7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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@kurjpetbjcjgy jo LEME\IC
e`m j tbkr saecc ertkry m iskesks
jo tbk hrei` (\EMH) Be``u Fec iaj ]urfu e`m Bkcsi`f i
Oi`ce`m
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• LEE (lkrkhrec eaycjim e`gijpetby)• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )
• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
•
_erie
• \pjremil saecc ertkry m iskesks j o hrei`
• LEE (lkrkhrec eaycjim e`gijpetby)
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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I` LEME\IC jvkr 8>> miookrk`t autetij`s bevk hkk` rkpjrtkm wjrcm-
wimk i` KGO rkpkets (5-<8 ) jo tbk kxtrelkccucer mjaei` jo
@jtlb< (@<KLM). Ajst jotk` tbk autetij` rksucts i` e` u`kvk`
`uahkr jo lystki`ks (kitbkr gei` jr cjss jo e` u`kvk` `uahkr jo lystki`ks).
@jtlb< u`mkrgjks < lckevegks (\5-\<). @ILM k`tkrs `ulckus
e`m rkgucetks tre`slriptij`
X H ^ D
X H ^ D
BMEl
BMEl
LjX
LjX
AEAC5
BEl
Bks/Bky
X H ^ D
X H ^ D
BMEl
BMEl
LjX
LjX
AEAC5
BEl
Bks/Bky
\ig`eci`g lkcc
Xklkivi`g lkcc (veslucer
sajjtb aus-
lck lkcc )
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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B&K
Ljccegk` I
@jtlb < kxtrelkccu cer
mjaei` (@<KLM, aeh 5K=)
B&K ^E\
e- sajjtb ausl ck
elti`
LEME\IC,lkrkhec wbitk
aettkr 0
Lj`trj c , lkrkhrec
Wbitk aettkr
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Lkrkhrec H cjjm Ocjw ^jsi trj` kaiss ij` tjajgrepby
^etik`t A/<= (strjfks) Lj`trjc A/<5
]ujai`k` kt ec, \trjfk 8>>=6<;45>?<-?7
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Ertkr ie c Lbe`gks i` LEME\IC i` Xkcet ij` tj ̂ etbjg k`ksis jo Mio ousk
Aykc i` Cjs s jo Lkrkhrec Wbitk Aettkr 4 Kxeai`et ij` jo Lkrkhrec Akmuc cery
Er tkriks hy Xklj` strult ij` jo \kr ie c \klt ij`s jo e` Eutj psy Lesk ..
Xkm zj`k4lj`ti`ujus cjss jo
_\ALs
Xkm mjttkm
zj`k4 mislj`ti`ujus
cjss jo _\ALsGrkk` zj`k4
i`tiaec oihrjus
tbilfk`i`g
Hcuk zj`k4
emvk`titiec oihrjsis
…
Jfkme kt ec. (\trjfk 8>>86<<48;?;-9)
– Ectbjugb i`tiaec
oihrjsis jr byeci`jsis
wes prksk`t, cuai`ec
jllcusij` wes slerlk.‑
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Bistjgrea jo i`tkr`ec mieaktkr jo ertkrijcks
hktwkk` LEME\IC e`m lj`trjc grjup i` W A
>
5>
8>
<>
; 8 > < ; ; > ? ; 2 > 9 ;
I`tkr`ec m ieaktkr jo ertkrijcks i` aa
^ k r l k ` t %
LEME\IC (̀ 1989)
lj`trjc (̀ 1852)B&K
^E\
Aiej kt ec. Hrei` ^etbjc 8>>=65=4<;2-<?=.
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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I` LEME\IC jl-
lcusij` jo e pk`k-
treti`g lkrkhrec
ertkry rksucts i` e
celu`er i`oerlt (3),
hkleusk pk`k-
treti`g ertkriks erktkrai`ec witbjut
ljccetkrec ocjw
orja tbk `kigb-
hjri`g ertkriks.
\tk`jsis Jllcusij`
e`m i`oerltkm t issu k
3 Celu`er
i`oerlt
Ljrtkx
Wbitkaettkr
Ljrtilec vs. wbitk aettkr ertk-
riks erk aktehjcileccy miookrk`t
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC4 Auct ip ck saecc (celu`er) i`oerlts i` WA e`m
hesec ge`g c ie, wbkrkes lkrkhrec ljrtkx is rkcet ivkcy s perkm
0 3
3
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC mjks `jt prjtklt orja lcessil risf oeltjrs4
E LEME\IC petik`t witb p.X5<<L autetij` bem ecsj skvkrk
akmiua-sizkm e`m saecc vksskc etbkrjslckrjsis i`lcumi`g
ljapcktk jllcusij` jo e` i`sucer hre`lb jo aimmck lkrkhrec
ertkry ( ) e`m e ljrtilec periktj-jllipitec i`oerlt ( ).
LEME\IC i`oerlts i` tbk wbitk aettkr ( 3 )
3
3
3
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC WA4 ertkrijcks
(@<KLM)
LEME\IC WA4 lepiccery
(@<KLM)
LEME\IC lkrkhrec ljrtkx4 saecc
ertkrijcks e`m e lepiccery ( ) (@<KLM)
I` LEME\IC hrei` @jtlb< kxtrelkccucer
mjaei` (@<KLM/GJA) elluaucetks
`jt j`cy j` WA ertkrijcks, hut ecsj j`WA lepicceriks (pkrilytks), es wkcc es
j` tbksk vksskcs i` lkrkhrec ljrtkx,
ectbjugb ljrtilec ertkrijcks erk `jt
kqueccy tbilfk`km.
\i`lk ertkrijpetby i` LEME\IC is gk-
`kreciskm, elluaucetij` jo @<KLM
le` ecsj hk sbjw` hy iaau`jstei`-
i`g e mieg`jstil sfi` hijpsy, hut...
`krvk
* 1 ertkriks
*
* *
*
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Kckltrj` ailrjgrepb jo e saecc mkraec ertkry4 wimk`km suhk`mjtbkciec
spelk ( ), irrkgucerity jo veslucer sajjtb auslck lkccs (_\ALs) e`m
gre`ucer jsaijpbicil aetkriec (GJA, errjws). K 1 k`mjtbkciua
Cuak`
_\AL
_\AL
_\AL
_\AL
_\AL
*
**
* K
K
K
*
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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\pklioil tj LEME\IC is kckltrj` ailrjsljpil oi`mi`g jo gre`ucer jsaijpbicil aetkriec (GJA * ) i` i`mk`tetij`s (`jtlbks) jo e`m ̀ kxt tj mkgk`kreti`g ves-lucer sajjtb auslck lkccs. GJA bes hkk` mkaj`stretkm i` ecc LEME\IC
lesks witb e @jtlb< autetij` rksucti`g i` e` u`kvk` `uahkr jo lystki`ks i`@jtlb< kxtrelkccucer mjaei`. (@ 1 `ulckus). ]iff e kt ec. Hrei` 8>>96 5<849<<-9<9
5 a a >.= a a
*
*
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Mkpjsitij` jo @jtlb< kxtre-
lkccucer mjaei` (@<KLM) le`
ecsj hk sbjw` witb iaau`j-
stei`i`g, hut KA mkaj`stre-
tij` jo GJA is ajrk rkciehck,ksp. et tbk hkgi``i`g jo tbk
miskesk witb j`cy saecc
eaju`ts jo @<KLM/GJA .
\fi` hijpsy4 @<KLM
iaau`jstei`i`g
`krvk*
*
3
59-yker-jcm aeck LEME\IC petik`t,
k ckltrj` a ilrjslj py jo e mkraec er tkry
3
@<KLM iaau`jstei`i`g
*
@krvk
* 1 ertkriks
]iff e kt ec. Hrei` 8>>96 5<849<<-9<9
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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]iff e kt ec. Hrei` 8>>96 5<849<<-9<9
LEME\IC e`m truk GJA Kcesti` oregak`ts
Gre`ucer mkhris jo u`f`jw` jrigi`
_eriehck lkcc mkhris
_eriehck lkcc mkhris
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Aizu`j kt ec.
I`tkr Akm =74
8>?7-78, 8>>2
\lbkim kt ec.
@kurjcjgy
75477=-?, 8>>2
Hress kt ec.
@KDA 8>>96
<?>45?;?-?;
GJA bes hkk` mkaj`stretkm i` ecc LEME\IC lesks witb e @jtlb< autetij` rksucti`g i` e` u`kvk` `uahkr jo lystki`ks i` @<KLM. Bjw ehjut `j`-lys lesks…:
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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]bk @J]LB < prjtki`
Miaitrijs _celbefis e`m \jpbie Fjssime
Hijakmilec Xkskerlb Oju`metij`, Elemkay jo Etbk`s
h-sbkkth-tur`
e-bkcix
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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CJ\\ JO θ-
sbkkt
\]X[L][XK
Erg
]rp
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Milbge`s kt ec. (\trjfk 8>>86<<4?7-75)4
┎ AHs oju`m i` ?9% jo 5? LEME\IC
petik`ts e`m i` `j lj`trjc suhdklts
(p1 >.>>5)
┎ Essjl. witb egk (p1 >.>>8)
┎ Cjletkm i` ljrtilec-suhljrtilec rkgij`s (<2%), wbitk aettkr (8>%),
tbeceaus (5<%), hrei`stka (5=%)
┎ 28% cjletkm jutsimk erkes
eppkeri`g bypkri`tk`sk j` ]8w
iaegks
Cks`if Jhkrstki` kt ec. (@kurjcjgy
8>>56 ;74 5>?? “5>7>)4
┎ AHs oju`m i` <5% jo <8 syaptj-
aetil LEME\IC petik`ts.
┎ Essjl. witb egk (p3 >.>>2)
┎ Essjl. witb e`tipcetkckt usk (p3
>.>8;)
┎ Cjletkm i` tbeceaus (?5%), suhljr-
tilec WA (8?%), lkrkhkccua (?%)
]8* AXI
AXI4 Cks`if Jhkrstki` kt ec.
Ailrjhckkms i` LEME\IC
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC tergkts spklio ilec cy wbitk
aettkr ertkr ijcks, wbkrkes g rky aettkr ertkrijcks erk hkttkr prk-
skrvkm e`m tbk tbi``kr weccs jo
grky aettkr ertkr ijcks erk ajrk
prj`k tj ckef (0 a ilrjhckkms ).
WA4 @<KLM
Ljrt kx4 @<KLM
Ljrtkx4 a ilrjh ckkm
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC WA4 o ihr i`jg k`
Ailrjh ckkm i` LEME\IC ljrtkx4
o ihr i`jgk`
Hrkefmjw ` jo HHH i` bypkr -
tk`sij` 4 o ihr i`jgk`
I` LEME\IC tbk HHH j o tbk
tbilf k`km ertkr ij cks eppkers
tj hk rkcet ivkcy i`telt4 kxtre-
veset ij` jo pcesae prj tki`s
is a i` iaec.
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Kpi ckpsy Lj`trj c
Oihri`jgk` kxtrevesetkm muri`g hrkefmjw` jo HHH mioousks tj
L\O e`m orja it is tefk` up hy lkrkhkccer ^urfi`dk lkccs (^L). I`
LEME\IC ^Ls rkaei` `kgetivk.
LEME\IC _F LEME\IC Af
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)• LEE (lkrkhrec eaycjim e`gijpetby)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
• _erie
• \pjremil saecc ertkry m iskesks j o hrei`
• LEE (lkrkhrec eaycjim e`gijpetby)
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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\ju re`mkr e`m Wëci`mkr j rigi` ec ert ilck ‑ Bkrkmitery auct i - i`oerlt mkak`-
tie ‑ ( El te @kurjp etbj c <948=7-8;=65977). E. \aecc i`oerlts i`4 5. r igbt or j`tec
wb itk aettkr , 8. r igbt lepsu ce i`tkr`e e`m <. ckot pec cimua + saecc celu`ks
i` tbk hesec ge`gc ie. e e`m h4 Lyst il i`oerlts i` pj`s (twj pet ik`ts ).
5.
8.
<
E
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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\wkmisb b krkmi tery au ct i -
i`oerlt mkak`t ie ( \wk-bAIM )
]bk oeaicy jo \jure`mkr
e`m Wëci`mkr (Bkrkmitery aucti-i`oerlt mkak`tie. Elte
@kurjpetbjc <948=7-8;=65977 )
wes cj`g lj`simkrkm tbkoirst puhc. LEME\IC oeaicy.
\wk-bAIM ]8w AXI
LEME\IC p.X5=5L
(Cjw kt ec. Hrei` 5<>4 <;7 “<?76 8>>7)
\wk-bAIM ]8w AXI
\wk-bAIM ]8w AXI
]8w AXI
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Oketurk \wk-bAIM LEME\IC (\tkvk`s
p.X5=5L)
@j. eookltkm 9 =
Ake` egk et j`skt ( µ \M) <=.? µ <.= =2 µ <
J`skt re`gk 89 - <2 <9-;7
Ake` egk et mketb ==.< µ =.< ?> µ 7
Muretij` (ykers) 55 µ 8.5 5=.? µ =.;
@j. jo strjfk kpisjmks 0= 0?
Cjw kt ec. Hrei` 8>>765<>, <;7 “<?7
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC, @<KLM \wk-bAIM, @<KLM
@
]bk ehsk`lk @jtlb <
KLM (L) e`m GJA (K)
i` \wk-bAIM e`m tbkir
prksk`lk i` LEME\IC
(M e`m O).
\wk-bAIM,
k ckltrj` ailrjsljpy K O
LEME\IC,
k cklt rj` ailrjsljpy @
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Miookrk`tiec mieg`jsis LEME\IC vs . \wk-bAIM
• Aigrei`k kpisjmks hkojrk j`skt jo strjfks ljaaj` i`
LEME\IC, `jt rkljrmkm i` \wk-bAIM
• AXI4 Cksij`s i` tkapjrec cjhks e`m lepsuce kxtkr`e
ljaaj` i` LEME\IC, u`usuec i` \wk-bAIM• \fi` hijpsy4 GJA rkgucercy oju`m i` LEME\IC, `jt i` \wk-
bAIM
I` = akahkrs jo tbk \wkmisb oeaicy `j petbjgk`il autetij`s
wkrk oju`m i` tbk k`tirk 2>95 hp rkemi`g oreak jo @J]LB<`jr lcker kvimk`lk ojr @J]LB< gk`k ci`fegk.
I` \tkvk`s oeaicy p.X5=5L autetij` lj`oirakm LEME\IC mg.
Cjw kt ec. Hrei` 8>>76 5<>, <;7 “<?7
Oigurks o rja Cjw kt ec Hrei` 8>>76 5<> <;7“<?7
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC, B&K
\wk-bAIM, e-\AE
\wk-bAIM, akmi`
LEME\IC, e-\AE
\wk-bAIM, B&K
LEME\IC, akmi`
\lckrjt il i`m ilks i`
\wk-bAIM vs .
LEME\IC4
Orj`tec wb itk aettkr 4
\wk-bAIM4 ake` >.<>
% witb ≫ >.;> =.=%
LEME\IC4 a ke` >.<9
% witb ≫ >.;> 7.=%
Hesec ge`gc ie4
\wk-bAIM4 ake` >.<<
% witb ≫ >.;> 2.<%
LEME\IC4 a ke` >.<?
% witb ≫ >.;> 59.9%
Lreggs kt ec. Hr ei`
^etbj c 8>5<68<4;=7-;;7
Oigurks o rja Cjw kt ec. Hrei` 8>>76 5<>, <;7 “<?7
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)• LEE (lkrkhrec eaycjim e`gijpetby)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
• _erie
• \pjremil saecc ertkry m iskesks j o hrei`
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
• LEE (lkrkhrec eaycjim e`gijpetby)
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Bkrjvili \ajjtb au slck elt i` Ljc cegk` I
B&K
Bkrjvili B&K
Oigs E-L orja Feciaj kt ec. Grkk`oikcm ½ s @kurjpetbjcjgy 2tb km. 8>>26 Oig. M ljurtksy jo Mr. B
_i`tkrs6 Oig. K orja Eceouzjoo kt ec. Kxp Gkrj`tjc =7 (8>58) 28;-<<
K
E H L
M O
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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\aecc vksskc miskesk
E4 Gre`u cer lj rt ilec
etrjpby
H e`m L4 saec c i`oerlts
E H
L
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Etbkrjslckrjsis i` saecc ertkriks is i` pri`lipck siaicer esi` cergkr vksskcs. I` hrei` ertkriks it lrkkps mjw` tj
saecckr hre`lbks e`m aey leusk stk`jsis jr jllcusij`.
Kahjci mktelbkm orja pcequks jo cergkr vksskcs le` cjmgk i`
saecc ertkriks e`m leusk jllcusij`.
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)• LEE (lkrkhrec eaycjim e`gijpetby)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
• _erie
• \pjremil saecc ertkry m iskesks j o hrei`
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
• LEE (lkrkhrec eaycjim e`gijpetby)
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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\wkmisb (p.FA?7>-?75@C)
Jutkr ceykr
I``kr ceykr Ocjrime (p.I75?_)
↞ Erltil (p.K?9<G), Mutlb (p.K?9<U),
Itecie` (p.K?9<F)
Ockaisb (p.E?98G)
↘
↕Ijwe (p.M?9=@)
_ 0 O (I`mie`e), _ 0 G jr 0 I(Cj`mj`)
e-sklrktesk
h-sklrktesk
g-sklrktesk
Itecie` rklkss (p.E?7<_) ↘
┎ Io tbk autetij` is i` Eh, eay-
cjim is autetkm jr aut+wt.
┎ Io tbk autetij` is i` ajcklu-
cks rkguceti`g Eh prjlkssi`g,
eaycjim is jo wicm typk (wt)
Jllipi tec ljr tkxLj`gj rkm ]bijo cevi`
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Eh5-=>
Jllipi te c ljrtkx
Eh5-=>
Lj`gj rkm
+ hirkor i`gk`l k B&K
Kckltrj` a ilrjslj py4 Eh eaycj im o ihr i cs
(Erl t il EM muk tj p .K?9<G autet ij` i` E θ^^)
]bijo cevi`
Lkrkhrec eay-
cj im e`gijpetby
(LEE ) eooklts
vksskcs jo ec c
s izk i`lcum i`g
sa ecc ertkr iks
mjw ` tj lep i c-
cer iks.
‑ mjuhck herrkci`g ‑
(bkrk tbk kxtkr`ec mieaktkrs erk tbk seak
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC LEEB&K Lj`gj rkm
LEME\IC
LEE
3
LEME\IC WA4 ertkr ij cks
(@<KLM)
LEME\IC
3
(bkrk tbk kxtkr`ec mieaktkrs erk tbk seak
ILB jo cjher typ k
Jllip i tec lj r tkx J i i cI` LEE E jotk`
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Jllip i tec lj rtkx,
Erlt il EM muk tj
p.K?9<G autetij`
i` E θ^^
Eh5-=>
Jllip i te c lj rtkx
i` spj rem il EM
E .
Oig. E4 Feciaj kt ec. Elte @kurjpetb Ljaa 8>5< i` pri`t, Oigs H e`m L4 ]bec kt ec. @kurjhijc
Egi`g <> (8>>9) 59<?-=2
I` LEE Eh jotk`
elluaucetks
ecsj i` lepi c ce-
r iks, i` skvkrk
lesks it leusks
lepi c cery jll -
cusij` e`m LHO
misturhe`lks,
hut r erkcy jvkrt
i` oerlts.
Lepi c cery LEEaey lj `t r ihu tk
tj tbk mkgk`kre-
t ij` jo ̀ kurj`s
i` E czbkiakr ½ s
miskesk hy ectkr- i`g/ iapeir i`g
LHO.
\lckrjt il i`mkx (\I)
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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( )
5 “ (i`tkr`ec mieaktkr/kxtkr`ec m ieaktkr)
i .k. tbk b igbkr tbk vecuk, tbk tb ilfkr is tb k wecc , e`m i` e`
jh c i tkretkm ertkry
\I 1 5
\LCKXJ]IL I@MKR (\I) E@ECY\I\**
\I \]MK_ ]-]K\]
LEME\IC
WA (`1989) >,7< >,5= ^3>.>5LJX]KR (`18;>) >,;? >,5< ^1>.><
LJ@]XJC\
WA (`1852) >,=; >,5?
LJX]KR (`18;2) >,;< >,5?lwEM
WA (`18<8) >,=< >,5<
LJX]KR (`1=5?) >,=2 >,5<
**4 hcjjm vksskcs witb kxtkr`ec mieaktkr ckss tbe` <> aa erk kxlcumkm.
^\5M9 EM
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Wkcckr kt ec. Hrei` ^etbjcjgy 8>>26 524 8;< “8??
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEXE\IC
(lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• Oirst rkpjrtkm hy Aekme kt ec. i` tbrkk sihci`g petik`ts orja e
Depe`ksk oeaicy prkciai`ericy i` 59?; e`m i` grketkr mkteic
597? witb \. Aekme es tbk oirst eutbjrs, bk`lk hkk` ecsj
lecckm Aekme sy`mrjak. Ehjut ;> lesks mieg`jskm, aei`cy i`
Depe`, hut ecsj i` Lbi`e e`m 5 Leulesie` petik`t i` \pei`.
• J`skt muri`g petik`t’s twk`tiks tj kercy tbirtiks es e veslucj-
petbil suhljrtilec k`lkpbecjpetby witbjut bypkrtk`sij`.
• @kurjcjgil syaptjas4 skvkrk psylbjajtjr mktkrijretij`
leuskm hy suhljrtilec k`lkpbecjpetby4 k.g. mysertbrie,myspbegie, kajtij`ec i`stehicity, e`m spestil geit.
• @j`-`kurjcjgil syaptjas erk k.g4 ecjpklie, vkrtkhrec misl
bkr`ietij`, e`m spj`mycjsis mkojrae`s.
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Yeaeajtj kt ec. @kurjpetbjcjgy e`m Eppcikm
@kurjhijcjgy <7, 9= “55<
LEXE\IC, ak`i`gkec ertkry LEXE\IC WA ertkry
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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, g y
LEXE\IC, WA ertkry Lj`trjc, ak`i`gkec ertkry
LEME\IC, ak`i`g. ertkry LEXE\IC, ak`i`g. ertkry
LEXE\IC4 oihrjus i`tiaec
prjciokretij`, spcitti`g jo tbk
i`tkr`ec kcestil ceai`e, e`m
jllesij`ec byeci`k mkgk-
`kretij`, hut rksucts j` cu-
ai`ec stk`jsis mislrkpe`t.
\lckrjtil i`mkx4 (` 1 8)
Lj`trjcs4 >.576 >.82
LEXE\IC WA4 >.8;6 >.<9
LEME\IC WA4 >.?5
Jimk kt ec. @kurjpetbjc, 8>>26 8245<8-5=8
LEXE\IC, WA ertkry
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Oirst Leulesie` (\pe`isb ) LEXE\IC petik`t 4
]8- e`m ]5-wkigbtkm AXI lbe`g ks4
•
Mioousk ckufjk`lkpbecjpetby i`vjcvi`g pkrivk`trilucer e`m mkkp WA,i`lcumi`g e`tkrijr tkapjrec cjhks e`m kxtkr`ec lepsucks.
• Auctipck celu`er i`oerlts mkkp WA e`m GA e`m hrei`stka (L e`m M).
• Auctipck ailrj-hckkms i` pj`s, hesec ge`gcie, e`m bkaispbkril suh-ljrtilec WA (K).
]8w AXI ]5w AXI ]8*-GK AXI
Ak`mijrjz kt ec. @kurjcjgy 8>586 7;4 8><<-<7
B mit cc t m i o h i
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)• LEE (lkrkhrec eaycjim e`gijpetby)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
• _erie
• \pjremil saecc ertkry m iskesks j o hrei`
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
• LEE (lkrkhrec eaycjim e`gijpetby)
^EMAEC
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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Mi`g kt ec D @kurjiaegi`g 8>5>6 8>45<=-5=>
^EMAEC
^EMAEC, ljccegk` I_ ^EMAEC,
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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^EMAEC, e-\AE
, g
^EMAEC, LM?2 a ilrjgie
^EMAEC, ve` Giksj` -kcestile
ve` Giksj`-kcestile
^EMAEC
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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^EMAEC
Begkc kt ec. Elte @kurjpetb 8>>=6 5>248<5-=>
^EMAEC
^EMAEC, ljccegk` I_
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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, g
^EMAEC , KA
• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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• Bkrkmitery sa ecc ertkry m iskesks j o hrei`
• LEME\IC (lkrkhrec eutjsjaec mjai`e`t ertkrijpetby witbsuhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby )
• \wk-bAIM (\wkmisb bkrkmitery aucti-i`oerlt mkak`tie)• LEE (lkrkhrec eaycjim e`gijpetby)
• LEXE\IC (lkrkhrec eutjsjaec rklkssivk ertkrijpetby witb
suhljrtilec i`oerlts e`m ckufjk`lkpbecjpetby)
• ^EMAEC (pj`ti`k eutjsjaec mjai`e`t ailrje`gijpetby e`m
ckufjk`lkpbecjpetby )• Xkt i`ec veslucjpetby w itb lkrkhrec ckufjmystrjp by (X_LC)
• LJC=E5 essjlietkm \EMH
• Oehry m iskesk
• _erie
• \pjremil saecc ertkry m iskesks j o hrei`
• Ertkr ij cjsl ckrjs is (–Hi`swe`gkr½s miskesk‑)
• LEE (lkrkhrec eaycjim e`gijpetby)
Xkti`ec veslucjpetby witb lkrkhrec ckufj-
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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p y
mystrjpby (X_LC), jo typk BKX@\
(bkrkmitery k`mjtbkcijpetby, rkti`jpetby,
`kpbrjpetby, witb strjfk-cifk kpisjmks),
is leuskm hy autetij`s i` ]XKR5 (tbrkk
priak rkpeir kxj`ulckesk 5) LM?2 ojr ailrjgcie
@kurjoiceak`t
Oigurks l jur tksy j o Mr. B _i`tkrs B&K
@jrmil LEME\IC rkskerlb grjup
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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@jrmil LEME\IC rkskerlb grjup
Lci`ilec stumiks
“ Aetti _iite`k` [ jo ], Ferjc I`st “ \use``e Xji`k [ jo ]
“ Euci _krffj`ikai [ jo B
^etbjcjgy
“ Be``u Feciaj [ jo B “ Ciise Ayccyfe`ges [ jo B
“ Ui`g Aiej [ jo ]
Gk`ktils
“ Ai``e ^ÿybÿ`k` [ jo B
“ Aeide \iitj`k` (`kk Du``e)* [ jo ]
“ ^ktre ^ese`k`* [ jo ]
^rjtkjails e`m ajcklucer hijcjgy
“ Aerl Heuae`` [ jo B
“ \eere ]iffe [ jo B
“ Aelikd Cecjwsfi [ jo B
“ [rhe` Ck`mebc e`m tkea Ferjc I`st
Iaegi`g e`m ^K] stumiks
“ Dube Xi``k [ jo ]
“\use``e Xji`k [ jo ]
Jpbtbecajcjgy
“ ]krj Fivkcí [ jo B
“^euce \uaae`k` [ jo B
[ jo B 1 [`iv jo Bkcsi`fi, Oi`ce`m [ jo ] 1 [`iv jo ]urfu, Oi`ce`m Ferjc I`st 1 Ferjci`sfe I`st, \wkmk`
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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LEME\IC4 ]8w AXI w itb lj`-
o cuk`t bypkri`tk`si t iks
Djk`suu
Bkcsi`fi
^jri
_eese
X5<<L X528L
↕
]urfu
7/29/2019 Hannu Kalimo - Neuropathology of CADASIL and other small artery diseases of the brain (SADB)
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]urfu ljet jo eras