marco düring - cadasil : lessons from neuroimaging

7/29/2019 Marco Dring - CADASIL : lessons from neuroimaging

1/40

Hubnen gy=

Yflfrz-]ad Wesearic Hfubnatjfb

Lessfbs hrfk beurfjka`jb`, part JJ

Karif Nrjb`

Jbstjtute hfr Ytrfde abn Nekebtja Wesearic

Kubjic, @erkaby


2/40

If`bjtjve prfhjle jb YXN prfiessjb` speen nehjijts


3/40

Nehjijts jb prfiessjb` speen


4/40

Glaid hfr jka jb`/JH/eti.Lesjfb _revalebie Kaps, b;715

LL

PKC5 15?

1 1>Lesjfb hrequebiy

Lesjfb hrequebiy

L


5/40

PKC patterb jb IANAYJL abn spfranji YXN

1% 15%

9% 3?%IANAYJL (b;9>9)

Ypfranji YXN (AY_Y4 b;53>)


6/40

If`bjtjve jkpajrkebt | kjxen patcflf`jes

AN XIJ

IANAYJL

pure XIJ

Icagrjat*, Mfutel*, Njic`abs* et al., Labiet Beurfl 7??:


7/40

Xfxel-gasen lesjfb sykptfk kappjb` (XLYK)

bfb-paraketrji test

^K^-G

]-s

ifre

1

PKC

?


8/40

Xfxel-gasen Lesjfb Yykptfk Kappjb`

sj`bjhjiabt vfxels= Laiubes

PKC

MCU-atlas (JIGK-N^J 31)

L

Nuerjb` et al, GWAJB 7?11


9/40

Wesults= XLYK jb spfranji YXN (b;531)


10/40

Abalysjs fb re`jfbal lesjfb vflukes


11/40

Jbterifrrelatjfb akfb` varjagles


12/40

Gayesjab betwfrd abalysjs

Nuerjb` et al., BeurfJka`e 7?17


13/40

Ifbilusjfb J

Ijriujt hj`ure= Lfkgarnj et al., 7???

Abterjfr tcalakji ranjatjfb

Nuerjb` et al., Grajb 7?11

Nuerjb` et al., Beurfjka`e 7?17


14/40

Grajb vfluke= strfb` prenjitfr hfr nehjijts

Nuerjb` et al., GWAJB 7?11


15/40

Atrfpcy ahheits all tjssue ifkpartkebts

Atrfpcy neijle `rfups


16/40

Ifrtjial kjirfjbharits at 0^

vab Xeluw, MIGHK 7?19Jka`es ifurtesy fh @M Gjessels


17/40

Jbijnebt laiubes abn atrfpcy



18/40

Jbijnebt lesjfbGaseljbe

-13 kfbtcs

(Hfllfw-up)

(+13 kfbtcs)

Experjkebtal futljbe


19/40

Yystekatji jnebtjhjiatjfb fh jbijnebt lesjfbs

Jbijnebt lesjfbGaseljbe Njhherebie Jka`e

#1

#7


20/40

Ketcfns

Nuerjb` et al., Beurflf`y 7?17


21/40

Ketcfns



22/40

Hreesurher | ifrtjial tcjidbess keasurekebts


23/40

Wesults


*p


24/40

We`jfbal ifrtjial tcjidbess & prfiessjb` speen


25/40

Gayesjab betwfrd abalysjs

Wj`cart et al., Beurfjka`e= Iljbjial 7?19


26/40

Ifbilusjfb JJ

Ijriujt hjùre= Lfkgarnj et al., 7???

Abterjfr tcalakji ranjatjfb

Nuerjb` et al., Grajb 7?11Nuerjb` et al., Beurfjkaè 7?17

Kenjal hrfbtal ifrtex


Wj`cart et al., BeurfJkaè= Iljbjial 7?19


27/40

Parnlaw, Ykjtc, ..., Icagrjat, ..., Nuerjb`, ..., Njic`abs, Labiet Beurfl 7?19

Enjtfrjal jb Labiet Beurfl 7?17

Gaseljbe Hfllfw up


28/40

Parnlaw, Ykjtc, Njic`absLabiet Beurfl 7?19

Gaseljbe Hfllfw-up


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Jnebtjhjiatjfb fh jbijnebt laiubes



30/40

Wesults | ratjb` fh jbijnebt laiubes



31/40

Watjb` exakple



32/40

Watjb` fh jbijnebt laiubes



33/40

Ypfranji a`e-relaten YXN



34/40

Xasiular abatfky

Yalakfb, 1:01


35/40

Xasiular tfpflf`y



36/40

PKC abn vasiular tfpflf`y

Yalakfb, 1:01

YXN severjty

I


37/40

Ifbilusjfb JJJ


A d l n t


38/40

Aidbfwlen`kebts

JYN Beurfjka`jb` Ubjt

Egru Gaydara

Kartjb Njicàbs

Kjicael Ewers

Gebbf @esjerjic

Karjya @fbjd

Laba Dakgejtz-Jlabdfvji

Wajber Kaljd

Wutcèr Wj`cart

Lee Yjkfb-Xerkft

Hubnjb`=

ICU Larjgfjsjre, A_-C_, _arjs

Cuùes Icagrjat

Hrabifjs ne @ujf

Nfkjbjque Cerv

Erji Mfuvebt

Kenjial Ubjversjty fh @razEnjtc Cfher

Ludas _jrpaker

Ytehab Wfpele

Ytepcab Yajler

Wejbcfln Yickjnt

G j t c j ll l nj


39/40

Grajb atrfpcy jb skall vessel njsease

Parnlaw, Ykjtc, ..., Nrjb`,..., Njic`abs, Labiet Beurfl (jb press)

G j l t nj t h n hj jt


40/40

Grajb vfluke= strfb` prenjitfr hfr nehjijts

marco düring - cadasil : lessons from neuroimaging

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