /*+2+,-'3! '2! /13! %'! *63+423! /6.67.1*'3! *+5+06'38! 9'3! *63+423!

/6.67.1*'3!%123!*&:;<=!3420!)4*>54.)?'38!9'3!12451*+'3!*'3!)*-3!/1.1/06.+30+,-'3!3420!-2'!

100'+20'!60'2%-'!%'!*1!3-73012/'!7*12/?'!3-30'204.+'**'!3'!0.1%-+3120!)1.!%'3!?>)'.3+@21-(!AB!

7+*106.1-(! 34-C'20! 13>560.+,-'3D! '2!)*1@'D! E! /4204-.3! F*4-3! 1C'/! 'FF'0! %'!5133'! *+5+068!G'3!

*63+423!3420!0>)+,-'5'20!%'!3+H@'!34-3/4.0+/1*!1*4.3!,-'!*&100'+20'!)6.+C'20.+/-*1+.'!'30!54+23!

/4230120'8! 91! ).+3'! %'! /420.130'! '30! .1))4.06'! %123! '2C+.42! BI! J! %'3! /13! )*-3! 4-! 54+23!

?454@H2'!'0! +20'23'8!:2!%+FF-3+42!42!473'.C'! 0>)+,-'5'20!-2'!1-@5'2010+42!%-!/4'FF+/+'20!

%'!%+FF-3+428!9&100'+20'!%'3!/1)3-*'3!+20'.2'3!'0!%-!/4.)3!/1**'-(!'30!)*-3!.1.'!,-'!/'**'!%'!*1!

3-73012/'!7*12/?'!34-3/4.0+/1*'8!=123!'2C+.42!I#J!%'3!/13D!+*!'(+30'!%'3!?>)'.3+@21-(!AB!1-!

2+C'1-!%'3!24>1-(!@.+3!/'20.1-(8!K2'!100'+20'!/4.0+/1*'!)1.F4+3!133'L!60'2%-'!3'!512+F'30'!)1.!

%'3!?>)'.3+@21-(!AB!'0!M9<NO8!='3! *63+423!3420!6@1*'5'20!)433+7*'3!1-!2+C'1-!%'! *1! F433'!

)4306.+'-.'! 1334/+6'3! E! *&100'+20'! 3-30'204.+'**'8! ='3! 017*'1-(! )*-3! 10>)+,-'3! 3420! F.6,-'203!

1C'/!%'3! *63+423!/6.67.1*'3!E!/4204-.3!2'03D!%&60'2%-'! *+5+06'D!1C'/!)1.F4+3!-2'!/45)43120'!

?654..1@+,-'8! 9'3! *63+423! )'-C'20! 3'! *+5+0'.! 1-! /'.C'*'0! P/6.67'**+0'Q! 4-! 1-! 0.42/! /6.67.1*!

P.?457'2/6)?1*+0'Q8!!

9'3! %+1@2430+/3! %+FF6.'20+'*3! 1-! 301%'! 1+@-! %'! *&6C4*-0+42! 3420! -2'! '2/6)?1*+0'! C+.1*'!

.6)*+/10+C'! P?'.)H3Q! 4-! -2'! 51*1%+'! %'! 9>5'D! %6F+2+0+C'5'20! 6/1.06'3! )1.! *'3! 60-%'3!

36.4*4@+,-'3D!-2!3>2%.45'!%&1/0+C10+42!%-!51/.4)?1@'!PC4+.!)*-3!*4+2Q!4-!)*-3!.1.'5'20!-2'!

100'+20'!560174*+,-'!%'! *1! 3-73012/'!7*12/?'!,-+!)'-0! 3'! .6C6*'.!%'! F1R42!7.-01*'! P51*1%+'3!

5+04/?42%.+1*'3!'2!)1.0+/-*+'.Q8!9'3!1206/6%'203!%'!*&'2F120D!-2'!%+30.+7-0+42!F13/+/-*1+.'!%'3!

*63+423!'0!*'3!'(15'23!7+4*4@+,-'3!%4+C'20!.64.+'20'.!*'!%+1@2430+/8!

9'!0.1+0'5'20!.')43'!3-.!%'3!74*-3!%'!;'0?>*).'%2+34*42'!PS4*-56%.4*TQ!3-.!U!4-!I!V4-.3!'2!

F42/0+42!%'!*1!.6)423'!%'!/?1,-'!'2F1208!

9&6C4*-0+42! 1/0-'**'! '30! )*-0W0! 7422'! 3123! .6/+%+C'! /?'L! X#! J! %'3! '2F1203! '0! 1C'/! -2'!

.6%-/0+42! +5)4.0120'! %'3! /?+FF.'3! %'! 54.01*+06! ,-13+! 2-**'8! ='3! 36,-'**'3! 2'-.4*4@+,-'3!

! "#$

%&'()*%!%+,-!).//+)-&*%!0.,%!""!1!0*%!2.%3!4*!)5%67*!0*!)&2505'*!*%-!8.)9*:*,-!05%27-&!0.,%!8.!

85--&).-7)*3!;,!<.5-!58!/+%*!8*!/)+=8(:*!07!05.9,+%-52!05<<&)*,-5*8!*,-)*!7,!&/5%+0*!.597!*-!5%+8&!

.'*2!.--*5,-*!0*!8.!%7=%-.,2*!=8.,2>*!07!%?%-(:*!,*)'*7@!2*,-).8!*-!8.!/)*:5()*!/+7%%&*!0A7,*!

:.8.05*!0*!8.!:?&85,*!B!>.7-!)5%67*!0*!2>)+,525-&!C%28&)+%*!*,!/8.67*D3!4.!)&/+,%*!0&<5,5-5'*!

,*!%*).!<+7),5*!67*!/.)!8A&'+87-5+,!%7)!/87%5*7)%!.,,&*%!C0.,%!,+-)*!%&)5*E!8*!0&8.5!8*!/87%!8+,9!

*,-)*!F!&/5%+0*%!.!&-&!0*!/)*%67*!"#!.,%D3!G*%!2)5-()*%!/87%!/)&2+2*%!+,-!0+,2!&-&!)*2>*)2>&%!

/+7)!*%%.?*)!0*!0&-*):5,*)! 8*! )5%67*!0*!)&2505'*3!H5,%5!7,*!,&')5-*!+/-567*E!0*%!.,-&2&0*,-%!

<.:585.7@E! 0*%! 2)5-()*%! IJK!2+))*%/+,0.,-! B! 2*7@! 0A7,*! %28&)+%*! *,! /8.67*! *-! 8A.=%*,2*! 0*!

%&67*88*!./)(%!8A&/5%+0*!%+,-!2+,%50&)&%!2+::*!0*%!2)5-()*%!0*!)5%67*!0*!)&2505'*3!

!

Sclérose en plaque (SEP)

4.!L;M!)*/)&%*,-*! 8.!/)*:5()*!2.7%*!,+,!-).7:.-567*!0*!>.,052./!%&'()*!.2675%!0*! 8A.078-*!

N*7,*3!L+,!5,250*,2*!*%-!0A*,'5)+,!O#P!"##!###!>.=5-.,-%!*,!Q).,2*3!;88*!*%-!2.).2-&)5%&*!/.)!

8A*@5%-*,2*! 0*! 8&%5+,%! 5,<8.::.-+5)*%! 0*! 8.! %7=%-.,2*! =8.,2>*! 07! %?%-(:*! ,*)'*7@! 2*,-).8!

05%%&:5,&*%! 0.,%! 8*! -*:/%! *-! 0.,%! 8A*%/.2*3! I8! ,A*@5%-*! /.%! 0*! :.)67*7)! 05.9,+%-567*!

%/&25<567*! *-! %+,! 05.9,+%-52! *%-! <.5-! %7)! 7,! *,%*:=8*! 0*! 2)5-()*%! 285,567*%E! =5+8+9567*%! *-!

).05+8+9567*%3!4.!L;M!0*! 8A*,<.,-! *%-! /87%! ).)*!67*! 2*88*!0*! 8A.078-*! *-! %.!/)&'.8*,2*!.'.,-!

8AR9*!0*!"S!.,%!*%-!*%-5:&*!*,-)*!F!B!O!1!0*!8A*,%*:=8*!0*%!L;M3!T,!0&=7-!.'.,-!8AR9*!0*!"#!

.,%!*%-!/+%%5=8*!C#EF!B!#E$1D!*-!0*!).)*%!2.%!+,-!&-&!0&2)5-%!0(%!8.!/)*:5()*!.,,&*!0*!'5*!C8.!

/87%!N*7,*!0*!,+-)*!%&)5*!.2-7*88*!.'.5-!F!.,%!8+)%!0*!%.!/)*:5()*!/+7%%&*D3!4.!L;M!0*!8A*,<.,-E!

+7-)*!%.!<)&67*,2*!.!0A.7-)*%!2.).2-&)5%-567*%!675!8.!05<<&)*,25*,-!0*!8.!/.->+8+95*!.078-*!U!%.!

/)&%*,-.-5+,!285,567*!*-!%*%!:.,5<*%-.-5+,%!*,!IJKE!%+,!&'+87-5+,!*-!%+,!/)+,+%-52E!*,<5,!8*%!

/+%%5=585-&%!->&)./*7-567*%!0*'.,-!-*,5)!2+:/-*!0*!8AR9*!0*!8A*,<.,-!*-!0*!8A*%/&).,2*!0*!'5*3!

4*!0&=7-!285,567*!/*7-!%*!:.,5<*%-*)!/.)!0*%!-)+7=8*%!0*!2+,%25*,2*!.'*2!%59,*%!0A.--*5,-*!07!

-)+,2! 2&)&=).8! %7)-+7-! 2>*V! 0*%! *,<.,-%! 0*! :+5,%! 0*! "#! .,%! *-! 7,*! 5,<*2-5+,! /)&2&0.,-! 8*%!

! "#$

%&'()%!()*+,-,'&.*)%!)%/! +)/+,*01)!23(%!)(0&+,(!*(!.*3+/!2)%!43%5!6)!2&3'(,%/&4!2&771+)(/&)-!

30)4!*()!89:;!)%/!2,(4!2&77&4&-)<!=!->,+&'&()!2>*(!+)/3+2!2)!2&3'(,%/&4!)/!2)!?+&%)!)(!4@3+')5!

A@)B! ->32,-)%4)(/<! -)! /3C-)3*!4-&(&.*)! +)%%)DC-)!2>303(/3')! =! -3!?3/@,-,'&)! 32*-/)! 30)4!2)%!

/+,*C-)%! %)(%&/&7%<! 2)%! /+,*C-)%! 2)! ->1.*&-&C+)! ,*! 2)%! %&'()%! D,/)*+%<! *()! (10+&/)! ,?/&.*)<!

10,-*3(/!?3+!?,*%%1)%!%1?3+1)%!2>3*!D,&(%!*(!D,&%5!

E(! 3*/+)! 1-1D)(/! ?)+D)//3(/! 2>,+&)(/)+! -)! 2&3'(,%/&4! )%/! ->1/*2)! 2*! 6AF! D,(/+3(/! *()!

D1(&('&/)! D,21+1)! GH! "#! -ID?@,4I/)%JK-L! 4@)B! MNO! 2)%! )(73(/%! )/! *()! ?+,/1&(,+34@&)!

3(,+D3-)! GH#<P! 'J-L! 4@)B! Q#O! 30)4! C3(2)%! ,-&',4-,(3-)%! 2>R'S! )(! 1-)4/+,?@,+T%)! 23(%!

%)*-)D)(/!-3!D,&/&1!2)%!43%!)/!?3+7,&%!2)!73U,(!+)/3+21)5!

6>)V?-,+3/&,(! RW9! 2,&/! %I%/1D3/&.*)D)(/! 4,D?,+/)+! *()! 1/*2)! 41+1C+3-)! )/! D12*--3&+)!

G2&%%1D&(3/&,(! %?3/&3-)! 2)%! -1%&,(%L5! E()! %1.*)(4)! )(! ?,(21+3/&,(! X"! 2)! ?+171+)(4)! )(!

&(0)+%&,(!+14*?1+3/&,(!)/!23(%!-)!?-3(!%3'&//3-)!GC,(()!1/*2)!2*!4,+?%!43--)*VL<!2)%!%1.*)(4)%!

XQ! XF8! )/! J,*! Y6:RW! %)-,(! ->Z')! 23(%! *(! 3*/+)! ?-3(<! *()! %1.*)(4)! 2)! 2&77*%&,(! )/! *()!

%1.*)(4)! 30)4! &([)4/&,(! 2)! S32,-&(&*D! G73&+)! -)%! &D3')%! &([)4/1)%! ?-*%&)*+%! D&(*/)%! 3?+T%!

->&([)4/&,(!2*!?+,2*&/L!%,(/!(14)%%3&+)%5!6>1/*2)!D12*--3&+)!?)*/!\/+)!-&D&/1)!G/)D?%!D34@&()!

2&%?,(&C-)<!4,,?1+3/&,(!2*!?3/&)(/L!=!2)%!%1.*)(4)%!%3'&//3-)%!GX"!30)4!C1(17&4)!2)!->&([)4/&,(!

2)!4,(/+3%/)!+13-&%1)!-,+%!2)!->1/*2)!41+1C+3-)L!)/!XQ5!6)%!D3(&7)%/3/&,(%!)(!RW9!2)!-3!F8]!2)!

->)(73(/! ?)*0)(/! \/+)! 2&771+)(/)%! 2)! 4)--)%! 2)! ->32*-/)5! 63! ?@3%)! %*C4-&(&.*)! )%/! C+T0)! 4@)B!

->)(73(/! 30)4! ?)*! 2)! -1%&,(%! 4,(%/&/*1)%! 303(/! -3! ?+)D&T+)! ?,*%%1)<! -3! D3/*+3/&,(! 2)! -3!

DI1-&()!%*&03(/!*(!'+32&)(/!43*2,^4+Z(&3-!?)*/! &(7-*)(4)+! -3! %&/*3/&,(!?-*/_/!?,%/1+&)*+)!2)%!

-1%&,(%! 4@)B! -)%! ?-*%! [)*()%! ?3/&)(/%5! 8(7&(<! -3! D3/*+&/1! &DD*(,-,'&.*)<! -3! 43?34&/1! 2)!

D&'+3/&,(%!2)%!4)--*-)%! &DD*()%!=! /+30)+%! -3!C3++&T+)!@1D3/,^)(41?@3-&.*)!)/! -3!43?34&/1!2)!

+1?3+3/&,(! 2)! -3! DI1-&()! ?+,?+)%! =! ->)(73(/! ?)*0)(/! )V?-&.*)+! 2)%! -1%&,(%! 2)! -3! %*C%/3(4)!

C-3(4@)!2>3%?)4/!)/!2>10,-*/&,(!%?14&7&.*)%5!R-!)%/!2,(4!?,%%&C-)!2)!0,&+!2)%!-1%&,(%!/I?&.*)%!

2)! F8]! 4@)B! ->)(73(/<! %)! D3(&7)%/3(/! ?3+! 2)%! @I?)+%&'(3*V! XQ! ,03-3&+)%! =! -&D&/)! ()//)<!

! "#$

%&'()*+,-,)'+! .*(.*+%)/01-)(*! -0! /'(.2! /-11*03! *,! %*! 2),0-,)'+! .(454(*+,)*11*6*+,!

.4()7*+,()/01-)(*8! 9'02! -7'+2! 6:6*! 6'+,(4! ;0*! 1-! .(42*+/*<! %=2! 1*! .(*6)*(! 4.)2'%*!

+*0('1'>);0*<!%*!/*2! 142)'+2!?)*+! 1)6),4*2!*,!'()*+,4*2!.*(.*+%)/01-)(*6*+,!-0!/'(.2!/-11*03!

4,-),! 2)>+)5)/-,)7*6*+,! .(4%)/,)7*! %*! (4/)%)7*! *,! %'+/! %*! @AB8! C*! 6:6*! 1*2! 142)'+2!

-..-(-)22-+,! *+! 5(-+/! DE.'2)>+-1! F"! 2'+,! )6.'(,-+,*2! G! +',*(! /-(! 2)! *11*2! +*! 2'+,! .-2!

,',-1*6*+,!2.4/)5);0*2!%0!%)->+'2,)/!%*!@AB<!*11*2!2'+,!5'(,*6*+,!47'/-,()/*2<!20(,'0,!2)!*11*2!

.*(2)2,*+,!20(!0+!*3-6*+!(4-1)24!G!H!6')2!IJ!,('02!+')(2!K!.4%)-,();0*2L8!M*.*+%-+,<!%&-0,(*2!

,E.*2! %*! 142)'+2! 2'+,! .'22)?1*2<! 6')+2! 47'/-,()/*2! /'66*! .-(! *3*6.1*! %*2! 142)'+2! %*!

20?2,-+/*! ?1-+/D*! .102! 4,*+%0*2! -7*/! N%=6*! .4()142)'++*1! *,! -2.*/,! .2*0%',06'(-18! O*2!

142)'+2!.(*+-+,!1*!/'+,(-2,*!2'+,!6')+2!5(4;0*+,*2!/D*P!1&*+5-+,!1'(2!%0!.(*6)*(!4.)2'%*!IQRS!

/D*P!1&-%01,*!*,!RTS!/D*P!1&*+5-+,L!(*+%-+,!1*!%)->+'2,)/!2.4/)5);0*!*+/'(*!.102!%)55)/)1*8!M*2!

.()2*2! %*! /'+,(-2,*! ,(-%0)2*+,! 1*! /-(-/,=(*! (4/*+,! %*2! 142)'+2! *,! .*(6*,,*+,! %*! 6'+,(*(! 1-!

%)2246)+-,)'+! ,*6.'(*11*!%*2!-+'6-1)*28!O*2!/(),=(*2!%*!U-(VD'5! ,(-%0)2-+,! 1-!%)2246)+-,)'+!

2.-,)-1*!%*2!142)'+2!*,!0,)1)242!/D*P!1&-%01,*!-7*/!0+*!?'++*!2*+2)?)1),4W2.4/)5)/),4<!2'+,!6')+2!

2)>+)5)/-,)52!%*!@AB!/D*P!1&*+5-+,!20(,'0,!-7-+,!"#!-+28!M&*2,!.'0(;0')!/*(,-)+*2!4;0).*2!'+,!

,*+,4!%*!6*,,(*!*+!47)%*+/*!%*2!/(),=(*2!%*!@AB!.('.(*2!G!1&*+5-+,8!X)+2)!R!'0!.102!%*2!/(),=(*2!

20)7-+,2!2'+,!+4/*22-)(*2!Y!

Z X0!6')+2!Q!142)'+2!I*,!+'+!$L!*+!DE.*(2)>+-1!FR!

Z X0!6')+2!R!142)'+2!I*,!+'+![L!.4()7*+,()/01-)(*2!

Z X0!6')+2!"!142)'+!2)4>*-+,!-0!+)7*-0!%0!,('+/!/4(4?(-18!

\-1D*0(*02*6*+,<! 1-! .*,),*! ,-)11*! %*2! )+/102)'+2! %-+2! /*2! 4,0%*2! +4/*22),*! 0+*! /'+5)(6-,)'+!

%-+2!%*2!>('0.*2!.102!)6.'(,-+,*8!!

O*2!142)'+2!64%011-)(*2!2'+,!,E.);0*6*+,!%*2!DE.*(2)>+-03!FR!G!1)6),*!?)*+!%45)+)*<!601,).1*2!

%-+2!.102!%*!Q#S!%*2! /-2! *,! 1*!.102! 2'07*+,! /*(7)/-03!.0)2! ,D'(-/);0*2!.0)2! 1'6?-)(*28!A+!

2->),,-1<!1-!142)'+!*2,!2'07*+,!%*!5'(6*!'7-1*!*,!2-!D-0,*0(!*2,!)+54()*0(*!G!%*03!7*(,=?(*28!A+!

! ""#

$%&$'(! )$! *+,+-.$,/&0! ,.121.03*&0''0! 0)*! ,+)*1.&04.0! +4! ,+)*1.+'$*1.$'0(! )$! )4.2$50! .0)*$3*!

&321.&04.0!6!430!/17&7+0''08!9+770!,+4.! '0)! '1)&+3)!0351,/$'&:40)(!430!,.&)0!;0!5+3*.$)*0!

0)*!43!)&-30!<!;=$5*&>&*1!?!;0!'$!,'$:408!

@0!;&$-3+)*&5!;&221.03*&0'!$4!)*$;0!$&-4!5+7,+.*0!+4*.0!50'4&!;0!'=ABCD!;1E6!;&)54*1(!50'4&!;0!

7$'$;&0!;0!@F70!G;&$-3+)*&5!)1.+'+-&:40H!0*!,'4)!.$.0703*(!5/0I!'0!E0430!032$3*(!43!)F3;.+70!

;=$5*&>$*&+3!;4!7$5.+,/$-0!G>+&.!,'4)! '+&3H!+4!5/0I!'0!,'4)!-.$3;(!430!>$)54'$.&*0!51.1J.$'0!

G>+&.!,'4)!'+&3H8!

@=1>+'4*&+3!;0!'$!KAL!;0!'=032$3*!0)*!.17&**03*0!G1>+'4*&+3!,$.!,+4))10H!;$3)!MNO!;0)!5$)(!'0)!

2+.70)!,.+-.0))&>0)!;15.&*0)!5/0I!'=$;4'*0!1*$3*!0%50,*&+330''0)8!P30!,+4))10!0)*!;12&3&0!,$.!

'=$,,$.&*&+3(! '$! .1$,,$.&*&+3! +4! '=$--.$>$*&+3! )$3)! /F,0.*/0.7&0! ;0! )&-30)! 304.+'+-&:40)!

;4.$3*!$4!7+&3)!QR!/04.0)!$>05!.1-.0))&+3! *+*$'0!+4!,$.*&0''0!0*!/$3;&5$,!.1)&;40'!,0.)&)*$3*!

03*.0!'0)!,+4))10)8!D04%!1>130703*)!;+&>03*!S*.0!)1,$.1)!;0!T#!E+4.)!,+4.!5+..0),+3;.0!6!;0)!

,+4))10)!;&)*&35*0)8!@0!;1'$&!03*.0!'$!,.07&U.0!0*!'$!;04%&U70!,+4))10!0)*!;0!"N!VWX!Q#!7+&)!03!

7+F0330!;$3)!3+*.0!0%,1.&03508!D0)!,+4))10)!4'*1.&04.0)!)+3*!;=$4*$3*!,'4)!,.+J$J'0)!:40!50!

;1'$&! 0)*! &321.&04.! 6!"!$38!@0)!$4*.0)! .&):40)!;0! .15&;&>0! G03!;0/+.)!;0)!5$.$5*1.&)*&:40)!;0)!

'1)&+3)!YZB!;15.&*0)!,'4)!/$4*H!)+3*!'0!)0%0!217&3&3(!'=$J)0350!;0!*.+4J'0)!;0!5+3)5&0350!'+.)!

;4!,.07&0.!1,&)+;0!0*!'=1>+'4*&+3!,.+-.0))&>08!@$!)4.>0340!;=43!/$3;&5$,!.0,.1)03*0!'0!)+45&!

7$E04.!;0!50**0!1>+'4*&+38!@0)! 2$5*04.)!;0! .&):40!,+4.! '$!5+3)*&*4*&+3!;=43!/$3;&5$,!)+3*! '0!

3+7J.0! ;0! ,+4))10)(! '$! J.&U>0*1! ;0)! &3*0.>$''0)! 03*.0! '0)! ,+4))10)(! '0)! )1:40''0)! ,0.)&)*$3*!

$,.U)! '$! ,.07&U.0! ,+4))10(! 430! ,.1)03*$*&+3! ,+'F)F7,*+7$*&:40! 0*! 430! 2+.70! ,.+-.0))&>0!

;=07J'108!P30!1>+'4*&+3!7+&3)!,1E+.$*&>0!;4!/$3;&5$,!$,.U)!430!>&3-*$&30!;=$3310)!;0!)4&>&!

$!1*1!;17+3*.10!,+4.!;0)!,$*&03*)!$>05!430!KAL!$F$3*!;1J4*1!$>$3*!Q#!$3)!5+7,$.$*&>0703*!6!

;0)!,$*&03*)!$>05!430!7$'$;&0!$F$3*!;1J4*10!03*.0!Q#!0*!R#!$3)8!D=$4*.0)!1*4;0)!6!,'4)!'+3-!

*0.70! )+3*! 6!7030.! ,+4.! >1.&2&0.! '0! 5$.$5*U.0!7+&3)! ;1'1*U.0! ;=430! KAL! $F$3*! ;1J4*1! ;$3)!

'=032$3508!

! """

#$!%&'(%$)$*%!+,)-,&%$!./0*$!-'&%!1$2!3,102!.$!4,10)5.&,16!1,&2!.$2!-,0225$2!$%!./'0%&$!-'&%!

.$2!%75&'-$0%(80$2!.(%$2!9!),.(:(+'%&(+$2!.$!1'!)'1'.($!;!%$12!1$2!(*%$&:$&,*2!,0!1$!<1'%(&')$&!

'+5%'%$=!#$0&!-&$2+&(-%(,*!$2%! &5'1(25$!'-&>2!'?(2!2-5+('1(25!$%!-&(2$!$*!+,)-%$!.$2!.(::5&$*%2!

:'+%$0&2!.$!&(280$!$*!-'&%(+01($&!./()'<$&($!-&,-&$2!@!1/$*:'*%=!

!

La neuromyélite optique (NMO) de Devic

A1! 2/'<(%! ./0*$! )'1'.($! (*:1'))'%,(&$! .5)B51(*(2'*%$! 25?>&$! $%! (.(,-'%7(80$! '?$+! '%%$(*%$!

251$+%(?$! .$2! *$&:2! ,-%(80$2! $%! .$! 1'! ),$11$! 5-(*(>&$! &$2-$+%'*%! 1$! +$&?$'0! '0! .530%! .$! 1'!

)'1'.($=! #'! :&580$*+$! .$! +$%%$! )'1'.($! $2%! (*+,**0$! +7$C! 1/$*:'*%! $%! +$! .('<*,2%(+! $2%!

&'&$)$*%!5?,805=!D$%%$!-'%7,1,<($!.,(%!E%&$!.(::5&$*+(5$!.$!1'!4FG!+'&!2'!-&(2$!$*!+7'&<$!$2%!

.(::5&$*%$=! #'!)(2$! $*! 5?(.$*+$! ./'0%,'*%(+,&-2! .$! 1/'80'-,&(*! H! .5+,0?$&%2! $*! IJJH! KA<L!

'*%(MNOP!:,0&*(%!0*!)'&80$0&!.$!1'!)'1'.($!'B'*%!0*$!2$*2(3(1(%5!.$!QRS!$%!0*$!2-5+(:(+(%5!

.$!T"S=!

U$2!+&(%>&$2!.('<*,2%(80$2!,*%!5%5!-&,-,252!'?$+!*5+$22(%5!.$!&50*(&!V!

- 0*!+&(%>&$!'32,10!V!'22,+('%(,*!./0*$!*5?&(%$!,-%(80$!$%W,0!./0*$!)B51(%$!'(<0X!

- .$0Y!.$2!%&,(2!+&(%>&$2!2$+,*.'(&$2!20(?'*%2!V!0*$!AZN!+5&53&'1$!*,&)'1$!'0!.530%!

.$2! 2(<*$2! +1(*(80$2!,0!*/5?,80'*%! -'2! 0*$!4FG[! 1'! -&52$*+$!./7B-$&2(<*'0Y!\I!

)5.011'(&$2! +,*%(<02! .$! 2(%0'%(,*! +$*%&,)5.011'(&$! $%! 5%$*.02! 20&! '0! ),(*2! R!

2$<)$*%2!?$&%53&'0Y[!1'!-&52$*+$!./A<L!'*%(MNO=!

U$2! 5%0.$2! )$*5$2! +7$C! 1/'.01%$! ,*%! ),*%&5! 80$! +7$C! 0*! -'%($*%! -&52$*%'*%! 0*! -&$)($&!

5-(2,.$! .$! )B51(%$! %&'*2?$&2$! '?$+[! 20&! 1/AZN[! .$2! 7B-$&2(<*'0Y! \I! 5%$*.02! $%! .$2! A<L!

'*%(MNO!-,2(%(:2[!1$!&(280$!.$!&5+(.(?$!'?$+!5?,10%(,*!?$&2!0*$!MNO!.$!U$?(+!.'*2!1/'**5$!

20(?'*%$!$2%!.$!]^S=!

40&!1$!-1'*!.$!1/()'<$&($[!)'1<&5!1$2!+&(%>&$2!-&5+5.$))$*%!.,**52[!1$!.('<*,2%(+!.(::5&$*%($1!

'?$+! 1'! 4FG! */$2%! -'2! %,0_,0&2! :'+(1$! @! -,2$&! '0! .530%! .$! 1'!)'1'.($=! F*! $::$%! .$2! 152(,*2!

! ""#

$%&%'&()*+! ,-.! %.%! &(//,&.%*+! $0*1! )2*-3(-.! 4(-+! *-56&,-! 78! 9! 4*+! $(+:! ;*! /)<+! +,<5*-.!

$*/*-4(-.=!)*<&!(+/*$.!*+.!4633%&*-.!4*!$*)<6!4*+!)%+6,-+!4%$&6.*+!4(-+!)(!>?@:!A!)26-5*&+*=!"B9!

4*+!*-3(-.+!(5*$!<-*!>?@!,-.!4*+!)%+6,-+!C%4<))(6&*+!%.*-4<*+!*-!0(<.*<&!$,CC*!$*))*+!4*!)(!

DEF! 4*!G*56$:! ;(! /,+6.656.%! 4*+! (-.6$,&/+! (-.6!DEF! *.! +<&.,<.! )2%5,)<.6,-! /*&C*..*-.! 4*!

3(6&*!)*!46(H-,+.6$!4%36-6.63:!

F<.&*!)2(..*6-.*!/(&.6$<)6I&*!4*+!-*&3+!,/.6J<*!*.!4*!)(!C,*))*=!$*..*!/(.0,),H6*!+*!46+.6-H<*!4*!

)(! >?@! /(&! )(! +%5%&6.%! 4*! +,-! %5,)<.6,-:! >6! )(! DEF! 4*! G*56$! %5,)<*! )*! /)<+! +,<5*-.! /(&!

/,<++%*=!)*+!&%$6465*+!+,-.!/)<+!3&%J<*-.*+!*.!)*+!4%)(6+!4*!&%$6465*!+,-.!/)<+!$,<&.+!J<*!4(-+!)(!

>?@!(5*$!<-*!&%$</%&(.6,-!C%46,$&*:!G(-+!)*+!+%&6*+!(4<).*+=!(<!',<.!4*!7!(-+!42%5,)<.6,-=!6)!

*K6+.*! L89!4*! 4%$I+! *.! 789!4*! /(.6*-.+! /(&(/)%H6J<*+:!;*+! &(&*+! 4,--%*+! 46+/,-6')*+! $0*1!

)2*-3(-.! C,-.&*-.! <-*! %5,)<.6,-! '*(<$,</! C,6-+! +%5I&*! (5*$! $,-+*&5(.6,-! 42(<.,-,C6*!

C,.&6$*!4(-+!MB!9!4*+!$(+!(/&I+!L!(-+!42%5,)<.6,-:!

;*! .&(6.*C*-.! $,C/,&.*! 4*+! 6-N*$.6,-+! 6-.&(! 5*6-*<+*+! 4*! 0(<.*+! 4,+*+! 4*! $,&.6$,O4*+:! P-!

.&(6.*C*-.!6CC<-,+<//&*++*<&!/(&!(-.6!QG!#8!/*<.!+*!46+$<.*&!&(/64*C*-.!*-!$(+!4*!&%$6465*:!

!

Myélite aigue transverse idiopathique

;(! CR%)6.*! (6H<*! .&(-+5*&+*! 646,/(.06J<*! *+.! <-*! (..*6-.*! 6-3)(CC(.,6&*! 42<-! +*HC*-.!

C%4<))(6&*! .,<$0(-.! )(! +<'+.(-$*! ')(-$0*! *.! )(! +<'+.(-$*! H&6+*=! +26-+.())(-.! +<&! <-*! /%&6,4*!

)6C6.%*! *.! %5,)<(-.! .R/6J<*C*-.! 4*! 3(S,-! C,-,/0(+6J<*:! G*+! $&6.I&*+! 426-$)<+6,-! *.!

42*K$)<+6,-!,-.!%.%!%.(')6+!/(&!T!U0*!U&(-+5*&+*!Q,-+,&.6<C!V,&W6-H!H&,</!X!*-!#88#!Y!

- $&6.I&*+!426-$)<+6,-!Y!4%36$6.!C,.*<&!*.Z,<!+*-+6.63!*.Z,<!.&,<')*+!5%+6$,+/06-$.%&6*-+!

(..&6'<(')*+! [! <-*! (..*6-.*!C%4<))(6&*=! +6H-*+! $)6-6J<*+! ,<! +RC/.\C*+! '6)(.%&(<K=!

C(6+! /(+! -%$*++(6&*C*-.! +RC%.&6J<*+=! -65*(<! +*-+6.63! $)6-6J<*! $)(6&*C*-.! 4%36-6=!

*K$)<+6,-! 42<-*! %.6,),H6*! $,C/&*++65*! *K.&(](K6()*! /(&! 6C(H*&6*=! (<.0*-.636$(.6,-!

42<-*!6-3)(CC(.6,-!4*!)(!C,*))*!%/6-6I&*!^/)%6,$R.,+*!4(-+!)*!;Q>!*.Z,<!/&6+*!4*!

! ""#

$%&'()*'+! ,-./00)1(+! )2(3*! 1&4+$'1%&! .+! 5).%01&1/,67! ./(-+! .81&*')00)'1%&! .+*!

'(%/90+*!&+/(%0%51:/+*!+&'(+!;!<+/(+*!+'!="!4%/(*>!

- $(1'3(+*! .8+?$0/*1%&!@! )&'-$-.+&'! .+! ().1%'<-()21+! ,-./00)1(+! .)&*! 0+*! "A! )&*!

2(-$-.+&'*7!)(5/,+&'B*6!$01&1:/+B*6!+&!C)D+/(!.8/&+!'<(%,9%*+!.+!08)('3(+!*21&)0+!

)&'-(1+/(+7! .%&&-+*! ().1%0%51:/+*! -D%:/)&'! /&+! ,)0C%(,)'1%&! )('-(1%ED+1&+/*+7!

.%&&-+*!$01&1:/+*!%/!*-(%0%51:/+*!-D%$)'(1$+*!.+!,)0).1+!.+!*F*'3,+!B*)($%G.%*+7!

H+<I+'7! 0/2/*7!J%/5+(%'EK4L5(+&67! *15&+*!.8)''+1&'+! ./!KMN!.)&*!/&! $%&'+?'+! .+!

,)0).1+*! 1&C+$'1+/*+*7! *15&+*! $01&1:/+*! %/! 2)()$01&1:/+*! +&! C)D+/(! .+! *$0-(%*+! +&!

20):/+7!)&'-$-.+&'*!.+!*15&+*!$01&1:/+*!-D%$)'+/(*!.+!&+/(%2)'<1+!%2'1:/+>!

O)&*!0)!2%2/0)'1%&!2-.1)'(1:/+7!+00+!'%/$<+!0+*!+&C)&'*!.+!*+?+!,)*$/01&!.)&*!0)!,)4%(1'-!.+*!

$)*7! P5-*! .+! Q! R! ;! )&*>! S+*! *F,2'T,+*! $01&1:/+*! *%&'! +**+&'1+00+,+&'! *+&*1'1D%E,%'+/(*!

)**%$1-*!%/!&%&!U!.+*!'(%/90+*!*2<1&$'-(1+&*>!

S+!.1)5&%*'1$!*+!C)1'!+&!/(5+&$+!2)(! 0)!(-)01*)'1%&!.8/&+!VWX!,-./00)1(+7!2)'<%0%51:/+!.)&*!

/&+!,)4%(1'-!.+*!$)*!BYAEQ;!Z!.+*!$)*!*+0%&!0+*!*-(1+*6>!S8+?),+&!.%1'!+?20%(+(!08+&*+,90+!.+!

0)!,%+00+! )D+$! .+*! *-:/+&$+*! +&! [=! *)51'')0+*! +'! )?1)0+*! +'! .+*! *-:/+&$+*! ["! *)&*! +'! )D+$!

1&4+$'1%&! .+! 5).%01&1/,! .)&*! )/! ,%1&*! /&! 20)&! .+! 08+*2)$+>! \&+! -'/.+! .+! 08+&$-2<)0+! +*'!

&-$+**)1(+! $%,2%(')&'! )/! ,%1&*! .+*! *-:/+&$+*! [=! [K]! +'^%/! _0)1(>! S)! `%&+! ,-./00)1(+!

2)'<%0%51:/+! )22)()1'! -0)(51+! */(! /&+! <)/'+/(! .+! #! U! ;! D+('39(+*! )D+$! +&! [=! )?1)07! /&!

<F2+(*15&)0! %$$/2)&'! )/! ,%1&*! .+/?! '1+(*! .+! 0)! */(C)$+! ,-./00)1(+>! S)! 2(1*+! .+! $%&'()*'+!

(+'(%/D-+!.)&*!,%1&*!.+!YA!Z!.+*!$)*!+*'!*%/D+&'!2-(1,-./00)1(+>!

S8-D%0/'1%&!.+!0)!,)0).1+!+*'!D)(1)90+!+'!0+*!*-(1+*!.+!0)!01''-()'/(+!*%&'!*%/D+&'!<-'-(%53&+*!

B,-0)&5+)&'! 0+*! C%(,+*! 1.1%2)'<1:/+*! +'! 0+*! C%(,+*! *+$%&.)1(+*6! +'! $%,2%('+&'!.+*! +CC+$'1C*!

(+*'(+1&'*! B,%1&*! .+! aA! 2)'1+&'*6>! N<+`! 08+&C)&'! 10! *+,90+! :/+! .+*! *-:/+00+*! )**+`! *-D3(+*!

5b&)&'! 08)$'1D1'-! :/%'1.1+&&+! */(D1+&&+&'! .)&*! "^#! .+*! $)*! )0%(*! :/+! "^#! 5/-(1**+&'! *)&*!

*-:/+00+*7!0+*!)/'(+*!5)(.)&'!/&+!5b&+!,%.-(-+>!S+*!C)$'+/(*!.+!9%&!2(%&%*'1$!*%&'!0)!*/(D+&/+!

! ""#

$%! &'! ('&'$)%! '*+$%&,! $%! -! './0! *.! $1&')! 23*45! $%! $)'6.3/5)20! $%/! 7'/! .)8%'*9!(1$*&&')4%/!

&1/)3..%&/0! *.%! '7/%.2%! $%! :&%)32;53/%! :%.$'.5! &'! :14)3$%! ')6*%0! *.! <')7&%! .3(74%! $%!

/%6(%.5/!(1$*&&')4%/!'55%).5/!%5!*.%!'7/%.2%!$%!&1/)3.!%.!=;:3/)6.'&!>"!%.!:14)3$%!')6*%?!

@%! 54')5%(%.5! 23./)/5%! %.! $%/! ).A%25)3./! ).54'8%).%*/%/! $%! ='*5%/! $3/%/! $%!

(%5=;&:4%$.)/3&3.%?!

@'! /*48%.*%! $%! 412)$)8%! $3)5! 53*A3*4/! <')4%! 4%2=%42=%4! *.! $)'6.3/5)2! $%! /2&143/%! %.! :&'B*%!

CDEF!$%/!2'/!$'./!.354%!/14)%G!(H(%!/I)&!/I'6)5!$I*.!(3$%!$I%.541%!).='7)5*%&!$'./!&'!JKL?!

!

Syndrome d’activation du macrophage (SAM)

@%!JMN!%/5!$1<).)!23((%!*.%!'25)8'5)3.!%5!*.%!:43&)<14'5)3.!.3.!('&)6.%!(')/! ).23.54O&1%!

$%/! &;(:=32;5%/! >! PQR! %5! $%/! ('243:='6%/! %.54').'.5! *.%! /12415)3.! ).'::43:4)1%! $%!

2;53S).%/!4%/:3./'7&%/!$%/!/)6.%/!2&).)B*%/!%5!7)3&36)B*%/?!@%!JMN!:%*5!/*48%.)4!,!53*5!T6%!

%5! &%/! 15)3&36)%/! /3.5! /3)5! :4)(')4%/! C61.15)B*%/!U! &'! &;(:=3=)/5)32;53/%! <'()(&)'&%0! &%!

/;.$43(%!$%!V4)/2%&&)0!&'!&;(:=3=)/5)32;53/%!&)1%!,!&IW!%5!&'!('&'$)%!$%!P=%$)'S+X)6'/=)G!3*!

/%23.$')4%/!C).<%25)%*/%/0!'*53)((*.%/0!5*(34'&%/0!)((*.)5')4%/G?!

@%/!/)6.%/!2&).)B*%/!61.14'*9!23(:345%.5!*.%!<)Y84%!%5!*.%!=1:'53/:&1.3(16'&)%?!Z.!5'7&%'*!

.%*43&36)B*%! %/5! :41/%.5! 2=%[! %.8)43.! E\!F! $%/! %.<'.5/! '8%2! )44)5'7)&)510! /3(.3&%.2%! %5]3*!

/;.$43(%!(1.).610!'55%).5%!$%/!.%4</!24T.)%./0!'5'9)%0!23.8*&/)3./?!

@%/! 24)5Y4%/! 7)3&36)B*%/! /3.5! *.%! 2;53:1.)%0! *.%! =;:3<)74).1()%0! *.%! =;:%454)6&;214)$1()%0!

*.%!=;:%4<144)5).1()%0!*.%!'*6(%.5'5)3.!<41B*%.5%!$%/!@QX!%5!$%/!/PQDE!C412%:5%*4!/3&*7&%!

$%! &I).5%4&%*S).%! DG?! @I)((*.3:=1.35;:'6%! C/'.6! %5! @PJG! (3.54%! *.%! '*6(%.5'5)3.! $%/!

&;(:=32;5%/!>!PQR^!%5!*.%!<3.25)3.!$)().*1%!83)4%!.*&&%!$%/!&;(:=32;5%/!_`?!@'!:41/%.2%!

$I*.%! =1(3:='632;53/%! $'./! '*! (3)./! *.! $%/! 5)//*/! :41&%81/! C(3%&&%! 3//%*/%0! <3)%0!

6'.6&)3./aG!23.<)4(%!&'!('&'$)%?!

! ""#

$%&! '()*%&! +%),-./(0-1-234)%&! -+(! .%,53&! *%! *'63+3,! .1)&3%),&! &(/*%&! *%! 2,/73('! *8/((%3+(%!

9',':,/1%! /7%9! .-),! 1%! &(/*%! ;<! )+%! 3+631(,/(3-+! *%&! 1=5.0-9=(%&! %(! 03&(3-9=(%&! 1353('%! /)>!

5'+3+2%&<!.-),!1%!&(/*%!;;<!)+%!/((%3+(%!&)..1'5%+(/3,%!/)!+37%/)!*)!./,%+90=5%!&-)&!?/9%+(!

%(! .-),! 1%! &(/*%! ;;;<! )+%! /((%3+(%! ./,%+90=5/(%)&%! .1)&! %>(%+&37%! /7%9! %+! .1)&! )+%! +'9,-&%!

(3&&)1/3,%@! $%&! /+-5/13%&! +%),-,/*3-1-234)%&! &-+(! '2/1%5%+(! )+! ,%61%(! *%! 1/! 2,/73('! *%!

18/((%3+(%!9',':,/1%@!$%&!1'&3-+&!73&3:1%&!%+!;AB!&-+(!7/,3'%&!%(!&-)7%+(!+-+!&.'93634)%&C!.,3&%!

*%! 9-+(,/&(%! 3+(%+&%! *%&! 5'+3+2%&! ./,6-3&! /&&-93'%! D! )+%! 7%+(,39)1-5'2/13%<! 1'&3-+&!

./,%+90=5/(%)&%&! 9-,(39-E&-)&9-,(39/1%&! +-*)1/3,%&! 0-5-2F+%&! -)! /++)1/3,%&! /7%9! .,3&%! *%!

9-+(,/&(%!%+!9-9/,*%!-)!0=.%,&32+/)>!GH!%+!.1/2%!*%!1/!&):&(/+9%!:1/+90%!.',37%+(,39)1/3,%@!

I+%! /(,-.03%! 9',':,/1%<! )+! /&.%9(! *%! +'9,-&%! 1/53+/3,%<! *%&! (,/+&6-,5/(3-+&! 0'5-,,/234)%&!

*%&! 1'&3-+&! ./,%+90=5/(%)&%&<! *%&! 9/193639/(3-+&! -+(! '2/1%5%+(! '('! *'9,3(&@! $%&! 1'&3-+&!

.%)7%+(!&3'2%,!*/+&!1%&!0'53&.0F,%&!9',':,/)>!-)!1%!9%,7%1%(@!$8/((%3+(%!*)!(,-+9!9',':,/1!%&(!

.1)&!,/,%5%+(!,/..-,('%@!J+!&.%9(,-;AB<!)+!*-):1%(!*%!1/9(/(%&!/&&-93'!D!)+%!'1'7/(3-+!*)!.39!

21)(/53+%K21)(/5/(%! /! '('! 3+(%,.,'('! 9-55%! 1%&! ('5-3+&! *8)+! 5'(/:-13&5%! /+/',-:3%@!

L%9-+*/3,%5%+(<!1/!90)(%!*)!.39!*%!M!N9'(=1!N&./,(/(%!OMNNP!%(!18/)25%+(/(3-+!*%!9%1)3!*%!

Q0-13+%!(,/*)3,/3%+(!1/!*%&(,)9(3-+!*)!./,%+90=5%@!$%&!1'&3-+&!.%)7%+(!'7-1)%,!%(!&%!5-*363%,!

/)!9-),&!*%!18'7-1)(3-+@!

$8'7-1)(3-+!*)!LNB!%&(!&'7F,%!%(!18/((%3+(%!+%),-1-234)%!%&(!*%!(,F&!5/)7/3&!.,-+-&(39@!I+%!

.,3&%! %+! 90/,2%! .,'9-9%! /5'13-,%! 1%! .,-+-&(39@! $/! 2,%66%! *%! 5-%11%! -&&%)&%! %&(! 1%! &%)1!

(,/3(%5%+(!9),/(36!/9()%11%5%+(!.-),!1%&!6-,5%&!2'+'(34)%&@!

!

!

!

!

! ""#

Maladies générales inflammatoires comportant une atteinte neurologique :

maladie de Behçet et lupus érythémateux disséminé

$%!&%'%()*!(*!+*,-*.!*/.!01*!2%/30'%4).*!&0'.)/5/.6&)70*!.803,%1.!.59)70*&*1.!'*/!,8&&*/!

2*4/! ':;<*! (*! =>%1/?! @*/! 3%/! 96()%.4)70*/! /042*1%1.! *1.4*! ">! *.! "#%1/! 81.! 6.6! 4%9984.6/?!

$:6.)8'8<)*!(*!'%!&%'%()*!*/.!)138110*?!$*/!34).A4*/!()%<18/.)70*/!/81.!01)70*&*1.!3')1)70*/!B!

! C!34).A4*!&%D*04B!%9,.8/*!E033%'*!F=!980//6*/G%1H!381/.%.6*!9%4!01!&6(*3)1!

! C! %0.4*/! 34).A4*/! F%0! &8)1/! IHB! %9,.8/*! <61).%'*J! 026).*! 80! 2%/30'%4).*! 46.)1)*11*J!

645.,A&*! 180*0KJ! 4%/,! 90/.0'*0K! 80! L8'')30').*! 16348.)70*J! ,59*4/*1/)E)').6! %0K! 98)1./! (*!

9)7M4*!

@*/! &%1)L*/.%.)81/! 1*048'8<)70*/! /042)*11*1.! 3,*N! O! P! =>! Q! (*/! 9%.)*1./! 46%')/%1.! (*/!

.%E'*%0K! 3')1)70*/! 98'5&849,*/! *1! 4%9984.! %2*3! (*/! '6/)81/! 9%4*13,5&%.*0/*/! *.G80! 01*!

%..*)1.*!2%/30'%)4*!*1!9%4.)30')*4!2*)1*0/*?!$:RST!3646E4%'*!&81.4*!/8).!(*/!,59*4/)<1%0K!UI!

'*! 9'0/! /802*1.! %0! 1)2*%0! (0! .4813! 3646E4%'J! (*/! 3%9/0'*/! )1.*41*/! 80! (0! .,%'%&0/J! /8).!

2)/0%')/*! 01*! .,48&E8/*!(*/! /)10/!2*)1*0K?!V*/! %18&%')*/! 9*02*1.! 98/*4! (*/! 948E'A&*/!(*!

()%<18/.)3!()LL64*1.)*'!%2*3!'%!WXY!80!01!1*048'090/!70%1(!':%..*)1.*!3646E4%'*!*/.!'%!94*&)A4*!

&%1)L*/.%.)81!(*!'%!&%'%()*?!

$:628'0.)81!/*!L%).!9%4!980//6*/!(*!/6264).6!)&9462)/)E'*!&%)/!':%..*)1.*!1*048'8<)70*!*/.!01*!

(*/!&%1)L*/.%.)81/!'*/!9'0/!<4%2*/!(*!'%!&%'%()*!/04.80.!*1!3%/!(:%..*)1.*!9%4*13,5&%.*0/*!*1!

RST?!Z1*!4*3811%)//%13*!946383*!*.!01*!94)/*!*1!3,%4<*!%(%9.6*!/81.!(813!*//*1.)*''*/!9804!

%&6')84*4!'*!94818/.)3?!

!

$*! '090/! 645.,6&%.*0K! ()//6&)16! F$X@H! (6E0.*! (%1/! *12)481! I>!Q! (*/! 3%/! %2%1.! "#! %1/?!

V,*N!"!9%.)*1.!/04!OJ!)'!*K)/.*!01*!%..*)1.*!1*0489/53,)%.4)70*!(A/!'%!946/*1.%.)81!)1).)%'*?!W*/!

&%1)L*/.%.)81/! /81.! .4A/! 2%4)6*/! %2*3! (*/! 369,%'6*/J! (*/! .480E'*/! (*! ':,0&*04! 28)4*! (*/!

.%E'*%0K! (*! 9/53,8/*! 80! 01*! 1624).*! 89.)70*J! 01*!&56').*! .4%1/2*4/*! 80! 01*!&61)1<).*?! $%!

! ""#

$%&'()%(! *+%)! ,--.*()/! ',$-%0,.&(! *1./! 2,.&(! &(-3(&-3(&! 0,! 4&5$()-(! *+,)/.-1&4$!

,)/.431$4310.4.*($6!

7+89:!-5&5;&,0(!($/!$1%'()/!4(%!$()$.;0(!()!*(31&$!*($!,--.*()/$!',$-%0,.&($!-,&!0($!05$.1)$!

$(!$./%()/!,%!).'(,%!*(!0,!<.-&1-.&-%0,/.1)!-5&5;&,0(!*,)$!,%!<1.)$!=>!?!@>!A!*($!-,$!(/!$1)/!

$,)$! /&,*%-/.1)! 4,&()-3B<,/(%$(! *5-(0,;0(6! C,)$! -(&/,.)$! -,$D! (00(! <1)/&(! *(! <%0/.40($!

3B4(&$.E),%F!GH!40%/I/! 01-,0.$5$!*,)$! 0,!$%;$/,)-(!;0,)-3(!1%!%)(!,/&143.(!-5&5;&,0(6!J)2.)!

(00(!'.$%,0.$(!4,&2,./(<()/!0($!05$.1)$!.$-35<.K%($!,&/5&.(00($6!

7(! *.,E)1$/.-! &(41$(! $%&! 0($! *1))5($! ;.101E.K%($! ()! 4,&/.-%0.(&! 0+,$$1-.,/.1)! *+%)(!

3B41-1<405<()/5<.(!(/!*+%)! /./&(!50('5!*+,)/.-1&4$!,)/.LCMN!),/.2!,!%)(!',0(%&!4&5*.-/.'(!

41$./.'(!*(!">>A!41%&!0(!*.,E)1$/.-6!

7+5'10%/.1)!*%!7JC!$(!2,./!4,&!41%$$5(!(/!$(&,./!40%$!E&,'(!()!-,$!*(!*5;%/!45*.,/&.K%(6!7,!

<1&/,0./5!*,)$! 0($!$5&.($!45*.,/&.K%($!($/!*(!"O!?!H>A6!7,!<1&;.*./5D! 0.5(!?! 0,!<,0,*.(!<,.$!

,%$$.!,%F!(22(/$!$(-1)*,.&($!*($!/&,./(<()/$!P-1&/.-1/35&,4.(!()!4,&/.-%0.(&Q!&($/(!50('5(6!

! ""#

Encéphalopathie associée à une thyroïdite d’Hashimoto

$%! &'()*+,-&.! ,/0%1'-2*&*! .1&! 3%! 4*)2.!,.! &'()*+,-&.! 3%! 5361! 4)786.9&.! :'.;! 3/.94%9&! .&! .33.!

&*6:'.)%-&!"<=>!,.1!.94%9&1!.9!?@.!1:*3%-).!%6A!BCDE!$%!5)75*9,7)%9:.! 472-9-9.!.1&! 4*)&.E!

$.1! 4*9:&-*91! &'()*+,-.99.1!1*9&!1*6F.9&!9*)2%3.1<!5%)4*-1! -3!.A-1&.!69.!'(5*&'()*+,-.!%3*)1!

86.!3.1!:%1!,.!&'()7*&*A-:*1.!1*9&!)%).1E!$%!16)F.96.!,.!3/%&&.-9&.!&'()*-,-.99.!5.6&!16)F.9-)!

,.1!%997.1!5361!&%),!86.!3/%&&.-9&.!9.6)*3*@-86.E!$/.9:75'%3*5%&'-.!,/0%1'-2*&*!,71-@9.!69!

1(9,)*2.!9.6)*3*@-86.!.&G*6!51(:'-%&)-86.!%11*:-7!H!3%!&'()*+,-&.!%F.:!737F%&-*9!,.1!%9&-:*)51!

%9&-&'()*+,-.91! I%9&-:*)51! %9&-2-:)*1*2-%6A<! %9&-:*)51! %9&-&'()*@3*J63-9.1KE! L.&&.!

.9:75'%3*5%&'-.!.1&! )%).2.9&!)%55*)&7.!.9!57,-%&)-.!.&!1*9!,-%@9*1&-:!.1&!5)*J%J3.2.9&!1*61!

.1&-27!5%)!27:*99%-11%9:.!,.!3%!5%&'*3*@-.E!!

L3-9-86.2.9&!-3!5.6&!1/%@-)!,.!3%!16)F.96.!J)6&%3.!,.!:*9F631-*91!5*6F%9&!7F*36.)!F.)1!69!7&%&!

,.!2%3!1%91!:%61.!-,.9&-4-%J3.!.&!1%91!%9&7:7,.9&!75-3.5&-86.<!,/69.!%3&7)%&-*9!,.!3%!:*91:-.9:.!

,.! 5)*4*9,.6)! F%)-%J3.<! ,/'72-5%)71-.1! )7:-,-F%9&.1<! ,/69! &%J3.%6! ,.! 51(:'*1.! %-@6.<! ,.!

3/%55%)-&-*9! ,.! 2*6F.2.9&1! %9*)2%6A! H! &(5.! ,.! 2(*:3*9-.1! *6! ,.! &).2J3.2.9&1E! L.1!

&%J3.%6A!16)F-.99.9&!3.!5361!1*6F.9&!:'.;!,.1!4-33.1!?@7.1!,.!5361!,.!"M!%91E!$.!,-%@9*1&-:!.1&!

:*94-)27!5%)! 3%!5*1-&-F-&7!,.!3%!).:'.):'.!,.1!%9&-:*)51!%9&-&'()*+,-.91E!$/NOP!:7)7J)%3.!.1&!

9*)2%3.! ,%91! .9F-)*9! QM!>!,.1! :%1! *6! 5.6&!2*9&).)! ,.1! %9*2%3-.1! 9*9! 157:-4-86.1! H! &(5.!

,/'(5.)1-@9%6A!,.!3%!16J1&%9:.!J3%9:'.!161&.9&*)-.33.E!$.1!1-@9.1!9.6)*3*@-86.1!)7@).11.9&!,.!

4%R*9! 15.:&%:63%-).! 1*61! &)%-&.2.9&! 5%)! :*)&-:*-,.1E! S9! :%1! ,.! 9*9! ).:*99%-11%9:.! ,.! 3%!

5%&'*3*@-.<!3/7F*36&-*9!:%)%:&7)-1&-86.!1.!4%-&!5%)!75-1*,.1!)7:-,-F%9&1E!

!

Références

"E!P6)&'(!CTU<!V%,.9!0C<!L*'.9!PS<!W%X1'-!OE!D:6&.!,-11.2-9%&.,!.9:.5'%3*2(.3-&-1! -9!:'-3,).9E!Y.,-%&)!=MM=Z!""M[.="!!=E!\.9.2J%62!C<!L'%2*3.1!T<!V.].)2.9!TE!D:6&.!,-11.2-9%&.,!.9:.5'%3*2(.3-&-1[!%! 3*9@^&.)2!4*33*_^65!1&6,(!*4!#`!5.,-%&)-:!5%&-.9&1E!T.6)*3*@(!=MM=Z!ab["==`^"=c"!

! ""#

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`%!O+(a'::!O.!-0=;UU!S.!4'33!5!'2!+:%!SJW!U'+2,='3!;U!9'81+2=17!),:219:'!37:'=;313%!4',=;:;><!?@@AB!C`D-,99:!?EF-HCGR$!#%! L=,99! Z.! O+(a'::! O.! &'(')*+,)! -B! U;=! 20'! W(2'=(+21;(+:! \'81+2=17! S-! -2,8<! Q=;,9%!/;(3'(3,3! 8'U1(121;(3! 9=;9;3'8! U;=! 9'81+2=17!),:219:'! 37:'=;313%! 4',=;:;><! ?@@AB! C`D-,99:!?EF-AG"?!!"@%!/+::'(!I56.!-0=;UU!S.!O=+(3;(!NS.!Z;2b'!&.!Z1!IL.!-2'90'(3!I.!O+(a'::!O%!SJW!1(!20'!81+>(;313!;U!9'81+2=17!),:219:'!37:'=;313%!4',=;:;><!?@@#B!A?F#C"G#CA!!""%!/+::'(!I56.!-0=;UU!S.!O=+(3;(!NS.!Z1!IL.!Z;2b'!&.!-2'90'(3!I.!O+(a'::!O%!J;:'!;U!SJW!1(!81UU'='(71+21;(!;U!6IcS!U=;)!S-!1(!701:8='(%!4',=;:;><!?@@#BA?F#C`G#A$!!"?%!Z;2b'!&c.!4;=20=;9!5Z.!N,22;(!Q5.!J;33!O.!-701UU)+(!5-.!N,(2'=!5Y%!-9'72=,)!;U!\'81+2=17!4',=;)<':1213!d9217+%!\'81+2=!?@@`F"??B!'"@$#G"@HA!!"$%!O+(a'::!O.!&'(')*+,)!-BZ'((;(!Y6.!X=3'::!c.!L'(('8<!5.!O+=Gd=!6.!K'1(30'(T'=!OQ.!Z,7701('221! /[.! \122;7T! -5%! 4',=;)<':1213! ;9217+GW>Q! 1(! 701:80;;8! 1(U:+))+2;=<!8')<':1(+21(>!/4-!813;=8'=3%!4',=;:;><!?@@`B!A@F$HHG$R?!!"H%!&=+(3P'=3'!)<':1213!7;(3;=21,)!a;=T1(>!>=;,9%!\=;9;3'8!81+>(;3217!7=12'=1+!+(8!(;3;:;><!;U!+7,2'!2=+(3P'=3'!)<':1213%!4',=;:;><!?@@?B!R#FH##GR@R!!"R%!I'U='3('!\.!N;::'(*'=>!N.!N,33;(!O!'2!+:%!67,2'!2=+(3P'=3'!)<':1213!1(!701:8='(F!7:1(17+:!7;,=3'!+(8!9=;>(;3217!U+72;=3%!5!/01:8!4',=;:!?@@$B!"`FH@"GH@C!!"C%! Z+(721(! /.! K1'=2:'a3T1! -.! S;='+,! /! '2! +:%! S<':12'! +1>,'! 2=+(3P'=3'! 181;9+201^,'!F!+99:17+21;(! 8'3! (;,P'+,M! 7=12e='3! 81+>(;321^,'3! f! ,('! 7;0;=2'! 8'! "A! 9+21'(23%! J'P!4',=;:%!?@@CB!"C?F#`@G#`#!!"A%! [12b>'=+:8! 4c.! S7/:+1(! LZ%! W)+>1(>! 70+=+72'=132173! ;U! 0');90+>;7<217!:<)90;01321;7<2;313%!\'81+2=!J+81;:!?@@$B!$$F!$#?GH@"!

! "#$

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