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Introduction: Relevance of fetal brain magnetic resonance imaging (MRI) in cases of cleft lip and/or palate (CL/P) is still discussed to date. The aim of our study was to review the contribution of fetal brain MRI for detecting cerebral anomalies in cases of CL/P comparing antenatal data with neonatal outcomes. Methods: A retrospective multicenter study was conducted from January 2010 to October 2020 in two multidisciplinary prenatal diagnosis centers among women with a fetal ultrasound (US) diagnosis of CL/P. Prenatal imaging and genetic analysis data were collected, as well as postnatal data, including outcomes of children who had an abnormal prenatal MRI. Results: Among the 202 fetuses with a US diagnosis of CL/P, 96 underwent US and fetal brain MRI. 19 brain MRIs were found to be abnormal: 14 (73.7%) involved CL/P associated with other US abnormalities and five (26.3%) involved isolated clefts, of which four were cleft lip and alveolus and secondary palate (CLP). MRI identified severe abnormalities that changed the prognoses of 3 cases of clefts associated with other US abnormalities. In contrast, MRI found only minor abnormalities for the five isolated clefts, with no postnatal disorders found in these children. Conclusion: Fetal brain MRI should be proposed in cases of clefts associated with other anomalies or if US examination is limited by local conditions. MRI could also be discussed in cases of isolated CLP but should not be performed in cases of isolated cleft lip.

BackgroundBalloon-assisted mechanical angioplasty for cerebral vasospasm following aneurysmal subarachnoid hemorrhage (aSAH) has a number of limitations, including transient occlusion of the spastic blood vessel. Comaneci is an FDA-approved device for temporary coil embolization assistance which has recently also been approved for the treatment of distal symptomatic refractory vasospasm. We aimed to report the feasibility, efficacy and safety of our experience with Comaneci angioplasty for refractory distal vasospasm (up to the second segment of the cerebral arteries) following aSAH.MethodsThis is a retrospective analysis of a prospective series of 18 patients included between April 2019 and June 2021 with aSAH and symptomatic vasospasm refractory to medical therapy, who were treated using Comaneci-17-asssisted mechanical distal angioplasty. Immediate angiographic results, procedure-related complications, and clinical outcomes were assessed. Inter-rater reliability of the scores was determined using the intraclass correlation coefficient.ResultsComaneci-assisted distal angioplasty was performed in 18 patients, corresponding to 31 target arteries. All distal anterior segments were easily accessible with the Comaneci-17 device. Vasospasm improvement after Comaneci mechanical angioplasty was seen in 22 distal arteries (71%) (weighted Cohen’s kappa (κw) 0.73, 95% CI 0.69 to 0.93). Vasospasm recurrence occurred in three patients (16.67%) and delayed cerebral infarction in three patients (16.67%), with a mean±SD delay between onset of symptoms and imaging follow-up (MRI/CT) of 32.61±8.93 days (κw0.98, 95% CI 0.88 to 1).ConclusionThis initial experience suggests that distal mechanical angioplasty performed with the Comaneci-17 device for refractory vasospasm following aSAH seems to be safe, with good feasibility and efficacy.

Patients with drug-resistant essential tremor (ET) may undergo Gamma Knife stereotactic radiosurgical thalamotomy (SRS-T), where the ventro-intermediate nucleus of the thalamus (Vim) is lesioned by focused beams of gamma radiations to induce clinical improvement. Here, we studied SRS-T impacts on left Vim dynamic functional connectivity (dFC, n = 23 ET patients scanned before and 1 year after intervention), and on surface-based morphometric brain features (n = 34 patients, including those from dFC analysis). In matched healthy controls (HCs), three dFC states were extracted from resting-state functional MRI data. In ET patients, state 1 spatial stability increased upon SRS-T (p = 0.0041). Lower pre-intervention spatial variability in state 2 expression, and more frequent expression of state 3 over state 1, correlated with greater clinical recovery (p = 0.015 and p = 0.008, respectively). ET morphometric profiles showed significantly lower similarity to HCs in 13 regions upon SRS-T (p ≤ 0.02), and a joint analysis revealed that before thalamotomy, morphometric similarity and states 2/3 mean spatial similarity to HCs were anticorrelated, a relationship that disappeared upon SRS-T (p

BACKGROUND/OBJECTIVE Aneurysmal subarachnoid hemorrhage (aSAH) is a life-threatening event with major complications such as delayed cerebral ischemia (DCI) or acute hydrocephalus and poor neurological outcome. DCI occurs most frequently 7 days after aSAH and can last for a prolonged period. The ability to predict these complications would allow the neuro-intensivist to identify patients at risk and select the most appropriate unit for hospitalization. To determine the most predictive radiological scales in grading subarachnoid or ventricular hemorrhage or both for functional outcome at 3 months in a large aSAH population, we conducted a single centre retrospective study in a neurocritical care unit. METHODS A 3-year single-centre retrospective cohort study of 230 patients hospitalized for aSAH was analysed. Initial computed tomography (CT) scans in patients hospitalized for aSAH were blindly assessed using eight grading systems: the Fisher grade, modified Fisher grade, Barrow Neurological Institute scale, Hijdra scale, Intraventricular Hemorrhage (IVH) score, Graeb score, and LeRoux score. We evaluated and compared these radiological scales for the early prediction of DCI, acute hydrocephalus, and poor neurological outcome at 3 months. RESULTS Of 200 patients with aSAH who survived to day 7 and were included for DCI analysis, 39% cases were complicated with DCI. The Hijdra scale was the best predictor for DCI, with a receiver operating characteristic area under the curve (ROCAUC) of 0.80 (95% confidence interval [CI], 0.74–0.85) compared to other scales (Fisher grade 0.52 (95% CI, 0.47–0.59) modified Fisher grade: 0.67 (95% CI, 0.60–0.73); Claassen Scale: 0.66 (95% CI, 0.59–0.72) and BNI Scale: 0.63 (95% CI, 0.56–0.70)). The IVH score was the most effective grading system for predicting acute hydrocephalus, with a ROCAUC of 0.85 (95% CI, 0.79–0.89). In multivariate analysis, the Hijdra scale was the best predictor of the occurrence of DCI (hazard ratio, 1.18; 95% CI, 1.10–1.25). CONCLUSIONS Although these results have yet to be prospectively confirmed, our findings suggest that the Hijdra scale may be a good predictor of DCI and could be useful in daily clinical practice.

International audience; Background and purposeExtent and dynamic of neurodegeneration in progressive multiple sclerosis (MS) might be reflected by global and regional brain perfusion, an outcome at the intercept between structure and function. Here, we provide a first insight into the evolution of brain perfusion and its association with disability in primary progressive MS (PPMS) over several years.MethodsSeventy-seven persons with PPMS were followed over up to 5 years. Visits included a 3-T magnetic resonance imaging with pulsed arterial spin labelling perfusion, the Timed 25-Foot Walk, 9-Hole Peg Test (NHPT), Symbol Digit Modalities Test (SDMT), and Expanded Disability Status Scale (EDSS). We extracted regional cerebral blood flow surrogates and compared them to 11 controls. Analyses focused on cortical and deep grey matter, the change over time, and associations with disability on the regional and global levels.ResultsBaseline brain perfusion of patients and controls was comparable for the cortex (p = 0.716) and deep grey matter (p = 0.095). EDSS disability increased mildly (p = 0.023), whereas brain perfusion decreased during follow-up (p < 0.001) and with disease duration (p = 0.009). Lower global perfusion correlated with higher disability as indicated by EDSS, NHPT, and Timed 25-Foot Walk (p < 0.001). The motor task NHPT showed associations with 20 grey matter regions. In contrast, better SDMT performance correlated with lower perfusion (p < 0.001) in seven predominantly frontal regions, indicating a functional maladaptation.ConclusionsDecreasing perfusion indicates a putative association with MS disease mechanisms such as neurodegeneration, reduced metabolism, and loss of resilience. A low alteration rate limits its use in clinical practice, but regional association patterns might provide a snapshot of adaptive and maladaptive functional reorganization.

Background and Purpose: Pediatric nontraumatic intracerebral hemorrhage accounts for half of stroke in children. Early diagnostic of the causative underlying lesion is the first step toward prevention of hemorrhagic recurrence. We aimed to investigate the performance of arterial spin labeling sequence (ASL) in the acute phase etiological workup for the detection of an arteriovenous shunt (AVS: including malformation and fistula), the most frequent cause of pediatric nontraumatic intracerebral hemorrhage. Methods: Children with a pediatric nontraumatic intracerebral hemorrhage between 2011 and 2019 enrolled in a prospective registry were retrospectively included if they had undergone ASL-magnetic resonance imaging before any etiological treatment. ASL sequences were reviewed using cerebral blood flow maps by 2 raters for the presence of an AVS. The diagnostic performance of ASL was compared with admission computed tomography angiography, other magnetic resonance imaging sequences including contrast-enhanced sequences and subsequent digital subtraction angiography. Results: A total of 121 patients with pediatric nontraumatic intracerebral hemorrhage were included (median age, 9.9 [interquartile range, 5.8–13]; male sex 48.8%) of whom 76 (63%) had a final diagnosis of AVS. Using digital subtraction angiography as an intermediate reference, visual ASL inspection had a sensitivity and a specificity of, respectively, 95.9% (95% CI, 88.5%–99.1%) and 79.0% (95% CI, 54.4%–94.0%). ASL had a sensitivity, specificity, and accuracy of 90.2%, 97.2%, and 92.5%, respectively for the detection of the presence of an AVS, with near perfect interrater agreement (κ=0.963 [95% CI, 0.912–1.0]). The performance of ASL alone was higher than that of other magnetic resonance imaging sequences, individually or combined, and higher than that of computed tomography angiography. Conclusions: ASL has strong diagnostic performance for the detection of AVS in the initial workup of intracerebral hemorrhage in children. If our findings are confirmed in other settings, ASL may be a helpful diagnostic imaging modality for patients with pediatric nontraumatic intracerebral hemorrhage. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifiers: 3618210420, 2217698.

International audience

Essential tremor (ET) is the most common movement disorder. Deep brain stimulation is the current gold standard for drug-resistant tremor, followed by radiofrequency lesioning. Stereotactic radiosurgery by Gamma Knife (GK) is considered as a minimally invasive alternative. The majority of procedures aim at the same target, thalamic ventro-intermediate nucleus (Vim). The primary aim is to assess the clinical response in relationship to neuroimaging changes, both at structural and functional level. All GK treatments are uniformly performed in our center using Guiot’s targeting and a radiation dose of 130 Gy. MR neuroimaging protocol includes structural imaging (T1-weighted and diffusion-weighted imaging [DWI]), resting-state functional MRI, and 18F-fluorodeoxyglucose-positron emission tomography. Neuroimaging changes are studied both at the level of the cerebello-thalamo-cortical tract (using the prior hypothesis based upon Vim’s circuitry: motor cortex, ipsilateral Vim, and contralateral cerebellar dentate nucleus) and also at global brain level (no prior hypothesis). This protocol aims at using modern neuroimaging techniques for studying Vim GK radiobiology for tremor, in relationship to clinical effects, particularly in ET patients. In perspective, using such an approach, patient selection could be based upon a specific brain connectome profile.

Essential tremor (ET) is the most common movement disorder. Its neural underpinnings remain unclear. Here, we quantified structural covariance between cortical thickness (CT), surface area (SA), and mean curvature (MC) estimates in patients with ET before and 1 year after ventro-intermediate nucleus stereotactic radiosurgical thalamotomy, and contrasted the observed patterns with those from matched healthy controls. For SA, complex rearrangements within a network of motion-related brain areas characterized patients with ET. This was complemented by MC alterations revolving around the left middle temporal cortex and the disappearance of positive-valued covariance across both modalities in the right fusiform gyrus. Recovery following thalamotomy involved MC readjustments in frontal brain centers, the amygdala, and the insula, capturing nonmotor characteristics of the disease. The appearance of negative-valued CT covariance between the left parahippocampal gyrus and hippocampus was another recovery mechanism involving high-level visual areas. This was complemented by the appearance of negative-valued CT/MC covariance, and positive-valued SA/MC covariance, in the right inferior temporal cortex and bilateral fusiform gyrus. Our results demonstrate that different morphometric properties provide complementary information to understand ET, and that their statistical cross-dependences are also valuable. They pinpoint several anatomical features of the disease and highlight routes of recovery following thalamotomy. Author Summary Doubts remain regarding the anatomical alterations underlying essential tremor, partly owing to heterogeneity in symptoms' severity and response to medication. Here, we studied drug-resistant patients clinically assessed and imaged before as well as 1 year after stereotactic radiosurgical thalamotomy, which significantly lowered tremor intensity. We extracted morphometric estimates of volume (subcortex and cerebellum), cortical thickness, surface area, and mean curvature (cortex), and quantified cross-regional statistical dependences across subjects (i.e., structural covariance or SC) for each measure, as well as cross-measure relationships for each region. Compared to matched healthy controls, patients showed altered surface area structural covariance within motion-related areas. Thalamotomy modulated mean curvature SC in frontal and subcortical centers. In both comparisons, SC and cross-measure relationship differences were also observed in visual areas.

Riassunto L’idrocefalo e una patologia frequente, dovuta a una causa malformativa, emorragica o infettiva nella maggior parte dei casi nei lattanti e nei neonati, di origine tumorale nel bambino grande. L’ecografia transfontanellare (ETF), la TC cerebrale e la risonanza magnetica (RM) sono i principali metodi di diagnostica per immagini. L’ETF e l’esame della diagnosi di certezza nel neonato. La TC cerebrale consente la diagnosi di certezza al di fuori del periodo neonatale, soprattutto in presenza di una sintomatologia acuta. La RM e l’esame di elezione per determinare la strategia terapeutica a partire dalle informazioni anatomiche e funzionali ottenute. La cinetica del liquido cerebrospinale (LCS) e complessa. Il LCS e prodotto soprattutto dall’epitelio dei plessi coroidei nelle cavita ventricolari. Il riassorbimento avviene essenzialmente attraverso le granulazioni o villosita aracnoidee (dette di Pacchioni). Il LCS si drena nei linfatici situati nei tessuti che circondano la lamina cribrosa dell’etmoide, ma anche nelle guaine dei nervi cranici e dei nervi spinali. Pertanto, tutte le anomalie della produzione e gli ostacoli al flusso o al riassorbimento del LCS possono essere responsabili di un idrocefalo. A questo flusso continuo si aggiunge un movimento di “avanti e indietro”, pulsatile, legato al battito cardiaco, responsabile di una forza sistolica centripeta che provoca lo spostamento del LCS. Ogni squilibrio in questo movimento di “avanti e indietro” puo essere responsabile di un idrocefalo.

International audience; Pituitary stalk interruption syndrome (PSIS) is a congenital pituitary anatomical defect. This syndrome is an antenatal developmental defect belonging to the holoprosencephaly phenotype spectrum. It is heterogeneous regarding clinical, biological and radiological presentation and is characterized by the following triad: thin (

Objective According to A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA) conservative treatment seems to be superior to any intervention for unruptured brain arteriovenous malformations (AVM). This study aims to evaluate safety and efficacy of upfront and repeated Gamma-Knife Radiosurgery (GKRS) in patients harbouring small AVM fulfilling the inclusion criteria of ARUBA. Methods A retrospective study was conducted to evaluate outcomes of unruptured naive brain AVM with a volume ⩽ 5cc eligible to ARUBA treated by GKRS with at least 3 years of follow-up. Results From 1992 to 2014, 249 patients were fulfilling the inclusion criteria of this study. The median age was 36 years [18-78]. The median treated volume of the nidus was 1.3 cc [0.4-5] and 63% of the AVM were in eloquent areas. Radiosurgery Based AVM score was 1-1.8 (76%), the Spetzler-Martin grade was II-III (73%), and the Virginia Radiosurgery AVM scale was ≤1point (75%). The overall AVM obliteration rate was 77.1% after up to 3 GKRS sessions. The median dose at the margin was 24 Gy [15-25] and the median follow-up was 45 months [36–205]. Eight patients (3.2%) experienced hemorrhage after GKRS, corresponding to a post-GKRS hemorrhage annual rate of 1.03%. Permanent symptomatic radio-induced changes rate was 2% (4 increased seizures, 1 neurological deficit). Conclusion The very low toxicity rate and the high occlusion rate are preaching in favor of upfront and repeated GKRS for unruptured small AVM (≤5cc).

Contexte L’angioplastie par ballonnet pour traiter les vasospasmes cerebraux a la suite d’une hemorragie sous-arachnoidienne presente des limites, notamment l’occlusion transitoire du vaisseau sanguin cible et d’eventuelles lesions endotheliales. Certaines etudes suggerent que l’angioplastie distale par ballonnet est sure et diminue le risque d’infarctus cerebral retarde et la recurrence du vasospasme. Comaneci (Rapid Medical, Yokneam, Israel) est un dispositif approuve par la FDA pour l’assistance temporaire a l’embolisation d’anevrisme par coiling et, depuis peu, pour le traitement des vasospasmes symptomatiques medicalement refractaires. Objectif L’angioplastie distale par Comaneci (au-dela du premier segment des arteres cerebrales) est utilisee dans notre centre depuis avril 2019. Notre objectif est de rapporter l’efficacite et la morbi-mortalite de l’angioplastie mecanique par Comaneci dans le traitement des vasospasmes distaux refractaires au traitement medical suite a une hemorragie sous-arachnoidienne sur rupture d’anevrisme. Methodes Treize patients ont ete inclus de facon prospective entre avril 2019 et janvier 2021 et ont beneficie d’une angioplastie mecanique par Comaneci 17 de leur vasospasmes distaux symptomatiques refractaires au traitement medicamenteux suite a une rupture d’anevrisme. Les resultats angiographiques immediats, les complications liees a la procedure et les resultats cliniques ont ete evalues. Resultats Chaque segment distal anterieur (n = 27) etait facilement accessible avec le dispositif Comaneci 17. Il a permis d’ameliorer le diametre des vaisseaux sur l’evaluation angiographiques pour 70 % des vaisseaux distaux (n = 19). Un deces est survenu par suite d’une rupture d’artere due a l’utilisation d’un dispositif d’angioplastie distale par ballonnet (refractaire a l’angioplastie mecanique par Comaneci 17). Aucune autre complication liee a l’angioplastie mecanique n’a ete relevee. Deux patients (15 %) ont presente une recidive de vasospasme et une ischemie cerebrale retardee est survenue chez deux patients (15 %). Conclusion Cette etude suggere la faisabilite et l’efficacite du Comaneci 17 dans le traitement du vasospasme cerebral distal medicalement refractaire avec l’avantage de ne pas necessiter d’occlusion transitoire du vaisseau cible comme c’est le cas avec une angioplastie par ballonnet.

Introduction Apprecier la realite histo-architecturale in vivo des noyaux gris centraux notamment des thalami est un veritable challenge en imagerie. La definition precise de cette anatomie est cependant un prerequis necessaire pour mesurer avec precision des alterations structurales parenchymateuses ou realiser le ciblage stereotaxique en neurochirurgie [1] . Materiel et methodes Des images tridimensionnelles MP2RAGE (Magnetization Prepared with two Rapid Acquisition Gradient Echoes sequence) [2] ont ete acquises en 0,6 mm isotropique a 7 T chez 60 volontaires sains. Une methode de normalisation « group-wize » [3] a permis de creer un template T1 haute resolution (0,5 mm3) a partir de 30 des 60 sujets ( Fig. 1 ). Deux neuroradiologues (R1 et R2) ont realise une segmentation de 24 regions de substance gris profonde et de douze noyaux thalamiques dans chaque hemisphere, selon l’atlas de Morel [4] . Un atlas moyenne (7TAMIbrainDGN) a partir des deux segmentations a ete valide par un troisieme neuroradiologue ( Fig. 2 ). Les structures correspondantes ont egalement ete extraites a partir de deux autres atlas numeriques (e-Morel et CIT168). Les valeurs quantitatives moyennes absolues des temps de relaxation T1 ont ete ensuite extraites apres correction du B1+. Un score de Dice a ete calcule pour mesurer la concordance inter-observateur et la variabilite inter-atlas. Resultats Les scores de Dice entre les deux neuroradiologues, entre le R1 et 7TAMIbrainDGN et entre R2 et 7TAMIbrainDGN etaient respectivement de 0,74 ± 0,13, 0,87 ± 0,07, 0,86 ± 0,08. Les Dice entre 7TAMIbrainDGN et l’atlas e-Morel etaient de 0,53 ± 0,16 et de 0,80 ± 0,07 entre 7TAMIbrainDGN et CIT168. Les temps de relaxation T1 variaient de 1322 ms a 2003 ms. Conclusion Cette etude a permis de creer un atlas des noyaux gris centraux et des thalamis a 7 T et de valider un processus de segmentation automatise avec extraction des temps de relaxation T1.

Dynamic susceptibility contrast (DSC) and arterial spin labeling (ASL) perfusion MRI are applied in pediatric brain tumor grading, but their value for clinical daily practice remains unclear. We explored the ability of ASL and DSC to distinguish low- and high-grade lesions, in an unselected cohort of pediatric cerebral tumors. We retrospectively compared standard perfusion outcomes including blood volume, blood flow, and time parameters from DSC and ASL at 1.5T or 3T MRI scanners of 46 treatment-naive patients by drawing ROI via consensus by two neuroradiologists on the solid portions of every tumor. The discriminant abilities of perfusion parameters were evaluated by receiver operating characteristic (ROC) over the entire cohort and depending on the tumor location and the magnetic field. ASL and DSC parameters showed overall low to moderate performances to distinguish low- and high-grade tumors (area under the curve: between 0.548 and 0.697). Discriminant abilities were better for tumors located supratentorially (AUC between 0.777 and 0.810) than infratentorially, where none of the metrics reached significance. We observed a better differentiation between low- and high-grade cancers at 3T than at 1.5-T. For infratentorial tumors, time parameters from DSC performed better than the commonly used metrics (AUC ≥ 0.8). DSC and ASL show moderate abilities to distinguish low- and high-grade brain tumors in an unselected cohort. Absolute value of K2, TMAX, tMIP, and normalized value of TMAX of the DSC appear as an alternative to conventional parameters for infratentorial tumors. Three Tesla evaluation should be favored over 1.5-Tesla.

This retrospective multi-center case series study illustrates the characteristics of optic pathways enlargement (OPE) in patients with typical Morning Glory Disc Anomaly. Our findings suggest OPE might be considered a malformation rather than a neoplasm.

Developmental dyslexia (DD) and developmental coordination disorder (DCD) are distinct diagnostic disorders. However, they also frequently co-occur and may share a common etiology. It was proposed conceptually a neural network framework that explains differences and commonalities between DD and DCD through impairments of distinct or intertwined cortico-subcortical connectivity pathways. The present study addressed this issue by exploring intrinsic cortico-striatal and cortico-cerebellar functional connectivity in a large (n = 136) resting-state fMRI cohort study of 8–12-year-old children with typical development and with DD and/or DCD. We delineated a set of cortico-subcortical functional circuits believed to be associated with the brain’s main functions (visual, somatomotor, dorsal attention, ventral attention, limbic, frontoparietal control, and default-mode). Next, we assessed, using general linear and multiple kernel models, whether and which circuits distinguished between the groups. Findings revealed that somatomotor cortico-cerebellar and frontoparietal cortico-striatal circuits are affected in the presence of DCD, including abnormalities in cortico-cerebellar connections targeting motor-related regions and cortico-striatal connections mapping onto posterior parietal cortex. Thus, DCD but not DD may be considered as an impairment of cortico-subcortical functional circuits.

Background: Aneurysmal subarachnoid haemorrhage (aSAH) is a life-threatening event with major complications such as delayed cerebral infarction (DCI) or acute hydrocephalus and poor neurological outcome. DCI occurs most frequently 7 days after aSAH and can last for a prolonged period. The ability to predict these complications would allow the neuro-intensivist to identify patients at risk and select the most appropriate unit for hospitalization. Methods: A 3-year single-centre retrospective cohort study was conducted in our neuroscience critical care unit. Initial computed tomography (CT) scans in patients hospitalized for aSAH were blindly assessed using eight grading systems: the Fisher scale, modified Fisher scale, Barrow Neurological Institute scale, Hijdra scale, Intraventricular Haemorrhage (IVH) score, Graeb score, and LeRoux score. We evaluated and compared these radiological scales for the early prediction of DCI, acute hydrocephalus, and poor neurological outcome at 3 months. Results: Of 200 patients with aSAH who survived to day 7 and were included for DCI analysis, 39% cases were complicated with DCI. The Hijdra scale was the best predictor for DCI, with a receiver operating characteristic area under the curve (ROCAUC) of 0.80 (95% confidence interval [CI], 0.74–0.85). The ideal cut-off score for all patients was 20/42, with a sensitivity of 85% (95% CI, 75%–94%) and specificity of 63% (95% CI, 54%–71%). The IVH score was the most effective grading system for predicting acute hydrocephalus, with a ROC AUC of 0.85 (95% CI, 0.79–0.89). In multivariate analysis, the Hijdra scale was the only independent predictor of the occurrence of DCI (hazard ratio, 1.18; 95% CI, 1.10–1.27). Conclusions: Although these results have yet to be prospectively confirmed, our findings suggest that the Hijdra scale may be a good predictor of DCI and could be useful in daily clinical practice.

Background Essential tremor (ET) is a common movement disorder. Resting-state functional magnetic resonance imaging is a noninvasive neuroimaging method acquired in absence of task. Objective Our study aimed to correlate pretherapeutic ventrolateral thalamus functional connectivity (FC) with clinical results 1 year after stereotactic radiosurgical thalamotomy (SRS-T) for drug-resistant ET. Data from 12 healthy control individuals were additionally included. Methods Resting state was acquired for 17 consecutive (right-handed) patients, before and 1 year after left unilateral SRS-T. Standard tremor scores were evaluated pretherapeutically and 1 year after SRS-T. Tremor network was investigated using region of interest, left ventrolateral ventral (VLV) cluster, obtained from pretherapeutic diffusion magnetic resonance imaging. Seed-based FC was obtained as correlations between the time courses of the VLV and that of every other voxel. The seed-connectivity maps were obtained pretherapeutically and correlated across all patients with clinical outcome 1 year after SRS-T. One-year magnetic resonance signature volume was always located inside VLV and did not correlate with reported seed-FC measures (P > 0.05). Results We report statistically significant correlations between pretherapeutic VLV FC with clinical outcome for 1) right visual association area (Brodmann area, BA19) predicting 1 year activities of daily living decrease (Punc = 0.02); 2) left fusiform gyrus (BA37) predicting 1 year head tremor score improvement (Punc = 0.04); and 3) posterior cingulate (left BA23, Puncor = 0.009), lateral temporal cortex (right BA21, Punc = 0.02) predicting time to tremor arrest. Conclusions Our results suggest that pretherapeutic resting-state seed-FC of left VLV predicts tremor arrest after SRS-T for ET. Visual areas are identified as the main regions in this correlation.

OBJECTIVE: To correlate pretherapeutic resting-state functional magnetic resonance imaging (rs-fMRI) measures with pretherapeutic head tremor presence and/or further improvement 1 year after stereotactic radiosurgical thalamotomy (SRS-T) for essential tremor (ET). METHODS: We prospectively collected head tremor scores (range, 0-3) and rs-fMRI data for a cohort of 17 consecutive ET patients in pretherapeutic and 1 year after SRS-T states. We additionally acquired rs-fMRI data for a healthy control (HC) group (n~= 12). Group-level independent component analysis (n~= 17 for pretherapeutic rs-fMRI) was applied to decompose neuroimaging data into 20 large-scale brain networks using a standard approach. Through spatial regression, we projected 1 year after SRS-T and HC rs-fMRI time points, on the same 20 brain networks. RESULTS: Pretherapeutic interconnectivity (IC) strength between the network including bilateral thalamus and limbic system with left supplementary motor area predicted head tremor improvement at 1 year after SRS-T (family-wise corrected P < 0.001, cluster size Kc~= 146). For the statistically significant cluster, IC strength was strongest in HCs (mean, 4.6; median, 3.8) compared with pre- (mean, 0.1; median, 0.2) or posttherapeutic (mean,~-0.2; median, 0.09) states. CONCLUSIONS: Baseline measures of IC between bilateral thalamus and limbic system with left supplementary motor area may predict head tremor arrest after thalamotomy. However, procedures such as SRS-T, for this particular clinical feature, do not align patients to HCs in terms of functional brain connectivity. We postulate that supplementary motor area is modulating head tremor appearance, by abnormal connectivity with the thalamolimbic system.

Resume La decouverte d’un perimetre cephalique (PC) inferieur au 3epercentile (p), au meme titre que le femur court ou l’hyperclarte de nuque a caryotype normal, demeure l’une des situations les plus difficiles pour le praticien en diagnostic prenatal. La microcephalie constitue une porte d’entree d’eventuelles pathologies cerebrales diverses; cependant l’objectif principal est d’identifier les situations graves accessibles en periode prenatale. Une mesure standardisee du PC, l’utilisation d’outils et de courbes de references adaptes, un suivi longitudinal des biometries cephaliques dans les situations a risques, et une analyse systematisee du systeme nerveux central peuvent permettre d’augmenter les performances diagnostiques de l’echographie souvent decevantes pour la microcephalie. La distinction precoce entre microcephalie associee ou isolee permet d’orienter rapidement la conduite a tenir et le conseil prenatal. L’IRM fœtale et la consultation de genetique sont fondamentaux dans ce contexte, ils permettent de preciser, au mieux, le diagnostic etiologique et d’apporter une aide au neuropediatre dans l’etablissement d’un pronostic souvent incertain. La recente augmentation des cas de microcephalie concomitante a l’epidemie du virus ZIKA est un argument supplementaire pour ameliorer nos pratiques et l’apprehension au quotidien des PC

Matrix metalloproteases MMP2 and MMP9 are involved in cancer angiogenesis and invasion. We recently demonstrated that plasma MMP2 and MMP9 levels could both predict response to bevacizumab in patients with recurrent high-grade glioma (HGG). We examined the potential relationship between MMP2/MMP9 plasma levels and glioma imaging characteristics. In this retrospective, monocentric study, MRI before bevacizumab administration for HGG patients was independently analyzed for contrast enhancement (CE) and FLAIR sequences. Contemporary MMP2 and MMP9 plasma levels were assessed using ELISA kits. We analyzed 28 patients with a median Karnofsky Performance Status of 70 (range 50-80). A diffuse pattern was observed in 14 patients (50%). We did not observe any correlation between baseline imaging features and plasma levels of MMP2 or MMP9. We found no association between baseline MMP levels and diffuse MRI patterns. In univariate analyses, diffuse pattern, multi-focal disease, tumor diameter, surface area, and volume had no impact on outcome, while the number of lobes involved in CE and crossing of the midline by CE were associated with a worse progression-free survival (p = 0.072 and p = 0.012, respectively) and overall survival (p = 0.012 and p

[\({}^{18}\)F]fluorodeoxyglucose (FDG) positron emission tomography (PET) aids in the localisation of the epileptogenic zone in patients with focal epilepsy, especially when magnetic resonance imaging (MRI) is normal or non-contributory. We propose a two-stage deep learning framework to support the clinical evaluation of patients with focal epilepsy by identifying candidate regions of hypometabolism in [18F]FDG PET scans. In the first stage, we train a generative adversarial network (GAN) to learn the mapping between healthy [18F]FDG PET and T1-weighted (T1w) MRI data. In the second stage, we synthesise pseudo-normal PET images from T1w MRI scans of patients with epilepsy to compare to the real PET scans. Comparing the estimated pseudo-PET images to the true PET scans in healthy control data, our GAN produced whole-brain mean absolute errors of \(0.053 \pm 0.015\), outperforming a U-Net (\(0.058 \pm 0.021\)) and a high-resolution dilated convolutional neural network (\(0.060 \pm 0.024\); all images scaled 0–1). In a sample of 20 epilepsy patients, we created Z-statistic images (with thresholding at +2.33) by subtracting the patient’s true PET scans from their estimated pseudo-normal PET images to identify regions of hypometabolism. Excellent sensitivity for lobar location of abnormalities (\(92.9 \pm 13.1\%\)) was observed for the seven cases with MR-visible epileptogenic lesions. For the 13 cases with non-contributory MR, a lower sensitivity of \(74.8 \pm 32.3\%\) was observed. Our method performed better than a statistical parametric mapping analysis. Our results highlight the potential of deep learning-based pseudo-normal [18F]FDG PET synthesis to contribute to the management of epilepsy.

OBJECTIVEThe tremor circuitry has commonly been hypothesized to be driven by one or multiple pacemakers within the cerebello-thalamo-cortical pathway, including the cerebellum, contralateral motor thalamus, and primary motor cortex. However, previous studies, using multiple methodologies, have advocated that tremor could be influenced by changes within the right extrastriate cortex, at both the structural and functional level. The purpose of this work was to evaluate the role of the extrastriate cortex in tremor generation and further arrest after left unilateral stereotactic radiosurgery thalamotomy (SRS-T).METHODSThe authors considered 12 healthy controls (HCs, group 1); 15 patients with essential tremor (ET, right-sided, drug-resistant; group 2) before left unilateral SRS-T; and the same 15 patients (group 3) 1 year after the intervention, to account for delayed effects. Blood oxygenation level–dependent functional MRI during resting state was used to characterize the dynamic interactions of the right extrastriate cortex, comparing HC subjects against patients with ET before and 1 year after SRS-T. In particular, the authors applied coactivation pattern analysis to extract recurring whole-brain spatial patterns of brain activity over time.RESULTSThe authors found 3 different sets of coactivating regions within the right extrastriate cortex in HCs and patients with pretherapeutic ET, reminiscent of the “cerebello-visuo-motor,” “thalamo-visuo-motor” (including the targeted thalamus), and “basal ganglia and extrastriate” networks. The occurrence of the first pattern was decreased in pretherapeutic ET compared to HCs, whereas the other two patterns showed increased occurrences. This suggests a misbalance between the more prominent cerebellar circuitry and the thalamo-visuo-motor and basal ganglia networks. Multiple regression analysis showed that pretherapeutic standard tremor scores negatively correlated with the increased occurrence of the thalamo-visuo-motor network, suggesting a compensatory pathophysiological trait. Clinical improvement after SRS-T was related to changes in occurrences of the basal ganglia and extrastriate cortex circuitry, which returned to HC values after the intervention, suggesting that the dynamics of the extrastriate cortex had a role in tremor generation and further arrest after the intervention.CONCLUSIONSThe data in this study point to a broader implication of the visual system in tremor generation, and not only through visual feedback, given its connections to the dorsal visual stream pathway and the cerebello-thalamo-cortical circuitry, with which its dynamic balance seems to be a crucial feature for reduced tremor. Furthermore, SRS-T seems to bring abnormal pretherapeutic connectivity of the extrastriate cortex to levels comparable to those of HC subjects.

Background: Although the incidence of sudden unexpected infant death (SUID) has decreased since the ‘Back to Sleep’ campaign in English-speaking countries and other preventive campaigns, the circum...

OBJECTIVE: Cortical thinning, previously identified during prodromal stages of Alzheimer's disease (AD), is a "candidate" biomarker implemented in AD clinical therapy trials. We investigated the effect of donepezil treatment on cortical thickness in mild cognitively impaired subjects with the amnestic syndrome of the hippocampal type, a prodromal at-risk group for progression to AD dementia. METHODS: Data were from a longitudinal analysis of a community-based multicenter suspected prodromal AD cohort diagnosed by the Free and Cued Selective Reminding Test (81 donepezil vs 92 placebo) enrolled in a double-blind, randomized, placebo-controlled parallel group design using donepezil (10 mg/day). The study started in November 2006 and concluded in August 2010. All subjects underwent 2 brain structural magnetic resonance imaging (MRI) scans, at baseline and at the end of the trial. Structural MRI images had been processed using the automated pipeline for longitudinal segmentation and surface reconstruction implemented in FreeSurfer. The primary outcome measure of this post hoc study was the annualized percentage change (APC) of cortical thickness. RESULTS: The donepezil group exhibited reduced APC cortical thinning compared to placebo in the rostral anterior cingulate (right: P = .048; left: P = .032), the orbitofrontal (right: P = .012; left: P < .048), and the right inferior frontal (P = .022) cortices and in the right insula (P = .010). These results were not statistically significant after Bonferroni correction likely due to insufficient power for cortical thickness measurements in the study group powered for the predefined hippocampus outcome. CONCLUSIONS: Our findings support the hypothesis that cortical thickness is a reliable candidate surrogate outcome in early predementia AD trials. In addition, donepezil treatment may have an impact on cortical structure/morphology in areas innervated by the medial and lateral cholinergic pathways. BACKGROUND: ClinicalTrials.gov identifier: NCT00403520. 77

Since December 2019, a novel coronavirus, also called severe acute respiratory syndrome CoV-2 (SARS-CoV-2), emerged in Wuhan, China, and caused a pandemic disease (COVID-19). Respiratory impairment is the most common symptom in patients with confirmed COVID-19; however, neurologic symptoms were documented in approximately 36%, including headache, disturbed consciousness, and paresthesia.1 The virus can take different pathways to involve the CNS: in one way through hematogenous or lymphatic route and in another way via the cribriform plate close to the olfactory bulb. Very few studies have shown CNS abnormalities related to COVID-19 on MRI. Herein, we report a case of SARS-CoV-2 brain lesions suggesting an acute demyelination.

Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymeningitis frequency was 4.1%. We report eight new cases with cranial, spinal or both involvements and a literature review of 46 pathological proven cases. We observed that IgG4-related pachymeningitis is in most cases not associated to extra-neurological manifestations of the disease. Only 27% of spinal and 40% of cranial IgG4-related pachymeningitis are associated with other disease localizations. First line treatment strategies included surgery and steroids. The use of immunosuppressants or rituximab was necessary in 18% of spinal and 54% of cranial localizations. Some patients remained with sequellae and clinical and/or radiological improvement can be difficult to obtain.

The embryological development of the central nervous system takes place during the neurulation process, which includes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD.This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5–S1 level, with a thickened filum terminale.The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale.

OBJECTIVEHemispherotomy is currently the most frequently performed surgical option for refractory epilepsy associated with large perinatal or childhood ischemic events. Such an approach may lead to good seizure control, but it has inherent functional consequences linked to the disconnection of functional cortices. The authors report on 6 consecutive patients who presented with severe epilepsy associated with hemiplegia due to stroke and who benefitted from a new, stereoelectroencephalography-guided partial disconnection technique.METHODSThe authors developed a new disconnection technique termed “tailored suprainsular partial hemispherotomy” (TSIPH). Disconnection always included premotor and motor cortex with variable anterior and posterior extent.RESULTSAt a mean follow-up of 28 months, there were no deaths and no patient had hydrocephalus. Motor degradation was observed in all patients in the 2 weeks after surgery, but all patients completely recovered. The 6 patients were seizure free (Engel class IA) at the last follow-up. No neuropsychological aggravation was observed.CONCLUSIONSTSIPH appears to be a conservative alternative to classic hemispherotomy, leading to favorable outcome in this series.

Objective To evaluate functional connectivity (FC) of the ventrolateral thalamus, a common target for drug-resistant essential tremor (ET), resting-state data were analyzed before and 1 year after stereotactic radiosurgical thalamotomy and compared against healthy controls (HCs). Methods In total, 17 consecutive patients with ET and 10 HCs were enrolled. Tremor network was investigated using the ventrolateral ventral (VLV) thalamic nucleus as the region of interest, extracted with automated segmentation from pretherapeutic diffusion magnetic resonance imaging. Temporal correlations of VLV at whole brain level were evaluated by comparing drug-naive patients with ET with HCs, and longitudinally, 1 year after stereotactic radiosurgical thalamotomy. 1 year thalamotomy MR signature was always located inside VLV and did not correlate with any of FC measures (P > 0.05). This suggested presence of longitudinal changes in VLV FC independently of the MR signature volume. Results Pretherapeutic ET displayed altered VLV FC with left primary sensory-motor cortex, pedunculopontine nucleus, dorsal anterior cingulate, left visual association, and left superior parietal areas. Pretherapeutic negative FC with primary somatosensory cortex and pedunculopontine nucleus correlated with poorer baseline tremor scores (Spearman = 0.04 and 0.01). Longitudinal study displayed changes within right dorsal attention (frontal eye-fields and posterior parietal) and salience (anterior insula) networks, as well as areas involved in hand movement planning or language production. Conclusions Our results demonstrated that patients with ET and HCs differ in their left VLV FC to primary somatosensory and supplementary motor, visual association, or brainstem areas (pedunculopontine nucleus). Longitudinal changes display reorganization of dorsal attention and salience networks after thalamotomy. Beside attentional gateway, they are also known for their major role in facilitating a rapid access to the motor system.

Monitoring of blood glucose is usually reported to reduce the risk of hypoglycemia in term newborns with high risk factors and for prematurity in neonatal intensive care unit patients. Differential diagnosis has rarely been discussed. In the eutrophic term newborn, hypoglycemia remains rare and an etiological diagnosis must be made. Intensive management of neonatal hypoglycemia is required to prevent neurodevelopmental defects. Without evident cause or if hypoglycemia persists, a systematic review of possible causes should be made. We report isolated glucocorticoid deficiency diagnosed in an infant at 10 months of age. This boy had neonatal hypoglycemia and mild jaundice that had not been investigated. During his first 9 months of life, he presented frequent infections. At 10 months of age, febrile seizures occurred associated with shock, hypoglycemia, hyponatremia, mild hyperpigmentation, and coma. He was diagnosed with hypocortisolemia and elevated ACTH levels. Brain injury was revealed by MRI after resuscitation, with hypoxic-ischemic and hypoglycemic encephalopathy. The molecular studies demonstrated the presence of p.Asp107Asn and previously unreported frameshift p.Pro281GlnfsX9 MC2R gene mutations. A substitutive hormone therapy was provided and during a follow-up of 12 months no adrenal crisis was noted. We report an unusual case of familial glucocorticoid deficiency with severe neurological injury. This case demonstrates the importance of an appropriate etiological diagnosis in neonatal hypoglycemia.

BACKGROUND The aim of this retrospective study was to evaluate the relevance of a systematic postoperative CT scan after neurosurgical craniotomy and to identify predictive factors of complications. METHODS This retrospective analysis included all the patients at our institution who benefited from a cerebral postoperative CT scan within 24 hours post-craniotomy. Patient characteristics and neuroimaging abnormalities were recorded. Predictive factors were identified using a recursive partitioning analysis. RESULTS A total of 633 patients were included. Of these, 17.9% of patients suffered from postoperative complications and 7.4% of them required a new surgery. The decision for reoperation was based on the neurological deterioration and the CT scan, but never on the CT scan alone. The mortality rate was 1.1%. The risk to be reoperated was correlated to the occurrence of a new postoperative neurological deficit (P

Objective The aim of this study is to evaluate the efficiency of a specific organizational model for early stroke management associated with repeated public awareness campaigns on stroke warning signs. Method Our model is based on initial telephone triage of potential candidates for an intravenous thrombolysis by an emergency physician before a 3-party conference including basic life support team on scene and a stroke neurologist. We performed a time series analysis for a period of 5 years and a half, comparing the number of emergency telephone calls with that of intravenous thrombolysis treatment realized. Results In our organizational model, repeated awareness public campaigns increased both the number of emergency calls for suspected stroke and the selection of potential candidates for intravenous thrombolysis. Results from the time series analysis suggest that educational campaigns are a major factor influencing our emergency medical service activity. This result is correlated with the number of performed intravenous thrombolyses by the stroke center especially within a 3-hour delay (Spearman ρ , P = .621, P = .000 and P = .439, P = .000, respectively). Conclusion Educational programs repeated each year are useful to the population for learning how to recognize stroke symptoms and send straight away an emergency call. Combining the emergency action with an early remote evaluation by the stroke center team and a direct admission in imaging department shortens the time-to-treatment delay. This model is reproducible in different health care systems.

Resume L’encephalopathie aigue necrosante (EAN) est une maladie neurologique rare declenchee a la suite d’une fievre le plus souvent d’origine virale affectant des nourrissons ou des enfants jusque la en bonne sante. L’evolution clinique se caracterise par une deterioration rapide de l’etat neurologique aboutissant presque toujours a un coma et necessitant une prise en charge en reanimation. Le diagnostic est conforte par l’imagerie par resonance magnetique (IRM) cerebrale qui montre des lesions specifiques de necrose symetrique et focale des thalami. L’EAN a ete associee dans de recentes etudes a l’existence d’une mutation autosomique dominante du gene de la proteine RAN-binding protein 2. Nous rapportons le cas d’une fratrie (trois enfants) d’origine tunisienne dans laquelle deux enfants ont presente de facon certaine une EAN a l’âge de 9 mois dans les suites immediates d’une infection virale. L’etude moleculaire a mis en evidence une mutation faux-sens du gene RAN-binding protein 2 chez les deux enfants pour lesquels un prelevement etait disponible. Parmi les tableaux d’encephalopathie aigue de l’enfant, la reconnaissance de cette nouvelle entite neurologique, rare, permet non seulement une prise en charge symptomatique precoce, mais aussi un conseil genetique plus precis.

International audience; BAP31 is one of the most abundant endoplasmic reticulum (ER) membrane proteins. It is a chaperone protein involved in several pathways , including ER-associated degradation, export of ER proteins to the Golgi apparatus, and programmed cell death. BAP31 is encoded by BCAP31, located in human Xq28 and highly expressed in neurons. We identified loss-of-function mutations in BCAP31 in seven individuals from three families. These persons suffered from motor and intellectual disabilities, dystonia, sensorineural deafness, and white-matter changes, which together define an X-linked syndrome. In the primary fibroblasts of affected individuals, we found that BCAP31 deficiency altered ER morphology and caused a disorganization of the Golgi apparatus in a significant proportion of cells. Contrary to what has been described with transient-RNA-interference experiments, we demonstrate that constitutive BCAP31 deficiency does not activate the unfolded protein response or cell-death effectors. Rather, our data demonstrate that the lack of BAP31 disturbs ER metabolism and impacts the Golgi apparatus, highlighting an important role for BAP31 in ER-to-Golgi crosstalk. These findings provide a molecular basis for a Mendelian syndrome and link intracellular protein trafficking to severe congenital brain dysfunction and deafness.

Background Existing methods to predict recovery after severe traumatic brain injury lack accuracy. The aim of this study is to determine the prognostic value of quantitative diffusion tensor imaging (DTI). Methods In a multicenter study, the authors prospectively enrolled 105 patients who remained comatose at least 7 days after traumatic brain injury. Patients underwent brain magnetic resonance imaging, including DTI in 20 preselected white matter tracts. Patients were evaluated at 1 yr with a modified Glasgow Outcome Scale. A composite DTI score was constructed for outcome prognostication on this training database and then validated on an independent database (n=38). DTI score was compared with the International Mission for Prognosis and Analysis of Clinical Trials Score. Results Using the DTI score for prediction of unfavorable outcome on the training database, the area under the receiver operating characteristic curve was 0.84 (95% CI: 0.75-0.91). The DTI score had a sensitivity of 64% and a specificity of 95% for the prediction of unfavorable outcome. On the validation-independent database, the area under the receiver operating characteristic curve was 0.80 (95% CI: 0.54-0.94). On the training database, reclassification methods showed significant improvement of classification accuracy (P < 0.05) compared with the International Mission for Prognosis and Analysis of Clinical Trials score. Similar results were observed on the validation database. Conclusions White matter assessment with quantitative DTI increases the accuracy of long-term outcome prediction compared with the available clinical/radiographic prognostic score.