关键词： Locus coeruleus   Magnetic resonance imaging   Neuromelanin   Noradrenaline   Alzheimer's disease   Parkinson's disease  

摘要： Purpose of Review Locus coeruleus (LC) is the main noradrenergic nucleus of the brain, and its degeneration is considered to be key in the pathogenesis of neurodegenerative diseases. In the last 15 years,MRI has been used to assess LC in vivo, both in healthy subjects and in patients suffering from neurological disorders. In this review, we summarize the main findings of LC-MRI studies, interpreting them in light of preclinical and histopathological data, and discussing its potential role as diagnostic and experimental tool. Recent findings LC-MRI findings were largely in agreement with neuropathological evidences;LC signal showed to be not significantly affected during normal aging and to correlate with cognitive performances. On the contrary, a marked reduction of LC signal was observed in patients suffering from neurodegenerative disorders, with specific features. Summary LC-MRI is a promising tool, which may be used in the future to explore LC pathophysiology as well as an early biomarker for degenerative diseases.

摘要： Post-viral anosmia in the setting of upper respiratory tract infections is usually related to mucosal congestion and nasal obstruction, resulting in a conductive olfactory loss; however, few patients with SARS-CoV-2-related anosmia had sinonasal symptoms, suggesting that mucosal congestion is an unlikely aetiology for anosmia in these cases.6 Olfactory epithelium support-cells residing in the olfactory cleft express the angiotensin-converting enzyme 2 receptor, identified as the binding antigen for SARS-CoV-2.6 Imaging of the olfactory cleft showed olfactory cleft opacification in 17 (74%) of cases.6 Moving proximally, olfactory nerve filia clumping, suggestive of inflammation, was observed in eight (35%) of patients, and olfactory bulb signal abnormalities, indicating degeneration or microhaemorrhage, were seen in 21 (91%) *** a case series of five individuals with AIDP and SARS-CoV-2 infection, the latency between respiratory symptom and neurological symptom onset was 14–30 days.7 Most patients presented with the classical findings of AIDP, with ascending paraparesis and hyporeflexia, conduction blocks and increased latency on nerve conduction studies, and albuminocytological dissociation in the CSF.7 However, uniquely to SARS-CoV-2-associated AIDP, patients also showed elevated systemic or CSF levels of IL-6 and IL-8, implicating an overactive inflammatory response in the pathogenesis of AIDP in these ***, vessel wall sequence MRI revealed contrast enhancement of the medium and large arterial vessel walls in these patients.2 This finding, coupled with the absence of CSF inflammatory markers and SARS-CoV-2-specific IgG antibodies, further supports the hypothesis for a systemic rather than CNS-specific inflammatory response contributing to a hypercoagulable state and endothelial cell dysfunction.2 An autopsy of one individual additionally showed systemic endotheliitis.2 Brain autopsy studies8 have described pathological changes that have

关键词： Cerebrovascular disorders   Thrombectomy   Stroke   Neurological improvement  

摘要： The predictive value of and the influencing factors associated with early neurological improvement (ENI) among patients with acute basilar artery occlusion (BAO) have not been well studied. The present study aimed to evaluate whether ENI predicted a better functional outcome and to identify the influencing factors of ENI. We performed a prospective observational analysis among 187 patients with acute BAO who underwent endovascular treatment (EVT) in Beijing Tiantan Hospital from January 2012 to July 2018. ENI was defined as having a drop on the National Institutes of Health Stroke Scale (NIHSS) by 8 or more scores or having a NIHSS of 0-1 within 24 h after EVT. A multivariate logistic regression model with backward selection was used to identify the influencing factors associated with ENI. ENI had a sensitivity of 0.69 and a specificity of 0.68 to predict a favorable outcome at 90 days after EVT. In addition, patients with ENIs had lower modified Rankin Scale score (mRS) (median: 2.0 vs. 5.0, p < 0.001) and were more likely to survive (95.2% vs. 72.0%, p < 0.001) and achieve functional independence (74.2% vs. 36.8%, p < 0.001). NIHSS before EVT, complete recanalization, white blood cell counts and general anesthetics were significant factors associated with ENI. A one-unit higher NIHSS and complete recanalization were associated with 1.04 (95% CI 1.01-1.08) and 2.71 (95% CI 1.14-6.45) times higher odds of achieving ENI, respectively. In conclusion, in patients with acute BAO, ENI within 24 hours after EVT can predict favorable outcomes at 90 day. Patients with higher NIHSS, lower white blood cell counts before surgery, without general anesthetics and patients with complete recanalization were more likely to achieve ENIs.

关键词： glutaric aciduria type 1   glutaric acidemia type 1   newborn screening   outcome   meta-analysis  

摘要： Purpose Glutaric aciduria type 1 (GA1), a rare inherited neurometabolic disorder, results in a complex movement disorder (MD) with predominant dystonia if untreated. Implementation into newborn screening (NBS) programs and adherence to recommended therapy are thought to improve the neurological outcome. Methods Systematic literature search for articles published from 2000 to 2019 was performed using the PRISMA protocol. Studies reporting on more than one individual identified by NBS were included. We investigated effects of interventional and noninterventional variables on neurological outcome. Results Fifteen publications reporting on 647 GA1 patients were included. In the NBS group (n = 261 patients), 195 patients remained asymptomatic (74.7%), while 66 patients (25.3%) developed a MD. Compared with the NBS group, a much higher proportion of patients (349/386;90.4%;p < 0.0001) diagnosed after the manifestation of neurologic symptoms had a MD and an abnormal motor development (285/349;81.7%;p < 0.0001). For NBS patients, deviations from the recommended diet increased the risk of insidious onset MD, while delayed start of emergency treatment increased the risk of acute onset MD. Conclusions This meta-analysis demonstrates that NBS programs for GA1 have an overall positive effect on the neurological outcome of affected individuals but their success critically depends on the quality of therapy.

关键词： COVID-19   SARS-CoV-2   Coronaviruses   Nervous System   Neurological manifestations  

摘要： Introduction. - The past two decades have been marked by three epidemics linked to emerging coronaviruses. The COVID-19 pandemic highlighted the existence of neurological manifestations associated with SARS-CoV-2 infection and raised the question of the neuropathogenicity of coronaviruses. The aim of this review was to summarize the current data about neurological manifestations and diseases linked to human coronaviruses. Material and methods. - Articles have been identified by searches of PubMed and Google scholar up to September 25, 2020, using a combination of coronavirus and neurology search terms and adding relevant references in the articles. Results. - We found five cohorts providing prevalence data of neurological symptoms among a total of 2533 hospitalized COVID-19 patients, and articles focusing on COVID-19 patients with neurological manifestations including a total of 580 patients. Neurological symptoms involved up to 73% of COVID-19 hospitalized patients, and were mostly headache, myalgias and impaired consciousness. Central nervous system (CNS) manifestations reported in COVID-19 were mostly non-specific encephalopathies that represented between 13% and 40% of all neurological manifestations;post-infectious syndromes including acute demyelinating encephalomyelitis (ADEM, n = 13), acute necrotizing encephalopathy (ANE, n = 4), Bickerstaff s encephalitis (n = 5), generalized myoclonus (n = 3) and acute transverse myelitis (n = 7);other encephalitis including limbic encephalitis (n = 9) and miscellaneous encephalitis with variable radiologic findings (n = 26);acute cerebrovascular diseases including ischemic strokes (between 1.3% and 4.7% of COVID-19 patients), hemorrhagic strokes (n = 17), cerebral venous thrombosis (n = 8) and posterior reversible encephalopathy (n = 5). Peripheral nervous system (PNS) manifestations reported in COVID-19 were the following: Guillain-Barre syndrome (n = 31) and variants including Miller Fisher syndrome (n = 3), polyneurit

关键词： cardiac arrest   LC-MS/MS   neurological outcome   proteomics   serum protein biomarkers  

摘要： Early prognostication of neurological outcome in comatose patients after cardiac arrest (CA) is vital for clinicians when assessing the survival time of sufferers and formulating appropriate treatment strategies to avoid the withdrawal of life-sustaining treatment (WLST) from patients. However, there is still a lack of sensitive and specific serum biomarkers for early and accurate identification of these patients. Using an isobaric tag for relative and absolute quantitation (iTRAQ)-based proteomic approach, we discovered 55 differentially expressed proteins, with 39 up-regulated secreted serum proteins and 16 down-regulated secreted serum proteins between three comatose CA survivors with good versus poor neurological recovery. Then, four proteins were selected and were validated via an enzyme-linked immunosorbent assay (ELISA) approach in a larger-scale sample containing 32 good neurological outcome patients and 46 poor neurological outcome patients, and it was confirmed that serum angiotensinogen (AGT) and alpha-1-antitrypsin (SERPINA1) were associated with neurological function and prognosis in CA survivors. A prognostic risk score was developed and calculated using a linear and logistic regression model based on a combination of AGT, SERPINA1 and neuron-specific enolase (NSE) with an area under the curve of 0.865 (P < .001), and the prognostic risk score was positively correlated with the CPC value (R = 0.708, P < .001). We propose that the results of the risk score assessment not only reveal changes in biomarkers during neurological recovery but also assist in enhancing current therapeutic strategies for comatose CA survivors.

关键词： Botulinum neurotoxin type A   IncobotulinumtoxinA   Chronic sialorrhea   Pediatric  

关键词： Artificial urinary sphincter   Neurological patients   Outcomes   Intrinsic sphincter deficiency   Stress urinary incontinence  

摘要： Purpose To report the outcomes of AUS in women with neurological SUI resulting from intrinsic sphincter deficiency after a follow-up from 3 to 20 years. Methods The charts of female with moderate to severe neurological SUI who underwent open or laparoscopic AUS implantation between November 1994 and July 2014 were reviewed retrospectively. All patients were operated by a single experienced surgeon. Primary endpoint was a postoperative continence categorized as complete continence (no pads used), improved incontinence or unchanged incontinence. Results Twenty-three women (mean age 54 years, range 19-77) underwent open or laparoscopic AUS implantation. At the last follow-up, 7 (30.4%) initial artificial urinary sphincters remained in situ and 9 (39.1%) patients had at least one revision or reimplantation. Sixteen patients were fully continent (69.6%), four (17.4%) had improved incontinence and three (13.0%) had unchanged incontinence. After a median follow-up of 11.6 years (3-22), 8 (34.8%) explanations in 7 patients and 16 (69.6%) revisions in 11 patients occurred. The average time without explanation or revision was 10.9 and 8.5 years, respectively. Survival rates without AUS explanation were 94.4%, 76.5%, 72.8%, 50% and without revision were 83.3%, 64.7%, 45.5% and 16.7% at 5, 10, 15 and 20 years, respectively. Conclusion Although all methods are imperfect at best, AUS remain toward our experience a good way to achieve long-term continence in female patients suffering from neurological SUI with preserved manual dexterity.

关键词： ADHD   drug allergy   epilepsy   neurologic   pregnancy  

摘要： Problem Drug allergies are increasingly common. Immunological factors, implicated in many neurological diseases, also influence an individual's susceptibility. We sought to ascertain a possible association between maternal drug allergy and long-term neurological-related hospitalizations in the offspring. Method of study This is a population-based cohort analysis, comparing the long-term risk of neurological-related hospitalization, involving a predefined set of ICD9 codes as recorded in hospital records, of children born to mothers with and without drug allergies. Deliveries occurred between the years 1991 and 2014 in a tertiary medical center. Twin pregnancies, fetal malformations, and cases of perinatal mortality were excluded. A Kaplan-Meier survival curve was constructed to compare cumulative neurological hospitalizations. A Cox proportional hazards model was used to control for time to event. Results The study included 242 342 patients, 9714 with known drug allergy (4%). Offspring born to mothers with drug allergies had significantly more neurological hospitalizations compared to controls (4.2% vs 3.1%;P <= .001;Kaplan-Meier log-rank testP <= .001), specifically for psychiatric disorders including eating disorders (0.3% vs 0.2%;P = .002), attention-deficit/hyperactivity disorder (0.124% vs 0.056%;P = .008), emotional disorders (0.8% vs 0.5%;P <= .001), and movement disorders (2.3% vs 1.8%;P = .002). While controlling for birth year, gestational age, maternal age, maternal diabetes, hypertensive disorders, and cesarean delivery, using a Cox proportional hazards model, maternal drug allergy was found to be an independent risk factor for neurological hospitalization of the offspring (adjusted HR 1.3, 95% CI 1.19-1.45P < .001). Conclusion Being born to a mother with known drug allergy is an independent risk factor for long-term neurological hospitalization of the offspring.