关键词： Rhabdomyolysis   HyhyperCKemia   Creatine kinase   Myopathy   Acute kidney injury  

摘要： The objective of this retrospective cohort study was to evaluate demographic, clinical and laboratory characteristics of patients with rhabdomyolysis as defined by a serum creatine kinase (sCK) activity > 950 U/L. A total of 248 patients were recruited from the Department of Neurology, Medical University of Vienna, between 01/2000 and 12/2017, with a median sCK activity of 2,160U/I (IQR 1,342-4,786). Seizures (31.9%), illicit drugs/alcohol (9.7%) and exercise (8.5%) were the most common trigger factors. Rhabdomyolysis incidence rates in specific neurological diseases as estimated by the ratio between rhabdomyolysis cases and the total number of cases with the corresponding disease were highest in myopathies (49.8/1,000 person-years, 95% CI 32.3-67.4), followed by epilepsy (16.4/1,000 person-years, 95% CI 12.8-20.0) and stroke (11.9/1,000 person-years, 95% CI 8.4-15.4). The half-life of sCK activity was 1.5 days in the total cohort. In myopathies, sCK activity was significantly higher as compared to other disease entities 7 days after the peak measurement (p = 0.0023). Acute kidney injury (AKI) developed in 18 patients (7.3%) with no AKI-related deaths during the study period. In conclusion, rhabdomyolysis occurred in a broad range of neurological entities but was associated with a favorable prognosis in most cases rarely resulting in AKI and death. (C) 2021 The Author(s). Published by Elsevier B.V.

关键词： Neurological disorders   Neuroscience   Biomedicine   general   Cancer Research   Metabolic Diseases   Infectious Diseases   Molecular Medicine   Neurosciences  

摘要： Emerging reports of rare neurological complications associated with COVID-19 infection and vaccinations are leading to regulatory, clinical and public health concerns. We undertook a self-controlled case series study to investigate hospital admissions from neurological complications in the 28 days after a first dose of ChAdOx1nCoV-19 (n = 20,417,752) or BNT162b2 (n = 12,134,782), and after a SARS-CoV-2-positive test (n = 2,005,280). There was an increased risk of Guillain-Barre syndrome (incidence rate ratio (IRR), 2.90;95% confidence interval (CI): 2.15-3.92 at 15-21 days after vaccination) and Bell's palsy (IRR, 1.29;95% CI: 1.08-1.56 at 15-21 days) with ChAdOx1nCoV-19. There was an increased risk of hemorrhagic stroke (IRR, 1.38;95% CI: 1.12-1.71 at 15-21 days) with BNT162b2. An independent Scottish cohort provided further support for the association between ChAdOx1nCoV and Guillain-Barre syndrome (IRR, 2.32;95% CI: 1.08-5.02 at 1-28 days). There was a substantially higher risk of all neurological outcomes in the 28 days after a positive SARS-CoV-2 test including Guillain-Barre syndrome (IRR, 5.25;95% CI: 3.00-9.18). Overall, we estimated 38 excess cases of Guillain-Barre syndrome per 10 million people receiving ChAdOx1nCoV-19 and 145 excess cases per 10 million people after a positive SARS-CoV-2 test. In summary, although we find an increased risk of neurological complications in those who received COVID-19 vaccines, the risk of these complications is greater following a positive SARS-CoV-2 test. A self-controlled case series analysis of nearly 32 million people in England shows an increased risk of rare neurological complications in those who received COVID-19 vaccines and following SARS-CoV-2 infection. The results highlight 38 excess cases of Guillain-Barre syndrome per 10 million ChAdOx1nCoV-19 vaccinations.

摘要： Aim To explore the breadth of pediatric neurological conditions for which neuromuscular electrical stimulation (NMES) has been studied. Method Databases (PubMed, Google Scholar, Scopus, and Embase) were searched from 2000 to 2020, using the search terms 'neuromuscular electrical stimulation' OR 'functional electrical stimulation' with at least one of the words 'pediatric OR child OR children OR adolescent', and without the words 'dysphagia OR implanted OR enuresis OR constipation'. Articles focused on adults or individuals with cerebral palsy (CP) were excluded. Results Thirty-five studies met the inclusion criteria, with a total of 353 pediatric participants (293 unique participants;mean age 7y 4mo, range 1wk-38y). NMES was applied in a range of pediatric conditions other than CP, including stroke, spinal cord injury, myelomeningocele, scoliosis, congenital clubfoot, obstetric brachial plexus injury, genetic neuromuscular diseases, and other neuromuscular conditions causing weakness. Interpretation All 35 studies concluded that NMES was well-tolerated and most studies suggested that NMES could augment traditional therapy methods to improve strength. Outcome measurements were heterogeneous. Further research on NMES with larger, randomized studies will help clarify its potential to improve physiology and mobility in pediatric patients with neuromuscular conditions.

关键词： Bone cysts   Aneurysmal   Spine   Neoplasms   Sodium tetradecyl sulfate   Sclerotherapy  

摘要： Objective To assess the clinical efficacy, technical feasibility, and safety profile of percutaneous sclerotherapy utilizing sodium tetradecyl-sulfate foam (STS) as a first-line treatment strategy for aggressive spinal aneurysmal bone cysts (sABCs) presenting with neurological compromise. Materials and methods Between July 2013 and September 2019, eight consecutive patients (5 males;3 females;mean age 22 +/- 17, range 7-52) underwent fluoroscopic/CT-guided intraosseous sclerotherapy for sABCs. Pain and/or neurological compromise was the primary indications. Procedural data, complications, imaging, and clinical results were analyzed. Results Technical success was achieved in all cases. Mean procedure time was 25 +/- 15 min (range 6-167);1 to 5 repeat treatment cycles (mean 3.7 +/- 1.2) utilizing a mean 2.6mls +/- 1.3 (range 1-6) of agitated 3% STS, with a DLP mean dose of 158 +/- 91 mGy*cm (range 62-331) per procedure. One reported a minor complication (pain), but no significant complications. Two patients had persistent neurological deficit due to cord compression despite successful sclerotherapy, requiring surgical resection (and were thereby excluded from the final outcome analysis). The remaining six patients demonstrated a significant reduction in tumor volume (p = 0.028), pain (p = 0.027), and SINS (spinal instability neoplastic score) (p = 0.027) at up to 5 years of follow-up (mean 20 +/- 16.7, range 7-51 months). Conclusion Percutaneous sclerotherapy with STS is a minimally invasive, technically feasible, safe, and effective first-line treatment for primary sABCs causing pain and neurological compromise, alleviating the need for extensive surgery. It is most effective with three or more treatment cycles, in patients with higher SINS, pain scores, or tumor volumes at the initial presentation.

关键词： Ionized calcium   Hypocalcemia   Spontaneous subarachnoid hemorrhage   Coagulation abnormalities  

摘要： Background Spontaneous subarachnoid hemorrhage (SSAH) is associated with significant morbidity and mortality. Pathophysiological processes following initial bleeding are complex and not fully understood. In this study, we aimed to determine whether a low level of ionized calcium (Ca++), an essential cofactor in the coagulation cascade and other cellular processes, is associated with adverse neurological outcome, development of early hydrocephalus, and symptomatic vasospasm among patients with SSAH. Methods This was a retrospective single-center cohort study of all patients admitted for SSAH between January 1, 2009, and April 31, 2020. The primary outcome was an unfavorable neurological status at discharge, defined as a modified Rankin Scale score greater than or equal to 3. Secondary outcomes were the development of early hydrocephalus and symptomatic vasospasm. Multivariable logistic regression was performed to determine whether Ca++ was an independent predictor of these outcomes. Results A total of 255 patients were included in the final analysis. Hypocalcemia, older age, admission Glasgow Coma Scale (GCS) score, and admission Hunt-Hess classification scale (H&H) grades IV and V were independently associated with unfavorable neurological outcome, with adjusted odds ratios (ORs) of 1.93 (95% confidence interval [CI] 1.1-3.4;p = 0.02) for each 0.1 mmol L-1 decrease in the Ca++ level, 1.04 (95% CI 1.01-1.08;p = 0.02) for each year increase, 0.82 (95% CI 0.68-0.99;p = 0.04), and 6.29 (95% CI 1.14-34.6;p = 0.03), respectively. Risk factors for the development of hydrocephalus were hypocalcemia and GCS score, with ORs of 1.85 (95% CI 1.26-2.71;p = 0.002) for each 0.1 mmol L-1 decrease in the Ca++ level and 0.83 (95% CI 0.73-0.94;p = 0.005), respectively. Ca++ was not associated with symptomatic vasospasm (OR 1.04 [95% CI 0.76-1.41];p = 0.81). Among patients with admission H&H grade I-III bleeding, hypocalcemia was independently associated with unfavorable neurological o

关键词： experimental models   gene therapy   in vivo tracking   mesenchymal stem cells   nervous system   neuroimmune   transplantation  

摘要： Neurological disorders are diseases of the central nervous system (CNS), characterized by a progressive degeneration of cells and deficiencies in neural functions. Mesenchymal stem cells (MSCs) are a promising therapy for diseases and disorders of the CNS. Increasing evidence suggests that their beneficial abilities can be attributed to their paracrine secretion of extracellular vesicles (EVs). Administration of EVs that contain a mixture of proteins, lipids, and nucleic acids, resembling the secretome of MSCs, has been shown to mimic most of the effects of the parental cells. Moreover, the small size and safety profile of EVs provide a number of advantages over cell transplantation. Intranasal (IN) administration of EVs has been established as an effective and reliable way to bypass the blood-brain barrier (BBB) and deliver drugs to the CNS. In addition to pharmacological drugs, EVs can be loaded with a diverse range of cargo designed to modulate gene expression and protein functions in recipient cells, and lead to immunomodulation, neurogenesis, neuroprotection, and degradation of protein aggregates. In this review, we will explore the proposed physiological pathways by which EVs migrate through the nasal route to the CNS where they can actively target a region of injury or inflammation and exert their therapeutic effects. We will summarize the functional outcomes observed in animal models of neurological diseases following IN treatment with MSC-derived EVs. We will also examine key mechanisms that have been suggested to mediate the beneficial effects of EV-based therapy.

关键词： Autoimmunity   Neuroimmunology   Biomedicine   general   Immunology  

摘要： The realization that autoantibodies can contribute to dysfunction of the brain has brought about a paradigm shift in neurological diseases over the past decade, offering up important novel diagnostic and therapeutic opportunities. Detection of specific autoantibodies to neuronal or glial targets has resulted in a better understanding of central nervous system autoimmunity and in the reclassification of some diseases previously thought to result from infectious, 'idiopathic' or psychogenic causes. The most prominent examples, such as aquaporin 4 autoantibodies in neuromyelitis optica or NMDAR autoantibodies in encephalitis, have stimulated an entire field of clinical and experimental studies on disease mechanisms and immunological abnormalities. Also, these findings inspired the search for additional autoantibodies, which has been very successful to date and has not yet reached its peak. This Review summarizes this rapid development at a point in time where preclinical studies have started delivering fundamental new data for mechanistic understanding, where new technologies are being introduced into this field, and - most importantly - where the first specifically tailored immunotherapeutic approaches are emerging.

关键词： COVID-19   SARS-CoV-2   encephalitis   blindness   neurology  

摘要： Background: There are growing reports of the neurological involvement among patients with coronavirus disease 2019 (COVID-19). Headache, confusion, and anosmia after olfactory nerve disruption are the most prevalent presentation of the neurological involvement related to COVID-19. However, small numbers of the central nervous system involvement have been reported. Case Report: A 49-year-old man was referred to our hospital with abrupt vision loss. Three weeks earlier he was admitted to the hospital based on his respiratory symptoms and was diagnosed with COVID-19 infection. Initial brain magnetic resonance imaging indicated diffuse restricted bilateral foci in both parietal and occipital lobes in favor of acute infarction. Diffuse weighted imaging demonstrated restricted bilateral hyperintense signals in parietal and occipital region. Occipital cortex biopsy showed brain tissue with focal infiltration of foamy macrophages mixed with reactive astrocytes and no plasma cell infiltration. Considering all of the evidence, post-COVID-19 encephalitis diagnosis was considered for the patient, and methyl prednisolone pulse therapy and intravenous immunoglobulin were initiated. Why Should an Emergency Physician Be Aware of This?: Although there are growing reports of neurological involvement among patients, blindness is rarely observed as a complication of post-COVID-19 encephalitis. To our knowledge, this is the first case of post-COVID-19 encephalitis that presented with bilateral vision loss primarily. This case may raise physicians' awareness of neurological complications of COVID-19. (C) 2021 Elsevier Inc. All rights reserved.

关键词： aortic calcification   coronary artery bypass   neuroprotection   neuroprotective device   perioperative stroke  

摘要： Patients with severely calcified aorta undergoing conventional cardiac surgery are at increased risk for postoperative neurologic deficits. Implementation of cerebroprotective devices may substantially reduce or even eliminate the risk of the adverse neurologic events, thus enabling surgical therapy, especially when interventional treatment cannot be considered an alternative option.

关键词： Biomarker   MiRNA   MiRNA-124a   Neurological deficit   Congenital heart disease   Pediatric cardiac surgery  

摘要： Brain injury is a major source of patient morbidity after cardiac surgery in children. New early accurate biomarkers are needed for the diagnosis of patients at risk for cerebral postoperative damage. Specific circulating miRNAs have been found as suitable biomarkers for many diseases. We tested whether miRNA-124a reflects neurological injury in pediatric patients following heart surgery. Serum samples were obtained from 34 patients before and six hours after heart surgery. MiRNAs-124a was quantified by RQ-PCR. MiRNA-124a levels six hours after heart surgery correlated with the neurological outcome of the patients. In children with neurological deficits, miRNA-124a levels increased while in those with no neurological deficits the levels decreased. MiRNA-124a was able, at six hours after the operation, to identify patients who are at risk for the appearance of neurological deficits. Circulating miRNA-124a is a potential biomarker for the appearance of neurological deficits in pediatric patients following heart surgery.