关键词： Cardiac arrest   Transfusion   Hemoglobin outcome   Prognosis  

摘要： Introduction: Hemoglobin (Hb) is a main determinant of tissue oxygen delivery and anemia could be particularly harmful in post-anoxic brain injury. The aim of this study was to evaluate the association of Hb and venous Hb oxygen saturation (SvO(2)/ScvO(2)) with long-term neurological outcome in patients admitted after cardiac arrest (CA). Methods: Analysis of adult CA patients admitted to the Department of Intensive Care of the Erasme University Hospital (Brussels, Belgium) over 9 years. We retrieved all data concerning CA characteristics as well as Hb during the first 48 h since injury as well as the need for red blood cells transfusions (RBCT). Minimum Hb and Hb oxygen saturation values were recorded. Neurological outcome was evaluated 3 months after CA. Unfavorable neurological outcome (UO) was defined as a Cerebral Performance Categories (CPC) score of 3-5. Results: We treated 414 patients patients with CA, including 231 (56%) out-of-hospital cardiac arrest (OHCA) and 158 (38%) with an initial shockable rhythm. Median Hb concentration on admission was 12.0 [9.9-13.7] g/dL and the lowest Hb concentration was 10.0 [8.1-11.0] g/dL;127 patients (31%) received at least one RBCT. Hb oxygen saturation on admission was 67 [59-74]%, while the lowest value was 60 [53-68]%. Low Hb and Hb oxygen saturation values were independently associated with UO;the optimal cut-off to predict UO was <9.9 g/dL and <60%, respectively. Conclusions: Low hemoglobin values and low values of oxygen venous saturation are significantly associated with unfavorable neurological outcome in adult patients resuscitated from cardiac arrest.

关键词： animal-assisted therapy   dog-assisted therapy   neurorehabilitation   child   dog  

摘要： Background Dog-assisted therapy (DAT) is increasingly applied in neurorehabilitation of patients with severe neurological impairments. To date, there are only anecdotal reports investigating its effects. Objectives This study was aimed to evaluate the potential of DAT in pediatric inpatient neurorehabilitation for severely neurologically impaired children and adolescents, to identify characteristics of patients receiving this therapy, characteristics of the therapy sessions, and to evaluate feasibility and extent of goal achievement. Methods We retrospectively analyzed 850 DAT sessions performed between 2010 and 2017 at an inpatient neurorehabilitation center. The dataset included 196 children and adolescents (Md = 5.50, 0.58-20.33 years) suffering from severe neurological impairments (disorders of consciousness in 37 patients) of various etiologies. We extracted information regarding patient and session characteristics, analyzed the predefined goals with content analysis, and examined to what extent the goals were met during DAT. Data were analyzed using descriptive statistics. Results Patients received an average of 4.34 therapy sessions. A total of 247 of 392 predefined goals (63%) were reached during DAT. The most frequently achieved goal was "enhancing fun" (83%), followed by "establishing contact and communication" (81%), and "relaxation" (71%). Only one critical incident regarding the dogs' safety occurred. Conclusion DAT is a feasible approach and appears to facilitate emotional, social, and psychological goals in children and adolescents with severe neurological impairment.

摘要： The European Academy of Neurology is 6 years old: challenges, opportunities and strategic priorities for the future European Academy of Neurology 2014-2019: foundation and growth The birth of the European Academy of Neurology (EAN) took place in Istanbul in 2014, on the occasion of the last (and joint) meeting of the ENS and EFNS. Front row: Prof. Fanz Fazekas (EAN President 2018-2020), Prof. Hanna Cock, Prof. Günther Deuschl (EAN President 2014-2018), Prof. Paul Boon and Prof. David Vodusek. [Extracted from the article]

关键词： KCND3   SCA19   22   ataxia   Kv4   3   neurodevelopmental disorder   cerebellum   movement disorders   parkinsonism   dystonia  

摘要： KCND3encodes the voltage-gated potassium ion channel subfamily D member 3, a six trans-membrane protein (Kv4.3), involved in the transient outward K(+)***3defect causes both cardiological and neurological syndromes. From a neurological perspective, Kv4.3 defect has been associated to SCA type 19/22, a complex neurological disorder encompassing a wide spectrum of clinical features beside ataxia. To better define the phenotypic spectrum and course ofKCND3-related neurological disorder, we review the clinical presentation and evolution in 68 reported cases. We delineated two main clinical phenotypes according to the age of onset. Neurodevelopmental disorder with epilepsy and/or movement disorders with ataxia later in the disease course characterized the early onset forms, while a prominent ataxic syndrome with possible cognitive decline, movement disorders, and peripheral neuropathy were observed in the late onset forms. Furthermore, we described a 37-year-old patient with a de novoKCND3variant [c.901T>C (***301Pro)], previously reported in dbSNP as rs79821338, and a clinical phenotype paradigmatic of the early onset forms with neurodevelopmental disorder, epilepsy, parkinsonism-dystonia, and ataxia in adulthood, further expanding the clinical spectrum of this condition.

关键词： cutis laxa   neurological findings   vascular tortuosity  

摘要： Cutis laxa is a heterogeneous group of diseases, characterized by abundant and wrinkled skin and a variable degree of intellectual disability. Cutis laxa, autosomal recessive, type IIIA and autosomal dominant 3 syndromes are caused by autosomal recessive or de novo pathogenic variants inALDH18A1. Autosomal recessive variants are known to lead to the most severe neurological phenotype, and very few patients have been described. We describe a 13-month-old patient with cutis laxa, autosomal recessive, type IIIA, with an extremely severe phenotype, including novel neurological findings. This description enlarges the neurological spectrum associated to cutis laxa, autosomal recessive, type IIIA, and provides an additional description of this syndrome.

关键词： COVID-19   SARS-CoV-2   Neurological manifestations   Neurological symptoms   Coronavirus  

摘要： Background As the COVID-19 pandemic developed, reports of neurological dysfunctions spanning the central and peripheral nervous systems have emerged. The spectrum of acute neurological dysfunctions may implicate direct viral invasion, para-infectious complications, neurological manifestations of systemic diseases, or co-incident neurological dysfunction in the context of high SARS-CoV-2 prevalence. A rapid and pragmatic approach to understanding the prevalence, phenotypes, pathophysiology and prognostic implications of COVID-19 neurological syndromes is urgently needed. Methods The Global Consortium to Study Neurological dysfunction in COVID-19 (GCS-NeuroCOVID), endorsed by the Neurocritical Care Society (NCS), was rapidly established to address this need in a tiered approach. Tier-1 consists of focused, pragmatic, low-cost, observational common data element (CDE) collection, which can be launched immediately at many sites in the first phase of this pandemic and is designed for expedited ethical board review with waiver-of-consent. Tier 2 consists of prospective functional and cognitive outcomes assessments with more detailed clinical, laboratory and radiographic data collection that would require informed consent. Tier 3 overlays Tiers 1 and 2 with experimental molecular, electrophysiology, pathology and imaging studies with longitudinal outcomes assessment and would require centers with specific resources. A multicenter pediatrics core has developed and launched a parallel study focusing on patients ages <18 years. Study sites are eligible for participation if they provide clinical care to COVID-19 patients and are able to conduct patient-oriented research under approval of an internal or global ethics committee. Hospitalized pediatric and adult patients with SARS-CoV-2 and with acute neurological signs or symptoms are eligible to participate. The primary study outcome is the overall prevalence of neurological complications among hospitalized COVID-19 patients, wh

关键词： Early neurological deterioration   Blood pressure   Intravenous thrombolysis   Oxidative stress   Blood-brain barrier   MMP-9   AQP4  

摘要： Objective: To study association between blood pressure (BP) and development of early neurological deterioration (END) in acute ischemic stroke patients with intra-venous rt-PA thrombolysis and its possible mechanism. Methods: We prospectively collected data of acute ischemic stroke patients with intravenous rt-PA thromboly-sis from March 2015 to June 2019. The collected data include general, clinical data and laboratory test. Moreover, serum levels or activity of malondialdehyde (MDA), superoxide dismutase (SOD), matrix metalloproteinase-9 (MMP-9), occludin (OCLN), ZO-1 and aquaporin 4(AQP-4) were determined. Results: A total of 357 acute ischemic stroke patients received intravenous rt-PA thrombolysis and 16 cases were eventually excluded. Finally, 341 patients were eligible in this study and 65 patients (19.06%) experienced END. Mean systolic blood pressure (SBP) within 24 h, serum levels or activity of MDA, SOD, MMP-9, ZO-1, OCLN, AQP-4 at 24 h after thrombolysis were the independent predictors for END in the total and hyper-tension population using multivariate logistic regression analysis, and mean SBP within 24 h was the best predictor for END. Receiver operating characteristic (ROC) analysis found that cutoff mean SBP for END was 148.16 mmHg, and sensitivity was 85.6%. The best target SBP level is 140@149 mmHg. Further, spearman correla-tion tests indicated that BP levels were directly proportional to serum levels or activity of MDA, MMP-9, ZO-1, OCLN, AQP-4 at 24 h after thrombolysis and neu-rological deterioration severity. Conclusions: END frequently occurs after thrombol-ysis, and the best predictor of END is SBP which may be mediated through oxidative stress induced blood-brain barrier disruption and AQP4 upregulation.

摘要： Measuring the outcomes of pediatric stroke is a challenge, and hitting the target has been difficult. There has been an explosion of research in pediatric stroke as clinicians and researchers have directed attention to what was once considered a rare disorder. Earlier investigators knew that systematic measurement of outcomes is essential but that measurement in pediatric stroke has been inconsistent in the literature. Only one stroke outcome measure is validated in children, the Pediatric Stroke Outcome Measure (PSOM). The PSOM has been used in a number of observational1 and descriptive studies.2,3 Over the years, investigators noted that the PSOM scored some patients as having more severe deficits than seemed clinically warranted. Accordingly, there was realization that the PSOM needed to be refined, but as one can imagine any attempt to modify or re-interpret a measure as important as the PSOM creates worries about how those modifications might affect the measure.

摘要： There has been significant media attention and a New York City Health Alert [1] surrounding “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19.” As of May 18, 2020, there were 145 suspected cases of this syndrome in New York City [2]. While this disease has been outlined as having components shock and similarities to Kawasaki disease, associated neurologic manifestations have not been described (https://***/sites/default/files/2020-05/COVID-19-Paediatric-multisystem-%20inflammatory%***). We have encountered several children with this condition and highlight two with major neurological complications.

关键词： Hypoxic-ischemic encephalopathy   Magnetic resonance imaging   Diffusion magnetic resonance imaging   Prognosis   Regression analysis  

摘要： Objective: To investigate the value of a model based on brain magnetic resonance imaging (MRI) performed in the subacute phase (between the 1st and 30th day) in predicting long-term neurological outcomes of adult hypoxic-ischemic encephalopathy (HIE) patients. Methods: Ninety-six adult HIE patients who underwent conventional MRI and diffusion-weighted imaging (DWI) during the subacute phase were retrospectively analyzed. Favorable (Cerebral Performance Categories (CPC) 1-2) and unfavorable outcome (CPC 3-5) groups were created based on patient neuro-logical status approximately three months after the onset of hypoxic-ischemic events. A multivariate stepwise regression model was applied after univariate analysis of MRI findings, and then the overall MRI score, Alberta Stroke Program Early Computed Tomography Score (ASPECTS), Bilateral ASPECTS (Bi-ASPECTS), modified ASPECTS (mASPECTS) and Bi- ASPECTS combined with posterior circulation ASPECTS (PC-ASPECTS) were calculated based on MRI findings. Receiver operating characteristic (ROC) curves were used to assess prognostic accuracy. Results: Both univariate and multivariate analyses showed the cerebral cortex and cerebellum, neostriatum, hippocampus, brainstem and postanoxic leukoencephalopathy were independent prognostic factors for unfavorable outcomes. The multivariate regression analysis resulted in an overall classification accuracy of 84.4%, a sensitivity of 84.2% (95% CI, 71.6-92.1%), and a specificity of 92.3% (95% CI, 78.0-98.0%) for unfavorable outcomes. The model had an areas under the ROC curves (AUC) of 0.944 (95% CI, 0.901-0.987);the MRI overall scores were 0.918 (95% CI, 0.866, 0.971), ASPECTS 0.839 (95% CI, 0.755, 0.923), Bi-ASPECTS 0.837 (95% CI, 0.753, 0.922), mASPECTS 0.851(95% CI, 0.771, 0.931) and Bi-ASPECTS+PC-ASPECTS 0.876 (95% CI, 0.806, 0.946). Conclusions: The multivariate model based on conventional MRI combined with DWI performed in the subacute phase could help predict the prognosis of adult