Fibromuscular dysplasia of cervicocephalic arteries: Prevalence of multisite involvement and prognosis.

BACKGROUND: Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic disease of small- to medium-sized arteries. The frequency of multisite involvement and its influence on prognosis has not been systematically assessed in patients with cervicocephalic FMD, and little is known about their mid-term clinical and arterial prognosis. The aim of our study was to assess the prevalence of renal involvement and clinical and arterial prognosis in patients with cervicocephalic FMD. METHODS: We reviewed clinical and radiological data of consecutive patients with a diagnosis of cervicocephalic FMD, admitted to our hospital between January 2000 and March 2010. Patients were identified retrospectively until December 2008, and prospectively from January 2009. For each cervical and intracranial artery, we recorded the presence and type (unifocal or multifocal) of FMD. We classified each FMD-related stenosis into four categories:<50%, 50-80%,>80% and occlusion. During the first six months of 2012, patients were scheduled for follow-up visit, including cervicocephalic follow-up imaging, and renal artery imaging, if not already available. On follow-up imaging, FMD-related stenosis was classified according to the same method used at baseline. Renal artery FMD was defined as the presence of the typical string of beads appearance, or as the presence of a unique stenosis of renal artery. Primary endpoints were stroke (ischemic or hemorrhagic), death, and progression of FMD lesions, defined by any increase in category of stenosis on follow-up imaging. RESULTS: Out of the 36 patients included (32 women), all with carotid artery involvement and 17 with associated vertebral artery involvement, 28 (78%) had ischemic symptoms and/or cervical artery dissection at the time of the diagnosis of FMD. Among the 30 patients who had renal artery imaging, 13 (43%) had renal FMD. Patients with renal artery disease did not differ from those without renal artery disease. After a median follow-up of 3.5 years, three patients had four strokes, one recurrent cervical dissection, one brain hemorrhage, and one fatal cardiac arrhythmia. Among the 31 patients who had follow-up imaging, two showed progression of cervicocephalic FMD (occlusion of carotid artery). Patients with renal involvement showed a non-significant trend toward a higher rate of stroke (P=0.17). CONCLUSIONS: In patients with cervicocephalic FMD, renal involvement is common. The risk of stroke, death or FMD progression was high in our cohort, suggesting that prognosis may not be as good as expected. This underlines the need for larger prospective studies to define the best treatment options.

Imaging of cervical artery dissection.

Cervical artery dissection (CAD) may affect the internal carotid and/or the vertebral arteries. CAD is the leading cause of ischemic stroke in patients younger than 45 years. Specific treatment (aspirin or anticoagulants) can be implemented once the diagnosis of CAD has been confirmed. This diagnosis is based on detection of a mural haematoma on ultrasound or on MRI. The diagnosis can be suspected on contrast-enhanced MRA (magnetic resonance angiography) or CT angiography, in case of long stenosis, sparing the internal carotid bulb, or suspended, at the junction of V2 and V3 segments of the vertebral artery, in patients with no signs of atheroma of the cervical arteries. MRI is recommended as the first line imaging screening tool, including a fat suppressed T1 weighted sequence, acquired in the axial or oblique plane at 1.5T, or 3D at 3T. Complete resolution of the lumen abnormality occurred in 80% of cases, and CAD recurrence is rare, encountered in less than 5% of cases. Interventional neuroradiology (angioplasty and/or stenting of the dissected vessel) may be envisaged in rare cases of haemodynamic effects with recurring clinical infarctions in the short-term.

[Cervical arteries dissection: diagnostic Color Doppler US criteria at the acute phase]. / Critères diagnostiques en échographie-Doppler des dissections artérielles cervicales à la phase aiguë.

PURPOSE: To estimate the value of duplex color-coded ultrasonography in the initial diagnosis of acute cervical artery dissection. MATERIAL AND METHOD: Retrospective study of 31 patients, mean age 45, sex ratio=1, referred for clinical suspicion of cervical artery dissection, confirmed by MRI. 46 dissected arteries were imaged. Ten patients presented multiple dissections. The evaluated sonographic diagnostic criteria were the direct signs of intra-mural hematoma: localized increased diameter of the artery, narrowed lumen, hypo and/or isoechoic intra-mural hematoma, intimal flap. The associated criteria studied were: the location of intra-mural hematoma and in case of an occlusion, the dissection of an other artery. RESULTS: We analyzed separately the arterial segments visualized in B-mode ultrasound (supra-bulbar internal carotid artery, vertebral artery from V0 to V3) and the arterial segments evaluated only by pulsed Doppler (intrapetrosal carotid artery, V3-V4 of the vertebral artery). With the above criteria, on arterial segments visualized in B-mode ultrasound, the diagnosis of dissection was done in 83% of cases during the initial examination but in only 30% of the arterial segments non visualized on ultrasonography. CONCLUSION: The direct signs of intra-mural hematoma on internal carotid and vertebral segments imaged by B-mode ultrasonography have a good sensitivity. Because results were compared to MRI, the specificity could not be assessed. In case of occlusion of an artery, multiple dissections are a strong argument for the diagnosis.

[Abnormalities of the basal ganglia and thalami in adults]. / Atteintes bilatérales des noyaux gris chez l'adulte.

Several diseases may cause non-specific MR signal abnormalities of the bilateral basal ganglia and thalami. As such, diagnosis of the underlying etiology may be difficult to achieve at imaging. In this review, we will present interpretative guidelines based on clinical data (mode of presentation, previous history, clinical symptoms, and evolution) and imaging data (type of signal abnormalities, location of lesions, and associated abnormalities). The main categories of diseases causing MR signal abnormalities of the bilateral basal ganglia and thalami in adults are reviewed: toxic, metabolic, vascular, tumoral, infectious and inflammatory diseases.

[Imaging of cervical arterial dissections: multi-center study and review of the literature]. / Imagerie des dissections des artères cervicales: enquête multicentrique et revue de la littérature.

Multiple imaging techniques are available to diagnose cervical arterial dissections. It may be possible to image the vessel wall hematoma (US, MRI, 2D or 3D MRA source images, CTA source images), the narrowed arterial lumen (MRA, CTA, catheter angiography), or the hemodynamic impact of the affected vessel. There is no available guideline in the literature regarding the imaging work-up at diagnosis or follow-up. The Société Française Neurovasculaire and the Société Française de Neuroradiologie have sponsored a multi-center study to evaluate current imaging strategies. Results from several centers, while being variable, do share some similarities: the preferred use of non-invasive or minimally invasive techniques to evaluate the vessel wall and the vascular lumen and/or hemodynamic impact, the use of fat suppressed sequences at MRI, the use of Gadolinium enhanced MRA, the limited interest for CT techniques, and the still important use of catheter angiography in selected cases.