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STUDY DESIGN Global cross-sectional survey. OBJECTIVE To validate the AO Spine Subaxial Cervical Spine Injury Classification by examining the perceived injury severity by surgeon across AO geographical regions and practice experience. SUMMARY OF BACKGROUND DATA Previous subaxial cervical spine injury classifications have been limited by subpar interobserver reliability and clinical applicability. In an attempt to create a universally validated scheme with prognostic value, AO Spine established a subaxial cervical spine injury classification involving four elements: (1) injury morphology, (2) facet injury involvement, (3) neurologic status, and (4) case-specific modifiers. METHODS A survey was sent to 272 AO Spine members across all geographic regions and with a variety of practice experience. Respondents graded the severity of each variable of the classification system on a scale from zero (low severity) to 100 (high severity). Primary outcome was to assess differences in perceived injury severity for each injury type over geographic regions and level of practice experience. RESULTS A total of 189 responses were received. Overall, the classification system exhibited a hierarchical progression in subtype injury severity scores. Only three subtypes showed a significant difference in injury severity score among geographic regions: F3 (floating lateral mass fracture, p:0.04), N3 (incomplete spinal cord injury, p:0.03), and M2 (critical disk herniation, p:0.04). When stratified by surgeon experience, pairwise comparison showed only 2 morphological subtypes, B1 (bony posterior tension band injury, p:0.02) and F2 (unstable facet fracture, p:0.03), and one neurologic subtype (N3, p:0.02) exhibited a significant difference in injury severity score. CONCLUSIONS The AO Spine Subaxial Cervical Spine Injury Classification System has shown to be reliable and suitable for proper patient management. The study shows this classification is substantially generalizable by geographic region and surgeon experience; and provides a consistent method of communication among physicians while covering the majority of subaxial cervical spine traumatic injuries.Level of Evidence: 4.

COVID-19 outbreak had a major impact on the organization of care in Italy, and a survey to evaluate provision of for arrhythmia during COVID-19 outbreak (March–April 2020) was launched. A total of 104 physicians from 84 Italian arrhythmia centres took part in the survey. The vast majority of participating centres (95.2%) reported a significant reduction in the number of elective pacemaker implantations during the outbreak period compared to the corresponding two months of year 2019 (50.0% of centres reported a reduction of > 50%). Similarly, 92.9% of participating centres reported a significant reduction in the number of implantable cardioverter-defibrillator (ICD) implantations for primary prevention, and 72.6% a significant reduction of ICD implantations for secondary prevention (> 50% in 65.5 and 44.0% of the centres, respectively). The majority of participating centres (77.4%) reported a significant reduction in the number of elective ablations (> 50% in 65.5% of the centres). Also the interventional procedures performed in an emergency setting, as well as acute management of atrial fibrillation had a marked reduction, thus leading to the conclusion that the impact of COVID-19 was disrupting the entire organization of health care, with a massive impact on the activities and procedures related to arrhythmia management in Italy. Electronic supplementary material The online version of this article (10.1007/s11739-020-02487-w) contains supplementary material, which is available to authorized users.

Background: Testicular adrenal rest tumors (TARTs) are benign neoplasms affecting patients with congenital adrenal hyperplasia (CAH). The prevalence of TART in adult patients with CAH is not well known. Ultrasonography (US) is the main tool for diagnosing TART and the role of contrast-enhanced US (CEUS) is never investigated. The aim of this study was to evaluate the TART prevalence in adults with CAH, by stratifying patients according to disease phenotype and assessing the diagnostic performance of US, color Doppler (CD) US and CEUS. Methods: Male patients >16 years old with certain diagnosis of CAH who underwent US for TARTs, between December 2015 and September 2019 were prospectively enrolled. The control group included patients without CAH affected by testicular lesions at US other than TARTs. Results: TARTs were identified in 16 of 52 patients (31%), of whom 15 (93.8%) displayed the salt-wasting (SW) form (P

Emerging evidence suggests that memory deficit in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease with varying impairment of motor abilities and cognitive profile, may be independent from executive dysfunction. Our multimodal magnetic resonance imaging (MRI) approach, including resting state functional MRI (RS-fMRI), diffusion tensor imaging (DTI) and voxel-based morphometry (VBM), aimed to investigate structural and functional changes within and beyond the Papez circuit in non-demented ALS patients (n = 32) compared with healthy controls (HCs, n = 21), and whether these changes correlated with neuropsychological measures of verbal and non-verbal memory. We revealed a decreased functional connectivity between bilateral hippocampus, bilateral parahippocampal gyri and cerebellum in ALS patients compared with HCs. Between-group comparisons revealed white matter abnormalities in the genu and body of the corpus callosum and bilateral cortico-spinal tracts, superior longitudinal and uncinate fasciculi in ALS patients (p

Objective: Advanced neuroimaging techniques may offer the potential to monitor disease spreading in amyotrophic lateral sclerosis (ALS). We aim to investigate brain functional and structural magnetic resonance imaging (MRI) changes in a cohort of ALS patients, examined at diagnosis and clinically monitored over 18 months, in order to early discriminate fast progressors (FPs) from slow progressors (SPs). Methods: Resting state functional MRI (RS-fMRI), diffusion tensor imaging (DTI) and voxel-based morphometry (VBM) analyses were performed at baseline in 54 patients with ALS and 22 HCs. ALS patients were classified a posteriori into FPs (n = 25) and SPs (n = 29) based on changes in Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised score from baseline to the 18-month assessment (ΔALSFRS-R), applying a k-means clustering algorithm. Results: At diagnosis, when compared to HCs, ALS patients showed reduced functional connectivity in both motor and extra-motor networks. When compared to SPs, at baseline, FPs showed decreased function connectivity in paracentral lobule (sensorimotor network), precuneus (in the default mode network), middle frontal gyri (frontoparietal networks) and increased functional connectivity in insular cortices (salience network). Structural analyses did not reveal significant differences in gray and white matter damage by comparing FPs to SPs. Receiver operating characteristic (ROC) curve analysis showed that functional connectivity increase in the left insula at baseline best discriminated FPs and SPs (area under the curve 78%). Conclusions: Impairment of extra-motor networks may appear early in ALS patients with faster disease progression, suggesting that a more widespread functional connectivity damage may be an indicator of poorer prognosis.

Objectives: To investigate the effects of cholecalciferol supplementation on the progression of motor disability in a cohort of amyotrophic lateral sclerosis (ALS) patients with low blood 25-hydroxyvitamin D3 [25(OH)D] levels, on the basis of the hypothesis of potential neuroprotective effects of vitamin D supplementation. Methods: Forty-eight ALS patients, 34 with deficient ( 0.05) and UMN (p > 0.05) among the patient groups who underwent 3 different doses of cholecalciferol. Conversely, the treatment with 75.000 IU/month or 100.000 IU/month induced a significant increase in serum levels of 25(OH)D in comparison with the supplementation with 50.000 IU/month; no significant differences were found between 75.000 IU/month and 100.000 IU/month. Conclusions: Our findings highlighted that 6-month supplementation of vitamin D in ALS patients had no significant effects on motor dysfunction. However, it is recommended to prevent medical complications of vitamin D deficiency in ALS patients as well as in other populations of neurodegenerative patients, characterized by low mobility and decreased sun exposure.

Aims: Peripheral neuropathy (PN) affects two-thirds of type 2 diabetes patients (T2DM). According to diabetic PN length-dependent pattern, neurophysiological evaluation of foot-sole nerves might increase NCS diagnostic sensitivity, hence allowing early diagnosis of PN. Thus, we aim to assess the ability of whole plantar nerve (WPN) conduction in diabetic PN early diagnosis. Methods: This is a single center prospective observational cohort study on 70 T2DM patients referred to Internal Medicine Unit of A.O.U. “Luigi Vanvitelli” between October 2019/October 2020. Primary endpoint was WPN efficacy assessment in PN early detection. As secondary, we evaluated (i) a potential cut-off of SNAPs amplitude by WPN and (ii) WPN diagnostic accuracy vs. gold-standard distal sural nerve conduction. Results: ROC curve analysis allowed to establish two potential cut-offs for people aged ≤60 years (AUROC: 0.83, 95%CI: 0.69–0.96, p < 0.001) and ≤60 years (AUROC: 0.76, 95%CI: 0.59–0.93, p = 0.017). In depth, we fixed a cut-off of WPN-SNAP amplitude of 4.55 μV and 2.65 μV, respectively, with subsequent 48 patients classified as PN-T2DM. Conclusions: Our data support WPN conduction study reliability in characterizing the most distal sensory nerve fibers at lower limbs. Thus, WPN may represent an extremely useful diagnostic tool for diabetic PN early detection.

COVID-19 pandemic has induced an urgent reorganization of the healthcare system to ensure continuity of care for patients affected by chronic neurological diseases including myasthenia gravis (MG). Due to the fluctuating nature of the disease, early detection of disease worsening, adverse events, and possibly life-threatening complications is mandatory. This work analyzes the main unresolved issues in the management of the myasthenic patient, the possibilities offered so far by digital technologies, and proposes an online evaluation protocol based on 4 simple tests to improve MG management. Telemedicine and Digital Technology might help neurologists in the clinical decision-making process of MG management, avoiding unnecessary in presence consultations and allowing a rational use of the time and space reduced by the pandemic.

ObjectiveAdvanced neuroimaging techniques may offer the potential to monitor disease progression in amyotrophic lateral sclerosis (ALS), a neurodegenerative, multisystem disease that still lacks therapeutic outcome measures. We aim to investigate longitudinal functional and structural magnetic resonance imaging (MRI) changes in a cohort of patients with ALS monitored for one year after diagnosis.MethodsResting state functional MRI, diffusion tensor imaging (DTI), and voxel-based morphometry analyses were performed in 22 patients with ALS examined by six-monthly MRI scans over one year.ResultsDuring the follow-up period, patients with ALS showed reduced functional connectivity only in some extramotor areas, such as the middle temporal gyrus in the left frontoparietal network after six months and in the left middle frontal gyrus in the default mode network after one year without showing longitudinal changes of cognitive functions. Moreover, after six months, we reported in the ALS group a decreased fractional anisotropy (P = .003, Bonferroni corrected) in the right uncinate fasciculus. Conversely, we did not reveal significant longitudinal changes of functional connectivity in the sensorimotor network, as well as of gray matter (GM) atrophy or of DTI metrics in motor areas, although clinical measures of motor disability showed significant decline throughout the three time points.ConclusionOur findings highlighted that progressive impairment of extramotor frontotemporal networks may precede the appearance of executive and language dysfunctions and GM changes in ALS. Functional connectivity changes in cognitive resting state networks might represent candidate radiological markers of disease progression.

Besides the prominent motor syndrome, some patients affected by amyotrophic lateral sclerosis (ALS) complain of many non-motor symptoms during the disease course, in particular chronic pain that significantly reduces the patients' quality of life. Complex regional pain syndrome (CRPS) is a rare painful condition, rarely described in ALS patients. We present the clinical case of a patient affected by spinal-onset ALS, who developed a type I CRPS (CRPS-I) at the upper limbs. To the best of our knowledge, only five cases of ALS-CRPS-I have been reported and they share some peculiar features: ALS spinal-onset with classic phenotype, rapid deterioration of quality of life, and a poor prognosis. Different mechanisms have been supposed in the pathogenesis of both CRPS and ALS, resulting in distinctive clinical presentations. Altered plasticity of brain sensory and motor areas might represent a common feature that seems to influence negatively ALS progression and prognosis.

Purpose The management of cervical facet dislocation injuries remains controversial. The main purpose of this investigation was to identify whether a surgeon’s geographic location or years in practice influences their preferred management of traumatic cervical facet dislocation injuries. Methods A survey was sent to 272 AO Spine members across all geographic regions and with a variety of practice experience. The survey included clinical case scenarios of cervical facet dislocation injuries and asked responders to select preferences among various diagnostic and management options. Results A total of 189 complete responses were received. Over 50% of responding surgeons in each region elected to initiate management of cervical facet dislocation injuries with an MRI, with 6 case exceptions. Overall, there was considerable agreement between American and European responders regarding management of these injuries, with only 3 cases exhibiting a significant difference. Additionally, results also exhibited considerable management agreement between those with ≤ 10 and > 10 years of practice experience, with only 2 case exceptions noted. Conclusion More than half of responders, regardless of geographical location or practice experience, identified MRI as a screening imaging modality when managing cervical facet dislocation injuries, regardless of the status of the spinal cord and prior to any additional intervention. Additionally, a majority of surgeons would elect an anterior approach for the surgical management of these injuries. The study found overall agreement in management preferences of cervical facet dislocation injuries around the globe.

Introduction Despite the well-described clinical efficacy of long-term subcutaneous immunoglobulin (LT-SCIg) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients, the neurophysiological effects of SCIg have been followed only for a short time and were not correlated with clinical parameters. Methods Fourteen CIDP patients were evaluated at baseline and after LT-SCIg administration for 24 to 48 months. Nerve conduction studies were performed and clinical features were assessed for: (a) overall strength, by Medical Research Council sum score; (b) sensory function, by Inflammatory Neuropathy Cause And Treatment score; (c) disability, by Rasch-built overall disability scale; (d) quality of life (QoL), by the EuroQol Visual Analog Scale. Results LT-SCIg treatment improved clinical and neurophysiological features, preserving strength and improving sensory deficits, disability, and QoL. Clinical scores correlated with the amplitude of distal motor action (dCMAP) and sensory nerve action (SNAP) potentials. Discussion LT-SCIg treatment demonstrates efficacy in maintaining and continuing clinical improvement at 24 to 48 months after start of treatment. dCMAP and SNAP amplitudes represent useful prognostic factors for functional outcome.