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Background and Purpose— Proximal large vessel occlusion (LVO) is present in up to 30% of minor strokes. The effectiveness of mechanical thrombectomy (MT) in the subgroup of minor stroke with LVO in the anterior circulation is still open to debate. Data about MT in this subgroup of patients are sparse, and their optimal management has not yet been defined. The purpose of this multicenter cohort study was to evaluate the effectiveness of MT in patients experiencing acute ischemic stroke (AIS) because of LVO in the anterior circulation, presenting with minor-to-mild stroke symptoms (National Institutes of Health Stroke Scale score of Methods— Multicenter cohort study involving 4 comprehensive stroke centers having 2 therapeutic approaches (urgent thrombectomy associated with best medical treatment [BMT] versus BMT first and MT if worsening occurs) about management of patients with minor and mild acute ischemic stroke harboring LVO in the anterior circulation. An intention-to-treat analysis was conducted. The primary end point was the rate of excellent outcome defined as the achievement of a modified Rankin Scale score of 0 to 1 at 3 months. Results— Three hundred one patients were included, 170 with urgent MT associated with BMT, and 131 with BMT alone as first-line treatment. Patients treated with MT were younger, more often received intravenous thrombolysis, and had shorter time to imaging. Twenty-four patients (18.0%) in the medical group had rescue MT because of neurological worsening. Overall, excellent outcome was achieved in 64.5% of patients, with no difference between the 2 groups. Stratified analysis according to key subgroups did not find heterogeneity in the treatment effect size. Conclusions— Minor-to-mild stroke patients with LVO achieved excellent and favorable functional outcomes at 3 months in similar proportions between urgent MT versus delayed MT associated with BMT. There is thus an urgent need for randomized trials to define the effectiveness of MT in this patient subgroup.

Object To date, experience of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of Huntington's disease (HD) has been limited to a small number of case reports. The aim of this study was to analyze long-term motor outcome of a cohort of HD patients treated with GPi DBS. Methods Seven patients with pharmacologically resistant chorea and functional impairment were included in a prospective open-label study from 2008 to 2011. The main outcome measure was the motor section of the Unified Huntington's Disease Rating Scale. The primary end point was reduction of chorea. Results Patients underwent MRI-guided bilateral GPi implantation. The median duration of follow-up was 3 years. A significant reduction of chorea was observed in all patients, with sustained therapeutic effect; the mean improvement on the chorea subscore was 58.34% at the 12-month follow-up visit (p = 0.018) and 59.8% at the 3-year visit (p = 0.040). Bradykinesia and dystonia showed a nonsignificant trend toward progressive worsening related to disease evolution and partly to DBS. The frequency of stimulation was 130 Hz for all patients. DBS-induced bradykinesia was managed by pulse-width reduction or bipolar settings. Levodopa mildly improved bradykinesia in 4 patients. Regular off-stimulation tests confirmed a persistent therapeutic effect of DBS on chorea. Conclusions GPi DBS may provide sustained chorea improvement in selected HD patients with pharmacologically resistant chorea, with transient benefit in physical aspects of quality of life before progression of behavioral and cognitive disorders. DBS therapy did not improve dystonia or bradykinesia. Further studies including quality of life measures are needed to evaluate the impact of DBS in the long-term outcome of HD.

Resume Introduction Les lesions cavitaires, definies par une degenerescence kystique de la substance blanche, ont ete decrites dans des atteintes hereditaires de la substance blanche (« leucodystrophies »). Cette atteinte est une des caracteristiques du childhood ataxia with central nervous system hypomyelination syndrome (CACH). Elles n’ont pas ete decrites dans la sclerose en plaques. Methodes Nous rapportons une serie retrospective multicentrique de 20 patients atteints de sclerose en plaques et presentant de larges lesions cavitaires. Les donnees cliniques, biologiques, electrophysiologiques, moleculaires et IRM ont ete collectees. Resultats Vingt patients ont ete inclus dans cette etude (6 hommes et 14 femmes). L’âge moyen au debut de la maladie etait de 37,6 ans (extremite 17–58 ans) et la duree moyenne d’evolution de 10 ans (extremite 2–20 ans). La moitie des patients (10/20) etait une forme primaire progressive. Le score EDSS moyen etait de 5,5 (extremite 2–8). Le score MMS moyen etait a 20/30. La recherche genetique de mutation du gene EIF2B codant pour le CACH syndrome a ete recherchee dans 7 cas et a toujours ete negative. Les lesions cavitaires etaient strictement supratentorielles et localisees au sein d’une leucopathie diffuse, avec une predominance posterieure chez la plupart des patients. Conclusion Les formes cavitaires de sclerose en plaques semblent representer un sous-groupe de patients caracterisee par une evolution clinique severe (forme primaire progressive, scores EDSS eleves et atteinte cognitive severe).

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BACKGROUND AND PURPOSE: Multiple sclerosis (MS) patients can present with atypical cavitary lesions mimicking vanishing white matter disease (VWMD). Our objective was to identify brain magnetic resonance imaging (MRI) findings that differentiate these two disorders. METHODS: A cross-sectional study was performed including 14 patients with MS with cavitary lesions and 14 patients with VWMD. Two neuroradiologists retrospectively reviewed the MRI including at least T1-, T2- and fluid-attenuated inversion recovery weighted images. RESULTS: The main differences included ovoid lesions perpendicular to the lateral ventricle, punctate isolated juxtacortical lesions (both 100% in MS versus 0% in VWMD) and symmetrical infratentorial hyperintensities (0% in MS versus 50% in VWMD). Other statistically significant differences included midbrain (79% in MS versus 29% in VWMD) and thalamus lesions (71% vs. 7%) as well as extensive external capsule involvement (29% vs. 86%) and extensive corpus callosum lesions (64% vs. 100%). Cavitary lesions usually had periventricular predominance in MS (36% vs. 0%) whereas they were more frequently anterior in VWMD (0% in MS versus 57% in VWMD). CONCLUSION: Despite many similar MRI findings, our results suggest that a careful analysis of the morphology and the location of the lesions is helpful to differentiate these distinct disorders.

Object Deep brain stimulation (DBS) is used for treating various types of dystonia. Multiple electrodes could be proposed to improve the therapeutic outcome enabling the targeting of specific neuronal populations not reached by the electrical field generated by the initially implanted electrode. The authors address the question of the feasibility and safety of staged multiple lead implantations in the sensorimotor internal globus pallidus (GPi) in primary generalized dystonia (PGD). Criteria for patient selection, surgical technique, target selection, electrical settings management, and clinical outcome are presented. Methods Sixteen patients (8 harbored the DYT1 gene mutation) presented with PGD and were enrolled in this study. Patients underwent clinical assessment using the Burke-Fahn-Marsden Dystonia Rating Scale preoperatively and during follow-up with DBS. Prior to the addition of electrodes, the authors confirmed, by turning off stimulation, that the patient was still benefiting from DBS and that DBS settings adjustment did not provide further improvement. The second target was defined according to the position of the first electrode, to the residual volume within the sensorimotor GPi, and according to residual symptoms. The second surgery followed the same protocol as the first and the new electrode were inserted using the same bur hole as the first electrode. Results The addition of a new pair of electrodes was followed by significant improvement in the whole population (p = 0.005), as well as in the DYT1-negative subgroup (p = 0.012) but not in the DYT1 subgroup (p = not significant). Nevertheless, some patients did not exhibit significant additional benefit. Seven hardware-related complications occurred during the entire follow-up, 3 prior to it, and 4 after the addition of the second pair of electrodes. Conclusions The addition of a second pair of electrodes in the GPi in patients with PGD with suboptimal or decaying benefit following the first surgery seems to be a safe procedure and is not followed by an increase in surgery-related complications. This staged procedure may provide further clinical improvement in patients with PGD in whom DBS effect is initially incomplete or when disease progression occurs over time. The position of the additional electrode within the GPi is determined by the available volume within the posteroventral GPi and by the distribution of the dystonic symptoms that need to be controlled.

Journal of Neuroradiology - In Press.Proof corrected by the author Available online since mardi 1 juillet 2014

The purpose of this study was to retrospectively determine the accuracy of MRI in identification of the morphologic features of median nerve dysfunction after surgical release of the median nerve for carpal tunnel syndrome.Two blinded readers independently evaluated axial 1.5-T MR images for retinacular regrowth, morphologic characteristics of the median nerve, and presence of mass effect, fibrosis, and carpal tunnel decompression. All 47 patients (11 men, 36 women; mean age, 55 years; range, 27-81 years) had undergone open surgical release of the median nerve for carpal tunnel syndrome. Thirty-five patients had electromyographic evidence of recurrent carpal tunnel syndrome. The other 12 patients did not have electrophysiologic evidence of recurrent carpal tunnel syndrome and were the control group.A statistically significant difference between the recurrent carpal tunnel syndrome and control groups was found for fibrosis (p = 0.009), nerve enhancement (p = 0.04), and median nerve width (p = 0.008) and ratio (p = 0.01) at the pisiform level.MRI may be used in association with electromyography for accurate postoperative evaluation of the carpal tunnel.

Resume Complementaire de l’examen clinique, l’electromyographie est devenue necessaire a la prise en charge chirurgicale des syndromes canalaires. Apres un bref rappel physiologique et technique, l’article detaille les causes d’erreur et les bases du diagnostic electromyographique des differents syndromes canalaires au membre superieur.

International audience; A 19-year-old man with no relevant medical history except a high velocity head trauma a few years earlier was admitted in our institution for sudden onset binocular diplopia. Head-CT performed afteer the trauma was depicted as being normal. He complained of chronic headaches for about three years, with recent worsening and painkillers resistance. Clinical examination found binocular ophthalmoplegia with paresis of both abducens nerves. Non-contrast CT-scan and CT angiography of the circle of Willis were normal. Brain MRI acquisition was performed on a 3T magnet (Skyra, Siemens, Germany) with a 32-channel head coil and included a dual-echo Susceptibility Weighted Imaging (SWI), with the following parameters: TE1/TE2 = 20/40 ms, TR = 47 ms, GRAPPA = 2, voxel size = 1 × 1 × 1.5 mm, 88 slices). Conventional sequences showed a subtle distension of the perioptic subarachnoid spaces on coronal T2 sequence (not shown), and a linear loss of signal on pial surface of the lefte lateral fissure (Fig. 1a). Susceptibility-weighted imaging was performed in order to quantify the related susceptibility effeect, through the use of quantitative susceptibility mapping. Marked pial signal loss on T2-GRE and susceptibility-weighted imaging (SWI) was observed at the level of the brainstem, lefte lateral fissure, cerebellar folia (Fig. 1b-f). Phase image was retrieved from SWI acquisition and underwent laplacian unwrapping as well as background field removal using regularization-enabled SHARP algorithm. Finally, total variation using split Bregman [1] method enabled images conversion to quantitative susceptibility maps (QSM). Reported susceptibility values were standardized according to the observed cerebrospinal fluid susceptibility. In normal appearing gray matteer (Fig. 1g-h), the value was 0.009 ± 0.1 ppm, while in cortico-pial affeected areas, measured susceptibility was 0.24 ± 0.1 ppm (Fig. 1f), suggesting a paramagnetic effeect. Discrete atrophy of the superior cerebellar vermis was also noted (Fig. 1k). T2-weighted images of the spinal cord showed a low signal lining on the spinal cord, suggesting hemosiderin deposit (Fig. 1i-j). No acute subarachnoid bleeding was present. Neither MR angiography of the intracranial vessels nor spinal MRI revealed any vascular malformation. No intra-axial hemorrhage was found. Fundoscopic examination revealed bilateral papillary subedema that was confirmed by the fluorescein angiogra-phy showing late papillary dye leakage. Lumbar puncture was finally performed and showed severe intracranial hypertension (57 cmH 2 O, normal range 7-15 cmH 2 0). Neither erythrocytes nor xantochromia were present. The diagnosis of superficial siderosis of the central nervous system was made based on the symptoms that were supported by radiological findings. Discussion

Background and purpose Proximal large vessel occlusion (LVO) is present in up to 30% of minor strokes. There is no proven effectiveness of MT in the subgroup of minor stroke with LVO in the anterior circulation and data about mechanical thrombectomy (MT) in this subgroup of patients are sparse, with optimal management of these patients being yet not definitely addressed. The purpose of this case-control study was to evaluate MT in patients suffering from acute ischemic stroke (AIS) and LVO in the anterior circulation, presenting with minor to mild stroke symptoms (NIHSS Material and methods Case-control study involving 4 comprehensive stroke centers, having two approaches regarding management of minor and mild AIS patients harboring LVO in the anterior circulation. An intention-to-treat analysis was conducted. The primary end point was the rate of excellent outcome defined as the achievement of a modified Rankin Scale score of 0–1 at 3 months. Results In total, 301 patients were included, 170 with MT associated to best medical management (BMM, case group) and 131 with BMM alone as first line treatment (control group). Patients treated with MT were younger, more often received intravenous thrombolysis, and had shorter time to imaging. Twenty-four patients (18.3%) belonging to the medical group had rescue MT due to neurologic worsening. Overall, excellent outcome was achieved in 64.5% of patients, with no difference between the two groups. Stratified analysis according to key subgroups did not find heterogeneity in the treatment effect size. Conclusion Patients having underwent MT or BMM achieve excellent and favorable functional outcome at 3 months in similar proportions. However, baseline characteristics were different between the 2 groups, highlighting the urgent need for randomized clinical trials in this subset of patients.

International audience; Etiologic diagnosis of adulthood leukodystrophy is challenging in neurologic practice. We describe here the clinico-radiological features of a novel autosomal dominant leukodystrophy in a single family. Clinical and MRI features were recorded in a three generation family. Exome sequencing was performed in two affected relatives and one healthy member. Four total relatives (3 women and 1 man, mean age at onset: 45, range 32-59) were followed: 2 for migraine and 2 for cognitive loss. MRI features were homogeneous in the four affected relatives: extensive and symmetrical white matter hyperintensities on T2-weighted images, with a posterior predominance, involvement of the middle cerebellar peduncles, corpus callosum and the posterior limb of the internal capsules. An extensive metabolic screening was negative. In addition, sequencing of pathogenic genes involved in dominant leukodystrophies (NOTCH3, LMNB1, GFAP, CSF1R) was negative. No mutation has been identified yet with exome sequencing. This report is peculiar because of dominant inheritance, adult onset, highly homogeneous white matter hyperintensities on T2-weighted MR images, predominant in the middle cerebellar peduncles and posterior part of internal capsule and absence of mutation of the genes involved in dominant leukodystrophies.

Discussion The spectrum of COL4A1-related disorders includes perinatal cerebral hemorrhage and porencephaly [2] , cerebral small vessel disease with retinal arteriolar tortuosity and leukoencephalopathy [3] , HANAC syndrome (Hereditary Angiopathy, Nephropathy, Aneurysms, and Muscle Cramps) and other eye abnormalities, including the Axenfeld-Rieger anomaly and cataract. A frequent white matter involvement is reported [3, 4] . Interestingly, some HANAC patients may have a normal brain MRI. However, except for one patient who suffered from migraine, these patients were free from neurologic symptoms [5, 6] . Recently, Weng et al. [7] reported COL4A1 variants in 2/96 patients with sporadic ICH. Their phenotype was not described in Introduction Ten to fifteen percent of strokes are related to an intracerebral hemorrhage (ICH). Etiologies are mainly arterial hypertension, amyloid deposition, and vascular malformations. In cases of a young age without vascular risk factors and vascular malformations, a genetic cause has to be suspected, especially a mutation in the gene coding for type IV collagen alpha 1 (COL4A1) [1] . We report a COL4A1 gene mutation in a 45-year-old woman with an isolated ICH in the absence of any other MRI abnormality, which further extends the clinical spectrum of COL4A1 mutations.

Background and Purpose Approximately 30% of strokes are cryptogenic despite an exhaustive in-hospital work-up. Analysis of clot composition following endovascular treatment could provide insight into stroke etiology. T-cells already have been shown to be a major component of vulnerable atherosclerotic carotid lesions. We therefore hypothesize that T-cell content in intracranial thrombi may also be a biomarker of atherothrombotic origin. Materials and Methods We histopathologically investigated 54 consecutive thrombi retrieved after mechanical thrombectomy in acute stroke patients. First, thrombi were classified as fibrin-dominant, erythrocyte-dominant or mixed pattern. We then performed quantitative analysis of CD3+ cells on immunohistochemically-stained thrombi and compared T-cell content between “atherothrombotic”, “cardioembolism” and “other causes” stroke subtypes. Results Fourteen (26%) thrombi were defined as fibrin-dominant, 15 (28%) as erythrocyte-dominant, 25 (46%) as mixed. The stroke cause was defined as “atherothrombotic” in 10 (18.5%), “cardioembolism” in 25 (46.3%), and “other causes” in 19 (35.2%). Number of T-cells was significantly higher in thrombi from the “atherothrombotic” group (53.60 ± 28.78) than in the other causes (21.77 ± 18.31; p

Objectifs Evaluer les resultats de l’IRM et de l’EMG pour syndrome du canal carpien recidivant apres neurolyse chirurgicale. Materiels et methodes Etude retrospective de 62 IRM et EMG apres neurolyse pour syndrome du canal carpien. Tous les patients presentant des anomalies cliniques post-operatoires evoquant une recidive ont ete explores par EMG et IRM post-operatoires. Toutes les interventions chirurgicales et les EMG ont ete realisees par le meme operateur. Les IRM (sequences axiales en T1 sans et avec IV de Gadolinium et T2) ont ete relues par deux radiologues, selon une grille analysant le retinaculum, le nerf median, la fibrose, les tendons et les gaines, les syndromes de masse ou variation anatomiques. Les constatations IRM sont comparees a l’evolution des EMG avant et apres l’intervention chirurgicale. Onze IRM, avec resolution des symptomes cliniques et electriques post-operatoires, forment le groupe temoin. Resultats L’IRM a detecte des anomalies du retinaculum et du nerf, des tenosynovites, des kystes qui expliquaient le non retour a la normale des donnees electriques. Conclusion L’IRM est utile, chez les patients ayant beneficie d’une liberation chirurgicale pour syndrome du canal carpien, en l’absence d’amelioration des donnees electriques post-operatoires.