E. Auffray-Calvier

Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature.

We report a caroticocavernous fistula (CCF) treated by an endovascular procedure in a young woman with Ehlers-Danlos syndrome type IV, with severe bilateral carotid and vertebral artery dysplasia. The CCF, which appeared after minor trauma, was successfully occluded by a detachable balloon introduced into the venous side by an arterial approach. Six previously published cases are reviewed, five successfully treated. The difficulties and risks of the endovascular procedure due to the vascular changes, are emphasised. The possibility of the venous approach is discussed.

Challenging the Diagnosis of Primary Angiitis of the Central Nervous System: A Single-center Retrospective Study

Objective. (1) To describe a series of adults assessed for suspected primary angiitis of the central nervous system (PACNS) and their final diagnosis; (2) to describe and compare presenting features of PACNS and reversible cerebral vasoconstriction syndrome (RCVS); and (3) to evaluate the specificity of the presenting features of RCVS. Methods. Patients evaluated at our institution between 2000 and 2008 for a possible CNS vasculitis and investigated by conventional angiography and/or brain biopsy were retrospectively analyzed. The inclusion criteria were a clinicoradiological presentation and cerebral angiography and/or brain biopsy raising the hypothesis of isolated cerebral vasculitis; and absence of identifiable etiology at the time of conventional angiogram and/or brain biopsy. Results. Among 58 cases evaluated, 37 met the inclusion criteria and 33 were included in the study. Thirteen patients had RCVS. Thunderclap headaches, the absence of a focal neurological deficit, a convexal subarachnoid hemorrhage and/or normal brain parenchyma on magnetic resonance imaging, and “string of beads” appearance on conventional angiography had high diagnostic value. Six patients had other noninflammatory vascular disorders (intracranial atherosclerosis, cryptogenic embolism, and genetic vasculopathy). Six patients had infection or malignancy. Eight patients were diagnosed with PACNS; their clinical presentation and disease course were heterogeneous. Brain biopsy was performed in 3 cases (positive in 1). Conclusion. RCVS is an important differential diagnosis of CNS vasculitis. Its particular presentation should allow rapid identification in order to avoid pointless investigations and treatment. The frequent lack of histological proof and heterogeneous presentation of PACNS illustrated the nosological uncertainties of this label.

Dégénérescence olivaire hypertrophique: Aspect IRM et évolution temporelle

Resume Objectif decrire l’aspect IRM de la degenerescence olivaire hypertrophique (DOH) secondaire a une lesion du triangle de Guillain et Mollaret. Patients et methodes nous presentons 15 cas de degenerescence olivaire hypertrophique chez 12 patients (4 femmes et 8 hommes) secondaires a une lesion de la fosse posterieure. L’IRM initiale etait realisee dans un delai de 3 semaines a 8 1/2 ans, avec acquisitions axiales, en echo de spin ponderee en densite protonique, T2 et T1 sans et apres injection de Gadolinium. De plus chez 6 patients, l’evolution du signal dans le temps etait analysee. Resultats neuf patients avaient une DOH unilaterale tandis que 3 presentaient une atteinte bilaterale. La DOH etait liee 8 fois a une lesion controlaterale du noyau dentele, 5 fois a une lesion homo-laterale du faisceau central du tegmentum et dans 2 cas a une atteinte contro-laterale du pedoncule cerebelleux superieur. La DOH etait visible en hypersignal densite protonique et T2 des 3 semaines. Apres injection de Gadolinium, il n’existait pas de rehaussement. Un hypersignal courbe central (HCC) pouvait etre rencontre apres 7 mois d’evolution. Enfin une decroissance progressive du signal etait observee a partir de 3 1/2 ans et les anomalies de signal persistaient apres 13 ans. Sur le plan clinique des myoclonies palatales etaient notees a 5 mois d’evolution chez un patient. Conclusion La degenerescence olivaire hypertrophique est une forme rare de degenerescence transsynaptique, qui associe un hypersigal T2 et un hypertrophie du noyau inferieur de l’olive bulbaire. Elle est secondaire a une lesion de triangle de Guillain et Mollaret et peut entrainer des myoclonies palatales.

Ischemia-modified Albumin and Heart Fatty Acid–binding Protein: Could Early Ischemic Cardiac Biomarkers Be Used in Acute Stroke Management?

BACKGROUND The detection of biomarkers such as ischemia-modified albumin (IMA) and heart fatty acid-binding protein (HFABP) is used in the early diagnosis of acute myocardial infarction. As these biomarkers are not organ specific, we tested them in the neurovascular field. METHODS A total of 41 patients with acute stroke were enrolled (31 ischemic strokes and 10 intracerebral hemorrhages). IMA and HFABP levels were measured in serum samples collected within 4.5 hours of stroke onset. Clinical, imaging, and outcome data were recorded. RESULTS No difference in baseline IMA or HFABP was found between patients with ischemic and hemorrhagic stroke. There was no correlation among biomarker levels at admission, National Institutes of Health Stroke Scale score, or stroke volume. Neither of the biomarkers could predict short-term prognosis. CONCLUSIONS IMA and HFABP do not appear to be relevant in acute stroke management.

Diagnostic et traitement des fistules vertébrovertébrales: Revue de la littérature : à propos de 8 observations

Resume Les fistules arterioveineuses vertebrovertebrales (F.A.V.V.) sont une pathologie vasculaire rare, decouverte de facon fortuite ou lors de l’exploration d’acouphenes pulsatiles, d’un souffle cervical, d’une pathologie ischemique du territoire vertebrobasilaire. Elles peuvent etre spontanees ou, plus souvent, post traumatiques. Notre serie comprend 8 patients âges de 20 a 77 ans, 4 femmes et 4 hommes (4 FVV post-traumatiques, 4 FVV spontanees) pris en charge sur une periode de 15 ans. Le bilan pre-therapeutique a comporte les examens suivants : echo-doppler (n = 4), IRM (n = 3) et surtout arteriographie (n = 8). 7 patients sur 8 ont beneficie d’un traitement realise avec succes par voie endovasculaire (5 occlusions par ballon, 1 occlusion par spire et 1 par manœuvre mecanique), sans complication ni recidive, a l’exception d’un cas. Nous confrontons nos resultats a ceux rapportes dans la litterature et proposons une mise au point sur cette pathologie et sa prise en charge therapeutique, laquelle s’est uniformisee a travers le developpement des techniques d’embolisation endovasculaire.Vertebrovertebral Arteriovenous Fistula (V.V.A.V.F.) is a relatively rare entity. It may be an incidental finding or be detected in patients presenting with pulsatile tinnitus, cervical bruit, or vertebro-basilar insufficiency. It can be spontaneous but it most frequently is post-traumatic in etiology. The authors report 8 patients, 4 women and 4 men aged between 20 to 77 years, with 4 post-traumatic V.V.A.V.F. and 4 spontaneous V.V.A.V.F. that were seen over a 15 year period. Imaging work-up included Doppler US (n=4), MRI 9n=3) and angiography (n=8). Seven of 8 patients were treated successfully using an endovascular technique (5 with balloon occlusion, 1 with coil embolization and 1 using a mechanical maneuver), without complication or recurrence, except in one case. We compare our results with published reports from the literature and review the underlying pathology and management strategies of V.V.A.V.F.

Anévrysme mycotique cérébral : Histoire naturelle et prise en charge thérapeutique

Resume Introduction les anevrysmes mycotiques cerebraux sont une complication rare et grave des endocardites infectieuses, qui engagent le pronostic vital et fonctionnel. Nous rapportons quatre observations de patients ayant presente une endocardite compliquee d’un anevrysme mycotique cerebral. Patients et methodes quatre hommes (âges de 24 ans a 63 ans) ont ete admis a l’hopital pour une endocardite compliquee d’un accident vasculaire cerebral (AVC) ischemique dans 2 cas et d’un AVC hemorragique dans les 2 autres, dont un avec hemorragie cerebromeningee associee. Le bilan neuroradiologique a mis en evidence un anevrysme mycotique cerebral. Discussion a la lumiere des donnees de la litterature, nous discutons de l’histoire naturelle et de la prise en charge des anevrysmes mycotiques cerebraux. Trois therapeutiques sont evoquees : l’antibiotherapie, la chirurgie et le traitement par voie endovasculaire. Conclusion les anevrysmes mycotiques cerebraux sont rares, leur mode de prise en charge est toujours controverse, ne faisant pas l’objet d’un consensus clair dans la litterature, et necessitant une approche therapeutique multidisciplinaire, au cas par cas, en privilegiant le traitement endovasculaire en premiere intention.

Imagerie des accidents vasculaires cerebraux en urgence

Resume Depuis 25 ans, les progres de la Neuroradiologie ont bouleverse la prise en charge de l’accident vasculaire cerebral (AVC). En effet, l’apparition du scanner dans les annees 70 a permis de differencier l’accident vasculaire cerebral ischemique de l’hemorragique. C’est maintenant l’imagerie par resonance magnetique (IRM) qui doit etre privilegiee car elle apporte de nouvelles informations essentielles a l’optimisation du traitement. Grâce a l’IRM de diffusion il est possible de visualiser, en moins d’une minute et precocement, l’etendue de la souffrance cerebrale. Le couplage de l’imagerie de diffusion avec la technique de perfusion permet de reperer le tissu hypoperfuse mais encore viable, menace de necrose, en l’absence de reperfusion. Parallelement, l’imagerie vasculaire IRM non invasive permet de rechercher la cause de l’AVC sur les vaisseaux a destinee cerebrale, mais aussi au niveau de la vascularisation cerebrale elle-meme. Le « sauvetage » de cette « zone de penombre » est au centre de la prise en charge en urgence de l’accident vasculaire ischemique. En cas d’accident vasculaire hemorragique, cette meme imagerie permet de rechercher une lesion vasculaire cerebrale causale (anevrysme, malformation arterio-veineuse, thrombophlebite etc.) et d’envisager son traitement, en privilegiant, quand c’est possible, la voie endovasculaire (Neuroradiologie Interventionnelle).

Cytomegalovirus encephalitis: Undescribed diffusion-weighted imaging characteristics. Original aspects of cases extracted from a retrospective study, and from literature review

INTRODUCTION There is paucity of studies analyzing DWI in MRI sequences when imaging cytomegalovirus (CMV) meningoencephalitis. The main objective of this study is to demonstrate that DWI sequence is mandatory when imaging immunocompromised patients presenting with encephalitic symptoms, as this sequence can reveal very peculiar lesions in the setting of CMV encephalitis. PATIENTS AND METHOD Three CSF PCR CMV positive cases were identified in a 13-year retrospective study with MRI scans including T1, FLAIR, DWI with automated ADC calculation, and T1 with contrast injection, and were reviewed by a senior neuroradiologist. RESULTS Our three cases presented multiple high-signal intensity punctiform lesions in DWI, which uniformly were in restricted diffusion. Each patient had multiple lesions (mean 19.3, range 12-30): 96.5% were supratentorial and 3.5% were infratentorial. Among supratentorial lesions, 62.5% were subependymal, 28.6% were periventricular and 8.9% were subcortical. Some lesions remained in a long lasting restricted diffusion state. All cases had FLAIR curvilinear periventricular high signal intensities. No contrast uptake was found. DISCUSSION Punctiform DWI lesions had a clear ventricle wall tropism, consistent with classical autopsy findings. The classical histological knowledge-compatible explanation for long lasting diffusion restriction is non-lethal cytotoxic edema owing to CMV inclusions. CONCLUSION Subependymal and periventricular punctiform restricted diffusion lesions in the setting of meningoencephalitis in immunocompromised patients seem highly evocative of CMV encephalitis. The diffusion sequence probably reveals focal lesions constitutive of cellular viral inclusions.

ARM des vaisseaux cervico-encéphaliques : technique, principales applications cliniques

Magnetic resonance angiography (MRA) is a very valuable tool in the routine evaluation of patients with stroke syndrome. It provides powerful noninvasive imaging of the cervical and intracranial vessels allowing the detection and the diagnosis of vascular anomalies. MRA usefully supplements, during the same examination, the analysis by MRI of the cerebral parenchyma. We will describe the indications of the various techniques (MRA with and without injection of contrast media) and show the value, artifacts and limitations of MRA in atherosclerotic stenosis or occlusive disease and in arterial dissections. This noninvasive vascular assessment will depend on the initial therapeutic orientation. Within the framework of the hemorrhagic stroke, we will discuss the role and the interest of dynamic MR angiography in the tracking and control of intracranial aneurysms and also the contribution of this newer sequences with gadolinium injection in the detection of cerebral vascular malformations.

Le syndrome de vasoconstriction cérébrale réversible

The reversible cerebral vasoconstriction syndrome (RCVS) is an under-estimated transient acute cerebrovascular disorder. It has long been mistaken as central nervous system vasculitis whereas it is now believed to result from an acute but prolonged vasospasm of cerebral arteries. This disorder can be precipitated by postpartum or vasoactive drug. However, it occurs spontaneously in a significant number of cases. The characteristic clinico-radiological presentation and disease course of the RCVS has been delineated only recently. Mean age at onset is 40-45 years, with a female predominance. A provocative factor can be identified in 12-60% out of the patients. Clinical presentation is predominantly marked by recurrent thunderclap headaches, but can be complicated with focal neurological deficit or seizures. Brain imaging is normal in most cases, but can reveal hemorrhagic or ischemic complications. Cortical subarachnoid hemorrhage is a suggestive finding. A posterior reversible encephalopathy syndrome (PRES) can be seen occasionally. Cerebral angiography reveals multifocal arterial narrowing with string and bead appearance. Cerebrospinal fluid reveals no or mild abnormalities. The disease resumes spontaneously within several days to weeks, whereas vasoconstriction reverses within 1 to 3 months. This clinico-radiological presentation should be promptly recognized in order to avoid unnecessary investigations and aggressive treatment, and lead to search for a triggering factor. Further studies are required in order to clarify the precipitating role of several drugs, and clinical trials are needed to reduce the occurrence of strokes.