关键词： Intradural   Extramedullary   Meningioma   Multinomial logistic regression   Spinal cord  

摘要： Among many factors leading to a worse functional prognosis in spinal meningioma (SM) surgery, in a previous study, we recognized anterior/anterolateral axial topography, sphincter involvement at first evaluation, surgery performed on a recurrence, and worse preoperative functional status. The purpose of this paper is to evaluate the cumulative weight of these factors on prognosis through a multinomial logistic regression model performed on an original evaluation scale designed by the authors on the ground of the experience of the neurosurgical departments of our University. The original SM database composed of 173 cases was classified in regard to sex, age, symptoms, axial and sagittal location, Simpson grade resection, and functional pre/postoperative status. Fine presurgical and follow-up reevaluations were available. The authors propose a scale (Spinal Meningiomas Prognostic Evaluation Score (SPES)) of preoperative evaluation to assess the surgery-related risk of neurological worsening experienced by the patients included in the present cohort. The authors describe a strong statistical association between the SPES and the follow-up Frankel and McCormick scores (r=-460 and .441, p .001, both). Through a univariate ANOVA analysis, we disclosed that patients presenting scores 2 and 3 had a significantly higher association to lesser Frankel and McCormick postoperative scores, in respect to patients presenting SPES scores 0-1 (univariate ANOVA, p .008 and .011). Anterior or anterolateral axial location, operating on a recurrence of SM, sphincter involvement, and worse functional grade at onset present, along with the SPES scores are fairly predictive and reliable in respect to the long-term results of patients suffering from SM.

关键词： CT   image fusion   MR   multimodal   SPECT  

摘要： Multimodal medical image fusion (MMIF) has a significant role for a better visualization of the diagnostic statistics, those help the medical professionals in the precise diagnosis of several critical diseases. This paper presents an improved fusion framework that uses the entire features extracted by the nonsubsampled shearlet transform (NSST) and adaptive biologically inspired neural model. The proposed scheme retains the required information without losing the resolution of the disease morphology. In the proposed method, the adaptive neural model based on local visibility and log Gabor energy based rules are applied to low and high-frequency components, respectively. The better fusion results obtained by the proposed approach are confirmed by a large extent of simulations on the different MR-SPECT and CT-MR neurological images. Based on all these simulated results, it states that the proposed approach is superior than the other approaches as it produces better visually fused images with improved computational measures.

关键词： spinal anaesthesia   local anaesthetics   bupivacaine   extended-release preparations   liposomes  

摘要： Background: An injectable liposomal bupivacaine suspension (EXPAREL (TM)) is approved by the US Food and Drug Administration for analgesia by tissue infiltration and interscalene brachial plexus, but not for use in the neuraxial space. This pilot study describes neurological and histological outcomes of escalating doses of this extended-release formulation of bupivacaine after subarachnoid administration. Methods: Twenty-five pigs (Sus scrofa domesticus) weighing 36.2 (4.4) kg were randomly assigned to one of five groups to receive a subarachnoid injection of sodium chloride 0.9%, 3 ml (negative control), preservative-free bupivacaine hydrochloride 0.5%, 3 ml (positive control), or one of three doses of liposomal bupivacaine suspension 1.33%: 1.5, 3, or 5 ml. After recovering from general anaesthesia, neurological outcomes were assessed by blinded observers. Three weeks later, the animals were sacrificed for histological evaluations of neurotoxicity. Results: Animals that received sodium chloride 0.9%, bupivacaine hydrochloride, or liposomal bupivacaine 1.5 ml recovered within 2, 5, or 4 h, respectively. Animals that received liposomal bupivacaine 3 or 5 ml exhibited signs of neuraxial block (decreased nociception and proprioception) up to 32 h after injection. No histological evidence of neurotoxicity was found in any of the groups. Conclusions: Subarachnoid administration of liposomal bupivacaine in pigs exhibited a dose-response effect, and resulted in longer duration of neuraxial block than bupivacaine hydrochloride without histological evidence of neurotoxicity. Our study contributes preliminary data to inform further toxicological assessments and regulatory approval before subarachnoid administration in humans.

关键词： Lumbar interbody fusion   Intraoperative monitoring   Somatosensory evoked potentials   Neurological deficit  

摘要： Background: Lumbar interbody spinal fusion (LIF) surgeries are performed to treat or prevent back pain in patients with degenerated intervertebral discs and a variety of spinal diseases. However, post-operative neurological complications may ensue. Intraoperative monitoring techniques have been used to predict and potentially reduce the risk of complications. Methods: This study examined the diagnostic accuracy of significant changes of somatosensory evoked potentials (SSEPs) to evaluate and predict post-operative neurological deficits after LIF. All patients underwent LIF at UPMC from 2010 to 2012. One thousand fifty-seven patients had pre-operative baseline and continuous intraoperative SSEP monitoring. Statistical analysis was completed using SPSS version 22. No relevant disclosure. Results: Patient outcomes were not significantly affected by age over 65, gender, obesity, and abnormal baselines. Lower extremity (LE) significant changes in SSEPs and LE loss of responses resulted in a sensitivity/specificity of 0.03/0.99 and 0.03/0.99;they had an AUC of 0.54/0.73 with a 95% confidence interval (CI) of [0.34, 0.74]/[0.29, 1.00]. Conclusions: Significant SSEP changes during LIF are a very specific but poorly sensitive indicator of peri-operative neurological deficits. The odds ratio for LE loss of responses was 29.14 with a 95% CI of 1.79-475.5, so LE SSEP loss of responses can serve as a biomarker of perioperative neurological deficits after LIF. Published by Elsevier Ltd.

关键词： Acoustic Neuroma   Hearing Disorders   Internal Auditory Canal   Cranial Nerves   Cerebellopontine Angle   Patients   Common Neoplasm   Hemorrhage   Tumours  

摘要： Vestibular schwannomas (VS) are the most common tumors involving the cerebellopontine angle (CPA) and the internal auditory canal (IAC). These tumors are usually slow-growing and commonly present with cranial nerve dysfunction such as hearing loss. Repeated intratumoral hemorrhage (ITH) is extremely rare with only four cases previously reported. We report the case of a 30 year old female with a right sided CP angle tumor who presented with vertigo and ataxia due to ITH. Her symptoms initially improved;however, three weeks later, she had acute onset of facial palsy and imaging confirmed rebleeding. Surgical pathology reported typical features of schwannoma. A literature review performed using the PubMed and EMBASE databases yielded four previous reports. A summary of these cases is presented and the features of ITH are discussed. Patients affected by repeated ITH present with sudden headache and ataxia. Rapid worsening of cranial nerve dysfunction such as hearing loss or facial nerve palsy is suggestive of ITH. (C) 2018 Elsevier Ltd. All rights reserved.

关键词： TCF20   22q13   Neurodevelopmental disorders   Smith-Magenis syndrome   Haploinsufficiency   Loss-of-function variants   Deletions  

摘要： BackgroundNeurodevelopmental disorders are genetically and phenotypically heterogeneous encompassing developmental delay (DD), intellectual disability (ID), autism spectrum disorders (ASDs), structural brain abnormalities, and neurological manifestations with variants in a large number of genes (hundreds) associated. To date, a few de novo mutations potentially disrupting TCF20 function in patients with ID, ASD, and hypotonia have been reported. TCF20 encodes a transcriptional co-regulator structurally related to RAI1, the dosage-sensitive gene responsible for Smith-Magenis syndrome (deletion/haploinsufficiency) and Potocki-Lupski syndrome (duplication/triplosensitivity).MethodsGenome-wide analyses by exome sequencing (ES) and chromosomal microarray analysis (CMA) identified individuals with heterozygous, likely damaging, loss-of-function alleles in TCF20. We implemented further molecular and clinical analyses to determine the inheritance of the pathogenic variant alleles and studied the spectrum of *** report 25 unique inactivating single nucleotide variants/indels (1 missense, 1 canonical splice-site variant, 18 frameshift, and 5 nonsense) and 4 deletions of TCF20. The pathogenic variants were detected in 32 patients and 4 affected parents from 31 unrelated families. Among cases with available parental samples, the variants were de novo in 20 instances and inherited from 4 symptomatic parents in 5, including in one set of monozygotic twins. Two pathogenic loss-of-function variants were recurrent in unrelated families. Patients presented with a phenotype characterized by developmental delay, intellectual disability, hypotonia, variable dysmorphic features, movement disorders, and sleep ***20 pathogenic variants are associated with a novel syndrome manifesting clinical characteristics similar to those observed in Smith-Magenis syndrome. Together with previously described cases, the clinical entity of TCF20-associated neurodev

关键词： Autoimmune diseases   Biology and life sciences   Cerebrovascular diseases   Clinical immunology   Clinical medicine   Clinical trials   Critical care and emergency medicine   Diagnostic medicine   Diarrhea   Drug research and development   Gastroenterology and hepatology   Guillain-Barre syndrome   Immunology   Inflammatory diseases   Medicine and health sciences   Neurology   Neurotrauma   Pathology and laboratory medicine   Pharmacology   Prognosis   Randomized controlled trials   Research and analysis methods   Research Article   Signs and symptoms   Stroke   Trauma medicine   Traumatic brain injury   Traumatic injury   Vascular medicine  

摘要： Background Randomized controlled trials (RCTs) pose specific challenges in rare and heterogeneous neurological diseases due to the small numbers of patients and heterogeneity in disease course. Two analytical approaches have been proposed to optimally handle these issues in RCTs: covariate adjustment and ordinal analysis. We investigated the potential gain in efficiency of these approaches in rare and heterogeneous neurological diseases, using Guillain-Barre syndrome (GBS) as an example. Methods We analyzed two published GBS trials with primary outcome 'at least one grade improvement' on the GBS disability scale. We estimated the treatment effect using logistic regression models with and without adjustment for prognostic factors. The difference between the unadjusted and adjusted estimates was disentangled in imbalance (random differences in baseline covariates between treatment arms) and stratification (change of the estimate due to covariate adjustment). Second, we applied proportional odds regression, which exploits the ordinal nature of the GBS disability score. The standard error of the estimated treatment effect indicated the statistical efficiency. Results Both trials were slightly imbalanced with respect to baseline characteristics, which was corrected in the adjusted analysis. Covariate adjustment increased the estimated treatment effect in the two trials by 8% and 18% respectively. Proportional odds analysis resulted in lower standard errors indicating more statistical power. Conclusion Covariate adjustment and proportional odds analysis most efficiently use the available data and ensure balance between the treatment arms to obtain reliable and valid treatment effect estimates. These approaches merit application in future trials in rare and heterogeneous neurological diseases like GBS.

关键词： SLEEP apnea syndromes   NEUROSCIENCES   LETTERS to the editor   HYDROCEPHALUS  

摘要： Magnetic resonance elastography (MRE) is an imaging technique for noninvasively and quantitatively assessing tissue stiffness, akin to palpation. MRE is further able assess the mechanical properties of tissues that cannot be reached by hand including the brain. The technique is a three-step process beginning with the introduction of shear waves into the tissue of interest by applying an external vibration. Next, the resulting motion is imaged using a phase-contrast MR pulse sequence with motion encoding gradients that are synchronized to the vibration. Finally, the measured displacement images are mathematically inverted to compute a map of the estimated stiffness. In the brain, the technique has demonstrated strong test-retest repeatability with typical errors of 1% for measuring global stiffness, 2% for measuring stiffness in the lobes of the brain, and 3-7% for measuring stiffness in subcortical gray matter. In healthy volunteers, multiple studies have confirmed that stiffness decreases with age, while more recent studies have demonstrated a strong relationship between viscoelasticity and behavioral performance. Furthermore, several studies have demonstrated the sensitivity of brain stiffness to neurodegeneration, as stiffness has been shown to decrease in multiple sclerosis and in several forms of dementia. Moreover, the spatial pattern of stiffness changes varies among these different classes of dementia. Finally, MRE is a promising tool for the preoperative assessment of intracranial tumors, as it can measure both tumor consistency and adherence to surrounding tissues. These factors are important predictors of surgical difficulty. In brief, MRE demonstrates potential value in a number of neurological diseases. However, significant opportunity remains to further refine the technique and better understand the underlying physiology.

关键词： Multiple sclerosis   Neutrophil-to-Lympocyte ratio   Monocyte-to-Lympocyte ratio   Brain atrophy   MRI   Patient-reported outcomes  

摘要： Background: Serum hematological indices such as the neutrophil-lymphocyte ratio (NLR) or monocyte-lymphocyte ratio (MLR) have been used as biomarkers of pathogenic inflammation and prognostication in multiple areas of medicine;recent evidence shows correlation with psychological parameters as well. Objectives/Aims: To characterize clinical, neuroimaging, and psycho-neuro-immunological associations with NLR and MLR in persons with multiple sclerosis (MS). Methods: We identified a large cohort of clinically well-defined patients from our longitudinal database that included MS-related outcomes, disease-modifying therapy, patient-reported outcome (PRO) measures, and quantified cerebral MRI at 1.5T. We queried hospital records for complete blood counts within 2months of each clinic visit and excluded those obtained during clinical relapses. Four hundred eighty-three patients, with a mean of 3 longitudinal observations each, were identified who met these criteria. Initial analyses assessed the association between NLR and MLR as the outcomes, and psychological and demographic predictors in univariable and multivariable models controlling for age, gender and treatment. The second set of analyses assessed the association between clinical and MRI outcomes including whole brain atrophy and T2-hyperintense lesion volume, with NLR and MLR as predictors in univariable and multivariable models. All analyses used a mixed effects linear or logistic regression model with repeated measures. Results: Unadjusted analyses demonstrated significant associations between higher (log-transformed) NLR (but not MLR) and PRO measures including increasing depression (p=0.01), fatigue (p<0.01), and decreased physical quality of life (p<0.01). Higher NLR and MLR strongly predicted increased MS-related disability as assessed by the Expanded Disability Status Scale, independent of all demographic, clinical, treatment-related, and psychosocial variables (p<0.001). Lastly, higher NLR and MLR significan