关键词： astrocytes   epilepsy   lipoprotein receptor-related protein   radial glia stem cells   reactive astrocytes   seizures   tissue plasminogen activator  

摘要： The Alzheimer disease-associated multifunctional low-density lipoprotein receptor-related protein-1 is expressed in the brain. Recent studies uncovered a role of this receptor for the appropriate functioning of neural stem cells, oligodendrocytes, and neurons. The constitutive knock-out (KO) of the receptor is embryonically lethal. To unravel the receptors' role in the developing brain we generated a mouse mutant by specifically targeting radial glia stem cells of the dorsal telencephalon. The low-density lipoprotein receptor-related protein-1 lineage-restricted KO female and male mice, in contrast to available models, developed a severe neurological phenotype with generalized seizures during early postnatal development. The mechanism leading to a buildup of hyperexcitability and emergence of seizures was traced to a failure in adequate astrocyte development and deteriorated postsynaptic density integrity. The detected impairments in the astrocytic lineage: precocious maturation, reactive gliosis, abolished tissue plasminogen activator uptake, and loss of functionality emphasize the importance of this glial cell type for synaptic signaling in the developing brain. Together, the obtained results highlight the relevance of astrocytic low-density lipoprotein receptor-related protein-1 for glutamatergic signaling in the context of neuron-glia interactions and stage this receptor as a contributing factor for epilepsy.

摘要： This article aims to highlight the impact of mentorship on the lives of youth with chronic conditions (YCC). Here, we focus on the concepts of mentoring and technology as a means to support transitioning YCC. This is in response to the urgent need for effective healthcare transition strategies and the increasing importance and prevalence of technology in healthcare and health systems. This article also highlights an e-mentoring program for youth with epilepsy, an intervention that bridges the fields of mentoring, transition, and technology. While there is need for further research in these areas, consideration of these factors are highly relevant to the effort to improve health for this generation of YCC. (C) 2020 Published by Elsevier Inc.

关键词： embolic stroke   infective endocarditis   intracranial hemorrhage   perioperative complications   septic emboli   valve replacement   valve surgery  

摘要： Background and purpose Ischaemic and hemorrhagic strokes are dreaded complications of infective endocarditis (IE). The timing of valve surgery for IE patients with stroke remains uncertain. The aim was to study perioperative neurological complications in relation to surgical timing. Methods The study cohort consisted of patients diagnosed with acute IE from January 2010 to December 2016. Early surgery was defined as valve surgery within 14 days of IE diagnosis, and late surgery as after 14 days. Neurological complications that occurred within 14 days post-surgery were considered perioperative and classified as new ischaemic stroke or hemorrhagic stroke, expansion of an existing intracranial hemorrhage and new-onset seizures. Perioperative neurological complications were compared by surgical timing and other variables, including pre-surgical imaging. Results Overall, 183 patients underwent valve surgery: 92 had early surgery at a median of 8 days (interquartile range 6-11);91 had late surgery at a median of 28 days (interquartile range 19-50). Twenty patients (10.9%) had 24 complications: 11 ischaemic, six intraparenchymal hemorrhages, three subarachnoid hemorrhages (SAHs) and four new-onset seizures. Rates of neurological complications were similar for early and late surgery groups (10.9% vs. 11%). Enterococcal IE was more common amongst patients with perioperative neurological complications (35% vs. 12.3%,P < 0.01). An acute infarct was present on pre-surgical magnetic resonance imaging of 134 patients (74%) and was not associated with perioperative neurological complications. Thirty-five patients (19.3%) had intracranial hemorrhage on pre-surgical imaging. SAH on pre-surgical imaging was associated with developing SAH perioperatively (66.7% vs. 13.5%,P < 0.01). Conclusion Early valve surgery for patients with IE complicated by stroke was not associated with perioperative neurological complications.

关键词： Depression screening   Epilepsy   Major depressive episode   The Neurological Disorders Depression   Inventory for Epilepsy   Mini-International Neuropsychiatric   Interview   Hospital Anxiety and Depression Scale  

摘要： Objective: To translate and validate the English version of the Neurologic Depression Disorders Inventory in Epilepsy (NDDI-E) into the Russian language as an instrument for rapid detection of major depressive episodes (MDE) for patients with epilepsy (PWE) from Russian Federation. Methods: One hundred and 75 consecutive PWE were included in the study. All patients were assessed with Mini-International Neuropsychiatric Interview (MINI 6.0.0), Hospital Anxiety and Depression Scale (HADS) and the Russian version of NDDI-E. Chi-square, Fisher's exact and Mann-Whitney tests were used to compare PWE with and without MDE. We analyzed internal structural validity, external validity, and receiver operator characteristics. Results: None of the participants had any difficulties in understanding the questions of NDDI-E. The internal consistency of the inventory was satisfactory (Cronbach's a = 0.856). Correlation between the NDDI-E and the HADS scores was moderate (r= 0.64, P < 0.001), indicating acceptable external validity. NDDI showed good capacity to detect MDE, with area under the curve of 0.919 (95% CI = 0.868-0.955;standard error: 0.019;P < 0.001). An optimal cut-off point with the highest Yuden's index (J = 0.699) was > 12. At this point NDDI-E showed sensitivity of 88.16% (95% CI = 78.7%-94.4%), specificity of 81.82% (95% CI = 72.8%-88.9%), positive predictive value of 59.3% (95% CI = 48.8%-69.0%), negative predictive value of 95.8% (95% CI = 92.5%-97.7%). Conclusion: Russian version of NDDI-E is an affordable and fast screening tool with a good combination of sensitivity and specificity. (C) 2020 Elsevier Inc. All rights reserved.

关键词： MLKI   Multiligament   Multiligamentous knee injury   Ligament knee injury   Biceps lemon's   Distal biceps   Biceps femoris avulsions  

摘要： Background: The aim of this study was to describe associated injuries in cases of distal biceps femoris avulsions (DBFA) as well as the incidence of neurological injury and radiographic abnormalities of the common peroneal nerve (CPN). Methods: A retrospective chart review was conducted of patients presenting to our office or trauma center with DBFA injuries. Demographic data was obtained as well as mechanism of injury. Assessment of concomitant injuries and presence of neurologic injury was completed via chart review and magnetic resonance imaging (MRI) review. The CPN was evaluated for signs of displacement or neuritis. Results: Sixteen patients were identified (mean age-at-injury 28.6 years, 87.5% male) with DBFA. Three patients (18.8%) sustained their injuries secondary to high energy trauma while 13 (81.3%) had injuries secondary to lower energy trauma. Nine patients (56.3%) initially presented with CPN palsy. All patients presenting with CPN palsy of any kind were found to have a displaced CPN on MRI and no patient with a normal nerve course had a CPN palsy. Conclusions: This case series demonstrates a strong association between DBFA and CPN palsy as well as multi-ligamentous knee injury (MLA). These injuries have a higher rate of CPN palsy than that typically reported for MLKI. Furthermore, these findings suggest that CPN displacement on MRI may be a clinically significant indicator of nerve injury. (C) 2020 Elsevier B.V. All rights reserved.

关键词： Airway disease   Pediatric airway   Airway surgery   Subglottic stenosis   Congenital tracheal stenosis   Quality of life  

摘要： Objectives: Anomalies of the larynx and trachea can cause respiratory distress in infants and older children. Depending on its nature, degree and extent of the disease invasive open surgery is indicated. Non-airway-related co-morbidities increase the challenges in its treatment. Neurological deficit poses a great challenge as it is associated with hypotonia and causes diminished laryngeal coordination. The definition of success in treatment of laryngotracheal disease has always focused on the post-operative functional outcomes: breathing, voice swallowing. The aim of this study is to describe a new dimension of success in the management of laryngotracheal disease in children with moderate neurological deficit, where the expected functional gain is less than in otherwise healthy children. Methods: This retrospective observational study includes all patients who have undergone open reconstructive airway surgery between 2012 and 2017. Control patients without neurological deficit and cases with moderate neurological deficit were included. Functional outcome data was obtained from clinical records and two questionnaires were filled in by the parents of the children: one the pediatric voice-handicap index (pVHI) and a quality of life questionnaire. Results: Thirty-two children were included of which ten had moderate neurological deficit. Both groups revealed post-operatively an improvement in the functional outcomes: breathing, voice and swallowing, however, as expected, a trend was observed towards less functional improvement in children with neurological deficit. Both groups reveal a remarkable gain in quality of life (QoL). Conclusion: Indicating the QoL to be an unidentified, dimension of success in the management of laryngotracheal disease in children with moderate neurological deficit.

关键词： Trigonal   Botulinum toxin A   Children   Neurological detrusor overactivity   Spinal cord injury  

摘要： Objective: To evaluate the efficacy and safety of trigonal botulinum toxin A (BTX-A) injections for children with neurological detrusor overactivity (NDO) secondary to spinal cord injury (SCI). Methods: From February 2012 to December 2018, children with NDO secondary to SCI were enrolled. All patients received 200U BTX-A intradetrusor injections including the trigone. Videourodynamic study was performed at baseline and 12 weeks after injection. The primary outcome measures were the presence of vesicoureteral reflux and standardized urodynamic measures. Secondary outcomes included incontinence quality of life questionnaire (I-QoL), voiding volume, urinary incontinence episodes, and complete dryness. Results: A total of 33 pediatric inpatients (28 male and 5 female) completed the study. No one developed VUR at week 0 or week 12. At the first instance of NDO, maximum detrusor pressure (PdetmaxFNDO) and NDO duration were reduced by 29.8% and 31.8%, respectively, whereas NDO volume (VFNDO) increased by 50.5%,12 weeks after injection. Mean urinary incontinence episodes were reduced by 31.7%, whereas voiding volume and IQOL were increased by 52.9% and 23.3%. 3 patients reported mild transient hematuria during the first week after injection. Conclusions: Our results suggest that the use of bladder-trigone-including intradetrusor BTX-A injection does not induce VUR, and is safe and effective in children with NDO secondary to SCI. (c) 2020 Elsevier Inc. All rights reserved.

关键词： Cerebral infarction   Thrombectomy   Perfusion-weighted MRI   Diffusion MRI  

摘要： Objectives Early recanalization and adequate collateral blood flow are surrogates for functional recovery in endovascular stroke treatment (EVT). We evaluated the prognostic value of pre- and immediate post-thrombectomy perfusion-weighted magnetic resonance imaging (PWI) parameters. Methods Consecutive patients with acute ischemic stroke who underwent EVT were enrolled. Lesion volumes and their corresponding changes on diffusion-weighted (DWI) and PWI were assessed. Outcome was measured with modified Rankin Scale (mRS) at 90 days, and early neurological improvement (> 8 points improvement on National Institutes of Health Stroke Scale [NIHSS] or 0 to 1) at 7 days. Results Fifty-two patients were enrolled. After control of initial NIHSS and recanalization status, post-thrombectomy time-to-peak (TTP) hypoperfused volume and TTP hypoperfused volume change remained independent predictors of favorable functional outcome (odds ratio [OR] = 0.13, 95% confidence interval [CI] = 0.03-0.54,p = 0.005;OR = 1.018, 95% CI = 1.00-1.03,p = 0.017), and early neurological improvement (OR = 0.20, 95% CI 0.07-0.58,p = 0.003;OR = 1.02, 95% CI = 1.00-1.03,p = 0.010). The areas under the curve of post-thrombectomy TTP hypoperfused volume and TTP hypoperfused volume change were 0.90 and 0.82 (cutoff 68 mL and 56 mL) for favorable outcome and 0.86 and 0.82 (cutoff 76 mL and 58 mL) for early neurological improvement, which had better prognostic values than other MR parameters and recanalization grades. Conclusions These results suggest a large amount of perfusion recovery on TTP is associated with favorable outcome as well as early neurological improvement after EVT, and may be a useful prognostic marker.

关键词： Acute headache   Neurological emergency   Secondary headache   Emergency room   Red flags  

摘要： Headache represents the second neurological cause of emergency room admittance. The differentiation of the small number of patients with life-threatening headaches from the overwhelming majority with benign primary headaches is an important problem in the emergency department since the misdiagnosis of a secondary headache can have serious consequences, including permanent neurologic deficits and death. The presence of one or more high-risk features (red flags) in patient clinical history or neurological examination warrants an urgent diagnostic workup including blood tests, neuroradiological studies, and lumbar puncture.

关键词： education   residency   EEG   epilepsy   neurology  

摘要： Aims. To assess the baseline EEG knowledge among adult neurology residents at our institution and their perspectives on EEG learning experience during residency. Methods. We evaluated baseline EEG knowledge and resident perception of EEG education utilizing an EEG quiz and an online EEG survey, respectively. The EEG quiz was divided in two parts, composed of normal (n=27) and abnormal (n=10) EEG examples. The EEG survey focused on the importance of EEG, EEG milestones and EEG education. Results. Twenty-one residents completed the EEG quiz;all 21 completed the normal EEG part whereas 19 of these 21 completed the abnormal EEG part. The overall score (mean +/- SEM) was 42 +/- 4.5% for the normal EEG part and 44 +/- 5.5% for the abnormal EEG part. The EEG survey was completed by 28 residents. Forty-three percent of the respondents reported not being able to read EEGs even with supervision. The most commonly reported education barriers were insufficient exposure, insufficient responsibility to read EEGs, and inability to link EEG learning to direct patient care. Conclusion. On average, adult neurology residents were able to correctly identify less than half of normal and abnormal EEG findings. Almost half of residents reported not being able to read EEGs even with supervision.