Type ndeg Age (DC) Type AVC Vasculopathie
1 SS 2e 35 ans Heacutemorragique Moya moya
2 SS 1er 23 ans Ischeacutemique
3 SS 3e 18 ans Heacutemorragique Moya moya deacutecours allogreffe
4 SS 1er 34 ans Heacutematome sous dural
5 SS 1er 25 ans Heacutemorragique Aneacutevrisme
6 SS 1er 27 ans Ischeacutemique
7 SS 1er 34 ans Heacutemorragique Dissection
8 SS 1er Heacutemorragique
9 Sb0 1er 29 ans Heacutematome extra dural grossesse
10 SC 1er 46 ans Heacutemorragique Aneacutevrisme
11 SC 1er 31 ans Heacutemorragique grossesse
Deacutecegraves lieacute agrave lrsquoAVC
Aneacutevrysme et meacutecanisme
Aneacutevrysme sans vasculopathie steacutenosante Freacutequence pop geacuteneacuterale Mutations favorisant ou responsable Evolution bull Hyperdeacutebit bull Heacutemolyse bull Inflammation endotheacuteliale bull Deacutegeacuteneacuterescence intimale fibro-eacutelastique
Aneacutevrysme sur Moya Moya Maladie dysplasiante Freacutequence Hyperdeacutebit du reacuteseau de suppleacuteance Risque heacutemorragique
Aneacutevrysmes du siphon carotidien
Embolisation des aneacutevrismes
Aneacutevrysme et traitement taille symptomatique localisation
Mr D 27 ans
bull AIC agrave 9 ans avec heacutemipareacutesie Dte seacutequellaire
bull Programme drsquoET bien conduit
bull IRM Infarctus sylvien Droit agrave 9 ans Multiples lacunes
bull Angio MR
Occlusion des deux carotides internes
reacuteseau Moya Moya perte de M1 bilat et de P2 G
Mr D 27 ans
MOYA MOYA risque heacutemorragique
Pas drsquoanti coagulant pas de fibrinolyse pas drsquoanti agreacutegant plaquettaire
5 Prise en charge Aigue et chronique
Prise en charge aiguumle de lrsquoaccident ceacutereacutebral
Echange transfusionnel en urgence
Bas deacutebit seacuterum physiologique pendant la saigneacutee Objectif 30 HbS (SS) et gt40 HbA (SC) Hb lt 11gdl Si pas drsquoeacutelectrophoregravese 3 ET (6 CG) successifs (heacutemostase)
1er
Protocole drsquoeacutevaluation de la perfusion ceacutereacutebrale (PCDREP)
inclusion M6 1 an 2 ans
IRM angio MR (Willis) perf Echo doppler Tc Scintigraphie de perfusion ceacutereacutebrale
Bilan neuro cognitif
Equipe multidisciplinaire Centre de reacutefeacuterence (UMGGR) neurologie neuro radiologie reacuteeacuteducation med nucleacuteaire neuro chirurgie reacutea neuro chirurgie
Consultation et bilan biologique tous les 6 mois
Bilan cognitif
Inteacuterecirct drsquoune approche multidisciplinaire
bull Pour le patient ndash Meacutecanisme de lrsquoaccident ceacutereacutebral ndash Theacuterapeutique (deacutecision colleacutegiale) ndash Deacutepistage preacutecoce de lrsquohypoperfusion preacutevention ndash Reeacuteducation ndash Suivi cognitif risque de deacutemence
bull Approches compleacutementaires et confrontation
des ideacutees dans un objectif de recherche
Cohorte PCDREP Recul 4 ans
Mortaliteacute 14 par an 586 4 ET 1 HU 100 vasculopathie Causes 1 inconnue 1 IDM 3 AVC heacutemorragiques
Reacutecidives AVC 2 par an 786 bull 3 heacutemorragiques (Moya
sous 23 ET) bull 5 ischeacutemiques
ndash 3 Vasculopathies sous ET ndash 1 Vasculopathie sous HU ndash 1 SAPL arteacuteriel malgreacute TAC
(INR 18)
Quelles sont les orientations agrave venir
Doppler Tc bull Plus sensible bull Plus preacutecoce bull Montre des acceacuteleacuterations
ndash Steacutenose anatomique ndash Steacutenose heacutemodynamique ndash Aspect MoyaMoya
bull Reacuteserve de vaso dilatation bull Montre le sens de circulation
du flux bull Deacutepistage en Afrique
Angio TDM ou ARM bull Montre les steacutenoses
anatomiques bull Stade peu reacuteversible sur
la vasculopathie bull Coupleacute agrave lrsquoIRM pour
visualiser le parenchyme ceacutereacutebral
bull Diffusion (AVC reacutecent)
Apport du doppler Tc dans le suivi de la vasculopathie ceacutereacutebrale dreacutepanocytaire adulte
Objectifs du Doppler transcracircnien
Enfants
Preacutevenir lrsquoapparition de lrsquoarteacuteriopathie ceacutereacutebrale (Etude STOP)
Adultes
Deacutepistage non fait Ou non accegraves agrave lrsquoangio TDM ou angio RM (Afrique)
Evolution apregraves modification theacuterapeutique
MAIS laquo NON REALISABLE CHEZ LrsquoADULTE DREPANOCYTAIRE raquo
Adultes
Preacutesentateur
Commentaires de preacutesentation
What is the doppler ultrasonography examination interest in adult SCD The main goal of doppler ultrasonography in children is to prevent the appearence of a cerebral arteriopathy before it occurs and then be visible on the MRA by the initiation of chronic blood exchange transfusions 13In adults the main goal is different since the arteriopathy did appear during childhood The primary goal is then to diagnose a cerebral arteriopathy in areas around the world were CT scan or MRI are not available or too expensive The other interest in Europe and USA is to monitor therapeutic changes in an easier way then MRI during the patientrsquos follow up 13However No study to detect stenosis and Moyamoya syndrome by doppler ultrasonography have been done and the question is even if it is feasible in adult SCD patients 13131313
Patients
Critegraveres drsquoinclusion dans lrsquoeacutetude PCDREP ndash Agegt18 ans ndash Dreacutepanocytose ndash ATCD drsquoatteinte clinique neurologique AVC AIT
eacutepilepsie atteinte psychiatrique
ndash Atteinte vasculaire ceacutereacutebrale (TDM IRM ou doppler dans lrsquoenfance)
ETOU
Preacutesentateur
Commentaires de preacutesentation
We underwent a study to answer this question as part of the PCDREP cohort study 13Inclusion criteria of PCDREP study13Agegt18 years13Sickle cell disease13History of clinical brain involvement overt stroke transient ischemia epilepsy or psychiatric disorder13And or13Previous imaging evoking a cerebral vasculopathy13
Ratio =
Meacutethode
bull IRM 3Tesla avec seacutequence ARM TOF bull Et Doppler Transcracircnien et des TSA le mecircme jour bull Doppler ratio des vitesses moyennes (TAMMx)
TAMMx le plus eacuteleveacute intra cracircnien
TAMMx carotide extracranienne ipsilateral
Enfant (STOP) TAMMx au niveau de la ceacutereacutebrale moyenne et de la carotide
interne intra cranienne
Pathologique au moins un ge 200 cms Conditionel au moins un ge 170 cms
Normal TOUS lt 170 cms
Preacutesentateur
Commentaires de preacutesentation
All patients had a 3T brain Magnetic Resonance Imaging (MRI) with a 3D time-of-flight Magnetic Resonance Angiography and Doppler ultrasonography performed on the same day An Ultrasound contrast agent was used when needed to increase the contrast during 15 minutes 13Doppler ultrasonography measure included the time-averaged mean of the maximum velocity (TAMMx) and a ratio was calculated between the intracranial arteries and the ipsilateral extracranial carotid 13This is an important difference compared to children where a doppler is considered pathologic if at least one velocity is upper or equal to 200 cms
Definitions de lrsquoarteacuteriopathie intra cracircnienne
ARM
bull Oslash pas de stenose ou lt 50
bull Arteacuteriopathie
stenose intracranienne ge50
ou occlusion
ou Moyamoya
Doppler Tc
Ratio des TAMMx ge3 Ou
deacutemodulation du lit drsquoaval (Moyamoya)
Preacutesentateur
Commentaires de preacutesentation
Patients were considered to have an arteriopathy if they had a stenosis superior to fifty percent andor had a vessel occlusion andor MoyaMoya collaterals13Arteriopathy was defined on Doppler ultrasonography if the time-averaged mean of the maximum velocity ratio was superior or equal to 3 or if very low velocities and blunted flow signal was present in favor of a Moya Moya syndrome13
Pas de steacutenose
Jeune femme de 19 ans avec un ATCD AVC (SAPL)
110
73
= 15
Preacutesentateur
Commentaires de preacutesentation
Here you can see a 19 year-old woman with a previous history of stroke due to an APLS no stenosis was found on MRA On Doppler Ultrasound the TAMMx ratio was lt 3 with a maximal intracranial mean velocity of 175 cms and 119 in the ipsilateral extracranial carotid
Steacutenose gt 50
Femme de 30 ans avec des ATCD drsquoAVC sur arteacuteriopathie ceacutereacutebrale (ACM)
138
31
= 445
Preacutesentateur
Commentaires de preacutesentation
In this case a 30 year-old woman with a known vasculopathy and previous history of strokes had a significant stenosis on the right MCA on MRA 13On Doppler Ultrasonography the time-averaged mean of the maximum ratio was gt3 evoking a stenosis in the right middle cerebral artery 1313
Syndrome Moyamoya
Une femme de 35 ans avec des ATCD drsquoAVC sur un syndrome de Moyamoya
Preacutesentateur
Commentaires de preacutesentation
Finally in this third exemple a 35 year-old woman with a known vasculopathy and previous history of strokes had an occlusion of the right MCA and the presence of Moya-Moya collaterals Correspondingly very low velocities and blunted flow signal on MCA without carotid stenosis or occlusion was in favor of a Moya Moya on the Doppler Ultrasonography13
Reacutesultats
bull 80 patients
bull Moyenne drsquoacircge 305 plusmn 79 ans
bull Sexe ratio (HF) 08
bull 77 ont eu un Doppler et une ARM
bull Produit de contrast ultrasonographique a eacuteteacute utiliseacute
chez 15 des patients (Sonovue reg)
Preacutesentateur
Commentaires de preacutesentation
80 patients were included in the study 3 patients presented a contra indication to MRI13Mean age 305 plusmn 79 years13Sex ratio (MF) 13Among 77 who had Doppler ultrasonography and magnetic resonance imaging 1338 patients (50) had intracranial arteriopathy on MRA including 17 patients presenting Moyamoya collaterals13Ultrasound contrast agent was required in 15 of patients (Sonovue reg)
bull 38 patients avec arteacuteriopathie ndash 10 steacutenose(s) ndash 11 occlusion(s) ndash 17 Moyamoya
bull 39 patients sans arteacuteriopathie
bull 33 patients ont eacuteteacute deacutepisteacutes ndash 5 steacutenose(s) ndash 11 occlusion(s) ndash 17 Moyamoya
bull 5 patients avec une steacutenose ont eacuteteacute manqueacutes
ARM Doppler
Reacutesultats
Sensibiliteacute = 87 Specificiteacute = 90
Valeur Preacutedictive Positive = 86 Valeur Preacutedictive Negative = 87
TOUTES LES MOYAMOYA
Preacutesentateur
Commentaires de preacutesentation
On Magnetic resonance angiography 38 patients had an intracranial arteriopathy 10 having a stenosis 11 an occlusion and 17 Moyamoya collaterals13On Doppler ultrasonography 33 patients had a severe arteriopathy 5 presented a stenosis 11 an occlusion and 17 Moyamoya 135 patients presenting a stenosis were missed13131313