关键词： ethics   adolescents   risk factors   treatment   quality of life  

摘要： In this article, the author discusses the impact of outdated productivity metrics such as relative value units on child neurology in U.S. Topics discussed include challenges associated with electronic medical record documentation; decrease in physician efficiency and improved productivity; and mentions that technology firm, Bell Laboratory hires researchers to have a historical perspective on site to help the young programmers be more productive.

关键词： Neuromuscular disorders   Trunk   Upper extremity   Kinematics   Daily activity  

摘要： Background: Trunk control is essential during seated activities. The trunk interacts with the upper extremities (UE) and head by being part of a kinematic chain and by providing a stable basis. When trunk control becomes impaired, it may have consequences for the execution of UE tasks. Aim: To review trunk involvement in body movement and stability when performing seated activities and its relation with UE and head movements in neurological patients with a flaccid trunk, with a focus on childhood and development with age. Methods and procedures: A search using PubMed was conducted and 32 out of 188 potentially eligible articles were included. Outcomes and results: Patients with a flaccid trunk (e.g. with spinal cord injury or cerebral palsy) tend to involve the trunk earlier while reaching than healthy persons. Different balance strategies are observed in different types of patients, like using the contralateral arm as counterweight, eliminating degrees of freedom, or reducing movement speed. Conclusions and implications: The key role of the trunk in performing activities should be kept in mind when developing interventions to improve seated task performance in neurological patients with a flaccid trunk.

关键词： Uric acid   ischaemic stroke   prognosis   neurological outcome   protective factor   sex   large-artery atherosclerosis   trial of ORG 10172 in acute stroke treatment (TOAST)  

摘要： Objective We aimed to evaluate the association between serum uric acid levels at the onset and prognostic outcome in patients with acute ischaemic stroke. Methods We retrospectively analysed the outcomes of 1166 patients with ischaemic stroke who were hospitalized in our centre during August 2008 to November 2012. Correlations of serum uric acid levels and prognostic outcomes were analysed. Results Men had higher serum uric acid levels and better neurological functional outcomes compared with women. There was a strong negative correlation between serum uric acid levels and unfavourable neurological functional outcomes. Generalized estimated equation analysis showed that a higher serum uric acid level (>237 mu mol/L) was a protective factor for neurological functional outcome in male, but not female, patients. Among five trial of ORG 10172 in acute stroke treatment classification subtypes, only patients with the large-artery atherosclerosis subtype had a significant protective effect of serum uric acid levels on neurological outcome. Conclusions Our study shows that high serum uric acid levels are a significant protective factor in men and in the large-artery atherosclerosis subtype in patients with ischaemic stroke. This is helpful for determining the prognostic value of serum uric acid levels for neurological outcome of acute ischaemic stroke.

关键词： Preterm   Infant   Ketamine   Neurodevelopmental outcome   Pain   Neurological outcome  

摘要： Objective: Although ketamine analgesia is effective in reducing pain and facilitating the tracheal intubation of newborns in the delivery room, no data on the neurological effects of this treatment are available. This study compared the neurodevelopmental outcomes at 2 years of age in a cohort of preterm newborns having received ketamine prior to tracheal intubation at birth (the ketamine group) and in a control group. Methods: We included newborns delivered at less than 33 weeks gestational age (WGA) having undergone tracheal intubation at birth. The Ages and Stages Questionnaire (ASQ) was completed at 1 and 2 years of age. The development quotient (DQ) was calculated from the revised Brunet-Lezine score assessed at a corrected age of 2 years. Results: There were no statistically significant differences between the ketamine group (n = 54 at 1 year and n = 51 at 2 years) and the control group (n = 16 at 1 and 2 years) in terms of the mean standard deviation DQ at the age of 2 (98 +/- 12 vs. 103 +/- 9, respectively;P = 0.17) and the ASQ score at the age of 2 (221 +/- 44 vs. 230 +/- 39, respectively;P = 0.55). Discussion: This prospective cohort of 51 preterm newborns having received ketamine at birth did not reveal any differences in terms of neurological development at the age of 2 (relative to a control group and the literature data). These preliminary results must be confirmed in a randomized trial with longer follow-up. (C) 2018 Elsevier Masson SAS. All rights reserved.

关键词： Patient video cases   Video cases   Video   Ranking of video cases  

摘要： Background: Teaching and learning with patient video cases may add authenticity, enhance diagnostic accuracy and improve chances of early diagnosis. The aim of this study is firstly to identify selection criteria for key Patient video cases (PVCs), secondly to identify trends in relevance of PVCs for learner levels and thirdly, to rank PVCs for learner levels. Methods: Based on a literature review, we identified criteria for key PVCs for use in paediatric neurology. We then performed a multi-round Delphi analysis to obtain agreement between 28 expert clinician teachers concerning key PVCs for four learner levels. Results: We identified two major criteria: key PVCs should demonstrate key movements, and these movements should be subtle and/or difficult to note. The expert clinician teachers subsequently assessed a list of 14 topics for key PVCs. We found a clear, increasing trend in relevance scores, from medical students to young residents to experienced residents and specialists. For medical students and residents, epileptic spasms, Down syndrome, developmental delay, cerebral palsy and absence epilepsy were highly ranked. For specialists, conditions like chorea, focal seizures or eye movement disorders topped the ranking list, although ranking was less clear for this group of advanced learners. Discussion and conclusion: Key PVCs should demonstrate movements that are difficult to note for learners. Ranked lists of key PVCs for teaching and learning at different learner levels are now available and may help institutions build validated local libraries of PVCs. (C) 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

关键词： acquired copper deficiency   spinal cord   gait disorder   zinc   bariatric surgery   neuropathy  

摘要： Background: Acquired copper deficiency (ACD) is a rare condition usually diagnosed from haematological changes. Aims: To characterise the diagnosis features and the evolution of patients with ACD revealed by neurological symptoms. Methods: Clinical, biological and magnetic resonance imaging (MRI) data were prospectively analysed at diagnosis and during follow up under copper supplementation. Results: Seven patients were studied over a 5-year period. Time to diagnosis ranged from 2.5 to 15 months. Subacute ascending paraesthesias and gait disorder were the first symptoms. All patients had a posterior cord syndrome (PCS) with sensory ataxic gait associated with superficial hypoesthesia of the feet;50% had also lateral cord signs. Electrodiagnostic tests diagnosed a lower limb sensory neuropathy in four patients. Spinal cord MRI was normal in three of seven patients. Anaemia and lymphopenia were diagnosed in six of seven patients. Serum copper was always low, and urinary copper was low or normal. Serum and urinary zinc were high in four patients. Decreased copper intake (stoma/parenteral nutrition, malnutrition, malabsorption with lack of vitamin supplementation after bariatric or other digestive surgeries) was found in four patients, and the chronic use of denture adhesive paste containing zinc was discovered in four patients. One patient had both the causes recorded. After copper supplementation, copper balance and then haematological disturbances were the first features to normalise gradually in 2 months. Radiological myelitis disappeared in 10 months, whereas neurological symptoms improved in six of seven patients after a mean follow up of 2 years. Conclusions: Progressive PCS with anaemia and lymphopenia must raise the possibility of an ACD. Early copper supplementation could increase the neurological prognosis.

关键词： adverse events   immunosuppression   nervous system   transplantation  

摘要： Controversy exists whether NE after LT are more frequently observed in children or adults. We aimed to compare the incidence and outcomes for NE after LT in pediatric and adult recipients. A single-center cohort study, including all LT between 2001 and 2013, was performed. Definition of NE included impaired consciousness, delirium, seizures, focal neurologic deficit, visual impairment, or slurred speech. A cohort of 443 consecutive LT recipients was included: 307 adults and 136 children. Cumulative incidence of NE was similar between adults 15% (n=41) and children 16% (n=20;P=.73) with a complete neurological recovery in 62% and 95% of the patients, respectively (P<.0001). Adults with NE had significantly lower survival (70% vs 76%;P=.015) with a HR of 2.36;this was similarly observed in children (45% vs 66%;HR 2.05, CI 0.66;6.34). Independent risk factors for NE in adults were pre-LT ascites, delta sodium, and post-LT hypomagnesemia, whereas in children pre-LT encephalopathy II and serum albumin were associated with NE. Although a similar incidence of NE after LT was observed, children were more likely to achieve neurological recovery. Risk factors for the development of NE are difficult to assess in both populations.

关键词： Critical care outcome   Critical illness   Interventional radiology   Intracranial vasospasm   Patient outcome assessment   Subarachnoid hemorrhage  

摘要： OBJECTIVE: An onsite access to neurointerventional radiology (NIR) may be useful for managing patients with aneurysmal subarachnoid hemorrhage (aSAH) after the aneurysm-securing procedure. We aimed to assess the association between neurological outcomes related to aSAH and onsite access to MR service. METHODS: This was a sequential period study of 47 patients with aSAH admitted consecutively during the pre-NIR period (January 2010 to June 2012) compared with 81 patients with aSAH admitted consecutively during the post-NIR period (January 2013 to June 2015) at an academic tertiary referral intensive care unit (ICU). The primary end point was the incidence of poor neurological outcome, defined as modified Rankin scale of >= 3 at 6 months from ictus. Secondary outcomes included incidence of symptomatic vasospasm (SV) and length of stay in ICU/hospital. RESULTS: The primary end point was observed in 18 of 47 (38%) patients during the pre-NIR period versus 25 of 81 (31%) patients during the post-NIR period (P = 0.39). The post-NIR period did not have an independent impact on neurological outcomes (adjusted odds ratio = 0.8, 95% confidence interval 0.3-2.1;P = 0.66). Of the patients who developed SV, 10 of 47 (21%) were during the pre-NIR period versus 33 of 81 (41%) during the post-NIR period (P = 0.02). The post-NIR period and higher Fisher grade were independent predictors of SV. Patients with SV had similar outcomes, but with longer stay in ICU during the post-NIR period compared with the pre-NIR period. CONCLUSIONS: Among patients with aSAH, the post-NIR period was associated with more frequent detection of SV, more endovascular procedures, longer hospital stay, but with no appreciable improvement in neurological outcomes either overall or in the subset of patients with SV.

关键词： Chemerin   Microglia   Inflammation   Germinal matrix hemorrhage   Neonate  

摘要： Chemerin, an adipokine, has been reported to reduce the production of pro-inflammatory cytokines and neutrophil infiltration. This study investigated the role of Chemerin and its natural receptor, ChemR23, as well as its downstream mediator calmodulin-dependent protein kinase kinase 2 (CAMKK2)/adenosine monophosphate-activated protein kinase (AMPK) /Nuclear factor erythroid 2-related factor 2 (Nrf2) following germinal matrix hemorrhage (GMH) in neonatal rats, with a specific focus on inflammation. GMH was induced by intraparenchymal injection of bacterial collagenase (0.3U) in P7 rat pups. The results demonstrated that human recombinant Chemerin (rh-Chemerin) improved neurological and morphological outcomes after GMH. Rh-Chemerin promoted accumulation and proliferation of M2 microglia in periventricular regions at 72 h. Rh-Chemerin increased phosphorylation of CAMKK2, AMPK and expression of Nrf2, and decreased IL-1 beta, IL-6 and TNF-alpha levels. Selective inhibition of ChemR23/CAMKK2/AMPK signaling in microglia via intracerebroventricular delivery of liposome-encapsulated specific ChemR23 (Lipo-alpha-NETA), CAMKK2 (Lipo-STO-609) and AMPK (Lipo-Dorsomorphin) inhibitor increased the expression levels of IL-1 beta, IL-6 and TNF-alpha, demonstrating that ChemR23/CAMKK2/AMPK signaling in microglia suppressed inflammatory response after GMH. Cumulatively, these data showed that rh-Chemerin ameliorated GMH-induced inflammatory response by promoting ChemR23/CAMKK2/AMPK/Nrf2 pathway, and M2 microglia may be a major mediator of this effect. Thus, rh-Chemerin can serve as a potential agent to reduce the inflammatory response following GMH. (C) 2018 Elsevier Inc. All rights reserved.