Automated electrical source imaging with scalp EEG to define the insular irritative zone: Comparison with simultaneous intracranial EEG

Objective To evaluate the accuracy of automated interictal low-density electrical source imaging (LD-ESI) to define the insular irritative zone (IZ) by comparing the simultaneous interictal ESI localization with the SEEG interictal activity.Methods Long-term simultaneous scalp electroencephalography (EEG) and stereo-EEG (SEEG) with at least one depth electrode exploring the operculo-insular region(s) were analyzed. Automated interictal ESI was performed on the scalp EEG using standardized low-resolution brain electromagnetic tomography (sLORETA) and individual head models. A two-step analysis was performed: i) sublobar concordance between cluster-based ESI localization and SEEG-based IZ; ii) time-locked ESI-/SEEG analysis. Diagnostic accuracy values were calculated using SEEG as reference standard. Subgroup analysis was carried out, based on the involvement of insular contacts in the seizure onset and patterns of insular interictal activity.Results Thirty patients were included in the study. ESI showed an overall accuracy of 53% (C.I. 29–76%). Sensitivity and specificity were calculated as 53% (C.I. 29–76%), 55% (C.I. 23–83%) respectively. Higher accuracy was found in patients with frequent and dominant interictal insular spikes.Conclusions LD-ESI defines with good accuracy the insular implication in the IZ, which is not possible with classical interictal scalp EEG interpretation.SignificanceAutomated LD-ESI may be a valuable additional tool to characterize the epileptogenic zone in epilepsies with suspected insular involvement.     

Clinical and advanced neurophysiology in the prognostic and diagnostic evaluation of disorders of consciousness: review of an IFCN-endorsed expert group

The analysis of spontaneous EEG activity and evoked potentials is a cornerstone of the instrumental evaluation of patients with disorders of consciousness (DoC). The past few years have witnessed an unprecedented surge in EEG-related research applied to the prediction and detection of recovery of consciousness after severe brain injury, opening up the prospect that new concepts and tools may be available at the bedside. This paper provides a comprehensive, critical overview of both consolidated and investigational electrophysiological techniques for the prognostic and diagnostic assessment of DoC. We describe conventional clinical EEG approaches, then focus on evoked and event-related potentials, and finally we analyze the potential of novel research findings. In doing so, we (i) draw a distinction between acute, prolonged and chronic phases of DoC, (ii) attempt to relate both clinical and research findings to the underlying neuronal processes and (iii) discuss technical and conceptual caveats. The primary aim of this narrative review is to bridge the gap between standard and emerging electrophysiological measures for the detection and prediction of recovery of consciousness. The ultimate scope is to provide a reference and common ground for academic researchers active in the field of neurophysiology and clinicians engaged in intensive care unit and rehabilitation.     

EEG power spectral density under Propofol and its association with burst suppression,a marker of cerebral fragility

ObjectiveUnder General Anesthesia (GA), age and Burst Suppression (BS) are associated with cognitive postoperative complications, yet how these parameters are related to per-operative EEG and hypnotic doses is unclear. In this prospective study, we address this question comparing age and BS occurrences with a new score (BP_TIVA) based on Propofol doses, EEG and alpha-band power spectral densities, evaluated for SEF₉₅ = 8–13 Hz.Methods59 patients (55 [34–67] yr, 67% female) undergoing neuroradiology or orthopedic surgery were included. Total IntraVenous Anesthesia was used for Propofol and analgesics infusion. Cerebral activity was monitored from a frontal electrodes montage EEG.ResultsBP_TIVA was inversely correlated with age (Pearson r = −0.78, p < 0.001), and was significantly lower (p < 0.001) when BS occurred during the GA first minutes (induction). Additionally, the age-free BP_TIVA score was better associated with BS at induction than age (AUC = 0.94 versus 0.82, p < 0.05).ConclusionWe designed BP_TIVA score based on hypnotics and EEG. It was correlated with age yet was better associated to BS occurring during GA induction, the latter being a cerebral fragility sign.SignificanceThis advocate for an approach based on evaluating the cerebral physiological age (« brain age ») to predict postoperative cognitive evolution.     

Evaluation of frequency-selective non-linear blending technique on brain CT in postoperative children with Moyamoya disease

ObjectiveTo evaluate whether a frequency-selective non-linear blending (BC) technique can improve tissue contrast and infarct detection on non-enhanced brain CT (NECT) in postoperative Moyamoya (MMD) patients.Materials and methodsFrom January 2010 to December 2017, 33 children (13 boys and 20 girls; mean age 9.1 ± 3.4 years) with MMD postoperatively underwent NECT followed by diffusion MRI. We compared the contrast-to-noise ratio (CNR) between gray matter (GM) and white matter (WM) in NECT and BC images and the CNR between the infarct lesion and adjacent normal-appearing brain in NECT and BC images using a paired t-test. We assessed image noise, GM-WM differentiation, artifacts, and overall quality using a Wilcoxon signed rank test. A McNemar two-tailed test was conducted to compare the diagnostic accuracy of infarct detection.ResultsThe CNR between GM and WM and the CNR of the infarct was better in BC images than in NECT images (3.9 ± 1.0 vs. 1.8 ± 0.6, P < 0.001 and 3.6 ± 0.3 vs. 1.9 ± 0.2, P < 0.001), with no difference in overall image quality observed. The sensitivity and specificity of infarct detection were 55.0% and 76.9% using NECT, and 70.0% and 69.2% using BC technique. The diagnostic accuracy of NECT and BC technique was 63.6% (21/33) and 69.7% (23/33), respectively.ConclusionThis study showed that the BC technique improved CNR and maintained image quality. This technique may also be used to identify ischemic brain changes in postoperative MMD patients by improving the CNR of the infarct lesion.     

Influence of inferior petrosal sinus drainage symmetry on detection of adenomas in Cushing's syndrome

BackgroundAsymmetric inferior petrosal sinuses (IPS) are not infrequently encountered during bilateral IPS sampling. There is little data on whether IPS symmetry influences success in predicting the adenoma side in patients with ACTH-dependent Cushing's syndrome (CS).ObjectiveTo assess the influence of IPS drainage patterns on detection of an adenoma in CS.MethodsRetrospective single-center cohort analysis reviewing records of patients with CS and negative MRI findings who subsequently underwent BIPSS.ResultsBIPSS was performed in 38 patients with a mean age of 45 ± 15 years. The overall technical success rate was 97% for bilateral cannulation. Asymmetric IPS were observed in 11 (39%) patients with Cushing's disease (CD). A side-to-side ACTH ratio was not significantly different between patients with symmetric outflow and those with asymmetric outflow at baseline (8.6 ± 2.7 versus 16.4 ± 6.0; P = 0.45), but ratios were significantly different after ovine corticotropin-releasing hormone (oCRH) stimulation (6.0 ± 2.5 versus 35.7 ± 22.5; P = 0.03). BIPSS correctly predicted the side of the adenoma in 25 (96%) patients with CD. Prediction was better when the venous outflow was symmetric (100%) rather than asymmetric (93%), although the difference was not significant (P = 0.42). Remission from CS was achieved in 32 patients (87%), independent of the symmetry of IPS.ConclusionsBearing in mind the sample size of this audit, asymmetric IPS at least do not seem to diminish the accuracy of diagnosis of ACTH-dependent CS, nor do they influence the clinical outcome.     

Is disease activity prior to fingolimod initiation predictive of response? Fingolimod as a “common” first line treatment

BackgroundIn countries where fingolimod is available as first-line therapy without restrictions, we have an opportunity to observe long-term efficacy profile of this drug in treatment-naive patients according to their initial disease activity.MethodsWe retrospectively analysed the data of RRMS patients treated with FTY, focusing on 2 groups: 17 highly active patients (HA) defined as follows: ≥ 2 relapses in the year before treatment initiation and either ≥ 1 Gd-enhancing T1 lesion or a significant increase in T2 lesion load from a baseline MRI; and 37 “not highly active” (NHA). We reviewed treatment efficacy (defined as NEDA-3), reasons for discontinuation and treatment tolerance in both groups.ResultsMean follow-up duration was 48.2 months, SD 18.4. Fingolimod efficiently reduced relapses (NHA 90.3% reduction, P < 0.001, HA 84.9%, P < 0.001), and new Gd enhancing lesions (NHA 85.4% reduction, P = 0.019, HA 92.3%, P = 0.043). The proportion of patients reaching NEDA-3 status was higher in the NHA group (NHA: 80% at 2 years and 66% at 4 years, HA: 58% at 2 years and 38% at 4 years, P = 0.042). Fingolimod was discontinued in 20 cases, mainly because of lack of efficacy (n = 15).ConclusionsFTY is efficient in reducing relapses and new Gd enhancing lesions in both HA and NHA patients although the probability of achieving NEDA-3 over time is higher in early-treated treatment-naive NHA patients.     

Diagnostic efficacy and safety of gadoteridol compared to gadobutrol and gadoteric acid in a large sample of CNS MRI studies at 1.5 T

PurposeTo evaluate safety and diagnostic accuracy of gadoteridol vs. other macrocyclic gadolinium-based contrast agents (GBCAs) in a large cohort of consecutive and non-selected patients referred for CE-MRI of the CNS.Material and methodsBetween November 2017 and March 2018, we prospectively enrolled a consecutive cohort of patients referred for neuroradiological CE-MRI (1.5 T MRI). Image quality and adverse events were assessed. Diagnostic performance was determined for a subgroup of patients with truth standard findings available. Comparison was made between patients receiving gadoteridol and patients receiving other macrocyclic GBCAs. Inter-reader agreement (kappa) between two expert neuroradiologists was calculated for the diagnosis of malignancy.ResultsOverall, 460 patients (220 M/240F; mean age 54 ± 16 years) were enrolled of which 230 received gadoteridol (Group 1) and 230 either gadoteric acid or gadobutrol [n = 83 (36.1%) and n = 147 (63.9%), respectively; Group 2]. Image quality was rated as good or excellent in both groups. The sensitivity, specificity and diagnostic accuracy for determination of malignancy was 88.2%, 96.5% and 95.4%, respectively, for Group 1 and 93.7%, 97.4% and 96.9%, respectively, for Group 2, with no significant differences between groups (P > 0.75) for any determination. Inter-reader agreement for the identification of malignancy was excellent [K = 0.877 (95%CI: 0.758–0.995) and K = 0.818 (95%CI: 0.663–0.972) for groups 1 and 2, respectively; P = 0.0913]. Adverse events occurred in 5 of 460 (1.09%) patients overall, with no significant difference (P = 0.972) between groups.ConclusionGadoteridol was safe and guaranteed good image quality without significant differences when compared to gadobutrol and gadoteric acid in a wide range of CNS pathologies.     

Leukoaraiosis and earlier neurological outcome after mechanical thrombectomy in acute ischemic stroke

Background and purposeThe aim of the study was to evaluate whether leukoaraiosis (LA) severity is associated with earlier neurological outcome in acute stroke patients undergoing mechanical thrombectomy.Materials and methodsIn this retrospective multicenter study, we evaluated 273 acute stroke patients treated with mechanical thrombectomy. LA severity was graded as 0–2 (absent-to-moderate) versus 3–4 (severe) according to the van Swieten scale. The main clinical outcome was the proportion of early neurological improvement and early neurological deterioration. Early neurological improvement was defined as a decrease of ≥ 4 points on the NIHSS, or an NIHSS score of zero 24 hours after baseline assessment. Early neurological deterioration was defined as an increase of ≥ 4 points on the NIHSS 24 hours after baseline assessment.ResultsThere was a significantly lower early neurological improvement rate (17.1% versus 39.2%; P = 0.006) and non-significantly higher early neurological deterioration rate (29.3% versus 17.7%; P = 0.084) in patients with severe LA (sLA) compared with patients with absent-to-moderate LA. In multivariable analysis, sLA was inversely associated with early neurological improvement (OR, 0.31; 95% CI, 0.13–0.78; P = 0.012). There was no significant association of sLA with early neurological deterioration. However, in patients without symptomatic intracranial hemorrhage, sLA was an independent predictor of early neurological deterioration (OR, 2.65; 95% CI, 1.09–6.45; P = 0.032).ConclusionssLA is a significant negative predictor of early neurological improvement and is an independent predictor of early neurological deterioration in patients without symptomatic intracranial hemorrhage.     

Nutrient Status in Patients with Frequent Episodic Tension-Type Headache: A Case-Control Study

ObjectiveTo investigate the relationship between frequent episodic tension-type headache (FE-TTH) and 25-hydroxyvitamin-D (25(OH)D), folate, vitamin B12, and magnesium.Design-MethodsA prospective case-control study involving adults with FETTH and age-sex matched healthy controls (HC) was performed. Individuals under the responsibility of the three provincial Health Centres of the prefecture of Trikala (Central Greece) were recruited during their regular check-up visits. The relationship between FETTH and serum levels of 25(OH)D, vitamin B12, folate, and magnesium was investigated (primary outcomes). Demographics, daily habits, somatometrics, psychometric and sleep quality measurements, laboratory indices, cardiovascular comorbidities and medications taken were also recorded and compared (secondary outcomes). Potential associations of the above-listed parameters with headache parameters (headache frequency, severity and analgesic consumption) were also examined (secondary outcomes).ResultsBetween September and December 2020, 30 patients with FETTH and 30 HC were successfully recruited. Demographics, comorbidities, regular medications, smoking habits, alcohol and coffee consumption, body mass index measurements, markers of systemic inflammation, folate and vitamin B12 levels were similar between the two groups (P > 0.05). Lower serum 25(OH)D was both univariately (P < 0.001) and multivariately [OR= 0.72, 95%CI = (0.55, 0.94) per 1 ng/ml increase] associated with FETTH, while serum magnesium was found lower in FETTH only according to the univariate approach (P = 0.036). Higher levels of depression (P = 0.050) and anxiety (P = 0.020), as well as poor quality of sleep (P = 0.008), were univariately associated with FETTH. Only the effect of anxiety remained significant following the multivariate logistic regression [OR= 7.90, 95%CI = (1.00, 62.47)]. Headache parameters were not associated with any one of the assessed variables.DiscussionLower serum 25(OH)D was related to the presence of FETTH. This finding could imply a potential role for vitamin D in the pathophysiology of TTH.     

Can we predict intraoperative blood loss in meningioma patients? Application of dynamic susceptibility contrast-enhanced magnetic resonance imaging

PurposeTo evaluate the potential of quantitative dynamic susceptibility contrast (DSC) perfusion MR imaging parameters as imaging biomarkers for predicting intraoperative blood loss in meningioma.MethodsFifty-one non-embolized meningioma patients who had undergone preoperative DSC perfusion MR imaging were retrospectively included. The corrected relative cerebral blood volume (rCBV) and leakage coefficient (K₂) of the entire enhanced tumor were obtained using leakage correction. Tumor volume, location, grade, and other clinical variables, were also analyzed. To investigate the vascularity and vascular permeability of meningiomas, and their correlation with predicting estimated blood loss (EBL) using preoperative DSC perfusion MR imaging, the authors proposed an index reflecting the inherent tendency of meningiomas to bleed after controlling volume (i.e., EBL/cm³). Simple regression was performed to identify predictors of EBL/cm³; subsequently, the relevant variables included in the stepwise multiple linear regression.ResultsOn univariate analysis, EBL/cm³ was correlated with rCBV (r = 0.677; P < 0.001), K₂ (r = 0.294; P = 0.036), and tumor volume (r = –0.312, P = 0.026). EBL/cm³ was not correlated with age (P = 0.873), sex (P = 0.404), tumor location (P = 0.327), or histological grade (P = 0.230). On multiple linear regression, rCBV (β = 0.663 [0.463–0.864], B = 1.293 [0.903–1.684; P < 0.001) and K₂ (β = 0.260 [0.060–0.460], B = 2.277 [0.523–4.031], P = 0.012), were the only independent predictors of EBL/cm³.ConclusionThe rCBV and K₂ derived from DSC perfusion MR imaging in meningiomas may serve as feasible tools for clinicians to predict intraoperative blood loss and facilitate surgical planning.     

Recording activity in proximal muscle networks with surface EMG in assessing infant motor development

ObjectiveTo develop methods for recording and analysing infant’s proximal muscle activations.MethodsSurface electromyography (sEMG) of truncal muscles was recorded in three months old infants (N = 18) during spontaneous movement and controlled postural changes. The infants were also divided into two groups according to motor performance. We developed an efficient method for removing dynamic cardiac artefacts to allow i) accurate estimation of individual muscle activations, as well as ii) quantitative characterization of muscle networks.ResultsThe automated removal of cardiac artefacts allowed quantitation of truncal muscle activity, which showed predictable effects during postural changes, and there were differences between high and low performing infants. The muscle networks showed consistent change in network density during spontaneous movements between supine and prone position. Moreover, activity correlations in individual pairs of back muscles linked to infant́s motor performance.ConclusionsThe hereby developed sEMG analysis methodology is feasible and may disclose differences between high and low performing infants. Analysis of the muscle networks may provide novel insight to central control of motility.SignificanceQuantitative analysis of infant’s muscle activity and muscle networks holds promise for an objective neurodevelopmental assessment of motor system.     

Grading meningiomas utilizing multiparametric MRI with inclusion of susceptibility weighted imaging and quantitative susceptibility mapping

Background and purposeThe ability to predict high-grade meningioma preoperatively is important for clinical surgical planning. The purpose of this study is to evaluate the performance of comprehensive multiparametric MRI, including susceptibility weighted imaging (SWI) and quantitative susceptibility mapping (QSM) in predicting high-grade meningioma both qualitatively and quantitatively.MethodsNinety-two low-grade and 37 higher grade meningiomas in 129 patients were included in this study. Morphological characteristics, quantitative histogram analysis of QSM and ADC images, and tumor size were evaluated to predict high-grade meningioma using univariate and multivariate analyses. Receiver operating characteristic (ROC) analyses were performed on the morphological characteristics. Associations between Ki-67 proliferative index (PI) and quantitative parameters were calculated using Pearson correlation analyses.ResultsFor predicting high-grade meningiomas, the best predictive model in multivariate logistic regression analyses included calcification (β = 0.874, P = 0.110), peritumoral edema (β = 0.554, P = 0.042), tumor border (β = 0.862, P = 0.024), tumor location (β = 0.545, P = 0.039) for morphological characteristics, and tumor size (β = 4 × 10⁻⁵, P = 0.004), QSM kurtosis (β = − 5 × 10⁻³, P = 0.058), QSM entropy (β = − 0.067, P = 0.054), maximum ADC (β = − 1.6 × 10⁻³, P = 0.003), ADC kurtosis (β = − 0.013, P = 0.014) for quantitative characteristics. ROC analyses on morphological characteristics resulted in an area under the curve (AUC) of 0.71 (0.61–0.81) for a combination of them. There were significant correlations between Ki-67 PI and mean ADC (r = − 0.277, P = 0.031), 25^th percentile of ADC (r = − 0.275, P = 0.032), and 50^th percentile of ADC (r = − 0.268, P = 0.037).ConclusionsAlthough SWI and QSM did not improve differentiation between low and high-grade meningiomas, combining morphological characteristics and quantitative metrics can help predict high-grade meningioma.     

Validation in French of the Montreal Cognitive Assessment 5-Minute,a brief cognitive screening test for phone administration

BackgroundThe prevalence of cognitive impairment and dementia is high and steadily increasing. Early detection of cognitive decline is crucial since some interventions can reduce the risk of progression to dementia. However, there is a lack of manageable scales for assessing cognitive functions outside specialized consultations. Recently, the MoCA-5 min, a short version of the Montreal Cognitive assessment (MoCA), phone-administered, was validated for screening for vascular cognitive impairment. The aim of the present study was to validate the MoCA-5 min in French in diverse clinical populations.MethodsThe Cantonese version of the MoCA-5 min was adapted for French language. Healthy volunteers and patients with possible or established cognitive impairment (Alzheimer's disease or related disorders, Parkinson's disease, Huntington's disease, type-2 diabetes) participated in the study. The original MoCA and the MoCA-5 min were administered, by phone, with a 30-day interval. Alternate forms were used to reduce learning effects.ResultsThe scores of the original MoCA and MoCA-5 min correlated significantly (Spearman rho = 0.751, P < 0.0001, 95% confidence interval 0.657 to 0.819). Internal consistency was good (Cronbach alpha = 0.795). The area under the ROC curve was 0.870 and the optimal cut-off value for separating patients with and without cognitive impairment with the MoCA-5 min was ≤ 27 with 87.32% sensitivity and 76.09% specificity. Interrater and test-retest reliability were adequate.ConclusionThis study demonstrates that the French version of the MoCA-5 min is a valid and reliable scale for detecting cognitive impairment in different clinical populations. It is administrable by phone and thus suitable for remote assessment as well as for large-scale screening and epidemiological studies.     

Relationship between circadian typology and risk-taking behaviors in adolescents: A cross-sectional study

ObjectiveThis study examined the relationship between circadian typology and risk-taking behavior.MethodsA cross-sectional study was conducted, involving 755 primary and junior school students aged 11–16 years. The Adolescent Risk-Taking Questionnaire and Morningness-Eveningness Questionnaire were administered to assess risk-taking behavior and circadian typology, respectively. Multiple linear regression analyses were performed to analyze the factors influencing risk-taking behavior.ResultsCircadian typology was negatively correlated with risk-taking behavior, with age and sex controlled for (Model 1). When family and class circumstances were added in Model 2, the significance of the association persisted (β = −1.67, 95% CI: [−2.93, −0.42], P = .009). However, the association between circadian typology and risk-taking behavior showed no significance (P = 0.050) when personality characteristics were controlled for in Model 3.ConclusionsThese results provide evidence indicating a relationship between circadian typology and risk-taking behavior, suggesting that evening circadian type is a risk factor for risk-taking behavior in adolescents.     

Altered neocortical tactile but preserved auditory early change detection responses in Friedreich ataxia

ObjectiveTo study using magnetoencephalography (MEG) the spatio-temporal dynamics of neocortical responses involved in sensory processing and early change detection in Friedreich ataxia (FRDA).MethodsTactile (TERs) and auditory (AERs) evoked responses, and early neocortical change detection responses indexed by the mismatch negativity (MMN) were recorded using tactile and auditory oddballs in sixteen FRDA patients and matched healthy subjects. Correlations between the maximal amplitude of each response, genotype and clinical parameters were investigated.ResultsEvoked responses were detectable in all FRDA patients but one. In patients, TERs were delayed and reduced in amplitude, while AERs were only delayed. Only tactile MMN responses at the contralateral secondary somatosensory cortex were altered in FRDA patients. Maximal amplitudes of TERs, AERs and tactile MMN correlated with genotype, but did not correlate with clinical parameters.ConclusionsIn FRDA, the amplitude of tactile MMN responses at SII cortex are reduced and correlate with the genotype, while auditory MMN responses are not altered.SignificanceSomatosensory pathways and tactile early change detection are selectively impaired in FRDA.     

Ictal gamma-band interactions localize ictogenic nodes of the epileptic network in focal cortical dysplasia

ObjectiveEpilepsy surgery fails in > 30% of patients with focal cortical dysplasia (FCD). The seizure persistence after surgery can be attributed to the inability to precisely localize the tissue with an endogenous potential to generate seizures. In this study, we aimed to identify the critical components of the epileptic network that were actively involved in seizure genesis.MethodsThe directed transfer function was applied to intracranial EEG recordings and the effective connectivity was determined with a high temporal and frequency resolution. Pre-ictal network properties were compared with ictal epochs to identify regions actively generating ictal activity and discriminate them from the areas of propagation.ResultsAnalysis of 276 seizures from 30 patients revealed the existence of a seizure-related network reconfiguration in the gamma-band (25–170 Hz; p < 0.005) – ictogenic nodes. Unlike seizure onset zone, resecting the majority of ictogenic nodes correlated with favorable outcomes (p < 0.012).ConclusionThe prerequisite to successful epilepsy surgery is the accurate identification of brain areas from which seizures arise. We show that in FCD-related epilepsy, gamma-band network markers can reliably identify and distinguish ictogenic areas in macroelectrode recordings, improve intracranial EEG interpretation and better delineate the epileptogenic zone.SignificanceIctogenic nodes localize the critical parts of the epileptogenic tissue and increase the diagnostic yield of intracranial evaluation.     

Short-term impact of alcohol detoxification on facial emotions recognition

Background and aimsAlcohol use disorder (AUD) is associated with impaired social cognition, including the disturbance of facial emotion recognition (FER). Previous studies have focused on the assessment of basic emotions decoding among patients with AUD, but the evolution of these performances in the early phase of alcohol withdrawal remains unknown.MethodsThis study was based on evolution of social cognition over a period of 21 days in two groups of individuals: a group of 20 AUD patients and a control group of 25 healthy individuals. AUD patients were tested on admission in a detoxification ward and after a 3-week stay. We evaluated FER with the Reading the Mind in the Eyes Test (RMET). We assessed empathy with a multidimensional questionnaire, the Interpersonal Reactivity Index (IRI). We measured anxiety and depression through the self-rating scale Hospital Anxiety and Depression (HAD). We hypothesized that FER would be impaired in AUD patients on admission and improve after detoxification, while being stable in the control group.ResultsRMET scores on admission and at discharge were inferior in AUD patients to those observed in HC (P = 2 × 10⁻⁶ and P = 0.033, respectively). In the patient group, the RMET score improved over the stay (P = 0.034). A time-by-group interaction for RMET score was observed (P = 0.003). IRI scores on admission were superior in AUD patients (P = 0.023) whichwas no longer observed at discharge (P = 0.54). This suggests that RMET might be more accurate in measuring theory of mind evolution in AUD patients after withdrawal. HAD scores on admission and at discharge were inferior in AUD patients compared to controls (P = 3 × 10⁻⁵ and P = 0.007, respectively). After controlling for HAD initial score, a time-by-group interaction was still observed for RMET scores (P = 0.026).ConclusionFER is impaired in patients with Alcohol Use Disorder compared to controls. This alteration improves after alcohol detoxification. We suggest the RMET could be used to follow the improvement of FER during the first month of abstinence, especially as RMET performance has been associated with maintenance of alcohol withdrawal.     

Nusinersen treatment in adults with severe spinal muscular atrophy: A real-life retrospective observational cohort study

Background and objectivesSpinal muscular atrophy (SMA) is a progressive neurodegenerative disease due to homozygous loss-of-function of the survival motor neuron gene SMN 1 with absence of the functional SMN protein. Nusinersen, a costly intrathecally administered drug approved in 2017 in Europe, induces alternative splicing of the SMN2 gene, which then produces functional SMN protein, whose amount generally increases with the number of SMN2 gene copies.MethodsWe retrospectively collected data from consecutive wheelchair-bound adults with SMA managed at a single center in 2018–2020. The following were collected at each injection, on days 1, 14, 28, 63, 183, and 303: 32-item Motor Function Measurement (MFM) total score and D2 and D3 subscores; the Canadian Occupational Performance Measure (COPM) performance and satisfaction scores; and lung function tests. The patients were divided into two groups based on whether their MFM total score was < or ≥ the mean (15.6%). Adverse events were recorded.ResultsWe identified 18 patients who received 4 to 8 Nusinersen injections. No significant improvements occurred over time in any of the MFM scores or lung function test results, which did not differ between groups. The COPM performance score improved significantly from day 0 to day 303 in the high-MFM group and the COPM satisfaction score in the overall population from D0 to D183. Half the patients achieved the minimal clinically important difference for both COPM scores.DiscussionThe overall stability of conventional motor assessment in this population with advanced disabilities is encouraging to use more sensitive tools based on self-perception and autonomy in daily life activities, such as COPM. Our finding of a significant COPM performance score improvement from days 0 to 303 only in the patients with initial MFM-32 scores above the mean in the population suggests that the severity of the baseline disabilities may affect treatment efficacy.Classification of evidence levelIV, retrospective observational cohort study.     

Safety and efficacy of the Silk flow diverter: Insight from the DIVERSION prospective cohort study

Background and purposeFlow diverters are considered as an essential tool in the stent-based treatment of complex intracranial aneurysms. We report here a subgroup analysis of the nationwide prospective DIVERSION study to investigate the safety and efficacy of the Silk flow diverter at 12 months follow-up.MethodsWe performed a subgroup analysis of patients included in the DIVERSION, a national prospective cohort study including all flow diverters placement between 2012 and 2014 in France, and treated with the Silk. The primary outcome was the morbi-mortality at 12 months, including death, morbidity event and aneurysm retreatment within 12 months post-treatment. All reported serious events were adjudicated by an independent Data Safety and Monitoring Board. Satisfactory occlusion was defined as 3 or 4 on Kamran's scale by an independent imaging core laboratory during follow-up.ResultsA total of 102 procedures involving 101 patients (mean age ± standard deviation, 54.3 ± 13.5 years) harbouring 118 aneurysms (113/118 located in the anterior circulation; mean size 8.2 ± 7.1 mm) were included. During the 12-month follow-up, 34 (33.3%) procedures experienced at least one morbi-mortality event: 3 deaths, 27 morbidity events and 4 retreatments. Overall, 1/3 deaths and 10/27 morbidity events were related to the device and/or the procedure, leading to a specific survival rate and a specific free-morbidity survival rate at 12 months of 98.98% [95% confidence interval, 92.98%–99.86%] and 89.73% [95%CI, 81.71%–94.36%], respectively. The rate of permanent-related neurological deficit was 5.9% within 12 months. One year follow-up imaging showed satisfactory occlusion in 82.2% of cases.ConclusionFlow diversion with the Silk device has a reasonable safety and effectiveness profile for the endovascular treatment of intracranial aneurysms.     

Serial MRI alterations of pediatric patients with beta-propeller protein associated neurodegeneration (BPAN)

Background and PurposeBeta-propeller protein-associated neurodegeneration (BPAN) is one subtype of neurodegeneration with brain iron accumulation. It is difficult to diagnose BPAN due to the non-specificity of their clinical findings and neuroimaging in early childhood. We experienced four pediatric patients with serial brain MRI and evaluated the alteration of the findings through their course.MethodsWe retrospectively reviewed the clinical findings and 21 MRI findings of the four patients with genetically confirmed pediatric BPAN. We also performed a quantitative MR assessment using the quantitative susceptibility mapping (QSM) values of the globus pallidus (GP), substantia nigra (SN), and deep cerebellar nuclei (DCN) compared to 10 age-matched disease controls.ResultsOnly one patient was suspected of BPAN based on imaging findings before the genetic diagnosis was made. The other three patients could not be suspected until their Whole-exome sequencings (WES) done. In all four cases, no abnormal signals were noted in the GP and SN at the initial brain MRI, but hypointensities were observed after the ages of 4–7 years on T2-weighted images and after the ages of 2–7 years on susceptibility-weighted images. In three patients, T2 hyperintensity in the bilateral DCN was persistently observed throughout the observational period. Three patients showed transient T2 hyperintensity and swelling in the GP, SN and/or DCN during the episodes of pyrexia and seizures. The other findings included cerebral and cerebellar atrophy, thinning of the corpus callosum, and delayed myelination. The QSM values of the GP and SN were significantly higher in the patients compared to the controls (P = 0.005, respectively), but that of the DCN did not differ significantly (P = 0.16).ConclusionBrain MRI is a useful method to establish the early diagnosis of BPAN.