关键词： anesthesia   acute   intravascular  

摘要： Dear Editor,Emergency intravascular thrombectomy is the best treatment for acute ischemic stroke [1]. However, it is controversial whether the use of general anesthesia during emergency intravascular thrombectomy affects patient recovery.

关键词： intraoperative monitoring   osmotic demyelination syndrome   post‐   transplant encephalopathy   post‐   transplant outcomes   sodium  

摘要： Rapid changes in serum sodium (Delta SNa) peri-liver transplant (LT) predispose to post-LT neurological complications (NC). We aimed to assess whether implementation of a protocol directed at limiting peri-LT Delta SNa reduced post-LT NC. A retrospective single-center review of adult LT recipients from 1/2016 to 10/2017 was performed. Patients with hyponatremia (SNa < 135 mEq/L) within 7 days of LT were analyzed in two eras: pre-protocol (1/2016-9/2016) and post-protocol (10/2016-10/2017). The primary outcome was the development of NC within 1 month of LT. Perioperative Delta SNa (Delta SNaPost-LT) was assessed as a secondary outcome. Among 85 and 107 patients who underwent LT pre- and post-protocol, 39 (46%) and 42 (39%) were hyponatremic within 7 days of LT, respectively. Significantly fewer patients in the post-protocol era developed NC vs. pre-protocol (7.1% vs. 25.6%, p = .02). Additionally, fewer LT recipients in the post-protocol era developed Delta SNaPost-LT >= 10 mEq/L (9.5% vs. 30.7%, p = .02). Intraoperatively, more patients post-protocol received hypotonic saline (33.3% vs. 2.6%, p < .01). Multivariable logistic regression revealed that transplantation in the post-protocol era was associated with significantly reduced odds (odds ratio 0.11, 95% confidence interval 0.01-0.50) of developing NC. In conclusion, the implementation of a multidisciplinary protocol aimed at reducing Delta SNa peri-LT was independently associated with a reduction in post-LT NC.

关键词： Children   Emergency   Acute strabismus   Squint   Red flags  

摘要： Objectives: Acute strabismus (AS) is the most common ocular motility disorder in children. In the emergency setting evaluation, the primary concern is to exclude a potentially dangerous underlying condition, requiring immediate intervention. Our first aim was to describe the epidemiology, clinical features, and underlying causes of AS in a cohort of children presenting to the emergency department (ED). Our second aim was to identify clinical features associated with a significant risk of underlying neurological emergencies (NEs). Design and setting: Clinical records of all patients under 18 years presenting for AS to the ED of the Bambino Gesu Children's Hospital over a 10-year period were retrospectively reviewed. A logistic regression model was applied to detect predictive variables associated with a higher risk of NEs. Results: 208 patients (M:F 1/4 1.19) were identified (0.35 cases per 1000 admission). Commonly associated symptoms included diplopia (18.3%), headache (23.1%), nausea or vomit (8.6%). Other ocular or neurological abnormalities were associated in 47.6% of patients. NEs accounted for 24.03% of all cases, mostly represented by brain tumours (8.65%). Ptosis, optic disk blurring, vomit, gait abnormalities and consciousness disorders were found to confer a significantly greater risk of an underlying NE. Conclusions: Potentially severe neurological conditions may affect almost one in four children presenting to the ED for AS. Brain malignancies are the most common dangerous cause. Presence of ptosis, papilledema, vomit, gait disorders, consciousness impairment, pupillary defects and multiple cranial nerves involvement should be considered as red flags.

关键词： Acute encephalopathy with biphasic seizures and late reduced diffusion   Children   Clinical prediction rule   Convulsion   Pediatrics   Prognosis   Seizure  

摘要： Objective: The clinical prediction rule (CPR) for acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was developed with an area under the receiver operating characteristic curve (AUC) of 0.95 ? 0.96. Our objective was to verify the AESD CPR in a new cohort and compare the utilities of three CPRs of acute encephalopathy: the Tada, Yokochi, and Nagase criteria. Methods: We reviewed the clinical data and medical charts of 580 consecutive patients (aged < 18 years) with febrile convulsive status epilepticus lasting for > 30 min in 2002 ? 2017 and measured the performance of the CPRs in predicting AESD and sequelae. Results: The CPRs predicted AESD with an AUC of 0.84 ? 0.88. The Tada criteria predicted AESD with a positive predictive value (PPV) of 0.25 and a negative predictive value (NPV) of 0.99. The Yokochi criteria predicted AESD with a PPV and NPV of 0.20 and 0.95, respectively, after 12 h. The Nagase criteria predicted AESD with a PPV and NPV of 0.14 and 1.00, respectively, after 6 h. The PPVs of the Tada, Yokochi, and Nagase criteria for sequelae were 0.28, 0.28, and 0.17, respectively;the corresponding NPVs were 0.97, 0.95, and 0.98, respectively. Conclusions: The effectiveness of the AESD CPR in a new cohort was lower than that in the derivation study. CPRs are not sufficient as diagnostic tests, but they are useful as screening tests. The Nagase criteria are the most effective for screening among the three CPRs due to their high NPV and swiftness. ? 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

关键词： Neurological disorders   Pediatric emergency department   Epileptic disorder   Seizures   Epilepsy  

摘要： Aim: Neurological disorders are one of the reasons for admission in pediatric emergency departments (PEDs). We aimed to evaluate the frequency of neurological disorders seen in a large tertiary PED. Methods: We conducted a one-year retrospective study that included 1471 medical records. Inclusion was based on the main complaint recorded by nurses at triage. We also retrieved the final diagnoses and the investigations performed in the PED. Results: About 3.4% of the yearly admissions was based on a neurologic complaint on arrival. The final diagnosis was of a neurologic disorder in 1237 children, 2% of which were admitted to the pediatric intensive care unit. An opinion from a child neurologist was requested for 33% of the children. Seizures were the most frequent reason for admission, followed by headaches. A previous visit to the PED in the past six months was a frequent finding (40%), and about one third of the patients with a neurologic diagnosis (except headaches) was already being followed by a child neurologist. Interpretation: Neurological disorders are frequent in our PED and are mainly represented by seizures and headaches. Appropriate training in epileptology might be helpful for healthcare professional working in PEDs . (c) 2021 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

关键词： rehabilitation   inertial measurement unit   force sensors   biofeedback   postural balance   gait   stroke   Parkinson's disease   mild cognitive impairment  

摘要： Wearable devices are used in rehabilitation to provide biofeedback about biomechanical or physiological body parameters to improve outcomes in people with neurological diseases. This is a promising approach that influences motor learning and patients' engagement. Nevertheless, it is not yet clear what the most commonly used sensor configurations are, and it is also not clear which biofeedback components are used for which pathology. To explore these aspects and estimate the effectiveness of wearable device biofeedback rehabilitation on balance and gait, we conducted a systematic review by electronic search on MEDLINE, PubMed, Web of Science, PEDro, and the Cochrane CENTRAL from inception to January 2020. Nineteen randomized controlled trials were included (Parkinson's n = 6;stroke n = 13;mild cognitive impairment n = 1). Wearable devices mostly provided real-time biofeedback during exercise, using biomechanical sensors and a positive reinforcement feedback strategy through auditory or visual modes. Some notable points that could be improved were identified in the included studies;these were helpful in providing practical design rules to maximize the prospective of wearable device biofeedback rehabilitation. Due to the current quality of the literature, it was not possible to achieve firm conclusions about the effectiveness of wearable device biofeedback rehabilitation. However, wearable device biofeedback rehabilitation seems to provide positive effects on dynamic balance and gait for PwND, but higher-quality RCTs with larger sample sizes are needed for stronger conclusions.

关键词： MCAO   BMSCs   PI3K   AKT   CRMP-2   GAP-43   Axon growth  

摘要： Background: Transplantation of bone marrow-derived mesenchymal stem cells (BMSCs) is a potential therapy for cerebral ischemia. However, the underlying protective mechanism remains undetermined. Here, we tested the hypothesis that transplantation of BMSCs via intravenous injection can alleviate neurological functional deficits through activating PI3K/AKT signaling pathway after cerebral ischemia in rats. Methods: A cerebral ischemic rat model was established by the 2 h middle cerebral artery occlusion (MCAO). Twenty-four hours later, BMSCs (1 x 10(6) in 1 ml PBS) from SD rats were injected into the tail vein. Neurological function was evaluated by modified neurological severity score (mNSS) and modified adhesive removal test before and on d1, d3, d7, d10 and d14 after MCAO. Protein expressions of AKT, GSK-3 beta, CRMP-2 and GAP-43 were detected by Western-bolt. NF-200 was detected by immunofluorescence. Results: BMSCs transplantation did not only significantly improve the mNSS score and the adhesive-removal somatosensory test after MCAO, but also increase the density of NF-200 and the expression of p-AKT, pGSK-3 beta and GAP-43, while decrease the expression of pCRMP-2. Meanwhile, these effects can be suppressed by LY294002, a specific inhibitor of PI3K/AKT. Conclusion: These data suggest that transplantation of BMSCs could promote axon growth and neurological deficit recovery after MCAO, which was associated with activation of PI3K/AKT /GSK-3 beta/CRMP-2 signaling pathway.

关键词： ABCC8   congenital hyperinsulinism   diazoxide   epilepsy   pancreatectomy  

摘要： Aim Congenital hyperinsulinism (CHI) is a heterogeneous disease with variable genetic etiology, histopathology, and clinical phenotype. This study aims to describe the clinical characteristics of persistent CHI and evaluate long-term neurological outcome and its risk factors in a cohort of Egyptian children. Methods Clinical, genetic, and biochemical data of 42 patients with CHI were collected. Patients were invited for neurological assessment, electroencephalogram, and magnetic resonance imaging of the brain. Results ABCC8 mutation was found in (61%) of cases who underwent genetic testing (17/28). Five cases with homozygous biparental ABCC8 mutation responded to combined diazoxide and octreotide without needing surgery. Seven out of twenty-one patients who had pancreatectomy (33%) developed diabetes after a median period of 4.8 (range:1-10) years following surgery. Fifty-five percent of our patients had neurodevelopmental impairment at follow-up. Logistic regression analysis has shown that delayed referral to tertiary centre for more than 8 days, delayed diagnosis of CHI for more than 14 days and hospital admission for more than 30 days, are significant predictors of unfavorable neurological sequelae in CHI;(OR = 12.7 [2.56], p = 0.001), (OR = 12.7 [2.9-56], p = 0.001), and (OR = 3.8 [0.14.5], p = 0.043), respectively. Conclusions ABCC8 mutation was the commonest genetic mutation underlying CHI in this study group. CHI cases with biparental homozygous ABCC8 mutation may show response to combined octreotide and diazoxide therapy. More than half of our patients had neurodevelopmental impairment at follow-up. Delayed referral to expert centre, delayed diagnosis and longer hospital stay are significant predictors of neurological disability in CHI cases.

关键词： Protocadherin 19   PCDH19   PCDH19-Clustering Epilepsy (PCDH19-CE)   Epilepsy   Synapses  

摘要： PCDH19-Clustering Epilepsy (PCDH19-CE) is an infantile onset disorder caused by mutation of the X-linked PCDH19 gene. Intriguingly, heterozygous females are affected while hemizygous males are not. While there is compelling evidence that this disorder stems from the coexistence of WT and PCDH19-null cells, the cellular mechanism underpinning the neurological phenotype remains unclear. Here, we investigate the impact of Pcdh19 WT and KO neuron mosaicism on synaptogenesis and network activity. Using our previously established knock-in and knock-out mouse models, together with CRISPR-Cas9 genome editing technology, we demonstrate a reduction in excitatory synaptic contacts between PCDH19-expressing and PCDH19-null neurons. Significantly altered neuronal morphology and neuronal network activities were also identified in the mixed populations. In addition, we show that in Pcdh19 heterozygous mice, where the coexistence of WT and KO neurons naturally occurs, aberrant contralateral axonal branching is present. Overall, our data show that mosaic expression of PCDH19 disrupts physiological neurite communication leading to abnormal neuronal activity, a hallmark of PCDH19-CE.

关键词： COVID-19   neurological   presentation   SARS-CoV-2   symptom  

摘要： Objective To characterize patients with coronavirus disease 2019 (COVID-19) who presented primarily with neurologic symptoms without typical COVID-19 symptoms of fever, cough, and dyspnea. Methods We retrospectively identified COVID-19-positive patients 18 years and older that had neurology symptoms on presentation requiring neurology consultation between March 14, 2020 and May 18, 2020. The patients were then classified into those with typical COVID-19 symptoms and those without. Demographic, clinical symptoms, laboratory result, and clinical outcomes were collected. Results Out of 282 patients who had neurology consult during this period, we identified 56 (mean age 69.2 years, 57% women) who tested COVID-19-positive and had neurologic symptoms on initial presentation. Of these, 23 patients (mean age 65.2 years, 52% women) had no typical COVID-19 symptoms while 33 did (mean age 72.2 years, 60% woman). In both groups, impaired consciousness was the most common initial neurologic symptom, followed by stroke, unsteady gait, headache, seizure, syncopal event, acute vision changes, and intracranial hemorrhage. Out of the 23 patients without typical COVID-19 symptoms on presentation, 10 went on to develop typical symptoms with 8 needing supplemental oxygen and one requiring mechanical ventilation. Conclusion Patients who have COVID-19 can present with serious neurologic symptoms such as impaired consciousness and stroke even without typical COVID-19 symptoms. Those without typical COVID-19 symptoms can later develop typical symptoms severe enough to need respiratory support.