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La maladie de Wilson ( A propos de 5 observation )

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(1)

!

!

!

!

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% & & & & &

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2(25+ 5 ! +( / 6 2*7/ /1+ 8 0 +9 6 1 72:/(+ 1 5 0*+98 6 78/*:( *8/(/;+<* =/(>1/8= 6 5+2; / 7+0 :+/(/;+<* 6 782+7 1 7 - 9+ '

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' ' ' 8/A *8 5B = 72(1/(/;+ ' 1 ' ' C' 8/A *8 5 *8/(/;+ ' ' ' ' ' 8/A *8 5 *8/(/;+ ' ' ' C ' 8/A *8 5 D2 78/. 7 8/(/;+ ' ? D

(2)
(3)

Au bon dieu

Au bon dieu

Au bon dieu

Au bon dieu

Tout puissant

Tout puissant

Tout puissant

Tout puissant

Qui m’a inspiré

Qui ma guidé dans le bon chemin

Je vous dois ce que je suis devenue

Louanges et remerciements

Pour votre clémence et miséricorde

(4)

A mon père

A mon père

A mon père

A mon père

Errahmouni mehmmed

Errahmouni mehmmed

Errahmouni mehmmed

Errahmouni mehmmed

Vous avez consenti par votre grand amour pour

moi et par votre sacrifice, de patience, de

bienveillance et d’encouragement à mon

éducation et ma formation ce qui a fait toute ma

force et m’a permis de mener à terme mes projets.

Je ne saurai jamais exprimer le précieux

sentiment d’un jeune homme auquel vous avez

tout donné et qui vous adresse l’expression

profonde d’une gratitude qui animera son cœur

(5)

A ma mère

A ma mère

A ma mère

A ma mère

Amirrar rhimou

Amirrar rhimou

Amirrar rhimou

Amirrar rhimou

Ma plus grande chance est de t’avoir pour mère.

Ton amour inquiet, ta confiance en moi, ta

tendresse débordante m’ont encouragé à

surmonter toutes les difficultés durant mon

long chemin et réaliser, enfin, le but assigné.

Aucune dédicace ne serait aussi belle pour

t’exprimer mon profond amour et ma gratitude.

(6)

A ma sœur nawal

A ma sœur nawal

A ma sœur nawal

A ma sœur nawal

Source de joie perpétuelle ! Tu m’as

toujours donné le meilleur de toi-même.

Les mots me manquent pour te dire

combien tu m’es chère.

Que dieu te garde.

(7)

A ma

A ma

A ma

A ma sœur Bouchra

sœur Bouchra

sœur Bouchra, , , , à son mari

sœur Bouchra

à son mari

à son mari

à son mari

omar et

omar et

omar et

omar et à ma ni

à ma ni

à ma ni

à ma nièce Aya

èce Aya

èce Aya

èce Aya

Je vous exprime à travers ce travail mes

sentiments fraternels et chers que j’ai pour

vous.

Je vous souhaite à tous, tout le bonheur

que vous méritez.

(8)

A mes frères :

A mes frères :

A mes frères :

A mes frères :

Nulle expression ne saurait exprimer

l’amour, la tendresse, et l’attachement que

j’ai pour vous.

Veuillez trouver ici l’expression de mes

souhaits les plus sincères de santé, de foi et de

(9)
(10)

A notre maître

A notre maître

A notre maître

A notre maître, , , , président de thèse

président de thèse

président de thèse

président de thèse ::::

Monsieur

Monsieur

Monsieur

Monsieur H

H

H

Hicham Tahri

icham Tahri

icham Tahri

icham Tahri,,,,

Professeur agrégé d’ophtalmologie au

Professeur agrégé d’ophtalmologie au

Professeur agrégé d’ophtalmologie au

Professeur agrégé d’ophtalmologie au

CHU Hassan II de Fè

CHU Hassan II de Fè

CHU Hassan II de Fè

CHU Hassan II de Fèssss

''''

Vous nous avez fait le grand honneur

d’accepter la présidence de notre jury. Votre

sagesse et vos qualités humaines resteront pour

nous le meilleur exemple. Nous vous

remercions vivement et nous vous exprimons

notre profond respect et notre sincère

gratitude.

(11)

A notre maître

A notre maître

A notre maître

A notre maître, , , , rapporteur de thèse

rapporteur de thèse

rapporteur de thèse

rapporteur de thèse ::::

Madame Ouafae Messouak

Madame Ouafae Messouak

Madame Ouafae Messouak

Madame Ouafae Messouak,,,,

Professeur agrégé de neurologie

Professeur agrégé de neurologie

Professeur agrégé de neurologie

Professeur agrégé de neurologie au

au

au

au

CHU Hassan II de Fès

CHU Hassan II de Fès

CHU Hassan II de Fès

CHU Hassan II de Fès....

Vous nous avez honoré en nous confiant ce

travail que vous nous avez inspiré et que vous

avez guidé dans sa réalisation par vos précieux

conseils et avec une grande sympathie.

Votre sens scientifique ; Votre méthode de

travail et votre dynamisme resteront pour nous

un exemple à suivre.

C’est ici pour nous l’occasion de vous témoigner

notre profonde reconnaissance et notre gratitude.

(12)

A notre maître, juge de thèse

A notre maître, juge de thèse

A notre maître, juge de thèse

A notre maître, juge de thèse ::::

Mon

Mon

Mon

Monsieur

sieur

sieur

sieur Mohammed Faouzi

Mohammed Faouzi

Mohammed Faouzi Bela

Mohammed Faouzi

Bela

Bela

Belahhhhsen

sen

sen....

sen

Profess

Profess

Profess

Professeur agrégé de neurologie

eur agrégé de neurologie

eur agrégé de neurologie

eur agrégé de neurologie au CHU

au CHU

au CHU

au CHU

Hassan II de Fès

Hassan II de Fès

Hassan II de Fès

Hassan II de Fès

''''

Nous vous remercions du grand honneur que

vous nous faites en acceptant aimablement de

faire partie de notre jury de thèse.

Qu’il nous soit permis de vous exprimer notre

profonde gratitude et notre respectueuse

considération.

(13)

A notre maître

A notre maître

A notre maître

A notre maître, , , , juge de thèse

juge de thèse

juge de thèse

juge de thèse ::::

Monsieur Mohammed El Abkari

Monsieur Mohammed El Abkari

Monsieur Mohammed El Abkari

Monsieur Mohammed El Abkari,,,,

Professeur agrégé d’hépato

Professeur agrégé d’hépato

Professeur agrégé d’hépato

Professeur agrégé d’hépato----gastro

gastro

gastro----

gastro

entérologie au CHU Hassan II de

entérologie au CHU Hassan II de

entérologie au CHU Hassan II de

entérologie au CHU Hassan II de

Fès

Fès

Fès

Fès....

Nous vous remercions d’avoir bien voulu

siéger parmi le jury de notre thèse.

Nous sommes honorés de votre présence

et veuillez trouver dans ce travail,

L’expression de notre reconnaissance et

notre profond respect.

(14)

2:( 5

2:( 5

2:( 5

2:( 5

27+ 8

27+ 8

27+ 8

27+ 8

2:( 5 2:8 9+27+/ 8 1+ 8 =287+ 4 78/5*07+/ + 7/8+<* *E+ 1 =287+ 4 (2 12(25+ 5 ! +( / 6 7*5 8 78/ = 07+9 ' ,. 27+ 7 7 1 7 /5 . 8 727+/ 5 /: 8927+/ ., : 8927+/ , . : 8927+/ .F : 8927+/ F . : 8927+/ .# : 8927+/ # 8/+ + 1 =287+ 4 (2 12(25+ 5 ! +( / 6 ( 5/ 5 (2 (+77 827*8 ' 2=+78 4 D 82(+7 *8 (2 12(25+ 5 ! +( / , . =+5 1+/(/;+ . = 07 ; 7+<* 5 (2 12(25+ ., D 82(+7 . *727+/ F. > +/=27 /(/;+ F., 0*+98 F.,., 2 8 =287+7+/ 5* 0*+98 F.,. 2==/87 5* *+98 F. 72:/(+ 1 5 0*+98 F.F / <* 0 5 *727+/ 4 +1=207 0 G ( 2(25 F. 9/(*7+/ 27*8 (( 5 (2 12(25+ 2=+78 4 2 +A 727+/ 0(+ +<* ,. /81 >1=7/127+<* 4 ,., 2 +A 727+/ + 2*;*82( ,. 2 +A 727+/ *8/(/;+<*

(15)

,. ., > 58/1 E782=>821+52( ,. . > H+ + ,. .F > 287 8+ ,. . 72E+ 7 78 1/8 =/ 7*82( 7 + 7 7+/ ( ,.F 2 +A 727+/ = >0 +278+<* ,. 2 +A 727+/ E782. *8/(/;+<* ,. ., 77 + 7 =27/:+(+2+8 7 ;2 78/.+ 7 7+ 2( ,. . 77 + 7 127/(/;+<* ,. .F 77 + 7 /0*(2+8 ,. . 77 + 7 8 2( ,. .# 77 + 7 / 7 /.287+0*(2+8 ,. .I 77 + 7 5/08+ + ,. .J 77 + 7 0285+/92 0*(2+8 ,. . 77 + 7 5 (2 = 2* 7 5 = 2 8 ' ,. . 77 + 7 +11* /(/;+<* . /81 2 >1=7/127+<* 2=+78 4 +2; / 7+0 =/ +7+A ,. 8;*1 7 0(+ +<* . 8;*1 7 :+/(/;+<* ., E21 / = 0+A+<* . +(2 0*=8+<* . ., 0*+98 7/72( . . 0*+98 (+:8 . .F 2 0 8*(/=(2 1+ . . 0*+98 *8+ 2+8 . .# 0*+98 =27+<*

(16)

F., +2; / 7+0 + 5+8 07 A21+(+2( F. +2; / 7+0 ; 7+<* 5+8 07 F.F 5+027+/ 5 5+2; / 7+0 ; 7+<* . 8;*1 7 825+/(/;+<* ., *8/. 12; 8+ . 12; 8+ =27+<* .F 25+/(/;+ / * #. ( 078/= > +/(/;+ I. 27/1/=27 /(/;+ J. 1280 7827 ;+<* - *+98 2=+78 4 +2; / 7+0 5+AA 8 7+ ( 2=+78 4 82+7 1 7 7 =8/ / 7+0 ,. 82+7 1 7 ,., + 7 7+<* ,. 2 . +0+((21+ ,.F 2 78+ 7+ ,. K+ 0 ,.# 7 7827 +/1/(>:527 ,.I 7+/E>52 7 ,.J 782+7 1 7 52 5 +7*27+/ = 0+A+<* ,. J., 27+ 7 2 >1=7/127+<* ,.J. 82+7 1 7 5B 78 7+ ,.J.F +88 / 5 0/1= ,.J. *AA+ 2 0 =27+<* 2+;* ,.J.# 52 7 (2 ;8/ ,.J.I 2 782 =(2 727+/ =27+<*

(17)

,. ., 82+7 1 7 5 A/81 =27+<* ,. . 82+7 1 7 5 A/81 *8/(/;+<* ,. .F 82+7 1 7 5)*92 7 ,. 2 *89 +((2 0 ,. ., + +7+27+/ 5* 782+7 1 7 2. =27+ 7 >1=7/127+<* :. =27+ 7 =8 >1=7/127+<*

,. .

Surveillance de l'efficacité de traitement

,. .F

Surveillance de la compliance au traitement

. 8/ / 7+0 %*278+ 1 =287+ 4 ,. + 0* +/ 7 0/11 72+8 *8 (2 8+ . %* ( 782+7 1 7 0 /+ +8 L F. / 0(* +/ 4 *1 +:(+/;82= +

(18)

2:( 5

2:8 9+27+/

2:( 5

2:( 5

2:8 9+27+/

2:8 9+27+/

2:( 5

2:8 9+27+/

C 4 2 2* 5 C2> 8. ( + 0 8 4 2(2 + 21+ /.782 A 82 4 2 =28727 21+ /.782 A 82 4 25 / + 78+.= / = 27 4 2*7/8+ 27+/ 7 1=/82+8 5B*7+(+ 27+/ 4 0/ 0 7827+/ 0/8=* 0*(2+8 1/> 1/;(/:+ 4 0 78 27+/ 2( 5 8 A 8 0 4 0 8*(/=(2 1+ 8+:(2; 4 + ;( 782 5 0/ A/8127+/ =/(>1/8= + 1 * 4 0*+98 D 4 A+:8// /;2 78/5*/5 2( : 4 1/;(/:+ ; 4 +11* /;(/:*(+ 4 +12; 8+ =28 8 / 2 0 12; 7+<* C: 4 H+(/:2 4 > (+ /(> 78/=/ 7+<* 4 /812( 4 *1 827+/ /81*( 2 ;*+ . 4 =/(>1 82 0 2+ 8 207+/ . 8 78+07+/ A82;1 7 ( ;7 =/(>1/8= + 1 4 8 A( E / 7 /7 5+ *E 4 7/1/5 +7/1 78+ 4 72*E 5 =8/7 8/1:+ D 4 9/(*1 ;(/:*(2+8 1/>

(19)

8888 1+ 8

1+ 8

1+ 8

1+ 8

287+

287+

287+

(20)

78/5*07+/

78/5*07+/

78/5*07+/

78/5*07+/ 4444

2 12(25+ 5 ! +( / /* (2 5 ; 8 0 0 =27/( 7+0*(2+8 7 * 7/E+0/ 0*=8+<* 8 5+72+8 M 5 782 1+ +/ 2*7/ /1+<* 8 0 +9 5* - * 1*727+/ M * + 87+/ /* * 5 ( 7+/ 5* ; J (/02(+ *8 ( 0 8/1/ /1 ,F<, 'F. < ,', 0/52 7 =/*8 * =8/7 + 782 1 1:82 2+8 5 782 =/87 5* 0*+98 M <*+ . 782N * 5+1+ *7+/ /* * 2: 0 5B E08 7+/ 5* 0*+98 52 (2 :+( 7 / 20. 0*1*(27+/ 7/E+<* 52 (B/8;2 + 1 7+ (( 1 7 52 ( A/+ M ( > 7 1 8. 9 *E 0 782( 7 (2 0/8 OIIP' B 7 * 12(25+ 828 M ( 7*5 7+1 7 <*B+( > 2 * A2 7 12(25 =/*8 F 2+ 2 0 O,# P OF P' 5 :*7 5 (2 >1=7/127/(/;+ 0(+ +<* 7 78 828 292 7 (3Q; 5 F 2 M (2 12(25+ 5 9 (/== ( =(* /*9 7 52 (2 =8 1+ 8 /* (2 5 *E+ 1 5 0 + 5 (2 9+ ' ( +; 0(+ +<* / 7 78 =/(>1/8= M 5/1+ =28 * >1=7/127/(/. ;+ =27+<* " =27+7 2+;* & $M * >1=7/127/(/;+ *8/(/;+<* "(2 5> 7/ + M( 78 1:( 1 7M( 78/*:( 5 (2 1280 ''''$ <*+ 7 9/(/ 7+ 8 200/1=2; 5 78/*. :( = >0 +278+<* "78/*:( 5 (2 = 8 / 2(+7 M 5* 028207 8 /* 5 = 8A/812 0 M * 727 5 =8 +A /* = >0 / $' * +9 2* /0*(2+8 6 (32 2* 5 C2> 8. ( + 8 7 /*9 7 =8 7 52 ( A/81 *8/(/;+<* O ,,P' A/81 - =8 727+/ 127/(/;+<* M 5/08+ + M / 7 /287+0*(2+8 M 8 2( /* 0285+2<* / 7 1/+ A8 . <* 7 ' 12 +A 727+/ 5 (2 12(25+ = *9 7 8 7 8 + /( M B2 /0+ 8 /*

*00 5 8 E=(+<*2 7 (2 928+ 7 5 72:( 2*E 0(+ +<* 8 0/ 78 O,F P'

5+2; / 7+0 7 =/ =28 (2 0/ A8/ 727+/ 27/1/.0(+ +<* 7 :+/(/;+<* " *=8 . 1+ M *=8*8+ M 0 8*(/=(2 1+ =(2 127+<* $' 2 *8/.+12; 8+ " M $ 8 9 ( 5 2 /12(+ 5 +; 2( 52 ( />2*E ;8+ M ( 7 2(21* M ( 78/ 0 0 8 :82(M (2

*: 72 0 :(2 0 M <*+ / 7 8 9 8 +:( 7/*7 0/11 ( +; 0(+ +<* B+( / 7 782+7 2 G 7R7 O, P'

(21)

( B2;+7 5 (B* 5 12(25+ 8 5+72+8 ( =(* A20+( - 782+7 8' 782+7 1 7 7 :2 *8 (B*7+(+ 27+/ 5B* 0 (27 *8 5* 0*+98 /* * + +:+7 *8 5 / 2: /8=7+/ + 7 7+ 2( ' 2 . +0+((21+ S 8/(/9/( T 8 7 * 782+7 1 7 5 8 A 8 0 M +( 5/+7 U78 78 =8+ ( =(* 7R7 =/ +:( 7 +( 5/+7 =8 58 - 9+ ' (B2: 0 5 7/*7 782+7 1 7M (B 9/(*7+/ =/ 72 5 (2 12(25+ 7 ( =(* /*9 7 1/87 (( OF P' 5 =+ 72; 5 (2 12(25+ 7 + 5+ = 2:( 52 (2 A8278+ 5* 12(25 - (2 8 0 80 5 A/81 2 >1=7/127+<* <*+ =8U7 7 1+ *E 2* 782+7 1 7' *+9+ 8 ;*(+ 8 5 =27+ 7 7 + 5+ = 2:( =/*8 32 *8 8 5 (2 7/( 82 0 7 5 (2 0/1=(+2 0 2* 782+7 1 7' 3 9/(*7+/ 7 ( =8/ / 7+0 5 = 5 7 5 (3 E7 +/ 5 ( +/ 0 8 :82( 7 =27+<* 02* =28 (3200*1*(27+/ 5* 0*+98 ' 2 0 78292+(M /* 7*5+ 8/ (2 =8 727+/ 0(+ +<* 5 (2 12(25+ 5 ! +(. / M 5 207*2(+7 0/ 0 8 2 7 ( 5+2; / 7+0 7 ( 782+7 1 7 5 0 77 12(25+ ' 5 *E+ 1 =287+ /* 7*5+ 8/ * 8+ 5 0+ < =27+ 7 2>2 7 7 *+9+ =/*8 (2 12(25+ 5 ! +( / 2* 2 2 M 5+ 0*72 7 ( 8 *(727 /:7 * 52 ( :*7 5 8 92(* 8 0 77 =27 /(/;+ 5 =/+ 7 5 9* 0(+ +<* M =2820(+ +<* 7 7 82= *. 7+<* '

(22)

+ 7/8+<*

+ 7/8+<*

+ 7/8+<*

+ 7/8+<*

8/+ ;82 5 = 2 *00 5 7 52 (3 + 7/+8 5 (2 12(25+ 5 ! +( / 5*. 82 7 =(* 53* + 0( O,# P O P O P' 2 =8 1+ 8 =287+ 0/ + 7 (2 5 08+=7+/ 0(+ +<* 7 (2 0/1=8 +/ 5 (2 = > +/=27 /(/;+ ' , I, 4 8 8+0 1 7+/ =/*8 (2 =8 1+ 8 A/+ (2 12(25+ 52 / (+98 S 82+7 *8 (2 12(25+ 5 A/+ T' , F 4 ! 7= 2( 5 08+7 * 5 /858 *8/(/;+<* =8/;8 +A 29 0 5> 287 8+ 8 1/8 + 7 7+/ (M 8+;+5+7 M 272E+ M +2(/88 7 (2:+(+7 1/7+/ (( M <*+ 72+7 /11 = *5/ 0( 8/ ' , 4 78*1= (( /*(+; (2 0/ E+ 7 0 5 0 > 58/1 29 0 * 0+88 / ' , V , F 4 08+=7+/ 5 (32 2* 9 87 = 8+0/8 =28 C2> 8 =*+ =28 ( + 8' , , 4 *8/(/;* 6 ' 'C ! +( / " 82+ $ =8/=/ ( 7 81 5 5 ; 8 . 0 0 ( 7+0*(2+8 =8/;8 +9 =/*8 5 +; 8 * 2AA 07+/ 828 7 0/ 7211 7 1/87 (( 028207 8+ =28 * 0+88 / 7 * 277 + 7 *8/(/;+<* M 29 0 5> 7/ + M * 5> 287 8+ M * 5> H+ + *89 2 7 0 G ( *) 7 ) * ' , ,F 4 *1= ( 9+ 2; <* (2 12(25+ 7 5* - * 78/*:( 5* 1 72:/(+ 1 0*=8+<* ' , , 4 2(( + 78/5*+7 ( 7 81 5 5 ; 8 0 0 =27/( 7+0*(2+8 7 =8/. =/ * 782 1+ +/ 2*7/ /12( 8 0 +9 5 (2 12(25+ ' , 4 *1+ ; 1 7 9+5 0 (3+1=(+027+/ 5 (2 *80 28; 0*+98 '

(23)

2 5 *E+ 1 = 2 92 8 9/(*7+/ 8 (2 9+ 5 12(25 29 0 (2 5 0/*9 87 =8/;8 +9 5 /*9 2*E 782+7 1 7 4 , #, 4 *1+ ; =8/=/ 0/11 782+7 1 7 =28 5+1 802=8/( " ' ' W$ + ) 072. :( ' , #I 4 ! 2( =8/=/ * 782+7 1 7 0 (27 *8 = 8 / (2 . +0+((21+ 6 * 5 8+9 5 = +0+((+ M 0 1 5+021 7 5 9+ 7 2(/8 ( 782+7 1 7 5 8 A 8 0 ' 1U1 0 80 *8 , I 2 5 9 (/== (3* 2; 53* /*9 (( 1/( 0*( *7+(+ 2:( 0 G ( =27+ 7 + 7/( 82 7 - (2 . +0+((21+ 4 (2 78+ 7 >( 7 7821+ /* 78+ . 7+ ' , I, 4 0 /*X+ H *7+(+ ( K+ 0 0/11 782+7 1 7' , JJ 4 //; 8/*5 0/11 0 - 1=(/> 8 ( K+ 0 =/*8 782+7 8 ( =27+ 7 ' , F 4 8 X 8 0/ A+81 (3+ 7 8U7 5* K+ 0 52 ( 782+7 1 7 5 (2 12(25+ ' 2 5 8 + 8 = 2 7 028207 8+ =28 ( 5 0/*9 87 ; 7+<* 4 , # 4 8>512 7 ( (/02(+ 7 ( ; 5 A 07* *E *8 ( 0 8/1/ /1 ,F' , F 4 ; J 5 (2 12(25+ 7 +5 7+A+ + 5 = 5211 7 =28 78/+ <*+= *((M 2 G+ 7 @212;* +' , # 4 =8/5*+7 5* ; 7 +5 7+A+ '

(24)

*E+ 1

*E+ 1

*E+ 1

*E+ 1

=287+ 4

=287+ 4

=287+ 4

=287+ 4

(25)

2

2

2

2 12(25+ 5 !

12(25+ 5 !

12(25+ 5 !

12(25+ 5 ! +( /

+( /

+( /

+( / 6 7*5

6 7*5

6 7*5 8 78/ =

6 7*5

8 78/ =

8 78/ = 07+9

8 78/ =

07+9

07+9

07+9

,

,

,

,.

.

. 27+

.

27+

27+

27+

7

7

7

7

7 1 7 /5

7 1 7 /5

7 1 7 /5

7 1 7 /5

78292+( 7 * 7*5 8 78/ = 07+9 8 2(+ 0 G 0+ < =27+ 7 =/87 *8 5 (2 12(25+ 5 ! +( / M 52 ( :*7 5B* 8 92(*27+/ 5 0 77 =27 /(/;+ 5* =/+ 7 5 9* 0(+ +<* M =2820(+ +<* 7 7 82= *7+<* ' /: 8927+/ / 7 7 0/((+; 52 ( 89+0 5 *8/(/;+ 5* 2 2 5 M 5*82 7 * = 8+/5 5 # 2 "5* ?2 9+ 8 F 2* 5 0 1:8 J$' /* ( 12(25 2>2 7 5 >1=7R1 *8/(/;+. <* 9/027 *8 29 0 /* 2 5 +; E782. *8/(/;+<* / 7 7 + 0(* 52 0 77 7*5 ' 0+ < 12(25 / 7 * 5 E21 =2820(+ +<* 0/1=8 2 7 4 - M :+(2 =27+<* M :+(2 8 2( 6 - +(2 0*=8+<* 6 - 12; 8+ 0 8 :82( M 0 /;82= + :5/1+ 2( 7 (2 A+:8/ 0/=+ 5+; 7+9 2*7 ' 3 E21 /= 72(1/(/;+<* 29 0 (2 (21= - A 7 M - (2 8 0 80 532 2* 0/8 M 2 7 8 2(+ 0 G 7/* ( 12(25 ' 5+2; / 7+0 =/ +7+A 5 (2 12(25+ 5 ! +( / 2 7 8 7 * 5 92 7 5 28;*. 1 7 0(+ +<* M :+/(/;+<* 7 825+/(/;+<* ' *8 ( =(2 7 82= *7+<* M 78/+ 5 / 12(25 / 7 7 /*1+ - (2 . +0+((21+ 7 5 *E 12(25 / 7 =8+ ( *(A27 5 K+ 0' 52 7 (3 / =+72(+ 27+/ ( 782+7 1 7 2 7 + 78/5*+7 - 5 5/ 08/+ 2 7 7 * 5/ 53 78 7+ 2 7 8 0/112 5 2=8 (2 /87+ 5 (3 R=+72( 29 0 * *89 +((2 0 0(+ +<* 82==8/0 ' * (B2 = 07 ; 7+<* 7 8 5+72+8 5 0 77 12(25+ M 3 <*U7 A21+(+2( - (2 8 . 0 80 5 02 +1+(2+8 2 7 8 2(+ =28 (3 E21 0(+ +<* 5 (2 A8278+ 5 5 *E 12(25 5 (2 8+ M 0 = 52 7 ( 2*78 A8278+ B/ 7 =2 7 E21+ =28 8 A* 5 (2 A21+(( '

(26)

.

.

.

. 8

8

8

8

727+/

727+/

727+/

727+/ 5

5

5

5

/:

/:

/:

/:

8927+/

8927+/

8927+/

8927+/

.

.

.

.,

,

,

,

:

:

:

:

8927+/

8927+/

8927+/

8927+/

,

,

,

,

( 32;+7 5 (( 'C Q; 5 , 2 M + * 5B* 128+2; 0/ 2 ;*+ 5 =8 . 1+ 8 5 ;8 M 5 *E+ 1 53* A8278+ 5 ' 2 2 7 0 5 7 M / /7 * =+ /5 5B+07 8 7 * 21 /88 =8+12+8 ' (3Q; 5 ,J 2 M (2 =27+ 7 2 =8 7 * 78/*:( 5 5 ;(*7+7+/ 7 5 (2 1280 M I 1/+ =(* 7285M (( 2 =8 7 * 5> 28. 7 8+ M * 1*7+ 1 7 * >= 8 +2(/88 ' 98+ 8 FM (2 =27+ 7 A*7 / =+72(+. 2* 89+0 5 *8/(/;+ M 0 G <*+ (3 E21 0(+ +<* 78/*92+76 2 727+/ 5 :/*7 7 (2 1280 / 7 +1=/ +:( M ( 8 A( E / 7 /.7 5+ *E 5 1 1:8 + A 8+ *8 / 7 =8 7 >1 78+<* 7 9+A M 8 A( E 2* *E 7 2:/(+ 7 * 2=82E+ 5 (2 1280 7 53 2:+((2; ' 3 E21 /= 72(1/(/;+<* - (2 (21= - A 7 2 8 9 ( * 2 2* 5 C2> 8. ( + 0 8 :+(27 82(' 8 *(727 5* :+(2 :+/(/;+<* 72+ 7 ( *+92 7 6 * 8/1:/= + - #', F 11FM * 127/08+7 2:2+ - F YM * 2:2+ - IFY' :+(2 0*=8+<* 72+7 = 87*8: 7 ( <* (2 0*=8 1+ 2:2+ - ' 1; ( " 4 ' V ,' 1; ($ 7 (2 0 8*(/=(2 1+ 1+ AA/ 58 Z ' J ; ( " 4 ' V ' ; ($' 8 7 5* :+(2 :+/(/;+<* 72+7 /812(' 3 0 8 :82( 2 1/ 78 * >. = 8 +; 2( + 7 8 2 7 ( 7 2(21* M ( />2*E ( 7+0*(2+8 53* A2[/ :+(27 . 82( 7 >1 78+<* "A+;',$' B 0 /;82= + 2:5/1+ 2( 2 /:) 07+9 * A/+ 7 8/; 5 0/ 7/*8 +88 ;*(+ 8 29 0 >=/78/= + 5* (/: ;2*0 M 2 =( /1 ;2(+ + 5+(2727+/ 5* 78/ 0 =/87 ' 2 D 2 1/ 78 (2 =8 0 5 F 0/85/ 928+<* *E 725 2* , F + A 8+ *8 5 (3\ /= 2; ' 5+2; 5+2; 5+2; 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 2 7/ 7+0 5 (2 12(25+ 5 ! +( / 2 7/ 7+0 5 (2 12(25+ 5 ! +( / 2 7/ 7+0 5 (2 12(25+ 5 ! +( / 2 7 8 78 78 78 7 **** *8 (*8 (*8 (*8 ( 08+7 808+7 808+7 808+7 8 0(+ +<*0(+ +<*0(+ +<*0(+ +<* 7 =287 =287 =287 =282.2.2.2. 0(+ +<* 0(+ +<* 0(+ +<* 0(+ +<* '''' 782+7 1 7 =28 (2 . +0+((21+ A*7 + 72*8 - 5 5/ =8/;8 +. 9 288+92 7 - , ; )M 2 /0+ - * 8 ;+1 2(+1 72+8 =2*98 0*+98 M * 782+7 1 7 =8 9 7+A 5 928+0 \ /= 2;+ - :2 5B* :U72:(/<*2 7 " $ ] 0=

(27)

0(+ +<* 6 * 8 ;8 +/ 5 +; *8/(/;+<* 29 0 (2 = 8 + 72 0 5 (B C ' B E21 0(+ +<* 5 (2 A8278+ 5 (2 12(25 B2 =* =2 U78 AA 07* =28 8 A* 5 (2 A21+(( '

8:8 ; 2(/;+<* 4

2 / G ?2 2 : /

2 + 5 E 2 12(25+ 5 ! +( /

(28)

+;*8 , 4 0 8 :82( 0/*= 2E+2( (2+8 1/ 782 7 5 >= 8 +; 2*E 2* +9 2* 5 7 2(21* M 5 />2*E ( 7+0*(2+8 53* A2[/ :+(27 82( 7 >1 78+. <* '

Figure 2 : IRM cérébrale en coupe axiale

FLAIR ne montre pas d'anomalie

(29)

.

.

.

.

:

:

:

:

8927+/

8927+/

8927+/

8927+/

( 32;+7 5 (3 A2 7 '@ Q; 5 , 2 M + * 5B* 128+2; 0/ 2 ;*+ 2>2 7 0/11 2 7 0 5 7 5 *E \ *8 5 0 5 *+7 - (2 12(25+ 5 ! +( / ' 8/+ 1/+ 292 7 (2 0/ *(727+/ M ( =27+ 7 2 =8 7 5 1/*9 1 7 2 /812*E 5 <*278 1 1:8 7>= 5 5> 7/ + =*+ (2 >1=7/127/(/;+ 3 7 8+0 + =28 (32==28+7+/ 53* 5> = 2;+ 7 53* 5> 287 8+ ' /9 1:8 M ( =27+ 7 A*7 / =+72(+ 2* 89+0 5 *8/(/;+ M 0 G <*+ (3 E21 0(+ +<* 78/*92+76 +; 5 /1: 8; 7 =/ +7+AM * 1280 =2 7+<* 29 0 277+7*5 5> 7/ +<* "=+ 5 1 7. 7 7 E7 +/ /* A( E+/ =(2 72+8 A/80 $ 29 0 5 1/*9 1 7 2 /812*E 5 A( E+/ 7 53 E7 +/ 5 =/+; 7 M 5/+;7 E7 +/ 7 255*07+/ M * >= 8. 7/ + ; 82(+ 7 78/*:( 5 0//85+ 27+/ 29 0 = 87*8:27+/ 5 (B =8 *9 5/+;7 G 7 5 (B =8 *9 5 128+/ 77 ' 3 E21 /= 72(1/(/;+<* - (2 (21= - A 7 2 1+ 9+5 0 * 2 2* 5 C2> 8. ( + 0 8 :+(27 82(' 8 7 5 (B E21 /. 127+<* 72+7 2 =287+0*(28+7 ' :+(2 :+/(/;+<* 2 7 8 2(+ 0 G 0 =27+ 7 7 +( 2 1/ 78 * 7 8/1:/= + - J ', F 11FM * 2:2+ - YM * 0>7/. (> " - FM# 7 - $M ( 8/(/;+ >= +(+7+<* 7 =27+7+<* 72+ 7 ;27+9 ' :+(2 0*=8+<* 72+7 = 87*8: 6 (2 0*=8 1+ 2:2+ - 'F 1; (M ( 72*E 5 0 8*(/=(2 1+ 1+ 7 + A 8+ *8 - ', ; (M ( 8 7 5* :+(2 :+/(/;+<* 72+7 /8. 12(' 2 0 8 :82( 2 1/ 78 * >=/5 +7 5 5 *E =*721 7 * 5+ . 08 7 02(0+A+027+/ 5 5 *E =2((+5*1 "( +/ :+(27 82( 5 />2*E ( 7+0*(2+8 $M 29 0 278/= + 0/87+0/. /* .0/87+02( "A+;'F$' B 0 /;82= + 2:5/1+ 2( 2 1+ 9+. 5 0 * 2 = 07 A29 *8 53 >= 87 +/ =/872( 29 0 A/+ 7 8/; "(2 827 7 2*;1 7 5 72+(( 7 53 0 / 78*07*8 /1/; M ( 78/ 0 =/87 7 5+(27 $ 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 2 7 8 7 * 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 2 7 8 7 * 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 2 7 8 7 * 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 2 7 8 7 * :2 2 7 *8 (:2 2 7 *8 (:2 2 7 *8 (:2 2 7 *8 ( 28;*28;28;28;***1111 7777 0(+ +<* 7 =2820(+ +<* * 0+7 0(+ +<* 7 =2820(+ +<* * 0+7 0(+ +<* 7 =2820(+ +<* * 0+7 0(+ +<* 7 =2820(+ +<* * 0+7 '''' 782+7 1 7 29 0 . +0+((21+ "

(30)

(2 A8278+ 5* 12(25 B2 =2 8 9 ( 5B2 /12(+ ' =8 5 *E 1/+ 5 782+7 1 7M ( 0*=8+= 2 7 288U7 82+ / 5 2 / 5+ =/ +:+(+7 M 8 1=(20 =28 ( *(A27 5 K+ 0 "K+ 2 H+ #1;$ ,0= 78/+ =8+ =28 )/*8' /* 782+7 1 7 (B 9/(*7+/ 2 7 128<* =28 * 8 ;8 +/ 1/5 8 5 +; 0(+ +<* '''' 8:8 ; 2(/;+<* 4

I

II

Cas index

III

Y S Mi N Kh Me

Maladie de Wilson

Cas décédé

Figure 3: TDM cérébrale en coupe axiale montrant

(3 >=/5 +7 5 5 *E =*721 29 0 278/= + 0/87+0/. /* .0/87+02( '

(31)

.

.

.

.F

F

F

F

:

:

:

:

8927+/

8927+/

8927+/

8927+/

F

F

F

F

( 32;+7 5 8 'C M Q; 5 ,J 2 M + * 53* 128+2; 0/ 2 ;*+ 5 =8 1+ 8 5 ;8 M 2 2 7 0 5 7 =27 /(/;+<* /72:( ' B + 7/+8 5 (2 12(25+ 292+7 5 . :*7 *A 1/+ 292 7 (2 0/ *(727+/ /^ ( =27+ 7 2 =8 7 * 78/*:( 5 0/1=/8. 7 1 7M 5 08+ 5 8+8 M * >= 8 +2(/88 M 5 5> = 2;+ M 78/*:( 5 (2 ;2; M 78/*:( 5 (2 1280 7 (B2==28+7+/ =8/;8 +9 53* 8 7285 + 7 (( 07* (' /9 1. :8 IM ( =27+ 7 A*7 / =+72(+ 2* 89+0 5 *8/(/;+ M 0 G <*+ (3 E21 0(+. +<* 78/*92+76 * 78/*:( 5 (2 1280 " >= 8 (/85/ M :82 8 78/=*( +/ 1280 - = 7+7 =2 M =+ 5 0/(( 2* /( 7 5+1+ *7+/ 5 :2(2 0 1 7 5 :82 $M ( / 7 =8 7 29 0 78 =+527+/ =+( =7/_5 5 5 *E 0R7 7 * >= 87/ + ; . 82(+ 29 0 = /1 5 (2 8/* 5 7 ' 2 0/ *(727+/ /= 72(1/(/;+<* 8 78/*. 92+7 * 2 2* 5 C' :+(27 82(' 8 7 5 (B E21 0(+ +<* 72+7 /812( 5 /8 5B* =( /1 ;2(+ ' *1 827+/ A/81*( 2 ;*+ 1/ 782+7 * 2 1+ 1/. 5 8 " :`, 'F; 5($M >=/0 8/1 " ` F '#; 5($M /81/0>72+8 " D ` a1F$ 7 * 8/1:/= + - J ', F 11F' :+(2 0*=8+<* 72+7 = 87*8: 7 ( <* (2 0*=8 1+ 2:2+ - 'F 1; (M (2 0 8*(/=(21+ 1+ AA/ 58 Z ', ; ( 7 (2 0*=8*. 8+ 5 ( 9 - ,F a; " Z# a; /* Z M a1/( $M ( 8 7 5* :+(2 :+/(/;+<* 72+7 2 =287+0*(28+7 ' B 0 8 :82( 2 /:) 07+9 , * 2 = 07 >=/. +; 2(M * 2 = 07 >= 8 +; 2( + 7 8 2 7 ( =*721 5 (/0* +; 8M 5 A2+ 0 2*E =>821+52*E 2* +9 2* 02= *(2+8 5 1U1 <*32* +9 2* 5* 1 . 0 = 2( 7 5* / M 8 = 072 7 ( />2*E 8/*; M 0 77 277 + 7 7 :+(27 82( 7 >. 1 78+<* "A+;' M#$' B 0 /;82= + 2:5/1+ 2( 2 1+ 9+5 0 M * A/+ 7 8/; 5 0/ 7/*8 +88 ;*(+ 8 M 29 0 * =( /1 ;2(+ A2+ 2 7 9/<* 8 * >= 87 +/ =/872( *8 A/+ 5 0+88 / ' 2 D 2 1/ 78 0/85/ 928+<* *E 725 2* +. 9 2* 5* , F + A 8+ *8 5 (3\ /= 2; ' 5+2; / 7+0 55+2; / 7+0 55+2; / 7+0 55+2; / 7+0 5 (2(2 12(25+ 5 ! +( /(2(2 12(25+ 5 ! +( /12(25+ 5 ! +( /12(25+ 5 ! +( / 2222 7777 72:(+ :2 2 7 *8 ( =2821 78 0(+ +<* 7 =2820(+ +<* * 0+7 72:(+72:(+ :2 2 7 *8 (:2 2 7 *8 ( =2821 78=2821 78 0(+ +<*0(+ +<* 7 =2820(+ +<*7 =2820(+ +<* * 0+7* 0+7 72:(+ :2 2 7 *8 ( =2821 78 0(+ +<* 7 =2820(+ +<* * 0+7 ''''

(32)

782+7 1 7 29 0 . +0+((21+ " # 1; $ 2 7 + 72*8 - 5 5/ 08/+ 2 7 M 288+92 7 - 0= ) 2*<* ( B 7 2 /0+ * 782+7 1 7 =8 9 7+A 5 (2 8*=. 7*8 5 928+0 \ /= 2;+ " (9/0285>( 1; b 0= )$' =8 * 2 5 782+7 . 1 7 29 0 * :/ AA+020+7 7 7/( 82 0 M ( =27+ 7 2 + 7 88/1=* ( 82+ / 5 2 / 5+ =/ +:+(+7 M 8 1=(20 =28 ( *(A27 5 K+ 0 "K+ 2 H+ #1;$ ,0=E )' * 0/*8 5 0/ *(727+/ M (2 *89 +((2 0 0(+ +<* 0/ 727 * 21 (+/82. 7+/ 5 78/*:( 5 (2 ;2; M 5 78/*:( 5 (2 1280 7 * 5+ =28+7+/ 5 78/*:( 5 5 ;(*7+7+/ M / /7 ;2( 1 7 * 77 21 (+/827+/ 5* 0/1=/87 1 7 7 5* +. 9 2* + 7 (( 07* ( 2 29/+8 (B2==28+7+/ 5 78/*:( = >0 +278+<* ' B E21 0(+ +. <* 78/*9 * 5+1+ *7+/ 5 :2(2 0 1 7 5 :82 2* 0/*8 5 (2 1280 M ( / 7 =8 7 29 0 78 =+527+/ =+( =7/_5 5* 0/7 58/+7 M * >= 87/ + 1+ +1 29 0 = /1 5 (2 8/* 5 7 ' G (2 A8278+ 5 0 =27+ 7M (B E21 0(+ +<* 72+7 78+07 1 7 /812('

(33)

33 8:8 ; 2(/;+<* 4

III

IV

Cas index Ah Kh Mu Ih Hi Me Ha An Fa

La maladie de Wilson

(34)

Figure 4 : coupe axiale d'IRM cérébrale en séquence montrant

5 >= 8 +; 2*E + 7 8 2 7 ( =*721 7 ( (/0* +; 8

.

Figure5 : coupe axiale d'IRM cérébrale en séquence Flair montrant des hypersignaux

d'une façon bilatérale et symétrique,

5 =*721 M 5 (/0* +; 8M 5 1U1 <* 5 A2+ 0 2*E =>821+52*E 2* +9 2* 02= *(2+8 '

(35)

.

.

.

.

:

:

:

:

8927+/

8927+/

8927+/

8927+/

( 32;+7 5 (( ' Q; 5 ,J 2 M 2>2 7 0/11 2 7 0 5 7 * =+ /5 53+07 8 - (3Q; 5 # 2 M * 21 /88 =8+12+8 7 (( 292+7 5 *E \ *8 5 0 5 *+7 - (2 12(25+ 5 ! +( / ' B + 7/+8 5 (2 12(25+ 292+7 5 :*7 78/+ 1/+ 292 7 (2 0/ *(727+/ /^ (( 2 =8 7 * 78/*:( 5 (2 ;2; M * 2H+ + 7 * 5+1+ *. 7+/ =8/;8 +9 5 8 5 1 7 0/(2+8 ' ?*+ JM (2 =27+ 7 A*7 / =+72(+ 2* 89+0 5 *8/(/;+ M 0 G <*+ (3 E21 *8/(/;+<* 78/*92+7 **** > 58/1> 58/1> 58/1> 58/1 E78E782.E78E782.2.2. =>821+52( 2H+ 7/ =>821+52( 2H+ 7/ =>821+52( 2H+ 7/ =>821+52( 2H+ 7/....8+;+58+;+58+;+5 MMMM ( 88+;+5 7 5 (B E21 0(+ +<* 8 78/*92+7 <*B* =( /. 1 ;2(+ A 81 ' 3 E21 /= 72(1/(/;+<* - (2 (21= - A 7 2 5 0/*9 87 (2 =8 . 0 5B* 2 2* 5 C2> 8. ( + 0 8 :+(27 82(' *1 827+/ A/81*( 2 ;*+ 1/ 782+7 * 2 1+ 1/5 8 " :` 'I; 5($M /81/0 8/1 " `F '# ; 5($ 7 1+08/0>72+8 " D `J a1F$' B2 2(> 5* :+(2 0*=8+<* 2 8 9 ( * :2+ 5 (2 0*=8 1+ 7 5 (2 0 8*(/=(21+ 1+ 2 /0+ - * 2*;1 727+/ +1=/872 7 5 (2 0*=8*8+ 5 ' 8 7 5* :+(2 :+/(/;+<* 72+7 78+07 1 7 /812(' B 0 8 . :82( 2 5 7 07 (2 =8 0 53* +; 2( + 7 8 2 7 ( />2*E ;8+ 0 782*E 5 A2[/ :+(27 82( 7 >1 78+<* M 0 7 >= 8 +; 2( + 7 8 ;2( 1 7 ( 1 0 = 2( 7 ( 7 ;1 7*1M 8 = 072 7 ( />2*E 8/*; "A+;' IMJ$' /7 2* + (2 =8 0 53* ( +/ /5*(2+8 0 7+1 78+<* 0/87+0/. /* .0/87+02( =28+ 72( 58/+7 M >=/ +. ; 2( , 7 >= 8 +; 2( 6 0 7 2 = 07 7 A29 *8 5B* 5 =R7 =28212; 7+.

<* ' B 0 /;82= + 2:5/1+ 2( 2 /:) 07+9 * 9/(*1+ * =( /1 ;2(+ /1/. ; M * A/+ 7 8/; 7 * 1+ +1 =2 0 1 7 + 782.= 8+7/ 2(' 2 D B2 =2 1/ 78 532 /12(+ ' 5+2; / 7+0 5 12(25+ 5 !5+2; / 7+0 5 12(25+ 5 !5+2; / 7+0 5 12(25+ 5 !5+2; / 7+0 5 12(25+ 5 ! +( /+( /+( /+( / 2 72 72 72 7 0/ A+81 =28 (0/ A+81 =28 (0/ A+81 =28 (0/ A+81 =28 ( 2 2 2 28888;*1;*1;*1;*1 7 0(+ +<*7 0(+ +<*7 0(+ +<*7 0(+ +<* 7 =2820(+ +<*7 =2820(+ +<*7 =2820(+ +<*7 =2820(+ +<* * 0+7* 0+7* 0+7* 0+7 '''' 782+7 1 7 =28 ( *(A27 5 K+ 0 "K+ 2 H+ #1;$ 2 7 + 72*8 ",0= 78/+ =8+ $ 29 0 * 7/( 82 0 E0 (( 7 M B 9/(*7+/ *8/(/;+<* 7 128<* =28 (2 72:+(+ 27+/ 5 (2 >1=7/127/(/;+ 6 (2 = 8 + 72 0 5 > 58/1 E782=>821+52( 2H+ 7/.8+;+5 29 0 78 1:( 1 7 5+ 08 7

(36)

5B277+7*5 M * 5> 287 8+ 7 * >= 8 2(+927+/ ' /* 782+7 1 7M (2 =27+ 7 2 =8 . 7 5 78/*:( = >0 +278+<* - 7>= 5B2;8 +9+7 7 5 5 + +:+7+/ <*+ / 7 7 8 ;8 /* * *8/( =7+<* " 2(5/($'

(37)

2 : 0 +;*8 I 4 0/*= 2E+2( 5B 0 8 :82( <* 0 1/ 782 7 * >= 8 +; 2( + 7 8 2 7 ( />2*E ;8+ 0 782*E"2M:$M ( 1 0 = 2( 7 ( 7 ;1 7*1"0$

(38)

+;*8 J 4 0/*= 2E+2( 5B 0 8 :82( <* 0 (2+8 1/ 782 7 * >= 8 +; 2( + 7 8 2 7 ( />2*E ;8+ 0 782*EM ( 1 0 = 2( 7 ( 7 ;1 7*1

(39)

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( 32;+7 5 8 ' M Q; 5 , 2 M 1 53* A8278+ 5 M +( B292+7 2*0* 2 7 0 5 7 =27 /(/;+<* 7 =28 7 B 72+ 7 =2 0/ 2 ;*+ ' (3Q; 5 ,J 2 M ( =27+ 7 =(2+; 2+7 5B* A2+:( 5 1 1:8 + A 8+ *8 29 0 5 0/ 7820. 7+/ 1* 0*(2+8 + 9/(/ 72+8 200 7* =28 (2 1280 7 (3 AA/87 2 /0+ - * 7/87+0/(+ =2 1/5+<* 5 (2 *<* ' 07/:8 JM ( =27+ 7 A*7 / =+72(+ 2* 89+0 5 *8/(/;+ M 0 G <*+ (3 E21 *8/(/;+<* 78/*92+76 5 1/*9 1 7 5> 7/ +<* 5 1 1:8 =(* 128<* - (3 1+0/8= ;2*0 M ( 8 A( E 8/7*(+ / 7 9+A ' B2 2(> 5* :+(2 0*=8+<* 1/ 782+7 * 0*=8 1+ 2:2+ -'Ia1/( (" 4, '#. 'J a1/( ($ 7 * 0 8*(/=(2 1+ 1+ 2:2+ - ', ; ( " 4 ' . ' ; ($' B 0 8 :82( 2 1+ 9+5 0 <* 0 A(2+8 * >= 8 +; 2( 5 :82 =/ 7 8+ *8 M 5 02= *( + 7 8 7 5 = 5/ 0*( 0 8 :82*E "A+;', $' 5+5+2.5+5+2.2.2. ; / 7+0 5 ; / 7+0 5 ; / 7+0 5 ; / 7+0 5 (2(2(2 12(25+ 5 ! +( / 2 7 8 7(2 12(25+ 5 ! +( / 2 7 8 712(25+ 5 ! +( / 2 7 8 712(25+ 5 ! +( / 2 7 8 7 **** 82+ / 5 08+7 882+ / 5 08+7 882+ / 5 08+7 882+ / 5 08+7 8 0(+ +<*0(+ +<*0(+ +<*0(+ +<* 7 =287 =287 =287 =282.2.2.2. 0(+ 0(+ 0(+ 0(+ ++++<*<*<*<* '''' 782+7 1 7 - :2 5 *(A27 5 K+ 0 "K+ 2 H+ # 1;$ ,0= E ) 2 7 + 72*8 7/*7 *89 +((2 7 (B 9/(*7+/ 0(+ +<* 6 ( =27+ 7 2 0/ * * 2;;82927+/ 5 (2 5> 7/ + 1=U0 2 7 (2 1280 M (3 2:+((2; 7 (32(+1 727+/ 7/*7 *(' /*78 5 K+ 2 H+ M * 1>/8 (2E2 7 " 20(/A $ 2 7 =8 08+7 - 5 5/ =8/;8 +9 ' =8 1/+ M ( =27+ 7 2 288U7 ( 782+7 1 7 *+7 - (B2;;82927+/ 5 (2 >1=7/12. 7/(/;+ *8/(/;+<* '

(40)

Figure 8 : coupes axiales d'IRM cérébrales en séquence T1 ne montrant pas

d'anomalies.

Figure 9 : coupes axiales d'IRM cérébrales en séquence montrant un hypersignal

5 :82 =/ 7 8+ *8 7 5 02= *( + 7 8 '

(41)

Figure 10 : IRM cérébrale en coupe axiale Flair

montrant un hypersignal

5 :82 =/ 7 8+ *8

7 5 02= *( + 7 8 '

(42)

8/

8/

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8/++++ + 1

+ 1

+ 1

+ 1

287+

287+

287+

287+ 4444

(43)

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

(44)

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7+<* 5 (2 12(25+

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. . . ., D, D, D, D 82(+782(+782(+782(+7 (* + *8 72= / 7 128<* (3 7*5 ; 7+<* 5 (2 12(25+ 5 ! +( / ' 32:/85M ( ; 2 7 (/02(+ *8 ( 0 8/1/ /1 ,F , #M ;8Q0 - * 7*5 (+2+ / 1 52 * ;82 5 A8278+ + 82 (/.282: O,,FP' *+ M ( ; 2 7 +5 7+A+ , F ;8Q0 2* 78292+( 5 78/+ <*+= 5+AA 8 7 O POIFPO ,IP'

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c * : 72 =/(>= =7+5 2== ( J /* ! =28 * ; <*+ 2 7 + /( 7 <*+ 2 I Y 53 /1/(/;+ 29 0 (3 J 8 =/ 2:( 5 (2 12(25+ 5 H O , P' ; 5 (2 12(25+ 5 H 7 E=8+1 52 5 /1:8 *E 7+ * M -(3 E0 =7+/ 5* A/+ ' ; 5 12(25+ 5 ! +( / 7 E=8+1 =8+ 0+=2( 1 7 52 ( A/+ M ( 0 89 2* 7 ( 8 + O P O,#IP'

(45)

. . . . *727+/*727+/*727+/*727+/ =*+ / 0(/ 2; M , FM =(* 5 F *727+/ =28=+(( *8 7/* ( ; / 7 7 +5 7+A+ 12+ 0 872+ / 7 E78U1 1 7 828 O ##P' =(* /*. 9 7M (( 7/*0 7 ( E/ M M , M ,I 7 , <*+ 0/88 =/ 5 7 2*E 5/12+ A/ 07+/ ( 5 (3 J O, P' +AA 8 7 1*727+/ / 7 7 =8/*9 6 5 ( 7+/ M + 87+/ M / M A82. 1 +A7M 2* +7 53 =+ 2; M E/ H+==+ ; 52 (2 8 ;+/ 0/52 7 O,IJP O ,IP' ((

782N 7 * 5 02(2; 5* 0258 5 ( 07*8 2:/*7+ 2 7 - (2 > 7 5 =8/7 + 78/ <* =8/A/ 5 1 7 1/5+A+ '

1*727+/ ( =(* A8 <* 11 7 8 0/ 78 "F - # Y 5 02 $ / 7 + J, ;( 7 + , J ;( O,#P O,, P' 872+ 1*727+/ / 7 = 0+A+<* 53* ;8/*= 7 +<* M (2 1*727+/ (2 =(* A8 <* 7 52 ( =/=*(27+/ *8/=

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

(46)

2:( 2* , 4 8+ 0+=2( 1*727+/ +5 7+A+ 52 (2 12(25+ 5 ! +( / 4 OIFP O,#JP D *72 7 D /;82= + 5 (2 1*727+/ 28+27+/ > +/(/;+<* 28207 8 5 (2 1*727+/ ((* 7827 *8 + , J ;(> *8/= " (( 12; YM /(/; #JYM 2852+; ,FYM (2 5M *8/= 5 (3 7 FJYM 82 0 M ;( 7 88 $ /*7 928+27+/ 12+ =2 7285+A' +7 200 =7 *8 2 G+ 7 2(' ", F$ + , I D( 727 V + 28 20H2 7 2(M ", #$ /12 7 2(' ", #$' 2 V 2/ 7 2(' ", $ *8/= " /(/; Y$ /*7 928+27+/ 2 G+ 7 2(' ", F$ D(> , IJ 28; 727 . + +7 200 =7 *8 /12 7 2(' ", #$' *8/= " * + M /((2 5 $ =27+<* /* *8/(/;+<* 9 8 2 2 G+ 7 2(' ", F$ + J, D( 727 V + "F,YM Y 5 * $ /*X 7 2(' ", #$ *8/= " * + $ :*7 7285+A 2 G+ 7 2(' ", F$ , =5 ( FFJ 727 V + +7 5 = / = /8>(27+/

(47)

" * Y$ 727 . + :*7 7285+A 78*H+ 7 2(' ", F$ *(( 7 2(' ", F$ 78*H+ 7 2(' ", #$ /12 7 2(' ", #$ , # 5 (J` , 5 ,J *8/= " (2 5 $ ( 9 7 81+ 2( /*5+2 / 7 2(' ", #$ =JI# 8 +;* 7 2(' ", #$ /*5+2 / 7 2(' ", #$ ,# 8 *8/= " +0+( $ ?2=/ E/ ,F H252 7 2(' " $ J , 5 ( J,5 , ?2=/ ,IY H252 7 2(' " $ ?2=/ , Y E/ C+1 7 2(' ", $ /8 "FJY$ d* 7 2(' ", 8;JJ , * E/ + H252 7 2(' " $ C* *52 7 2(' " $ * 8 72(+ F + ?2=/ 5 ( ;(> ?2=/ *(1+ 2 7 C+1 7 2(' ", $ +1+G*

(48)

7 2(' ", $ H252 7 2(' " $ F5 (7>8 ?2=/ A*(1+ 2 7 5 1 5 ( = ?2=/ A*(1+ 2 7 5 1 F 5 (D ?2=/ C* *52 7 2(' " $ + ,,Y E/ , C+1 7 2( ", $ 8;, , 8/ 2+X2 JY ! * 7 2(' ", $ d* 7 2( ", $ + E/ , C+1 7 2( ", $ , J 8 2+X2 ! * 7 2(' ", $ d* 7 2( ", $ * + " Y$ 2 G+ 7 2(' ", F$ , := 5 ( FFJ 727 . + D(> F 8 /12 7 2(' ", #$ 8; , D(> ?2=/ /7>= 5+ =2827 2H +72 7 2(' " $ , 5 ( #,, ?2=/ /7>= 5+ =2827 2H +72 7 2(' " $ (2 J 2( ?2=/ /7>= 5+ =2827 2H +72 7 2(' " $ , J 8 72(+ /12 7 2(' ", #$

(49)

/ 72 +02 2 7 2(' ", J$ ?2=/ ", Y$ H252 7 2(' " $ /8 ", Y$

F

F

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F F F F....,,,, *+98*+98*+98*+98 0/ * 5 , JF =28 (3 0/11 /(+;/. ( 1 7 7+ (M ( *+98 7 * 1+08/ *78+1 7 A2+ 2 7 =287+ 5 5/*G 1 72*E (/*85 + 5+ = 2:( 2*E A/ 0. 7+/ :+/(/;+<* /812( OJFP' ( 7 0 2+8 2* +9 2* 5* +7 207+A 5 /1. :8 *E G>1 2;+ 2 7 0/11 0/A207 *8 52 (2 0272(> 5 8 207+/ 53/E>5/8 5*07+/ O, IP' G>1 0*=8+<* + 7 89+ 7 52 5 /1:8 *E 5/12+ 0 ((*(2+8 7 ( <* 2 0 8*(/=(2 1+ /* A 88/E>52 7 8 <*+ =/*8 ( 782 A 87 5 8 7 > . 7 5 (3 1 "028 0 *+98 1/+ 5 1; ) 3200/1=2; 5 (32 1+ $' /*8 ( 2*78 > 7 1 G>127+<* 0 +72 7 5* 0*+98 M 0+7/ 4 O ,IP . 1/ /21+ /E>52 / 7 0 2+8 - (2 =+;1 727+/ 7 - (2 8 ;*(27+/ 5 *8/782 1 77 *8 7 *8/= =7+5 ' . 2 (> >( /E>52 7 7+ (( - (3 78 7+ 5* 7+ * 0/ )/ 07+A 52 ( =/*. 1/ M (3/ 7 (3 (2 7+ 5 (32==28 +( 0285+/92 0*(2+8 ' . 2 >7/0 8/1 ./E>52 + 7 89+ 7 52 ( 1 72:/(+ 1 /E>527+AM ( A/ 07+/ . 1 7 5 0 89 2*M (2 > 7 5 (3 1 7 (2 > 7 5 = / = /(+=+5 '

(50)

F F F

F....,,,,...,., 2,, 22 8 =287+7+/ 5* 028 =287+7+/ 5* 08 =287+7+/ 5* 08 =287+7+/ 5* 0*+98*+98*+98*+98

3/8;2 + 1 53* 25*(7 2+ M 0/ 7+ 7 5 J - ,# 1; 5 *+98 M / ( 8 . 78/*9 ;82 5 <*2 7+7 52 ( A/+ ", Y$M />2*E =+;1 7 5 0 89 2*M ( 1* 0( M ( 8 + 7 ( / O P' 2 ( 7+ * M ( 0*+98 3 7 )212+ /* A/81 (+. :8 M +( 7 7/*)/*8 (+ - 5 20+5 21+ M - 5 =8/7 + "0*=8/.=8/7 + $ 7 - (2 0 8*(/=(2 1+ ' F F F F....,,,,.... 2==/87 5*2==/87 5*2==/87 5*2==/87 5* *+98*+98*+98*+98 3 2 8 0/112 5 * 2==/87 <*/7+5+ 5 F a; H; =/+5 0/8=/8 ( ) "/* M, 1; )$ 52 ( 02 53* /11 25*(7 7 a; H; =/*8 ( /*88+ / M 0 8 0/112 527+/ 72+ 7 A/ 5 *8 5 7*5 5 2==/87 0 2+8 =/*8 0/1= 8 (2 = 87 <*/7+5+ 5 0*+98 5* 2* 1 72:/(+ 1 7 - (3 E08 7+/ ' 72:( 2*E 7 F A/*8 + 7 8 = 07+9 1 7 (2 7 *8 5 0*+98 - 0/ /11 8 <*/7+. 5+ 1 7 2:( 2* 4 2==/87 0/ +(( 0*+98 "1; )$ 532=8 (32; 0 A82 [2+ 5 0*8+7 2 +72+8 5 2(+1 7 O P' A2 7 5 , - F 2 M A2 7 5 - I 2 , A2 7 5 I - 2 ,M A2 7 5 , - , 2 ,M# 5/( 0 7 " $ 5 ,F - , 2 ,M# /11 25*(7 11 25*(7 ,M# 11 0 + 7 11 2((2+72 7 8 / Q; ,M#

(51)

2:( 2* F 4 / 0 7827+/ 1/> 5 0*+98 52 ( 2(+1 7 O, #P' >= 532(+1 7 / 0 7827+/ a; H; . 8*+7 5 1 8 "=/+ / M 08* 720 7 1/((* <* $ ,M . +2 5 "9/(2+(( M =/8 M :\ *AM 2; 2*$ FM . e *A 7 =8/5*+7 (2+7+ 8 " (2+7M : *88 $ ,MJI . ;*1 "- A *+(( 2(+1 72+8 MA+ 98 M=(2 7 . 820+ $ ,M,J . 8*+7 " 28:8 A8*+7+ 8 > 0/1=8+ ( 28:8 A8*+7+ 8 78/=+02*E $ M . D82+ 7 0 8 2( M . *+( 7 ;82+ MIF . /+E , M . / 5+1 7 7 =+0 IMJI . /+ / "> 0/1=8+ 9+ M :+ 8 7 =+8+7* *E$ M F F F F.... 72:/(+ 1 572:/(+ 1 5 0*+9872:/(+ 1 572:/(+ 1 5 0*+980*+980*+98 32=8 5 7*5 8 2(+ 29 0 5 + /7/= 825+/207+A 5* 0*+98 M / 78/*9 <* (2 12) *8 =287+ 5 0*+98 7 2: /8: - =287+8 5 (3 7/120 7 5 5*/5 *1 7 <*3+( 277 + 7 0/ 0 7827+/ 12E+12( 52 ( 2 ; 5 , - F *8 2=8 (3251+ + 7827+/ /82( O, JP' 2 (3 7 8/0>7 M ( 0*+98 7 /+7 0/1=( E - (2 1 72((/7 +/ + M /+7 (+ -5 21+ /20+5 M (2 1 72((/7 +/ + )/* * ;82 5 8R( 52 (3 /1 / 72 + 5* 0*+. 98 8 ;*(2 7 / =2 2; - 7829 8 (2 =28/+ + 7 7+ 2( O P' *8 ( =(2 ; 82(M ( 1 72:/(+ 1 0 ((*(2+8 5 0*+98 52 ( 7+ * 5 (3/8;2 + 1 A2+7 + 7 89 +8 * 72= 53+ 0/8=/827+/ <*+ 0 +7 (3207+/ 53* 8 . 5*072 1 1:82 2+8 M 0/8 + 0/ * <*+ 8 5*+7 ( * =*+ ( * A82 0 +7 (2 1 1:82 0>7/=(2 1+<* ;8Q0 2* 782 =/87 *8 ,=' 2+7 12+ 7 2 7 <* 52 ( 0/1=287+1 7 0>7/=(2 1+<* ( 0*+98 = *7 U78 7/0H =28 ( 1 72((/7 +/. + /^ +( 7 =8+ 0 28; =28 * 1/( 0*( 0 2= 8/ (2 =8/7 + ,= "/* =8/7 + 7E,$M =287 2+8 0>7/ /(+<* = 0+A+<* 5* 782 =/87 *8 ! +( / 6 (3 J

(52)

"A+;*8 ,,$M O ,P 7 ( =2 2; 5* 0*+98 5 (2 =8/7 + 0 2= 8/ ,= -(3 J 3 AA 07* 2=8 0 2 ; 1 7 0/ A/8127+/ ( 25 <*27 OFIP' 2 =8/7 + J 7 (/02(+ 2* +9 2* 5* 82 .D/(;+ 0 ((*(2+8 "A+;*8 , $M (( =8 7 * 5*2(+7 5 A/ 07+/ =8/:2:( 1 7 53/8+;+ =/ 7 V 7825*07+/ (( ' (( 2 7 1+ 9+5 0 52 ( A/+ M ( > 7 1 89 *E 7 ( 8 + O,J,P' =8/7 + 5 782 =/87 5 1 72*E (/*85 / 7 * +1+(+7*5 M 12+ (3 J 2 5 028207 8+ 7+<* =287+0*(+ 8 "A+;*8 ,,$ /7211 7 4 c /7+A C D 6 +7 5 = / = /8>(27+/ "- (3/8+;+ 5 (32== ((27+/ 2 5 7>= $ OIFP O,, P' c /7+A D = 81 772 7 (2 A+E27+/ - (3 " :+ 5+ ; 5/12+ $ c <* 0 dD Dd d <*+ 8 (+ 5/12+ 5 A+E27+/ 2* ;1 7 782 1 1:82 2+8 OFP OF P O P' cI 1/7+A 8 = 7 D dd - (3 E78 1+7 7 81+ 2( 3 J M * 7*5 8 0 7 2 5 1/ 78 <* 0 1/7+A 2>2 7 * = 0+A+0+7 =/*8 ( 0*+98 7 5 (+ 8 I 1/( 5 0*+98 - * 1/( 5 .7 81+ 2( 53 J OIJPO ,P' c /7+A M 7 +1=(+<* 52 (2 8 ;*(27+/ 5* A(*E 5 0*+98 c /7+A M 7 +7* 2* +9 2* 5 I 1 (+0 782 1 1:82 2+8

5 *E 5 8 + 8 1/7+A / 7 0/ 89 =/*8 ( 782 =/87 5 1 72*E (/*85 OFP OF P O P' 77 =8/7 + = 81 7 (3+ 0/8=/827+/ 5* 0*+98 52 ( 0*=8/=8/7 + 5/ 7 (32=/0 8*(/=(2 1+ 72 7 (/02(+ 2* +9 2* 5* 8 7+0*(*1 5/=(2 1+<* 7 /* 5* 0/1=287+1 7 82 .;/(;+ M 12+ (( = 81 7 2* + ( 8 (28;2; 5* 0*+98 52 (2 :+( =28 ( :+2+ 53* 782A+0 9 +0*(2+8 5 0( 0 =28 (3 ( 927+/ 5 (2 0/ 0 . 7827+/ 0*+98 52 (3 =27/0>7 OIFP O, P O I P "A+;*8 , 7 ,F$'

(53)

+;*8 ,,` 78*07*8 5 (3 2 J OIIP'

+;*8 , 4 ( 5 *E /02(+ 27+/ "782 D/(;+ 7 9 +0*( $ 5 (B J 52 (B =27/0>7 OIJP'

(54)

+;*8 ,F 4 82 =/87 5* 0*+98 52 (3 =27/0>7 /812( 7 02 5 12(25+ 5 ! +( / OIIP' F F F F...F.FFF //// <*<*<*<* 0000 5555 1*727+/1*727+/1*727+/1*727+/ 4444 1=2071=2071=2071=207 0000 G (G (G (G ( 12(2512(2512(2512(25 G ( ! +( / + M (2 =8/7 + J 7 5 A 07* * 9/+8 2: 7 ' 0*+. 98 B277 + 7 5/ 0 + ( 02 2(+0*( :+(+2+8 + ( +7 5 > 7 5 (2 0 8*(/=(2 . 1+ ' 5 0/*( * 5+1+ *7+/ 5 (B E08 7+/ :+(+2+8 5* 0*+98 =27+<* 7 * AA/ 58 1 7 5 (2 0 8*(/=(2 1+ <*+ 7 82=+5 1 7 5 ;825 82+ / 5 (B2:. 0 5B+ 0/8=/827+/ 5* 0*+98 "A+;*8 ,F$' 2 :2(2 0 0*=8+<* 7 5/ 0 =/ +7+9 O,IP O, IP' 77 5+1+ *7+/ 5 (3 E08 7+/ :+(+2+8 5* 0*+98 =27+<* 782N * AA 7 7/E+<* *8 (3 =27/0>7 "1/87 0 ((*(2+8 $ 7 * 200*1*(27+/ 5 0*+98 52 ( 7+ . * E782 =27+<* M =287+0*(+ 8 ( 0 89 2*M (2 0/8 7 ( 8 + O IJP' 2 0 8*(/=(2 1+ 2==287+ 7 2* ;8/*= 5 f ;(/:*(+ M 7 > 7 7+ /* (2 A/81 5 (32=/0 8*(/=(2 1+ 52 ( 8 7+0*(*1 5/=(2 1+<* M (( + 0/8. =/8 ( 0*+98 52 (32==28 +( 5 D/(;+ =/*8 ( =/87 8 /8 (2 0 ((*( ' 2 0 8*(/=(2 .

(55)

1+ 7 ( =8+ 0+=2( 782 =/87 *8 5* 0*+98 52 ( =(2 12M (2 0/ 0 7827+/ =(2 . 127+<* /812( 5 0 77 =8/7 + 7 5 - 1; (' (2 5+1+ *7+/ /* (32: 0 53+ 0/8=/827+/ 5* 0*+98 - (32=/0 8*(/=(2 1+ 782N * 5+1+ *7+/ 5 (2 (+: 827+/ 0 ((*(2+8 5 0 8*(/=(2 1+ 7 5/ 0 * 5+1+ *7+/ 5 / 72*E 2 ;*+ "/: 89 0 G #Y 5 12(25 $ OIIP OFP' 5/ 2; 5 (2 0 8*(/=(2 1+ 82+7 5/ 0 * ( 1 7 +1=/872 7 12+ *87/*7 0/1. =( 1 72+8 5* 5+2; / 7+0 5 (2 12(25+ 5 ! +( / ' F F F F.... 9/(*7+/9/(*7+/9/(*7+/9/(*7+/ 27*8 (( 5 (2 12(25+27*8 (( 5 (2 12(25+27*8 (( 5 (2 12(25+27*8 (( 5 (2 12(25+ * 0/*8 5 (2 = 2 + +7+2( <*+ 0/88 =/ 5 2*E , - =8 1+ 8 2 5 (2 9+ M ( 0*+98 3200*1*( 52 ( =27/0>7 M - 0 725 ( 12(25 / 7 2 >1=7/127+<* O J P' * 0/*8 5 (2 0/ 5 = 2 M 5 I - J 2 M ( 0*+98 7 8 5+ 78+:* 5* 0>7/. =(2 1 =27/0>72+8 9 8 ( (> / /1 <*+ ( <* 782 7M / 7 * AA 7 =8/7 0. 7 *8M ( =(* /*9 7 0 782 A 87 7 =8/;8 +A 2 12 +A 727+/ 0(+ +<* /81+ 5 +; 53 =27+7 1/5 8 M 12+ =28A/+ 0 77 8 5+ 78+:*7+/ = *7 U78 :8*72( 7 782N 8 * 08/ =27/0>72+8 29 0 * =27+7 2+;* 7 * 8 (28;2; 12 +A 5* 0*+98 52 ( 2 ;6 /*80 53 1/(> O, IP OF#P' A+ M 2* 0/*8 53* F 1 = 2 M (2 0/ 0 7827+/ 5 0*+98 (+:8 =(2 127+. <* 2*;1 7 29 0 ( 927+/ 5 (2 0*=8*8+ 7 200*1*(27+/ 5 0*+98 52 ( 7+ . * E782 =27+<* ' 0 725 M +( E+ 7 A8 <* 11 7 5 ( +/ 0 8/ +<* 7 (( <*3* =27+7 0 8/ +<* 207+9 /* * 0+88 / ' (7 8+ *8 1 7M ( 0*+98 =8/9/<* 5 ( +/ 5 />2*E ;8+ 0 782*EM 8 =/ 2. :( 5 12 +A 727+/ *8/(/;+<* 0(2 +<* 1 7 5 08+7 O,#,P O ,FP'

(56)

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, , , ,...,.,,, 12 +A12 +A12 +A12 +A 727+/727+/727+/727+/ + 2*;*82(+ 2*;*82(+ 2*;*82(+ 2*;*82( 12 +A 727+/ 0(+ +<* = *9 7 =8 7 8 /* 5+9 8 A/81 7 / 7 78 7 8/; 72 7 52 ( *8 =8 727+/ <* 52 (2 527 532==28+7+/ 5 =8 1+ 8 78/*:( OJ P' 2 12(25+ 8 9 ( 0 G #Y 5 =27+ 7 =28 * >1=7/127/(/;+ (+ -(3277 + 7 =27+<* M 0 G F#Y 5 =27+ 7 =28 5 +; *8/(/;+<* 7 0 G , Y 5 =27+ 7 =28 5 78/*:( = >0 +278+<* OF P O, P' 2 ( 2*78 02 ( 12 +A 727+/ / 7 127/(/;+<* 6 8 2( /* / 7 /287+0*(2+8 O P' 3 + 7/+8 27*8 (( 2 7 2+ + 5 08+7 "532=8 /:> 1/5+A+ $ OJ P' . >1=7/127+<* 4 200*1*(27+/ 5* 0*+98 52 ( 0>7/ /( 53 =27/0>7 ' . +: 827+/ 7 8 5+ 78+:*7+/ 5* 0*+98 ' 2. >1=7/127+<* ' :. 08/ =27+<* ' 0. 1+ 1/(>7+<* ' +. 0 ' ++. 1+ +/ =/ 72 /* /* 782+7 1 7' . 00*1*(27+/ 7+ *(2+8 0 8/ +<* 5* 0*+98 ' 2. >1=7/127+<* " =/ 72 /* 782+7 $ :. 08/ =27+<* ' 0. 8 :82( "A/81 *8/= >0 +278+<* $

(57)

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

7 0/ A+81 52 * 5 *E+ 1 7 1= =28 ( 5+2; / 7+0 ; 7+<* A21+(+2( O#P'

12 +A 727+/ =27+<* =8 5/1+ 7 0 G (3 A2 7 2(/8 <* ( 12 +. A 727+/ *8/(/;+<* / 7 =(* A8 <* 7 2*.5 (- 5 , 2 O, P' 3277 + 7 =27+<* 9/(* 5 A2[/ 928+2:( M =28A/+ 78 ( 7 1 7 :+ 7/( 8 9/+8 72:+(+. (2+ 2 7 3 E7 8+/8+ 8 ( +; *8/(/;+<* O # P' , , , ,.... 2 +A2 +A2 +A2 +A 727+/727+/727+/727+/ *8/(/;+<**8/(/;+<**8/(/;+<**8/(/;+<* 12 +A 727+/ *8/(/;+<* 2==282+ 7 52 (2 5 *E+ 1 - (2 78/+. + 1 5 0 + M 0/ 0 8 2 7 ( 25/( 0 7 7 ( 25*(7 ) * ' 2 =(*=287 5 =8 727+/ *8/(/;+<* 5 :*7 7 5 A2[/ + +5+ * M O P 12+ 0 872+ 02 5 =8 727+/ 2+;* *89+ 7 2=8 * 782*127+ 1 M * + 7 89 7+/ 0 +8*8;+02( /* * 200/*0 1 7 O,,P O,IP'

(58)

2 (2 12(25+ 5 ! +( / M ( 12 +A 727+/ *8/(/;+<* 3/: + 7 =2 - * 0 8/ /(/;+ 5/ 7 ( 5 ;8 5 8 0*= 827+/ 5 = 5 5 (3 7 5* 5 5/112; 78*07*82*E 5* > 7 1 89 *E 292 7 5 0/11 0 8 ( 782+7 1 7 O IFP' 872+ / 7 2> 5 A2+8 5 0(2 +A+027+/ 5 /* .;8/*= 0(+ +<* A/ 07+/ 5 (2 8 (27+/ 29 0 (3 OF P' . 8 1+ 8 /* .;8/*= 4 028207 8+ =28 * :825>H+ + M * 8+;+5+7 M 5 78/*. :( 0/; +7+A 7 = >0 +278+<* 29 0 5+(2727+/ 5* F 1 78+0*( ' . *E+ 1 /* .;8/*= 4 82((+ 5 =27+ 7 29 0 * 272E+ M * 78 1/8M * 02=2. 0+7 A/ 07+/ (( 8 5*+7 7 5 ( +/ A/02( 7 2(21+<* ' . 8 + 8 /* .;8/*= 4 82 1:( 5 =27+ 7 29 0 5 5> H+ + 9 8 M * 5> 287 8+ M 5 78/*:( 5 (2 = 8 / 2(+7 M 7 5 ( +/ A/02( 52 ( =*721 7 ( =2((+5*1 O JP' /8 5B* 0/((/<* 27+/ 2( A82 [2+ *8 (2 12(25+ 5 ! +( / /8;2 + =28 ( M ( 72:( 2* *8/(/;+<* 0(2 +<* 5 (2 12(25+ 2 7 =8 7 5+AA 8 11 7 (+ 29 0 (2 = > +/=27 /(/;+ 4 . > 58/1 E782=>821+52( =28 277 + 7 5 9/+ +;8/. 78+2( 4 29 0 * 5> 28. 7 8+ M * > 58/1 2H+ 7/.8+;+5 " >=/H+ + M 8+;+5+7 2E+2( M 5+AA+0*(7 53+ +7+27+/ 5 1/*9 1 7 $ 7 * 78 1:( 1 7 5 8 =/ ' . > 58/1 5> 7/ +<* =28 277 + 7 5 ;2 ;(+/ 5 :2 4 29 0 5 = 87*8:27+/ 5 (2 =/ 7*8 7 5 1/*9 1 7 0 /8 /.27 7/ +<* ' . > 58/1 272E+<* =28 277 + 7 5 9/+ 0 8 : ((/.7 2(21+<* 4 29 0 * 78 1. :( 1 7 =/ 7*82( 7 + 7 7+/ ( "78 1:( 1 7 :277 1 7 532+( 7 * 272E+ 5 1 1:8 $' > 58/1 / 7 828 1 7 + /( ' > 7/ + M 2H+ + 7 272E+ / 7 ( =(* /*. 9 7 =8 7 - 5 + 7 +7 928+2:( 0 G * 1U1 =27+ 7 O P'

(59)

, , ,

,.... ...,.,,, > 58/1> 58/1> 58/1> 58/1 E782=>821+52(E782=>821+52( 4444E782=>821+52(E782=>821+52(

2 >1=7/127/(/;+ E782=>821+52( 7 82==/87 52 #Y 5 02 (/ ( 8+ O P O# P OJ#P O, P O P'

2 (2 A/81 =28H+ / + O P - 5 :*7 =8 0/0 <*+ =8 5/1+ 0 G (325*(7 M (2 8+;+5+7 7 5+AA* 5 7>= E782=>821+52(M - =8 5/1+ 2 0 2E+2( ' * 5 :*7M 0 77 8+;+5+7 12 +A 7 <*3- (2 1280 M (2 727+/ 5 :/*7 7 + 72:( M (2 5 1280 7 82+5+ 29 0 7 52 0 - (3 >= 8.(/85/ ' 28 (2 *+7 M 0 77 8+;+5+7 7/*0 ( 1/*9 1 7 A+ M 8 52 7 5+AA+0+( ( ; 7 E 0*7 ' 77 >= 87/ + 2AA 07 2* + ( 1* 0( 5* 9+ 2; M 5* = 28> E 7 5 (2 (2 ;* ' ( 5 0/*( 4 . 5> = 2;+ 4 = /1 A8 <* 7 7 /*9 7 =8 0/0 ' (( 7 8 =/ . 2:( 53* >= 8 +2(/8 M ( 8+ <* 5 A2* 8/*7 7 +1=/872 7' . 5> 287 8+ 4 78 A8 <* 7 M (( 7 53+ 7 +7 928+2:( M ( 5 :+7 9 8:2( 7 82( 7+ 02 5 M 8 52 7 (2 9/+E 2 / /* (2 =28/( + + 7 ((+;+:( M 29 0 7/ 2(+7 5 8+8 7 5* =( *8 5> 7/ +<* ' . A20+ ! +( / + 29 0 * 9+ 2; A+; M ( 782+7 * = * /*(+; =28 (2 5> 7/ + 7 (2 8+;+5+7 ' 2 :/*0 7 78/*9 87 29 0 * 2 = 07 5* /*8+8 = 812. 7M =28A/+ 285/ +<* (/8 5 1/*9 1 7 9/(/ 72+8 O, IP O I P' * (2 A8 <* 0 5 (2 >1=7/127/(/;+ E782=>821+52( M +( A2*7 8 0 80 8 > 7 127+<* 1 7 (2 12(25+ 5 ! +( / 5 92 7 7/*7 78 1:( 1 7 53277+7*5 /* 7/*7 > 58/1 =28H+ / + 292 7 2 O P' , , , ,.... .... > H+> H+> H+> H+ ++++

A/81 5> H+ 7+<* =8 5/1+ 7 0 G (3 A2 7MO IPO F P 5+ 7+ ;* 78/+ A/81 =8+ 0+=2( 4O,JJPO, IP

(60)

. (2 A/81 272E+<* 29 0 78 1/8 + 7 7+/ ( 7 =/ 7*82( 7 272E+ 5 1 1:8 ' . (2 A/81 2H+ 7+<* 29 0 8+;+5+7 M :825>H+ + 7 78 1/8 5 8 =/ O,I P' A/81 / 7 828 1 7 =*8 7 (2 12)/8+7 / 7 1+E7 /* <* 7+ (( 52 (3 9/(*7+/ ' , , , ,.... ...F.FFF > 287 8+> 287 8+> 287 8+> 287 8+ 3 7 ( 1/5 5 =8 727+/ ( =(* A8 <* 7' (( 7 =8 7 0 G JY 5 02 OF P O #IP O JP O P' 2 5> 287 8+ 7 ( =(* /*9 7 1+E7 M 5> 7/ +<* M 272E+<* M >=/H+ 7+<* 7 = *5/:*(:2+8 ' . 2 5> 287 8+ >=/H+ 7+<* 200/1=2; (2 A/81 =28H+ / + M 29 0 5+AA+0*(7 -(3+ +7+27+/ 7 * >=/= / + ' . 2 5> 287 8+ 5> 7/ +<* 32 /0+ - * 5> 7/ + A20+2( M (+ ;*2( 29 0 * 0 *0 /. 7 1 7 7 * 7+1:8 782 ;( O,I P'

. 2 5> 287 8+ 272E+<* M 29 0 =2827+/ 5 >((2: 9/+8 E=(/ +9 "H(2E/= / + $ 9/+7 52 (2 A/81 = *5/ 0( 8 * ' . 2 5> 287 8+ = *5/:*(:2+8 29 0 * 9/+E A/80 7 1/ /7/ OF P' 2 5> 287 8+ = *7 B2 /0+ 8 - * /*8+8 5> 7/ +<* M * >= 8 2(+927+/ 7 * 78/*:( 5 (2 5 ;(*7+7+/ ' , , ,

,.... .... 72E+72E+72E+72E+ 7777 78 1/8 =/ 7*82( 7 + 778 1/8 =/ 7*82( 7 + 778 1/8 =/ 7*82( 7 + 778 1/8 =/ 7*82( 7 + 7 7+/7+/7+/7+/ ((((

77 A/81 7 =8 5/1+ 2 7 0 G (325*(7 ' 2 5 1280 A2+7 29 0 * 2*;1 727+/ 5* =/(>;/ 5 * 7 727+/ M 5 =2 +88 ;*(+ 8 M 29 0 5 288U7 M ( 78/ 0 / 0+(( 9 8 (3292 7 "272E+ 727+<* $' 78 1:( 1 7 7 5 ;82 5 21=(+7*5 M =/ 7*82( 7 + 7 7+/ (M =8/E+12( 7 5+ 72(' 78 1:( 1 7 + 7 8A 8 29 0 (2 ; . 7* (( M * 5> 1 78+ M * 2 > 0 8/ /1 78+ 7 * 5> 5+25/0/0+ + ' 78 1:( .

(61)

1 7 =/ 7*82( 7 :+ 9+ +:( 2* 12+ 7+ 5 =/ 7*8 M ; 82( 1 7 2*E =/+; 7 7 2*E =2*( "272E+ 0+ 7+<* $' 3277 + 7 5 9/+ 5 727/.8*:8/.7 2(21+<* "= 5/ 0*( 0 8 : (( *E *= . 8+ *8$ 7 8 =/ 2:( ' 32*78 12 +A 727+/ *8/(/;+<* 1/+ A8 <* 7 / 7 7 5 08+7 6 5 +; =>821+52*E 7 = *5/:*(:2+8 6 +; 5 2:+ H+M 5 255/HM 5 /AA12 M * >= 88 A( E+ 7 5 0(/ * OJ P O F P' E0 =7+/ (( 1 7M 5 08+ =+( =7+<* ; 82(+ /* =287+ (( M 1>/. 0(/ +<* /* 727 5 12( = *9 7 8 9 ( 8 (2 12(25+ M (( 8 =/ 5 7 /*9 7 12( 2* 782+7 1 7 2 7+0/1+7+2( O,I P' 78/*:( 0/; +7+A / 7 782 +7/+8 7 2778+:*2:( 2*E 0 2 ; 1 7 1 72. :/(+<* 6 ( =27+ 7 =8 7 7 5 78/*:( 5 1 1/+8 M 5 (2 8 0/ 2+ 2 0 M 5 (B277 7+/ 7 (B2(7 827+/ 5 = 8A/812 0 + 7 (( 07* (( 8 2(+ 2 7 828 1 7 * 72. :( 2* 5 5 1 0 782+72:( O,,,P O,# P O,I P O JP O P'

, , , ,...F.FFF 12 +A12 +A12 +A12 +A 727+/727+/727+/727+/ = >0 +278+<*= >0 +278+<*= >0 +278+<*= >0 +278+<* 78/*:( = >0 +278+<* A/ 7 =287+ + 8 7 5 (2 12(25+ ' 77 /7+/ 2 7 *87/*7 5 7 81+ =28 + ; 7 88+/ O,P O IP O #P' ( / 7 + 2*;*82*E "52 ,#Y 5 02 $ /* 200/1=2; 7 ( 12 +A 727+/ *8/(/;+<* ' ( / 7 2(/8 532*72 7 =(* 9 8 <* (2 >1=7/127/(/;+ *8/(/;+<* 7 292 0 O,, P' 3 9 72+( 5 12 +A 727+/ = >0 +278+<* 7 0/ +5 82:( M =28 /858 5 A8 <* 0 / 8 78/*9 4 g 8/*:( 7 >1+<* 6 ( 32;+7 A8 <* 11 7 53* 5 =8 +/ 5 ;829+7 928+2:( M 29 0 =28A/+ 5 200 1 (2 0/(+<* 9/+8 78/*:( :+=/(2+8 O,P' 7 727+9 5 *+0+5 / 7 =2 828 O P' 0/ 727 2* + * >= 8 1/7+9+7 7 * (2:+(+7 5 (3 *1 *8'

(62)

g 8/*:( 5 0/1=/87 1 76 1:( 5 /5+A+027+/ 5 0/1=/87 1 7M +88+72:+(+. 7 M 2;8 +9+7 M 0/( 8 +11/7+9 O, P <*+ *89+ 7 - (325/( 0 7 7 = *9 7 =2. 82N78 :2 2( 7 8 7285 8 ( 5+2; / 7+0 O, P' (3 E78U1 / = *7 0/ 727 8 5 78/*:( 5 0/1=/87 1 7 29 0 9+/( 0 7 207 2 7+. /0+2*E O P' g 8/*:( = >0 /7+<* 29 0 0/1=/87 1 7 :+G288 M 8 782+7 /0+2( 7 = >0 / 0 +G/. = 8 +A/81 ' 5 (+8 M 2((*0+ 27+/ 7 = 8 / 2(+7 0 +G/7>=+<* / 7 :+ 5 . 08+7 O,P O FP O F P' 872+ 78/*:( = >0 +278+<* / 7 8 9 8 +:( 2=8 ( 782+7 1 7 2(/8 <* 532*78 ( / 7 =2 ' , , ,

,.... 12 +A12 +A12 +A12 +A 727+/727+/727+/727+/ E782E782E782E782 *8/(/;*8/(/;*8/(/;*8/(/;++++<*<*<*<* , , , ,... .. ..,.,,, 77 + 777 + 777 + 777 + 7 =27/:+(+2+8=27/:+(+2+8=27/:+(+2+8=27/:+(+2+8 7 ;2 78/7 ;27 ;27 ;2 78/78/78/...+ 7.+ 7+ 7+ 7 7+ 2(7+ 2(7+ 2(7+ 2( 2 12(25+ =27+<* 7 ( 1/5 5 =8 727+/ ( =(* A8 <* 7 0 G (3 A2 7 - * Q; 1/> 5 - ,F 2 O,# P' 1/5 5 8 9 (27+/ 5 (3277 + 7 =27+<* 7 928+2:( A/ 07+/ 5* 725 9/(*7+AM ( = *7 32;+8 53* ( 927+/ 5 G>1 =27+<* 0 G 5 =27+ 7 2 >1=7/127+<* M 53* =27+7 0 8/ +. <* 207+9 29 0 * ( 927+/ 1/5 8 12+ = 8 + 72 7 5 782 21+ 2 O, P' +; =27+<* = *9 7 7825*+8 =28 * + *AA+ 2 0 =27+<* 2+;* M * =27+7 2+;* M * 0+88 / 5 0/1= O J P' B+ *AA+ 2 0 =27+<* 2+;* B+ *AA+ 2 0 =27+<* 2+;* B+ *AA+ 2 0 =27+<* 2+;* B+ *AA+ 2 0 =27+<* 2+;* 3/: 89 7+ (( 1 7 0 G ( A2 7 M ( 25/( 0 7 /* ( 25*(7 ) * ' (( 028207 8+ =28 (3+ 72((27+/ :8*72( 53* +07 8 + 7 M 53* 2 0+7 M 53* A+ 98 7 =28A/+ 53* 0 = 2(/=27 + ' +/(/;+. <* 1 7M (3 ( 927+/ 5 782 21+ 2 8+<* 7 2:+7* (( 1 7 + A 8+ *8 - , A/+ ( 92( *8 /812( M =8 5/1+ 2 7 *8 ( "0/ 782+8 1 7 - 0 <*+ 7 /:. 89 52 ( 2*78 A/81 5B =27+7 $'

(63)

78/*:( 5 (2 082 02* =28 5+1+ *7+/ 5 A207 *8 5 0/2;*(27+/ " A. A/ 58 Z# Y$M = *9 7 + 7 8A 8 8 29 0 (2 :+/= + =27+<* O JP' 3 =27+7 2+;* 3 =27+7 2+;* 3 =27+7 2+;* 3 =27+7 2+;* = *7 1+1 8 * =27+7 9+82( M (( 12 +A 7 =28 * 2 . 7 + M * +07 8 =8/;8 +9 1 7 08/+ 2 7M * A+ 98 M 5 5/*( *8 2:5/1+ 2( M 5 287 82(;+ 7 =28A/+ 5B* =27/ =( /1 ;2(+ O P' :+(2 :+/(/;+<* 5 . 0/*98 * >= 8:+(+8*:+ 1+ 1+E7 7 * 0>7/(> ' 2 0/ E+ 7 0 5B* =27+7 9+82( " 6 6 '''$ = *7 5 0( 0 8 * =27+7 A*(1+ 2 7 ' 2 0+88 / =27+<* 2 0+88 / =27+<* 2 0+88 / =27+<* 2 0+88 / =27+<* 0/ 7+7* - * 725 78 =8 0/0 5 (2 12(25+ M (( = *7 8 7 8 (/ ;7 1= 0/1= 0/11 (( = *7 5 0/1= 8 =28 (32==28+7+/ 53* 5 0/1=(+027+/ 0(2 +<* 6 * 2 0+7 M * 0+80*(27+/ 0/((27 82( 2:5/. 1+ 2( 7 * >= 87 +/ =/872( 0/1=(+<* 5 928+0 / /= 2;+ M 5 / . 5 1 5 1 1:8 + A 8+ *8 M 5 =( /1 ;2(+ 7 5B =27/1 ;2(+ ' 72:( 2* 5 0+88 / 5 0/1= 7 ( 1/5 5 8 9 (27+/ 5 (2 12(25+ 0 G ( *) 7 ) *. ' 3+ 0+5 0 5 0280+ /1 =27/0 ((*(2+8 7 =(* A2+:( 52 (2 12(25+ 5 ! +( / <* 52 ( 2*78 7+/(/;+ 5 0+88 / O,,JP O, FP' /* ( ) * =27+ 7 <*+ =8 7 7 * 277 + 7 =27+<* 0 8/ +<* + E=(+<* 29 0 /* 2 0+88 / 5 982+ 7 U78 5 =+ 7 =/*8 (2 12(25+ 5 ! +( / ' 2 ( 2 ( 2 ( 2 (+7 +2+7 +2+7 +2+7 +2 :+(+2+8:+(+2+8:+(+2+8:+(+2+8 4444 2 0+88 / =27+<* 7 (3 1/(> 1:( 7 U78 * 72. :( 2* 7>=+<* =/*8 (2 A/8127+/ 5 02(0*( 5 9/+ :+(+2+8 O # P' 02(0*( / 7 5 028207 8+ 7+<* =287+0*(+ 8 6 532==28 0 * +A/81 M 5 0/*( *8 9 87 A/ 0 7 # Y 5 ( *8 0/1=/ +7+/ 7/72( A2+7 5 0 /( 7 8/(' 2 (+7 +2 :+(+2+8 82+7 8 =/ 2:( 5 5/*( *8 2:5/1+ 2( =8 7 =28 ( A2 7 7 +( A2*7 A2+8 > 7 127+<* 1 7 (3 0 /;82= + - (2 8 0 80 5 (+7 +2 '

(64)

78/*:( ;2 78/.+ 7 7+ 2*E 27>=+<* = *9 7 U78 =8 7 6 5 2* M 5 9/1+ 1 7 M 5/*( *8 7 5+ 7 +/ 2:5/1+ 2( M * 2 /8 E+ 7 * 12(2+ O P' , , , ,.... .... 77 + 777 + 777 + 777 + 7 127/(/;+<*127/(/;+<*127/(/;+<*127/(/;+<* 4444 32 1+ 1/(>7+<* M (2 7 8/1:/= + M (2 ( *0/. *78/= + 7 ( 78/*:( 5 (2 082 / 7 ( 2 /12(+ <*+ 7/*0 7 ( ( 1 7 A+;*8 5* 2 ; 2* 0/*8 5 (2 12(25+ 5 ! +( / ' 32 1+ 1/(>7+<* 2==282N7 ( =(* /*9 7 0 G 5 *) 7 5 1/+ 5 # 2 - (3/002 +/ 53* 5 0/1= 27+/ =27+<* 2+;* 9 8 M (3 1/(> 7 (+ -(3+ +:+7+/ 5 G>1 8>7 8/0>72+8 7 - * ;82 5 <*2 7+7 5 0*+98 (+: 8 :8*72( 1 7 - (2 *+7 5 08/ 5 =27/0>7 M 0 <*+ + 5*+7 * 2 1+ 1/. (>7+<* 2+;*h - 7 7 5 //1: ;27+A 52 J Y 5 02 7 (32==28+7+/ 53* +07 8 -:+(*8+:+ / 0/ )*;* O JP' 2 ;27+9+7 5 7 7 5 //1: 5/ 7 (2 +:+(+7 7 9/+ + 5 # YM = 81 7 53 (+1+ 8 * 1/(> 2*7/.+11* 7 53+ 72*8 8 - 7/87 * 0/87+0/7 82=+ ' 32 2(> 5* A8/77+ 2 ;*+ <*+ = *7 1/ 78 8 (2 =8 0 5 0/8= 5 + G O #PM /* 0/8 5 =/ 07*27+/ :2 /= +( M = 81 7 292 7 7/*7 5 32 *8 8M 0/1=7 7 * 5 (2 7 8/1:/= + A8 <* 11 7 2 /0+ M 5 (32: 0 5 0 +G/0>7 /* 0/8 5 =282 +7 + 782. 8>7 8/0>72+8 ' 32 1+ 32;;829 02 53 1/882;+ 5+; 7+9 =28 8*=7*8 5 928+0 / /= 2;+ ' 3 AA 7 /E>527+A 5* 0*+98 0+80*(2 7 7 8 =/ 2:( 53* 7 8/1:/= + 7 5B* ( *0/ *78/= + 0/11 (( = *9 7 U78 /: 89 02 53 >= 8 =( + 1 (+ - (3 >= 87 +/ =/872( '

(65)

, , , ,.... ...F.FFF 3277 + 7 /0*(2+83277 + 7 /0*(2+83277 + 7 /0*(2+83277 + 7 /0*(2+8 (( 028207 8+ =28 (2 =8 0 53* 2 2* 0/8 5 0/*( *8 :8* 5/8 /* 9 87 M /11 (B2 2* 5 C2> 8 V ( + 0 8M (/02(+ - (2 = 8+= 8+ 5 (2 0/8 M 2 (28; *8 928+ 78 , 7 F 11 "A+;' , M,#$' ( /00*= ( =R( *= 8+ *8 =*+ ( =R( + A 8+ *8 7 3 7 5 - 7/*7 (2 0+80/ A 8 0 M +( 8 A( 7 (2 *80 28; 0*+98 5 (2 A20 =/ 7 8+ *8 5 (2 1 1:82 5 0 1 7 O ,,P' 7 2 2* 7 5 7 072:( -(3\ +( * 7 =28A/+ = *7 U78 /:) 07+9 <*3- (2 (21= - A 7 ' ; 82( :+(27 82(M +( = *7 U78 E0 =7+/ (( 1 7 * +(27 82(' ( 7 =8 <* 7/*)/*8 =8 7 52 ( A/81 *8/(/;+<* ' ( 7 * ( . 1 7 A/ 521 72( 2* 5+2; / 7+0 O, P 1U1 3+( 7 /: 89 2* 0/*8 53 =27 /=27 + 0 /( 727+<* =8/(/ ; / ! +( / + 6 7 (( <* (2 0+88 / :+(+2+8 =8+1+7+9 M (B =27+7 0 8/ +<* 207+9 M (2 0 /( 72 0 8/ +<* + 782 /* E782 =27+<* 7 (2 0+88 / 08>=7/; +<* ' 2 =8 0 5 (B2 2* 0/8 32 =2 5 0/88 (27+/ 29 0 (2 0*=8*8+ 12+ (( 7 0/88 ( 29 0 (2 9 8+7 5 (2 12(25+ ' =8 782+7 1 7M +( = *7 8 ;8 8 =8/;8 +9 1 7 + 9 8 5 2 A/8127+/ M 7/72( 1 7 /* =287+ (( 1 7' 2+ +( = *7 )212+ 5+ =282N78 M 7 / 9/(*7+/ 7 + 5 = 52 7 5 (B AA+020+7 5* 782+7 1 7 O,,JP' 7+78 E0 =7+/ (M * 02728207 7/*8 /( 7 5 08+7 ",J Y 5 02 $M ( B2;+7 5B* 5+ <* ;8+ /* :8* <*+ + ; 2* 0 78 5* 08+ 72((+ M 5B/^ =287 7 5 5 . =R7 825+2+8 8 1:(2 7 2*E = 72( 5B* 7/*8 /( "A+;',I$ + /* :+(27 82(M ;U 2 7 =2 (2 9+ +/ ( =(* /*9 7' 77 02728207 8 ;8 M 5 A2[/ =(* 8 ;*. (+ 8 2=8 ( 782+7 1 7 O,,JP'

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a

b

Figure 15 : localisation de l'anneau de kayser-fleischer (a : vue face, b : vue profil)[302]

+;*8 , 4 (3 E21 - (2 (21= - A 7

/ 78 * 2 2* 5 C2> 8.A( + 0 8M 0(2+8 1 7 9* 0/ 78 (B+8+ 9 87 0(2+8' OFP

(67)

, , , ,.... .... 77 + 7 877 + 7 877 + 7 877 + 7 8 2(2(2(2( 3277 + 7 8 2( 7 <*2 +1 7 0/ 72 7 12+ 8 7 ( =(* /*9 7 (27 7 ' 12 +A 727+/ 8 2( / 7 /*9 7 (+ - 5 ( +/ 7*:*(2+8 =8/E+12( 7 828 1 7 - * 277 + 7 ;(/1 8*(2+8 O P' 3277 + 7 7*:*(2+8 ; 58 * 5 A2*7 5 8 2: /8=7+/ 7*:*(2+8 =28 277 + 7 =8/E+12( 29 0 4 c >= 821+ /0+5*8+ ' c D(*0/ *8+ + 7 81+77 7 M A8*07/ *8+ ' c 8+0/ *8+ ' c >= 802(0+*8+ 7 = 8/02(0+ / ' c >= 8= / = 27*8+ ' 3 1:( 5 0 = 87*8:27+/ 1 72:/(+<* = *7 /002 +/ 8 5 +7 +2 8 2. ( >1=7/127+<* /* / ' %* (<* 02 5 > 58/1 5 2 0/ + *8 277 + 7 7*:*(2+8 =8/E+12( / 7 7 5 08+7 ' 20+5/ 7*:*(2+8 =28 277 + 7 5+ 72( 7 8 78/*9 828 1 7 5 1U1 <*3* + *AA+ 2 0 8 2( ' 3277 + 7 ;(/1 8*(2+8 0/ + 7 - * 5+1+ *7+/ 5 (2 A+(7827+/ ;(/1 8*(2+8 7 5* A(*E 2 ;*+ O, P' (( 12 +A 7 =28 * =8/7 + *8+ 6 *87/*7 2(:*1+ *. 8+ M * 127*8+ + 7 81+77 7 M 8 0+5+92 7 <*+ = *7 U78 * >1=7R1 + 2*;*82( 5 (2 12(25+ ' > 58/1 =27/8 2( 29 0 A/ 07+/ 7*:*(2+8 /812( 0/1=(+<* ; 82. ( 1 7 (3+ *AA+ 2 0 =27+<* 7 +( 0/ 9+ 7 53> 9+7 8 ( 5+*8 7+<* ' ;(/1 8*(/ = 8+7 29 0 > 58/1 = 8/7+<* - 0/1=( E +11* /* -; 7 2* + 5 08+7 O,,JP'

(68)

, , , ,.... ...#.### 77 + 777 + 777 + 777 + 7 / 7 // 7 / ./ 7 // 7 /... 287+0*(2+8287+0*(2+8287+0*(2+8287+0*(2+8 (( / 7 =2 E0 =7+/ (( M / 7 *87/*7 A8 <* 7 52 ( =/=*(2. 7+/ 2 +27+<* 29 0 * =8 5+( 07+/ 52 0 872+ A21+(( ' 3277 + 7 / 7 /.287+0*(2+8 7 =(*8+A207/8+ (( 4 c 28 AA 7 7/E+<* 5+8 07 5* 0*+98 *8 ( 0287+(2; M *8 (2 7821 / * 7 =28 5 . =R7 5 0*+98 + 782 > /9+2( O ,P' c 28 (320+5/ 7*:*(2+8 8 2( M A*+7 8 2( 5 = / = /8 7 5 02(0+*1M 0 77 27. 7 + 7 8 2( 7 8 =/ 2:( 53* 5 1+ 82(+ 27+/ / * ' c 28 (3+ *AA+ 2 0 =27+<* <*+ 2:/*7+7 - * / 7 /287 8/=27 + >= 878/= +<* 5/*(/*8 * 5 =/+; 7 7 5 0 9+(( O,,JP' * +9 2* / *EM / 0/ 727 5 / 7 /=27 + (+ - (2 5 1+ 82(+ 27+/ M A2+7 53/ 7 /=/8/ =8 0/0 <*+ 82+7 /: 892:( 0 G Y 5 12(25 M * / . 7 /12(20+ M * / 7 /5> 78/= + M 5 A8207*8 =/ 72 M <* (<* 02 53/ 7 /0 / 58+7 5+ <*2 7 / 7 7 82==/87 O ,P' * +9 2* 287+0*(2+8 M 7/*7 ( 287+0*(27+/ = *9 7 U78 277 + 7 M / 0/ . 727 5 82+5 *8 7 5 8 78+07+/ 287+0*(2+8 *87/*7 5 2 0 7 5 ; /*E 5 1U1 <*3* >= 8(2E+7 287+0*(2+8 ' 02 53287 8+7 8 0*88 7 / 7 7 5 . 08+7 =(*7R7 0 G (3 A2 7' +AA 8 7 2 /12(+ 825+/(/;+<* 8 78/*9 O,,JP 4 c / 5 27+/ A(/* 7 +88 ;*(+ 8 /* 0 / 582( ' c 2(0+A+027+/ =282.287+0*(2+8 2*E =/+ 7 53+ 87+/ 02= *(/.(+;21 72+8 ' c +/ - 7>= 53 0/0 7 5 8/ +/ =+= > 2+8 ' c +/ 5+ 0/9 87 :82( 7 / 7 /0 / 58/ 9 87 :82( E=(+<* 7 ( 5/*( *8 (/1:2+8 ' 0 / 58/02(0+ / 5 ; /*E 7 0 / 58/12(20+ 8/7*(+ / 7 1 7+/ '

(69)

, , , ,.... ...I.III 77 + 777 + 777 + 777 + 7 5/08+ +5/08+ +5/08+ +5/08+ + = 87*8:27+/ 5/08+ + / 7 (+ - (2 7/E+0+7 5* 0*+98 7 *82)/*. 7 5 78/*:( + 5*+7 =28 (3+ *AA+ 2 0 =27+<* O, P' > 1 /88 > 1 /88 > 1 /88 > 1 /88 4 1 /88 =8+12+8 /* 0/ 52+8 7 82==/87 0 G # Y 5 A 11 M =28 5> A/ 07+/ /928+ ' 72*E 53/ 78/; 7 A2+:( 82+ / 5 A2+:( 0/ 0 7827+/ 5 M 7 /* 5 (3+ *AA+ 2 0 5 =8/5*07+/ 53\ 78/; O I P' 3\ 78/; 7 0 2+8 =/*8 12+ 7 +8 (2 1+7/ 5 0 ((*( 5 ;82 *(/ 2 7 (2 7 0 2+8 =/*8 (328/1272 A/((+0*(2+8 M 8 92 0 (3+ 7/E+027+/ 0*=8+<* + 7 8A 8 29 0 (3207+9+7 5 (328/1272 A/((+0*(2+8 0 0+ E=(+<* 82+7 ( 78/*:( 5 (3/9*(27+/ 7 =28A/+ 5 29/87 1 7 =/ 72 O, P' 7 03 7 =280 <* ( 782+7 1 7 7 02=2:( 53+ 9 8 8 0 78/*:( ( 5+2; / 7+0 =8 . 0/0 7 +1=/872 7' >= 8=2827 >8/_5+ 7 0/ 52+8 2* 5 =R7 0*=8+<* O, P' 7/( 82 0 2* ;(*0/ = *7 2==282N78 8 (27+/ 29 0 =2 08 27/=27 + ' 8 7285 =*: 872+8 7 5 08+7 0 G (325/( 0 7' , , , ,... .. ..J.JJJ 77 + 777 + 777 + 777 + 7 0285+/92 0*(2+80285+/92 0*(2+80285+/92 0*(2+80285+/92 0*(2+8 2 =8 0 5 0*+98 - 5 5/ 2; ( 9 2* +9 2* 5* 0\ *8M *87/*7 52 ( A2+ 0 2* 5 + M 782N 5 78/*:( 5 (2 0/ 5*07+/ M * 0285+/1>/=27 + 0/ . ; 7+9 29 0 1>/0285+7 7 A+:8/ + 7 8 7+7+ (( O,##PM 5+AA 8 7 7>= 5328>7 1+

/7211 7 (2 A+:8+((27+/ 9 78+0*(2+8 <*+ 8 7 (2 02* 7+ (( 5 1/87 0285+2. <* *:+7 O,,JP'

8 78/*9 5 2 /12(+ ( 078/0285+/;82= +<* *+92 7 O,##P 4 . >= 878/= + 9 78+0*(2+8 58/+7 /* ;2*0 /* ;(/:2( '

(70)

. =/(28+ 27+/ =8 0/0 ' . /* 5 02(2; ' . 9 8 +/ 5 / 5 ' . E782 > 7/( =8 127*8 2*8+0*(2+8 /* 9 78+0*(2+8 ' . +:8+((27+/ 278+2( ' . (/0 + / 278+2(' . (/0 2*8+0*(/.9 78+0*(2+8 7>= /:+7G ' 3277 + 7 5 > 7 1 89 *E 2*7/ /1 O PM =28 200*1*(27+/ 5 0*+98 2* +9 2* 5* 78/ 0 0 8 :82(M E=/ ( 12(25 - =8 7 8 * >=/7 +/ /87 / 72. 7+<* 5 1U1 <*3* 8 =/ 2 /812( - (2 12 \ *98 5 92( 2(92 O P' 3277 + 7 0285+2<* *89+ 7 0 G ( 12(25 ! +( / + 2 =8 5/1+ 2 0 5 E 7 0 G 5 *) 7 5 5+AA 8 7 Q; OF#P' , , , ,... .. ... 77 + 777 + 777 + 777 + 7 5 (2 = 2* 7 55 (2 = 2* 7 55 (2 = 2* 7 55 (2 = 2* 7 5 = 2= 2= 2= 2 8888 78/*:( 5 (2 =+;1 727+/ 0*72 = *9 7 U78 128<* 12+ / 7 728. 5+A M / /7 (2 =8 0 5B* >= 8=+;1 727+/ :8* Q78 <*+ 8 = 07 ( 1*. <* * 7 =8 5/1+ 2* 5/ 5 =+ 5 7 (2 A20 2 7 8+ *8 5 )21: M (+ - * 5 =R7 5 1 (2 + ( (/ ; 5 (2 )/ 07+/ 5 81/ =+5 81+<* ' (* *( 2G*8 *8 ( / ;( / 7 5 08+7 0/11 <*+92( 7 5 (B2 2* 0/8 12+ / 7 828+ +1 7 / = 0+A+<* O,IP' , , , ,.... .... 77 + 7 +11* /(/;+<*77 + 7 +11* /(/;+<*77 + 7 +11* /(/;+<*77 + 7 +11* /(/;+<* B2 /0+27+/ - * =27+7 2*7/.+11* 7 0/ * ' 2 /0+27+/ - * 5 8. 127/1>/ +7 M * 1>2 7 + M * (*=* M * 0( 8/5 81+ /* * > 58/1 5 D//5 =2 7*8 M * 028 0 +11* /;(/:*(+ " ;$ 7 =(*7R7 82==/87 29 0 (2 = +. 0+((21+ O,,JP'

(71)

.

.

.

. /81

/81

/81

/81

2 >1=7/127+<*

2 >1=7/127+<*

2 >1=7/127+<*

2 >1=7/127+<*

( B2;+7 5B* 1:( 5 02 5 =+ 7 725 =8 0(+ +<* M 2>2 7 /* / * 2 7 0 5 7 A21+(+2( 5 (2 12(25+ 5 ! +( / ' 5 =+ 72; A2+7 +5 2( 1 7 9 8 (BQ; 5 F - 2 52 (2 A8278+ 5B* A2 7 0 G ( <* ( 9+ 7 5BU78 8 0/ * 0 77 12(25+ ' B E21 :+/(/;+<* 78/*9 <* (2 :2(2 0 0*=8+<* 7 =/ +7+9 ' (B2: 0 5B2 7 0 5 7 A21+(+2(M 0B 7 2:+7* (( 1 7 - (2 *+7 5 (2 5 0/*. 9 87 - (B E21 + 7/(/;+<* 5* A/+ M 5 ( +/ 5B2 = 07 =287+0*(+ 8 <* ( 5+2; / 7+0 5 (2 12(25+ 5 ! +( / 5/+7 U78 * = 07 ' 2 0 02 (2 :+/(/;+ 1/( 0*(2+8 2 * 2==/87 5+2; / 7+<* +1=/872 7 O, P' ( 7 AA 7 A/ 521 72( 5 8 0/ 2N78 (2 12(25+ 5 ! +( / - 0 725 /^ ( 782+. 7 1 7 7 0/ 7211 7 AA+020 O #P'

Figure

Figure 2 :  IRM cérébrale en coupe axiale  FLAIR ne montre pas d'anomalie
Figure 3: TDM cérébrale en coupe axiale montrant (3 &gt;=/5 +7 5 5 *E =*721 29 0 278/= + 0/87+0/
Figure  4 :    coupe  axiale  d'IRM  cérébrale  en  séquence    montrant 5 &gt;= 8 +; 2*E + 7 8 2 7 ( =*721 7 ( (/0* +; 8
Figure  8 :    coupes  axiales  d'IRM  cérébrales  en  séquence  T1  ne  montrant  pas  d'anomalies
+3

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