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Twenty seven years Experience in theTwenty seven years Experience in the
Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)
The fourth annual
International Neurosurgery Conference27-30 December 2008
Quintana Leonidas , Massaro Paolo, Gonzalez Francisco, Yokota Patricio, Segovia Miguel
Department of Neurosurgery- Valparaso University - Faculty of Medicine
Chile
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ChronicChronic cerebrovascularcerebrovascularocclusiveocclusive
diseasedisease
Arteritis (Arteritis ( endarteritisendarteritis))
GeneticGenetic immunologicalimmunological basebase
NameName: cigarette smoke in the air: cigarette smoke in the air
MoyamoyaMoyamoya ininjapanesejapanese
Twenty seven years Experience in theTwenty seven years Experience in the
Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)
Jiro Suzuki
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StagesStages ofofprogressionprogression
(( SuzukisSuzukis ClassicationClassication:: ArchArch NeurolNeurol ,, VolVol 20, 28820, 288--299,1969)299,1969)
1
2
3
4
5
6
PROGRESSIVE STENOSIS OF INTRACRANIAL INTERNAL CAROTID ARTERIES , ACA & MCA
PROGRESSIVE APPEARANCE OF A MESH OF VESSELS OF SMALL DIAMETER IN THE REGION
OF BASAL GANGLIA, AS A FORM OF COLLATERAL CIRCULATION
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ISCHEMIC FORMISCHEMIC FORM
ChildsChilds
T.I.AT.I.A
InfarctionInfarction
CerebralCerebral atrophyatrophy
HEMORRHAGIC FORMHEMORRHAGIC FORM
YoungYoung adultadult
VentricularVentricularhemorrhagehemorrhage
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What is the meaning of moyamoya vessels?
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VASCULAR ENDOTHELIAL GROWTH FACTOR EXPRESSION AND ANGIOGENESIS
INDUCED BY CHRONIC CEREBRAL HYPOPERFUSION IN RAT BRAINJian Hai, M.D., Ph.D. Shi-Ting Li, M.D., Qi Lin, M.S., Qing-Gang Pan, M.S., Fei Gao, Ph.D., Mei-Xiu Ding, M.D.
Neurosurgery 53:963-972, 2003Conclusions: These results demonstrated that chronic cerebral hypoperfusion could
Induce sustained up-regulation of VEGF mRNA and protein expression in rat brain,
which was correlated with angiogenesis.
Enhanced brain angiogenesis in chronic cerebral hypoperfusion after
administration of plasmid human vascular endothelial growth factor in
combination with indirect vasoreconstructive surgeryNOBORU KUSAKA, M.D., KENJI SUGIU, M.D., KOJI TOKUNAGA, M.D.,ATSUSHI KATSUMATA, M.D., AYUMI NISHIDA, M.D.,
KATSUNARI NAMBA, M.D.,HIROFUMI HAMADA, M.D., HIROYUKI NAKASHIMA, M.D., AND ISAO DATE, M.D.
J Neurosurg 103:882890, 2005
Conclusions. In rat models of chronic cerebral hypoperfusion, administration of
phVEGF combined with indirect vasoreconstructive surgery significantly increased
capillary density in the brain. The authors results indicate that administration of
phVEGF may be an effective therapy in patients with chronic cerebral hypoperfusion,such as those with moyamoya disease.
Chronic ischemia Genetic mech. mRNA VEGF Angiogenesis
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ISCHEMIA
HYPOXIA
cell elected
ANGIOGENESIS
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15 cases ( 7 males, 8 females. Age : 6 46 y.o ;media : 25,4 y. old)
Name Age Gender StrokeType Moyamoya clinical pattern
R.A. 6 M Ischemic TypicalL.M. 8 M Ischemic Typical
J.N. 9 F Ischemic Typical
Y.M. 11 M Ischemic Typical
L.O. 15 M Ischemic Atypical (unilat)
F.G. 16 M Ischemic TypicalF.C. 16 F Ischemic Typical
Ischemic cases7 cases ; 6 typical , 1 atypical
Twenty seven years Experience in theTwenty seven years Experience in the
Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)
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15 cases ( 7 males, 8 females. Age : 6 46 y.o ;media : 25,4 y. old)
Name Age Gender StrokeType Moyamoya clinical pattern
J.G. 18 F Hemorrhagic Atypical
L.E. 25 F Hemorrhagic TypicalH.N. 36 M Hemorrhagic Atypical (unilat)
V.S. 37 F Hemorrhagic Atypical (unilat)
R.C. 42 F Hemorrhagic Atypical (unilat)
J.G. 46 F Hemorrhagic Atypical (unilat)F.M. 46 M Hemorrhagic Typical
C.R. 51 F Hemorrhagic Typical
Hemorrhagic cases
8 cases ; 3 typical , 5 atypical
Twenty seven years Experience in theTwenty seven years Experience in the
Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)
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Angiographical criteria to diagnose the Moyamoya Disease(Research committee on progressive occlusive disease of the circle of Willis,Ministry of
Health and Welfare of Japan 1978)
1-Stenosis or occlusions at the terminal portion of intracranial ICA
and/or the proximal portion of the ACA and/or the MCA.
2- Abnormal vascular mesh, the moyamoya vessels, observed inthe neighborhood of the above mentioned areas in the arterial
phase.
3- Above mentioned findings are recognized bilaterally.
We observed 6 atypical cases ( CUASI MOYAMOYA )
or Akim Moyamoya ( 40%)
4 cases Hemorrhagic Stroke unilaterally moyamoya vessels
1 case Hemorrhagic Stroke early occlusion of cervical ICA bilat.
1 case Ischemic Stroke unilaterally moyamoya vessels
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CASE N 6 J.G. 18 years old , female
Mild headache, left hemiparesis , with complete recovery.
AORTIC ARCH RIGHT CERVICAL LEFT CEREVICAL
ICA ICA
RIGHT ICA AP LEFT ICA AP VERTEBRAL
CASE N 2 J N 9 ld f l
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CASE N 2 J.N. , 9 years old, female
Difficult learning, speech disturbance, right progressive
hemiparesis, focal convulsions.
VERTEBRAL LAT. VERTEBRAL AP.
RIGHT ICA AP RIGHT ICA LATERAL LEFT ICA AP LEFT ICA LATERAL
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ENCEPHALO-DURO-ARTERIO-SINANGIOSIS ( EDAS )
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ENCEPHALO-DURO-ARTERIO-GALEO-SINANGIOSIS
BURR HOLES PERIOSTIAL -SINANGIOSIS1
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2 ENCEPHALO-DURO-ARTERIO-GALEO-SINANGIOSISBURR HOLES PERIOSTIAL -SINANGIOSIS
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ANGIOGRAPHICAL CONTROL ( 6-12 monthes )
RIGHT ICA LAT. PREOP. RIGHT ICA LATERAL RIGHT ICA LATERAL
POSTOP. 1 POSTOP. 2
LEFT ICA LAT. PREOP. LEFT ICA LATERAL LEFT ICA LATERAL
POSTOP. 1 POSTOP. 2
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SURGICAL TREATMENT
10 first operations (1982- 2000) : EDAS
5 last operations (2001- 2007) :EDAS + wide galeal flap
Encephalo-duro-galeo-
arterio- sinangiosis
+burr holes and periostialsinangiosis
MEDICAL TREATMENT
Vasodilators drugsCorticosteroids
Platelets antiaggregants
Nootropics drugs
NO UTILITY
DEMONSTRATED
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TABLE FOR FUNCTIONAL EVALUATION
I Return to a normal life
II Activity with mild limitations
III Activity with severe limitationsIV Vegetative state
V Died
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ANALYSIS OF FUNCTIONAL RESULTS
ACCORDING WITH THE INITIAL DAMAGE
Ischemic cases Hemorrhagic cases Grade
2 TIA 1 small ICH ; 1 IVH I
3 Infarction (2 temp;1 front.) 1 IVH; 1IVH+ lobar ICH II
1 Hemispheric Infarction 2 severe IVH
2 IVH + basal ganglia ICH III
1 Bilateral multiple infarctions IV
Initial clinical presentation and time of evolution
of Moyamoya Disease is strongly related
with the final outcome
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RESULTS AT 6 TO 12 MONTHS AFTER OPERATIONClnical results
I Return to normal life 4 cases
II Activity with mild limitations 5 cases
III Activity with severe limitations 5 casesIV Vegetative state 1 case
V Death 0 cases
Angiographical results
14/15 Presented signs of effective revascularization1/15 Not presented signs of effective revascularization
The revascularization signs were present between 6-12 months
after the operation, and the donnor artery with wider caliber
EXCELLENTEXCELLENT
OR GOODOR GOOD
BADBAD
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OUTCOMEFollow up between 2 27 years
I Return to a normal life 4 cases
II Activity with mild limitations 5 casesIII Activity with severe limitations 5 cases
IV Vegetative state 0 cases
V Died 1 cases
Related with:
1- Early Diagnosis
2- Effective treatment
EXCELLENTEXCELLENT
OR GOODOR GOOD
POORPOOR
CONCLUSIONS
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CONCLUSIONS
1- THE PATTERN OF MOYAMOYA DISEASE IS SOMEWHATDIFFERENT IN OUR EXPERIENCE, FROM THE JAPANESE
EXPERIENCE , WITH MANY CASES CATALOGUED AS CUASI
MOYA MOYA,PERHAPS WE SHOULD SAY WESTERN
MOYAMOYA.AND ITS MORE FREQUENTLY SEEN, IN OUR EXPERIENCE, IN THE
HEMORRHAGIC FORM OF MOYA MOYA DISEASE.
2- OUR EXPERIENCE SHOWS THAT THE MOST IMPORTANTPOINTS RELATED WITH THE CLINICAL RESULTS ARE THE
INITIAL NEUROLOGICAL DAMAGE, AND THE EARLY DIAGNOSIS
AND TREATMENT
3-WE EMPLOYED THE WIDELY USED TECHNIQUE OF EDAS, AND
LATER, EDAS PLUS WIDER GALEAL FLAP, AND BURR HOLES,
WITH GOOD ANGIOGRAPHICAL RESULTS AND RELATIVE GOOD
CLINICAL RESULTS.
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Twenty seven years Experience in theTwenty seven years Experience in the
Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)
The fourth annual
International Neurosurgery Conference27-30 December 2008
Quintana Leonidas , Massaro Paolo, Gonzalez Francisco, Yokota Patricio, Segovia Miguel
Department of Neurosurgery- Valparaso University - Faculty of Medicine
Chile
THANK YOU VERY MUCH !!!THANK YOU VERY MUCH !!!
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