A WORLDWIDE ENIGMA STUDY ON EPILEPSY-RELATED GRAY AND WHITE MATTER COMPROMISE ACROSS THE ADULT LIFESPAN.

Objectives: Temporal lobe epilepsy (TLE) is commonly associated with mesiotemporal pathology and widespread alterations of grey and white matter structures. Evidence supports a progressive condition although the temporal evolution of TLE is poorly defined. This ENIGMA-Epilepsy study utilized multimodal magnetic resonance imaging (MRI) data to investigate structural alterations in TLE patients across the adult lifespan. We charted both grey and white matter changes and explored the covariance of age-related alterations in both compartments. Methods: We studied 769 TLE patients and 885 healthy controls across an age range of 17-73 years, from multiple international sites. To assess potentially non-linear lifespan changes in TLE, we harmonized data and combined median split assessments with cross-sectional sliding window analyses of grey and white matter age-related changes. Covariance analyses examined the coupling of grey and white matter lifespan curves. Results: In TLE, age was associated with a robust grey matter thickness/volume decline across a broad cortico-subcortical territory, extending beyond the mesiotemporal disease epicentre. White matter changes were also widespread across multiple tracts with peak effects in temporo-limbic fibers. While changes spanned the adult time window, changes accelerated in cortical thickness, subcortical volume, and fractional anisotropy (all decreased), and mean diffusivity (increased) after age 55 years. Covariance analyses revealed strong limbic associations between white matter tracts and subcortical structures with cortical regions. Conclusions: This study highlights the profound impact of TLE on lifespan changes in grey and white matter structures, with an acceleration of aging-related processes in later decades of life. Our findings motivate future longitudinal studies across the lifespan and emphasize the importance of prompt diagnosis as well as intervention in patients.

Effect of anesthetic strategies on distal stroke thrombectomy in the anterior and posterior cerebral artery.

BACKGROUND: Numerous questions regarding procedural details of distal stroke thrombectomy remain unanswered. This study assesses the effect of anesthetic strategies on procedural, clinical and safety outcomes following thrombectomy for distal medium vessel occlusions (DMVOs). METHODS: Patients with isolated DMVO stroke from the TOPMOST registry were analyzed with regard to anesthetic strategies (ie, conscious sedation (CS), local (LA) or general anesthesia (GA)). Occlusions were in the P2/P3 or A2-A4 segments of the posterior and anterior cerebral arteries (PCA and ACA), respectively. The primary endpoint was the rate of complete reperfusion (modified Thrombolysis in Cerebral Infarction score 3) and the secondary endpoint was the rate of modified Rankin Scale score 0-1. Safety endpoints were the occurrence of symptomatic intracranial hemorrhage and mortality. RESULTS: Overall, 233 patients were included. The median age was 75 years (range 64-82), 50.6% (n=118) were female, and the baseline National Institutes of Health Stroke Scale score was 8 (IQR 4-12). DMVOs were in the PCA in 59.7% (n=139) and in the ACA in 40.3% (n=94). Thrombectomy was performed under LA±CS (51.1%, n=119) and GA (48.9%, n=114). Complete reperfusion was reached in 73.9% (n=88) and 71.9% (n=82) in the LA±CS and GA groups, respectively (P=0.729). In subgroup analysis, thrombectomy for ACA DMVO favored GA over LA±CS (aOR 3.07, 95% CI 1.24 to 7.57, P=0.015). Rates of secondary and safety outcomes were similar in the LA±CS and GA groups. CONCLUSION: LA±CS compared with GA resulted in similar reperfusion rates after thrombectomy for DMVO stroke of the ACA and PCA. GA may facilitate achieving complete reperfusion in DMVO stroke of the ACA. Safety and functional long-term outcomes were comparable in both groups.

Opsoclonus-Myoclonus-Ataxia Syndrome Due to Covid-19.

Opsoclonus-myoclonus syndrome (OMS) as a rare neurological encephalopathic entity associated with non-specific infections or cancer processes has been repeatedly described in the setting of SARS-CoV-2 infection. We report a case of a 53-year-old man with SARS-CoV-2 infection, who developed clinical features of opsoclonus-myoclonus ataxia syndrome including cognitive impairments with a prolonged course of disease. Of particular note, cerebrospinal fluid (CSF) analysis revealed the production of myelin oligodendrocyte glycoprotein (MOG) antibodies, suggesting an underlying neuroimmunological mechanism associated with infection with the novel SARS-CoV-2 virus.

Quantitative Diffusion-Weighted MRI of Neuroblastoma.

Neuroblastoma is the most common extracranial, malignant, solid tumor found in children. In more than one-third of cases, the tumor is in an advanced stage, with limited resectability. The treatment options include resection, with or without (neo-/) adjuvant therapy, and conservative therapy, the latter even with curative intent. Contrast-enhanced MRI is used for staging and therapy monitoring. Diffusion-weighted imaging (DWI) is often included. DWI allows for a calculation of the apparent diffusion coefficient (ADC) for quantitative assessment. Histological tumor characteristics can be derived from ADC maps. Monitoring the response to treatment is possible using ADC maps, with an increase in ADC values in cases of a response to therapy. Changes in the ADC value precede volume reduction. The usual criteria for determining the response to therapy can therefore be supplemented by ADC values. While these changes have been observed in neuroblastoma, early changes in the ADC value in response to therapy are less well described. In this study, we evaluated whether there is an early change in the ADC values in neuroblastoma under therapy; if this change depends on the form of therapy; and whether this change may serve as a prognostic marker. We retrospectively evaluated neuroblastoma cases treated in our institution between June 2007 and August 2014. The examinations were grouped as 'prestaging'; 'intermediate staging'; 'final staging'; and 'follow-up'. A classification of "progress", "stable disease", or "regress" was made. For the determination of ADC values, regions of interest were drawn along the borders of all tumor manifestations. To calculate ADC changes (∆ADC), the respective MRI of the prestaging was used as a reference point or, in the case of therapies that took place directly after previous therapies, the associated previous staging. In the follow-up examinations, the previous examination was used as a reference point. The ∆ADC were grouped into ∆ADCregress for regressive disease, ∆ADCstable for stable disease, and ∆ADC for progressive disease. In addition, examinations at 60 to 120 days from the baseline were grouped as er∆ADCregress, er∆ADCstable, and er∆ADCprogress. Any differences were tested for significance using the Mann-Whitney test (level of significance: p < 0.05). In total, 34 patients with 40 evaluable tumor manifestations and 121 diffusion-weighted MRI examinations were finally included. Twenty-seven patients had INSS stage IV neuroblastoma, and seven had INSS stage III neuroblastoma. A positive N-Myc expression was found in 11 tumor diseases, and 17 patients tested negative for N-Myc (with six cases having no information). 26 patients were assigned to the high-risk group according to INRG and eight patients to the intermediate-risk group. There was a significant difference in mean ADC values from the high-risk group compared to those from the intermediate-risk group, according to INRG. The differences between the mean ∆ADC values (absolute and percentage) according to the course of the disease were significant: between ∆ADCregress and ∆ADCstable, between ∆ADCprogress and ∆ADCstable, as well as between ∆ADCregress and ∆ADCprogress. The differences between the mean er∆ADC values (absolute and percentage) according to the course of the disease were significant: between er∆ADCregress and er∆ADCstable, as well as between er∆ADCregress and er∆ADCprogress. Forms of therapy, N-Myc status, and risk groups showed no further significant differences in mean ADC values and ∆ADC/er∆ADC. A clear connection between the ADC changes and the response to therapy could be demonstrated. This held true even within the first 120 days after the start of therapy: an increase in the ADC value corresponds to a probable response to therapy, while a decrease predicts progression. Minimal or no changes were seen in cases of stable disease.

Association Between Net Water Uptake and Functional Outcome in Patients With Low ASPECTS Brain Lesions: Results From the I-LAST Study.

BACKGROUND AND OBJECTIVES: The effect of mechanical thrombectomy (MT) on functional outcome in patients with ischemic stroke with low ASPECTS is still uncertain. ASPECTS rating is based on the presence of ischemic hypoattenuation relative to normal; however, the degree of hypoattenuation, which directly reflects net uptake of water, is currently not considered an imaging biomarker in stroke triage. We hypothesized that the effect of thrombectomy on functional outcome in low ASPECTS patients depends on early lesion water uptake. METHODS: For this multicenter observational study, patients with anterior circulation stroke with ASPECTS ≤5 were consecutively analyzed. Net water uptake (NWU) was assessed as a quantitative imaging biomarker in admission CT. The primary end point was the rate of favorable functional outcome defined as modified Rankin Scale score 0-3 at day 90. The effect of recanalization on functional outcome was analyzed according to the degree of NWU within the early infarct lesion. RESULTS: A total of 254 patients were included, of which 148 (58%) underwent MT. The median ASPECTS was 4 (interquartile range [IQR] 3-5), and the median NWU was 11.4% (IQR 8.9%-15.1%). The rate of favorable outcome was 27.6% in patients with low NWU (<11.4%) vs 6.3% in patients with high NWU (≥11.4%; p < 0.0001). In multivariable logistic regression analysis, NWU was an independent predictor of outcome, whereas vessel recanalization (modified thrombolysis in cerebral infarction ≥2b) was only significantly associated with better outcomes if NWU was lower than 12.6%. In inverse-probability weighting analysis, recanalization was associated with 20.7% (p = 0.01) increase in favorable outcome in patients with low NWU compared with 9.1% (p = 0.06) in patients with high NWU. DISCUSSION: Early NWU was independently associated with clinical outcome and might serve as an indicator of futile MT in low ASPECTS patients. NWU could be tested as a tool to select low ASPECTS patients for MT. TRIAL REGISTRATION INFORMATION: The study is registered within the ClinicalTrials.gov Protocol Registration and Results System (NCT04862507).

Structural network alterations in focal and generalized epilepsy assessed in a worldwide ENIGMA study follow axes of epilepsy risk gene expression.

Epilepsy is associated with genetic risk factors and cortico-subcortical network alterations, but associations between neurobiological mechanisms and macroscale connectomics remain unclear. This multisite ENIGMA-Epilepsy study examined whole-brain structural covariance networks in patients with epilepsy and related findings to postmortem epilepsy risk gene expression patterns. Brain network analysis included 578 adults with temporal lobe epilepsy (TLE), 288 adults with idiopathic generalized epilepsy (IGE), and 1328 healthy controls from 18 centres worldwide. Graph theoretical analysis of structural covariance networks revealed increased clustering and path length in orbitofrontal and temporal regions in TLE, suggesting a shift towards network regularization. Conversely, people with IGE showed decreased clustering and path length in fronto-temporo-parietal cortices, indicating a random network configuration. Syndrome-specific topological alterations reflected expression patterns of risk genes for hippocampal sclerosis in TLE and for generalized epilepsy in IGE. These imaging-transcriptomic signatures could potentially guide diagnosis or tailor therapeutic approaches to specific epilepsy syndromes.

Topographic divergence of atypical cortical asymmetry and atrophy patterns in temporal lobe epilepsy.

Temporal lobe epilepsy, a common drug-resistant epilepsy in adults, is primarily a limbic network disorder associated with predominant unilateral hippocampal pathology. Structural MRI has provided an in vivo window into whole-brain grey matter structural alterations in temporal lobe epilepsy relative to controls, by either mapping (i) atypical inter-hemispheric asymmetry; or (ii) regional atrophy. However, similarities and differences of both atypical asymmetry and regional atrophy measures have not been systematically investigated. Here, we addressed this gap using the multisite ENIGMA-Epilepsy dataset comprising MRI brain morphological measures in 732 temporal lobe epilepsy patients and 1418 healthy controls. We compared spatial distributions of grey matter asymmetry and atrophy in temporal lobe epilepsy, contextualized their topographies relative to spatial gradients in cortical microstructure and functional connectivity calculated using 207 healthy controls obtained from Human Connectome Project and an independent dataset containing 23 temporal lobe epilepsy patients and 53 healthy controls and examined clinical associations using machine learning. We identified a marked divergence in the spatial distribution of atypical inter-hemispheric asymmetry and regional atrophy mapping. The former revealed a temporo-limbic disease signature while the latter showed diffuse and bilateral patterns. Our findings were robust across individual sites and patients. Cortical atrophy was significantly correlated with disease duration and age at seizure onset, while degrees of asymmetry did not show a significant relationship to these clinical variables. Our findings highlight that the mapping of atypical inter-hemispheric asymmetry and regional atrophy tap into two complementary aspects of temporal lobe epilepsy-related pathology, with the former revealing primary substrates in ipsilateral limbic circuits and the latter capturing bilateral disease effects. These findings refine our notion of the neuropathology of temporal lobe epilepsy and may inform future discovery and validation of complementary MRI biomarkers in temporal lobe epilepsy.

Thrombectomy for secondary distal, medium vessel occlusions of the posterior circulation: seeking complete reperfusion.

BACKGROUND: Whether to approach distal occlusions endovascularly or not in medium-sized vessels secondary to proximal large vessel occlusion stroke remains unanswered. OBJECTIVE: To investigates the technical feasibility and safety of thrombectomy for secondary posterior circulation distal, medium vessel occlusions (DMVO). METHODS: TOPMOST (Treatment fOr Primary Medium vessel Occlusion STroke) is an international, retrospective, multicenter, observational registry of patients treated for distal cerebral artery occlusions. This study subanalysis endovascularly treated occlusions of the posterior cerebral artery in the P2 and P3 segment secondary preprocedural or periprocedural thrombus migration between January 2014 and June 2020. Technical feasibility was evaluated with the modified Thrombolysis in Cerebral Infarction (mTICI) scale. Procedural safety was assessed by the occurrence of symptomatic intracranial hemorrhage (sICH) and intervention-related serious adverse events. RESULTS: Among 71 patients with secondary posterior circulation DMVO who met the inclusion criteria, occlusions were present in 80.3% (57/71) located in the P2 segment and in 19.7% (14/71) in the P3 segment. Periprocedural migration occurred in 54.9% (39/71) and preprocedural migration in 45.1% (32/71) of cases. The first reperfusion attempt led in 38% (27/71) of all cases to mTICI 3. On multivariable logistic regression analysis, increased numbers of reperfusion attempts (adjusted odds ratio (aOR)=0.39, 95% CI 0.29 to 0.88, p=0.009) and preprocedural migration (aOR=4.70, 95% CI,1.35 to 16.35, p=0.015) were significantly associated with mTICI 3. sICH occurred in 2.8% (2/71). CONCLUSION: Thrombectomy for secondary posterior circulation DMVO seems to be safe and technically feasible. Even though thrombi that have migrated preprocedurally may be easier to retract, successful reperfusion can be achieved in the majority of patients with secondary DMVO of the P2 and P3 segment.

CT imaging-based approaches to cochlear duct length estimation-a human temporal bone study.

OBJECTIVES: Knowledge about cochlear duct length (CDL) may assist electrode choice in cochlear implantation (CI). However, no gold standard for clinical applicable estimation of CDL exists. The aim of this study is (1) to determine the most reliable radiological imaging method and imaging processing software for measuring CDL from clinical routine imaging and (2) to accurately predict the insertion depth of the CI electrode. METHODS: Twenty human temporal bones were examined using different sectional imaging techniques (high-resolution computed tomography [HRCT] and cone beam computed tomography [CBCT]). CDL was measured using three methods: length estimation using (1) a dedicated preclinical 3D reconstruction software, (2) the established A-value method, and (3) a clinically approved otosurgical planning software. Temporal bones were implanted with a 31.5-mm CI electrode and measurements were compared to a reference based on the CI electrode insertion angle measured by radiographs in Stenvers projection (CDLreference). RESULTS: A mean cochlear coverage of 74% (SD 7.4%) was found. The CDLreference showed significant differences to each other method (p < 0.001). The strongest correlation to the CDLreference was found for the otosurgical planning software-based method obtained from HRCT (CDLSW-HRCT; r = 0.87, p < 0.001) and from CBCT (CDLSW-CBCT; r = 0.76, p < 0.001). Overall, CDL was underestimated by each applied method. The inter-rater reliability was fair for the CDL estimation based on 3D reconstruction from CBCT (CDL3D-CBCT; intra-class correlation coefficient [ICC] = 0.43), good for CDL estimation based on 3D reconstruction from HRCT (CDL3D-HRCT; ICC = 0.71), poor for CDL estimation based on the A-value method from HRCT (CDLA-HRCT; ICC = 0.29), and excellent for CDL estimation based on the A-value method from CBCT (CDLA-CBCT; ICC = 0.87) as well as for the CDLSW-HRCT (ICC = 0.94), CDLSW-CBCT (ICC = 0.94) and CDLreference (ICC = 0.87). CONCLUSIONS: All approaches would have led to an electrode choice of rather too short electrodes. Concerning treatment decisions based on CDL measurements, the otosurgical planning software-based method has to be recommended. The best inter-rater reliability was found for CDLA-CBCT, for CDLSW-HRCT, for CDLSW-CBCT, and for CDLreference. KEY POINTS: • Clinically applicable calculations using high-resolution CT and cone beam CT underestimate the cochlear size. • Ten percent of cochlear duct length need to be added to current calculations in order to predict the postoperative CI electrode position. • The clinically approved otosurgical planning software-based method software is the most suitable to estimate the cochlear duct length and shows an excellent inter-rater reliability.

CT Hypoperfusion-Hypodensity Mismatch to Identify Patients With Acute Ischemic Stroke Within 4.5 Hours of Symptom Onset.

BACKGROUND AND OBJECTIVES: To test the hypothesis that CT hypoperfusion-hypodensity mismatch identifies patients with ischemic stroke within 4.5 hours of symptom onset. METHODS: We therefore performed the Retrospective Multicenter Hypoperfusion-Hypodensity Mismatch for The identification of Patients With Stroke Within 4.5 Hours study of patients with acute ischemic stroke and known time of symptom onset. The predictive values of hypoperfusion-hypodensity mismatch for the identification of patients with symptom onset within 4.5 hours were the main outcome measure. RESULTS: Of 666 patients, 548 (82.3%) had multimodal CT within 4.5 hours and 118 (17.7%) beyond 4.5 hours. Hypoperfusion-hypodensity mismatch was visible in 516 (94.2%) patients with symptom onset within and in 30 (25.4%) patients beyond 4.5 hours. CT hypoperfusion-hypodensity mismatch identified patients within 4.5 hours of stroke onset with 94.2% (95% confidence interval [CI] 91.9%-95.8%) sensitivity, 74.6% (95% CI 66.0%-81.6%) specificity, 94.5% (95% CI 92.3%-96.1%) positive predictive value, and 73.3% (95% CI 64.8%-80.4%) negative predictive value. Interobserver agreement for hypoperfusion-hypodensity mismatch was substantial (κ = 0.61, 95% CI 0.53-0.69). DISCUSSION: Patients with acute ischemic stroke with absence of a hypodensity on native CT (NCCT) within the hypoperfused core lesion on perfusion CT (hypoperfusion-hypodensity mismatch) are likely to be within the time window of thrombolysis. Applying this method may guide the decision to use thrombolysis in patients with unknown time of stroke onset. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov Identifier: NCT04277728. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that CT hypoperfusion-hypodensity mismatch identifies patients with stroke within 4.5 hours of onset.

Artificial intelligence for classification of temporal lobe epilepsy with ROI-level MRI data: A worldwide ENIGMA-Epilepsy study.

Artificial intelligence has recently gained popularity across different medical fields to aid in the detection of diseases based on pathology samples or medical imaging findings. Brain magnetic resonance imaging (MRI) is a key assessment tool for patients with temporal lobe epilepsy (TLE). The role of machine learning and artificial intelligence to increase detection of brain abnormalities in TLE remains inconclusive. We used support vector machine (SV) and deep learning (DL) models based on region of interest (ROI-based) structural (n = 336) and diffusion (n = 863) brain MRI data from patients with TLE with ("lesional") and without ("non-lesional") radiographic features suggestive of underlying hippocampal sclerosis from the multinational (multi-center) ENIGMA-Epilepsy consortium. Our data showed that models to identify TLE performed better or similar (68-75%) compared to models to lateralize the side of TLE (56-73%, except structural-based) based on diffusion data with the opposite pattern seen for structural data (67-75% to diagnose vs. 83% to lateralize). In other aspects, structural and diffusion-based models showed similar classification accuracies. Our classification models for patients with hippocampal sclerosis were more accurate (68-76%) than models that stratified non-lesional patients (53-62%). Overall, SV and DL models performed similarly with several instances in which SV mildly outperformed DL. We discuss the relative performance of these models with ROI-level data and the implications for future applications of machine learning and artificial intelligence in epilepsy care.

Evaluating Common Cavity Cochlear Deformities Using CT Images and 3D Reconstruction.

OBJECTIVES: The aim of this study is to compare the common cavity (CC) with the normal anatomy inner ear in order to evaluate whether the cavity is representing both the cochlear and the vestibular parts of the inner ear and to revisit CC deformity from a three-dimensional (3D) perspective. METHODS: High-resolution computed tomography image datasets of 17 temporal bones initially identified as CC were evaluated with 3D reconstruction and multiplanar image analysis using a free available software for 3D segmentation of the inner ear. All 3D images of CC were compared to a normal inner ear. Maximum and minimum diameter of the CC were correlated with the circumference of the CC in an axial plane. RESULTS: In 13 cases (76%), CC represented only the vestibular part of the inner ear and did not represent CC as defined here and by Sennaroglu, Kontorinis, and Khan. True CC was correctly diagnosed in only one case (6%). In three cases (18%), a rudimentary part of the cochlear portion could be identified. The axes' length of the elliptical cavity showed a strong positive linear relation to the circumference of the cavity (long axis: r = 0.94; P < .0001; short axis: r = 0.68; P = .0029). CONCLUSION: This study supports the assumption that many reported CC cases only represent the vestibular part of the inner ear and are therefore cases of cochlear aplasia. 3D segmentation and systematic analysis of CT-imaging add clinical value to the comprehension of the morphology of the anatomical structures of the inner ear. LEVEL OF EVIDENCE: 2C Laryngoscope, 131:386-391, 2021.

Mapping Cochlear Duct Length to Electrically Evoked Compound Action Potentials in Cochlear Implantation.

OBJECTIVE: Objective measurements may assist in indicating cochlear implants and in predicting outcomes of cochlear implantation surgery. Using electrically evoked compound action potentials (ECAP), information about the function of the auditory nerve can be obtained by analyzing responses to electrical stimulation transmitted and derived by the recording electrode. The aim of this study was to determine whether ECAP characteristics differ depending on the stimulated intracochlear region and the size of the cochlea. STUDY DESIGN: Retrospective cohort study. SETTING: University Medical center, tertiary academic referral center. PATIENTS: Patients undergoing cochlear implant surgery between 2015 and 2018. INTERVENTION: Cochlear implantation with FLEXsoft electrode arrays (length 31.5 mm, 12 stimulating channels). MAIN OUTCOME MEASURES: The cochlear duct length (CDL) and the cochlear coverage (CC) were measured using a new computed tomography-based software and correlated to the postoperative speech performance. Additionally, ECAP were measured and associated to the CDL. RESULTS: A total of 59 ears of 53 cochlear implant users with a mean age of 63.6 (SD 14.9) years were included. The mean estimated CDL was 35.0 (SD 2.2) mm. The mean CC was 90.3% (SD 5.5%). A total of 4,873 ECAP were measured. A statistically significant, moderate, negative correlation between the ECAP slope and the site of stimulation was found (r = -0.29, 95% confidence interval: -0.32 to -0.27, p < 0.0001). No correlation between the CC and the speech performance was found (r = -0.08, 95% confidence interval: -0.33 to 0.18 p = 0.52). CONCLUSION: ECAP slopes seem to be a reliable tool to identify the electrode's position inside the cochlea and also showed correlations to the anatomy of the patient. A combination of objective measurements such as anatomical parameters and ECAPs are helpful to assist the postoperative fitting and are promising tools to improve patient care.

Artificial Intelligence and Big Data. / Künstliche Intelligenz und Big Data.

Medical images play an important role in ophthalmology and radiology. Medical image analysis has greatly benefited from the application of "deep learning" techniques in clinical and experimental radiology. Clinical applications and their relevance for radiological imaging in ophthalmology are presented.

Network-based atrophy modeling in the common epilepsies: A worldwide ENIGMA study.

Epilepsy is increasingly conceptualized as a network disorder. In this cross-sectional mega-analysis, we integrated neuroimaging and connectome analysis to identify network associations with atrophy patterns in 1021 adults with epilepsy compared to 1564 healthy controls from 19 international sites. In temporal lobe epilepsy, areas of atrophy colocalized with highly interconnected cortical hub regions, whereas idiopathic generalized epilepsy showed preferential subcortical hub involvement. These morphological abnormalities were anchored to the connectivity profiles of distinct disease epicenters, pointing to temporo-limbic cortices in temporal lobe epilepsy and fronto-central cortices in idiopathic generalized epilepsy. Negative effects of age on atrophy further revealed a strong influence of connectome architecture in temporal lobe, but not idiopathic generalized, epilepsy. Our findings were reproduced across individual sites and single patients and were robust across different analytical methods. Through worldwide collaboration in ENIGMA-Epilepsy, we provided deeper insights into the macroscale features that shape the pathophysiology of common epilepsies.

White matter abnormalities across different epilepsy syndromes in adults: an ENIGMA-Epilepsy study.

The epilepsies are commonly accompanied by widespread abnormalities in cerebral white matter. ENIGMA-Epilepsy is a large quantitative brain imaging consortium, aggregating data to investigate patterns of neuroimaging abnormalities in common epilepsy syndromes, including temporal lobe epilepsy, extratemporal epilepsy, and genetic generalized epilepsy. Our goal was to rank the most robust white matter microstructural differences across and within syndromes in a multicentre sample of adult epilepsy patients. Diffusion-weighted MRI data were analysed from 1069 healthy controls and 1249 patients: temporal lobe epilepsy with hippocampal sclerosis (n = 599), temporal lobe epilepsy with normal MRI (n = 275), genetic generalized epilepsy (n = 182) and non-lesional extratemporal epilepsy (n = 193). A harmonized protocol using tract-based spatial statistics was used to derive skeletonized maps of fractional anisotropy and mean diffusivity for each participant, and fibre tracts were segmented using a diffusion MRI atlas. Data were harmonized to correct for scanner-specific variations in diffusion measures using a batch-effect correction tool (ComBat). Analyses of covariance, adjusting for age and sex, examined differences between each epilepsy syndrome and controls for each white matter tract (Bonferroni corrected at P < 0.001). Across 'all epilepsies' lower fractional anisotropy was observed in most fibre tracts with small to medium effect sizes, especially in the corpus callosum, cingulum and external capsule. There were also less robust increases in mean diffusivity. Syndrome-specific fractional anisotropy and mean diffusivity differences were most pronounced in patients with hippocampal sclerosis in the ipsilateral parahippocampal cingulum and external capsule, with smaller effects across most other tracts. Individuals with temporal lobe epilepsy and normal MRI showed a similar pattern of greater ipsilateral than contralateral abnormalities, but less marked than those in patients with hippocampal sclerosis. Patients with generalized and extratemporal epilepsies had pronounced reductions in fractional anisotropy in the corpus callosum, corona radiata and external capsule, and increased mean diffusivity of the anterior corona radiata. Earlier age of seizure onset and longer disease duration were associated with a greater extent of diffusion abnormalities in patients with hippocampal sclerosis. We demonstrate microstructural abnormalities across major association, commissural, and projection fibres in a large multicentre study of epilepsy. Overall, patients with epilepsy showed white matter abnormalities in the corpus callosum, cingulum and external capsule, with differing severity across epilepsy syndromes. These data further define the spectrum of white matter abnormalities in common epilepsy syndromes, yielding more detailed insights into pathological substrates that may explain cognitive and psychiatric co-morbidities and be used to guide biomarker studies of treatment outcomes and/or genetic research.

Differentiation of retropharyngeal calcific tendinitis and retropharyngeal abscess: a case series and review of the literature.

INTRODUCTION: Retropharyngeal calcific tendinitis (RCT) is a self-limiting aseptic inflammation of the tendon of the longus colli muscle, which can be clinically and radiologically misdiagnosed as abscess formation. This is a particular challenge for ENT specialists. However, articles about RCT are highly underrepresented in ENT journals and existing articles in ENT journals almost exclusively report overtreatment. METHODS: This study presents five patients, in which the diagnosis of RCT was delayed and of which one patient underwent incision and draining of a suspected retropharyngeal abscess under general anesthesia. In addition, the literature on the reported cases of RCT, between 1990 and 2020 was reviewed. For each case, epidemiological characteristics, complaints on presentation, symptoms, imaging and laboratory finding and treatment were summarized and compared to our own findings. RESULTS: In all the five patients, the correct diagnosis was delayed. One patient underwent incision and draining of a suspected RA under general anesthesia. All patients received antibiotic treatment. The literature review revealed a total of 116 reported cases of RCT. A total of 99 CT scans and 72 MRI showed soft tissue swelling in 89.6% and calcifications in 91.4% of the cases, 6.9% received invasive treatment. CONCLUSION: This article emphasizes the importance of knowledge about RCT and its management to avoid invasive and potentially harmful treatment. The focus in establishing the correct diagnosis of RCT is the identification and correct interpretation of clinical symptoms together with the specific radiological findings.

Ischemic lesion growth in acute stroke: Water uptake quantification distinguishes between edema and tissue infarct.

Infarct growth from the early ischemic core to the total infarct lesion volume (LV) is often used as an outcome variable of treatment effects, but can be overestimated due to vasogenic edema. The purpose of this study was (1) to assess two components of early lesion growth by distinguishing between water uptake and true net infarct growth and (2) to investigate potential treatment effects on edema-corrected net lesion growth. Sixty-two M1-MCA-stroke patients with acute multimodal and follow-up CT (FCT) were included. Ischemic lesion growth was calculated by subtracting the initial CTP-derived ischemic core volume from the LV in the FCT. To determine edema-corrected net lesion growth, net water uptake of the ischemic lesion on FCT was quantified and subtracted from the volume of uncorrected lesion growth. The mean lesion growth without edema correction was 20.4 mL (95% CI: 8.2-32.5 mL). The mean net lesion growth after edema correction was 7.3 mL (95% CI: -2.1-16.7 mL; p < 0.0001). Lesion growth was significantly overestimated due to ischemic edema when determined in early-FCT imaging. In 18 patients, LV was lower than the initial ischemic core volume by CTP. These apparently "reversible" core lesions were more likely in patients with shorter times from symptom onset to imaging and higher recanalization rates.

Surgical approach for complete cochlear coverage in EAS-patients after residual hearing loss.

INTRODUCTION: In cases with residual-hearing (RH) loss after cochlear implantation, a safe method is needed to provide full spectral resolution and as much auditory information as possible without implant replacement. Aim of this study was to prove the feasibility of accessing a partially inserted cochlear-implant-electrode for complete insertion to its maximum length through the external ear canal using a transcanal approach. METHODS: Two CI electrodes were customized with 18 stimulating channels. The electrode design enables the use of 12 active channels available for electrical stimulation inside the cochlea both after partial and full insertion. 10 CI electrodes were implanted in 10 fresh human cadaveric temporal bones. After initial partial insertion by posterior tympanotomy, the electrode was inserted to its maximum length via a transcanal approach. Radiographs and CT scans were performed to confirm the electrode position. The electrodes were investigated via x-ray after removal. RESULTS: X-ray and CT-scans confirmed the electrode prototypes covering an angular insertion depth between 236° to 307° after initial insertion. Accessing the electrode in the middle ear space was feasible and insertion to its full length was successful. Post-insertion CT confirmed insertion of the 28mm and 31.5mm electrode arrays covering an angular insertion depth between 360° and 540° respectively. No tip foldovers were detected. CONCLUSION: This study confirms the feasibility of extending the electrode insertion to its maximum insertion length using a transcanal approach in temporal bone specimens. This constitutes a second stage procedure on demand in EAS-surgery. This may be beneficial for EAS-patients providing electrical stimulation beyond the basal turn of the cochlea once the functional residual hearing is lost, without replacing the entire CI.

ERASER.

Background and Purpose- Using a novel study design with virtual comparators based on predictive modeling, we investigated whether next-generation mechanical thrombectomy devices improve outcomes in patients with ischemic stroke. We hypothesized that this new study design shows that a next-generation mechanical thrombectomy system is superior to intravenous tPA (tissue-type plasminogen activator) therapy (IVT) alone. Methods- ERASER (Eric Acute Stroke Recanalization) was an investigator-initiated, prospective, multicenter, single-arm (virtual 2-arm) study that evaluated the effectiveness of a new recanalization device together with a specific intermediate catheter (Embolus Retriever with Interlinked Cages/SOFIA, Microvention) in stroke patients with internal carotid artery or middle cerebral artery occlusions. The primary end point was the volume of saved tissue. Volume of saved tissue was defined as the difference of actual infarct volume and brain volume predicted to develop infarction using a machine learning model based on data from intravenous tPA therapy patients. Results- Eighty-one patients were enrolled. The median patient age was 71 years (interquartile range, 61-77). National Institutes of Health Stroke Scale score was 14 (interquartile range, 12-18). The actual infarct volume was smaller than predicted by the intravenous tPA therapy model, with a median volume of saved tissue of 50 mL (interquartile range, 19-103; P<0.0001). Good clinical outcome (modified Rankin Scale, 0-2 at 90 days) was observed in 48 out of 69 (70%). The recanalization rate (Thrombolysis in Cerebral Infarction 2b/3) was 95%. Conclusions- ERASER is the first mechanical thrombectomy study with a primary end point based on predictive analytics enabling intraindividual virtual comparisons. The next-generation mechanical thrombectomy method resulted in smaller infarcts than predicted after intravenous tPA therapy alone and showed a high rate of good clinical outcome. The novel study design with virtual comparisons is promising for further application and testing in the neurovascular arena. Clinical Trial Registration- URL: https://www.clinicaltrials.gov . Unique identifier: NCT02534701.