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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.

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

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.

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.

Essential tremor (ET) is a prevalent movement disorder characterized by marked clinical heterogeneity. Here, we explored the morphometric underpinnings of this cross-subject variability on a cohort of 34 patients with right-dominant drug-resistant ET and 29 matched healthy controls (HCs). For each brain region, group-wise morphometric data was modelled by a multivariate Gaussian to account for morphometric features' (co)variance. No group differences were found in terms of mean values, highlighting the limits of more basic group comparison approaches. Variance in surface area was higher in ET in the left lingual and caudal anterior cingulate cortices, while variance in mean curvature was lower in the right superior temporal cortex and pars triangularis, left supramarginal gyrus and bilateral paracentral gyrus. Heterogeneity further extended to the right putamen, for which a mixture of two Gaussians fitted the ET data better than a single one. Partial Least Squares analysis revealed the rich clinical relevance of the ET population's heterogeneity: first, increased head tremor and longer symptoms' duration were accompanied by broadly lower cortical gyrification. Second, more severe upper limb tremor and impairments in daily life activities characterized the patients whose morphometric profiles were more atypical compared to the average ET population, irrespective of the exact nature of the alterations. Our results provide candidate morphometric substrates for two different types of clinical variability in ET. They also demonstrate the importance of relying on analytical approaches that can efficiently handle multivariate data and enable to test more sophisticated hypotheses regarding its organization.

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International audience; INTRODUCTION: There is a growing interest in personality evaluation in Parkinson's disease (PD), following observations of specific temperaments in PD patients. Therefore, our objective was to evaluate personality dimensions from the Temperament and Character Inventory (TCI) in a cohort of fluctuating PD patients considered for deep brain stimulation. METHODS: Fluctuating PD patients from the PREDISTIM cohort were included. Description of TCI dimensions and comparison with a French normative cohort were performed. Pearson correlations between TCI dimensions and motor, behavioral and cognitive variables were investigated. Structural and internal consistency analysis of the TCI were further assessed. RESULTS: The 570 PD patients presented significant higher scores in Harm Avoidance, Reward Dependence, Persistence, Self-Directedness and Cooperativeness and significant lower scores in Self-Transcendence compared to the French normative cohort; only Novelty Seeking scores were not different. Harm Avoidance and Self-directedness scores were correlated with PDQ-39 total, HAMD, HAMA scores, and anxiolytic/antidepressant treatment. Novelty Seeking scores were correlated with impulsivity. Pearson correlations between TCI dimensions, principal component analysis of TCI sub-dimensions and Cronbach's alpha coefficients showed adequate psychometric proprieties. CONCLUSION: The TCI seems to be an adequate tool to evaluate personality dimensions in PD with good structural and internal consistencies. These fluctuating PD patients also have specific personality dimensions compared to normative French population. Moreover, Harm Avoidance and Self-Directedness scores are associated with anxio-depressive state or quality of life and, and Novelty Seeking scores with impulsivity.

AI brain tumour segmentation and brain extraction algorithms promise better diagnostic and follow-up of brain tumours in adults. The development of such tools for paediatric populations is restricted by limited training data but careful adaption of adult algorithms to paediatric population might be a solution. Here, we aim exploring the transferability of algorithms for brain (HD-BET) and tumour segmentation (HD-GLIOMA) in adults to paediatric imaging studies.In a retrospective cohort, we compared automated segmentation with expert masks. We used the dice coefficient for evaluating the similarity and multivariate regressions for the influence of covariates. We explored the feasibility of automatic tumor classification based on diffusion data.In 42 patients (mean age 7 years, 9 below 2 years, 26 males), segmentation was excellent for brain extraction (mean dice 0.99, range 0.85-1), moderate for segmentation of contrast-enhancing tumours (mean dice 0.67, range 0-1), and weak for non-enhancing T2-signal abnormalities (mean dice 0.41). Precision was better for enhancing tumour parts (p 0.001) and for malignant histology (p = 0.006 and p = 0.012) but independent from myelinisation as indicated by the age (p = 0.472). Automated tumour grading based on mean diffusivity (MD) values from automated masks was good (AUC = 0.86) but tended to be less accurate than MD values from expert masks (AUC = 1, p = 0.208).HD-BET provides a reliable extraction of the paediatric brain. HD-GLIOMA works moderately for contrast-enhancing tumours parts. Without optimization, brain tumor AI algorithms trained on adults and used on paediatric patients may yield acceptable results depending on the clinical scenario.

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.

International audience; We present a new consensus atlas of deep grey nuclei obtained by shape-based averaging of manual segmentation of two experienced neuroradiologists and optimized from 7T MP2RAGE images acquired at (0.6mm) 3 in 60 healthy subjects. A group-wise normalization method was used to build a high-contrast and high-resolution T1-weighted brain template (0.5mm) 3 using data from 30 out of the 60 controls. Delineation of 24 deep grey nuclei per hemisphere, including the claustrum and twelve thalamic nuclei, was then performed by two expert neuroradiologists and reviewed by a third neuroradiologist according to tissue contrast and external references based on the Morel atlas. Corresponding deep grey matter structures were also extracted from the Morel and CIT168 atlases. The data-derived, Morel and CIT168 atlases were all applied at the individual level using non-linear registration to fit the subject reference and to extract absolute mean quantitative T1 values derived from the 3D-MP2RAGE volumes, after correction for residual B1 + biases. Three metrics (The Dice and the volumetric similarity coefficients, and a novel Hausdorff distance) were used to estimate the inter-rater agreement of manual MRI segmentation and inter-atlas variability, and these metrics were measured to quantify biases due to image registration and their impact on the measurements of the quantitative T1 values was highlighted. This represents a fully-automated segmentation process permitting the extraction of unbiased normative T1 values in a population of young healthy controls as a reference for characterizing subtle structural alterations of deep grey nuclei relevant to a range of neurological diseases.

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 (

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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).

International audience; Objective: This longitudinal study aimed to measure the time course of intellectual changes after pediatric focal resective epilepsy surgery and to identify their predictors.Methods: We analyzed a cohort of 81 school-aged children with focal epilepsy and intractable seizures who underwent neurosurgery (focal resection) from 2000 to 2018 in La Timone Hospital (Marseille). Neuropsychological assessments were carried out before and then 1, 2, 3, and 5 years after epilepsy surgery.Results: Eighty-one patients with a median age at surgery of 13.74 years [4.25] were enrolled. Overall, 45 of the 81 (55%) recruited patients were improved after the surgery on at least one of the five domains of the Wechsler Intelligence Scale. Temporal lobe localization and postoperative seizure freedom were the main prognostic factors impacting intellectual outcome (improvement and decline) after epilepsy surgery. Younger patients at surgery were less likely to have a postoperative IQ decline. Intellectual improvement after epilepsy surgery could be delayed for up to 5 years after surgery and concerned all intellectual domains except the Verbal Comprehension Index (VCI). Intellectual decline after epilepsy surgery occurred mainly during the first two years after the surgery and was reflected in full-scale intelligence quotient (FSIQ) and Working Memory Index (WMI).Conclusions: Our study points out that children and adolescents with TLE who achieved freedom from seizure after epilepsy surgery are the leading candidates for achieving postoperative intellectual improvement. This enhancement in intellectual function shows a long time course, whereas intellectual decline is evidenced earlier.

Summary Background The CNS manifestations of COVID-19 in children have primarily been described in case reports, which limit the ability to appreciate the full spectrum of the disease in paediatric patients. We aimed to identify enough cases that could be evaluated in aggregate to better understand the neuroimaging manifestations of COVID-19 in the paediatric population. Methods An international call for cases of children with encephalopathy related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and abnormal neuroimaging findings was made. Clinical history and associated plasma and cerebrospinal fluid data were requested. These data were reviewed by a central neuroradiology panel, a child neurologist, and a paediatric infectious diseases expert. The children were categorised on the basis of their time of probable exposure to SARS-CoV-2. In addition, cases were excluded when a direct link to SARS-CoV-2 infection could not be established or an established alternate diagnostic cause could be hypothesised. The accepted referral centre imaging data, from ten countries, were remotely reviewed by a central panel of five paediatric neuroradiologists and a consensus opinion obtained on the imaging findings. Findings 38 children with neurological disease related to SARS-CoV-2 infection were identified from France (n=13), the UK (n=8), the USA (n=5), Brazil (n=4), Argentina (n=4), India (n=2), Peru (n=1), and Saudi Arabia (n=1). Recurring patterns of disease were identified, with neuroimaging abnormalities ranging from mild to severe. The most common imaging patterns were postinfectious immune-mediated acute disseminated encephalomyelitis-like changes of the brain (16 patients), myelitis (eight patients), and neural enhancement (13 patients). Cranial nerve enhancement could occur in the absence of corresponding neurological symptoms. Splenial lesions (seven patients) and myositis (four patients) were predominantly observed in children with multisystem inflammatory syndrome. Cerebrovascular complications in children were less common than in adults. Significant pre-existing conditions were absent and most children had favourable outcomes. However, fatal atypical CNS co-infections developed in four previously healthy children infected with SARS-CoV-2. Interpretation Acute-phase and delayed-phase SARS-CoV-2-related CNS abnormalities are seen in children. Recurring patterns of disease and atypical neuroimaging manifestations can be found and should be recognised being as potentially due to SARS-CoV-2 infection as an underlying aetiological factor. Studies of paediatric specific cohorts are needed to better understand the effects of SARS-CoV-2 infection on the CNS at presentation and on long-term follow-up in children. Funding American Society of Pediatric Neuroradiology, University of Manchester (Manchester, UK). Video Abstract Neuroimaging manifestations in children with SARS-CoV-2 infection

BackgroundRecurrence following obliteration of brain arteriovenous malformations (AVMs) is common in children surgically treated, but recurrences following endovascular (EVT) and radiosurgical approaches are scantily reported.ObjectiveTo analyze the rates and risk factors for AVM recurrence after obliteration in a single-center cohort of children with ruptured AVMs treated with multimodal approaches, and to carry out a comprehensive review and meta-analysis of current data.MethodsChildren with ruptured AVMs between 2000 and 2019 enrolled in a prospective registry were retrospectively screened and included after angiographically determined obliteration to differentiate children with/without recurrence. A complementary systematic review and meta-analysis of studies investigating AVM recurrence in children between 2000 and 2020 was aggregated to explore the overall recurrence rates across treatment modalities by analyzing surgery versus other treatments.ResultsSeventy children with obliterated AVMs were included. AVM recurrences (n=10) were more commonly treated with EVT as final treatment (60% in the recurrence vs 13.3% in the no-recurrence group, p=0.018). Infratentorial locations were associated with earlier and more frequent recurrences (adjusted relative risk=4.62, 95% CI 1.08 to 19.04; p=0.04).In the aggregate analysis, the pooled rate of AVM recurrence was 10.9% (95% CI 8.7% to 13.5%). Younger age at presentation was associated with more frequent recurrences (RR per year increase, 0.97, 95% CI 0.93 to 0.99; p=0.046).ConclusionLocation of infratentorial AVMs and younger age at presentation may be associated with earlier and more frequent recurrences. The higher rates of recurrence in patients with AVMs obliterated with EVT questions its role in an intent-to-cure approach and reinforces its position as an adjunct to surgery and/or radiosurgery.

International audience; We describe the clinical, electroencephalography (EEG), and developmental features of a patient with developmental and epileptic encephalopathy due to a homozygous pathogenic variation of mitochondrial glutamate/H+ symporter SLC25A22. Epilepsy began during the first week of life with focal onset seizures. Interictal EEG revealed a suppression-burst pattern with extensive periods of non-activity. The prospective follow-up confirmed developmental encephalopathy as well as ongoing active epilepsy and almost no sign of development at 8 years of age. We confirm in the following paper that SLC25A22 recessive variations may cause a severe developmental and epileptic encephalopathy characterized by a suppression-burst pattern. On the basis of an in-depth literature review, we also provide an overview of this rare genetic cause of neonatal onset epilepsy.

Giant hypothalamic hamartomas (GHH) are rare neonatal intracerebral congenital malformations responsible for gelastic epilepsy and/or endocrine disturbances. Sacrococcygeal teratomas (SCT) are fetal neoplasms associated with perinatal morbidity and mortality, especially hemorrhagic complications in giant examples (GSCT). Here, we describe an immature ruptured GSCT complicated by hemorrhagic shock at 32-week gestation boy requiring an emergency delivery, followed immediately by urgent surgical removal. A brain lesion resembling a GHH was also present on the antenatal MRI. In order to exclude metastatic immature teratoma or glioma, a biopsy was performed by a retro-sigmoidal approach, which confirmed the nature of the hamartoma. Here, we describe for the first time the association of a ruptured immature GSCT associated with a GHH.

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.

Essential tremor (ET) is a neurological disease with both motor and nonmotor manifestations; however, little is known about its underlying brain basis. Furthermore, the overall organization of the brain network in ET remains largely unexplored. We investigated the topological properties of brain functional network, derived from resting¿state functional magnetic resonance imaging (MRI) data, in 23 ET patients versus 23 healthy controls. Graph theory analysis was used to assess the functional network organization. At the global level, the functional network of ET patients was characterized by lower small¿worldness values than healthy controls¿less clustered functionality of the brain. At the regional level, compared with the healthy controls, ET patients showed significantly higher values of global efficiency, cost and degree, and a shorter average path length in the left inferior frontal gyrus (pars opercularis), right inferior temporal gyrus (posterior division and temporo¿occipital part), right inferior lateral occipital cortex, left paracingulate, bilateral precuneus bilaterally, left lingual gyrus, right hippocampus, left amygdala, nucleus accumbens bilaterally, and left middle temporal gyrus (posterior part). In addition, ET patients showed significant higher local efficiency and clustering coefficient values in frontal medial cortex bilaterally, subcallosal cortex, posterior cingulate cortex, parahippocampal gyri bilaterally (posterior division), right lingual gyrus, right cerebellar flocculus, right postcentral gyrus, right inferior semilunar lobule of cerebellum and culmen of vermis. Finally, the right intracalcarine cortex and the left orbitofrontal cortex showed a shorter average path length in ET patients, while the left frontal operculum and the right planum polare showed a higher betweenness centrality in ET patients. In conclusion, the efficiency of the overall brain functional network in ET is disrupted. Further, our results support the concept that ET is a disorder that disrupts widespread brain regions, including those outside of the brain regions responsible for tremor., Spanish Ministry of Economy and Competitiveness, Grant/Award Number: DPI‐2015‐72638‐EXP; European Commission, Grant/Award Number: ICT‐2011‐287739; Spanish Ministry of Economy and Competitiveness, Grant/Award Number: DPI‐2015‐68664‐C4‐1‐R; Claire O'Neil Essential Tremor Research Fund; Spanish Health Research Agency, Grant/Award Numbers: FIS PI16/00451, FIS PI12/01602; Ministry of Economy and Competitiveness, Grant/Award Number: RTC‐2015‐3967‐1; National Institutes of Health, Grant/Award Numbers: NINDS #R01 NS088257, NINDS #R01 NS073872, NINDS #R01 NS085136, NINDS #R01 NS094607, NINDS #R01 NS39422, Sí

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.