关键词： general movements   hypoxic-ischaemic encephalopathy   infant neurological examination   minor neurological finding   perinatal asphyxia  

摘要： Aim To characterise the spectrum of findings in sequential neurological examinations, general movements (GM) assessment and magnetic resonance imaging (MRI) of infants with perinatal asphyxia. Methods The prospective cohort study of term infants with perinatal asphyxia treated at Helsinki University Hospital's neonatal units in 2016-2020 used Hammersmith Neonatal Neurological Examination (HNNE) and brain MRI at 2 weeks and Hammersmith Infant Neurological Examination (HINE) and GM assessment at 3 months of age. Results Analysis included 50 infants: 33 displaying perinatal asphyxia without hypoxic-ischaemic encephalopathy (HIE), seven with HIE1 and 10 with HIE2. Of the infants with atypical HNNE findings, 24/25 perinatal asphyxia without HIE cases, 5/6 HIE1 cases and all 10 HIE2 cases showed atypical findings in the HINE. The HINE identified atypical spontaneous movements significantly more often in infants with white matter T2 hyperintensity. Conclusion In this cohort, most infants with perinatal asphyxia, with or without HIE, presented atypical neurological findings in sequential examinations. The profile of neurological findings for children with perinatal asphyxia without HIE resembled that of children with HIE. White matter T2 hyperintensity was associated with atypical spontaneous movements in the HINE and was a frequent MRI finding also in perinatal asphyxia without HIE.

关键词： treatable autoimmune encephalitides   N-methyl  

摘要： Since the launch of The Lancet Neurology in 2002, the subsequent two decades have seen a revolution in our understanding of the neurological diseases affecting children. [...]the care of a patient requires two levels of diagnosis, an aetiological diagnosis and the diagnosis of the epilepsy syndrome, to ensure that comorbidities are recognised and management is optimal. New ways to access somatic variants include the analysis of cell-free DNA from CSF allowing, for the first time, mutation identification of brain-limited variants without the need of using brain tissue.12 A challenge for understanding neurobiology is the increasingly apparent overlap between the molecular determinants of paediatric neurological diseases, such as autism spectrum disorder, intellectual disability, movement disorders, cerebral palsy, and epilepsy, not to mention later onset diseases such as schizophrenia. Administration of an antisense oligonucleotide once every 3 months, designed to result in retention of exon 7 of SMN2 pre-mRNA to produce increased full length SMN2, has life-changing outcomes, compared with the natural history of this disease.19 An alternative form of gene therapy for this disease has also been successful using a safe, adeno-associated viral (AAV) vector.

关键词： Ischemic stroke   Middle cerebral artery occlusion   Gut microbiome   Neuroinflammation   Neuroplasticity   Dietary protein  

摘要： Background: Moderate dietary protein restriction confers neuroprotection when applied before ischemic stroke. How a moderately protein-reduced diet influences stroke recovery when administered after stroke, is a clinically relevant question. This question has not yet been investigated. Methods: Male C57BL6/J mice were exposed to transient intraluminal middle cerebral artery occlusion. Immediately after the stroke, mice were randomized to two normocaloric diets: a moderately protein-reduced diet containing 8% protein (PRD) or normal diet containing 20% protein (ND). Post-stroke neurological deficits were evaluated by a comprehensive test battery. Antioxidant and neuroinflammatory responses in the brain and liver were evaluated by Western blot and RTqPCR. Stroke-induced brain injury, microvascular integrity, glial responses, and neuroplasticity were assessed by immunohistochemistry. Fecal microbiota analysis was performed using 16S ribosomal RNA amplicon sequencing. Results: We show that PRD reduces brain infarct volume after three days and enhances neurological and, specifically, motor-coordination recovery over six weeks in stroke mice. The recovery-promoting effects of PRD were associated with increased antioxidant responses and reduced neuroinflammation. Histochemical studies revealed that PRD increased long-term neuronal survival, increased peri-infarct microvascular density, reduced microglia/ macrophage accumulation, increased contralesional pyramidal tract plasticity, and reduced brain atrophy. Fecal microbiota analysis showed reduced bacterial richness and diversity in ischemic mice on ND starting at 7 dpi. PRD restored bacterial richness and diversity at these time points. Conclusion: Moderate dietary protein restriction initiated post-ischemic stroke induces neurological recovery, brain remodeling, and neuroplasticity in mice by mechanisms involving antiinflammation and, in the post-acute phase, commensal gut microbiota rebalancing.

关键词： cytokine   immunoadsorption   immunoglobulin   neurological autoimmune diseases   plasma exchange  

摘要： Background Therapeutic plasma exchange (TPE) and immunoadsorption (IA) are first or second line treatment options in patients with neurological autoimmune diseases, including multiple sclerosis, neuromyelitis optica spectrum disorders (NMSOD), chronic inflammatory demyelinating polyneuropathy, acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barre syndrome), and autoimmune encephalitis. Methods In this prospective randomized controlled monocentric study, we assessed safety and efficacy of therapy with IA or TPE in patients with neurological autoimmune diseases. Treatment response was assessed using various neurological scores as well by measuring immunoglobulin and cytokine concentrations. Clinical outcome was evaluated by application of specific scores for the underlying diseases. Results A total of 32 patients were analyzed. Among these, 19 patients were treated with TPE and 13 patients with IA. IA and TPE therapy showed a comparable significant treatment response. In patients with MS and NMOSD, mean EDSS before and after treatment showed a significant reduction after treatment with IA. We observed a significant reduction of the pro-inflammatory cytokines IL-12, lL-17, IL-6, INF-gamma, and tumor necrosis factor alpha during IA treatment, whereas this reduction was not seen in patients treated with TPE. Conclusions In summary, both IA and TPE were effective and safe procedures for treating neurological autoimmune diseases. However, there was a trend towards longer therapy response in patients treated with IA compared to TPE, possibly related to a reduction in plasma levels of pro-inflammatory cytokines seen only in the IA-treated group.

关键词： Stroke   COVID-19   Thrombolysis   Treatment delay   Outcomes   Mortality  

摘要： Introduction We have demonstrated in a multicenter cohort that the COVID-19 pandemic has led to a delay in intravenous thrombolysis (IVT) among stroke patients. Whether this delay contributes to meaningful short-term outcome differences in these patients warranted further exploration. Methods We conducted a nested observational cohort study of adult acute ischemic stroke patients receiving IVT from 9 comprehensive stroke centers across 7 U.S states. Patients admitted prior to the COVID-19 pandemic (1/1/2019-02/29/2020) were compared to patients admitted during the early pandemic (3/1/2020-7/31/2020). Multivariable logistic regression was used to estimate the effect of IVT delay on discharge to hospice or death, with treatment delay on admission during COVID-19 included as an interaction term. Results Of the 676 thrombolysed patients, the median age was 70 (IQR 58-81) years, 313 were female (46.3%), and the median NIHSS was 8 (IQR 4-16). Longer treatment delays were observed during COVID-19 (median 46 vs 38 min, p = 0.01) and were associated with higher in-hospital death/hospice discharge irrespective of admission period (OR per hour 1.08, 95% CI 1.01-1.17, p = 0.03). This effect was strengthened after multivariable adjustment (aOR 1.15, 95% CI 1.07-1.24, p < 0.001). There was no interaction of treatment delay on admission during COVID-19 (p(interaction) = 0.65). Every one-hour delay in IVT was also associated with 7% lower odds of being discharged to home or acute inpatient rehabilitation facility (aOR 0.93, 95% CI 0.89-0.97, p < 0.001). Conclusion Treatment delays observed during the COVID-19 pandemic led to greater early mortality and hospice care, with a lower probability of discharge to home/rehabilitation facility. There was no effect modification of treatment delay on admission during the pandemic, indicating that treatment delay at any time contributes similarly to these short-term outcomes.

摘要： This review explores children's self-reported outcome measurements in pediatric neurology. We examine the following questions: (1) What is meant by patient-reported health, functioning, and quality of life outcomes? (2) How can patients express whether the interventions they receive do more good than harm? (3) Why and how should pediatric neurology patients help determine the outcomes of interest? (4) What tools and recommendations are available to evaluate the outcomes of interest? Applying patients' perspectives across the processes of evaluation of medical interventions has become an important expectation. These developments, consistent with current healthcare goals, coincide with the evolution of pediatric neurology into a sophisticated diagnostic-interventional field that aims to prolong survival, decrease impairments and symptoms, and improve patients' well-being - the recognized essential endpoints of interest in all medicine.

关键词： ECMO   COVID-19   Stroke   Intracranial haemorrhage  

摘要： Background Patients with Coronavirus disease 2019 (COVID-19)-related acute respiratory disease (ARDS) increasingly receive extracorporeal membrane oxygenation (ECMO) support. While ECMO has been shown to increase risk of stroke, few studies have examined this association in COVID-19 patients. Objective We conducted a systematic review to characterise neurological events during ECMO support in COVID-19 patients. Design Systematic review of cohort and large case series of COVID-19 patients who received ECMO support. Data Sources Studies retrieved from PubMed, EMBASE, Cochrane, Cochrane COVID-19 Study Register, Web of Science, Scopus, ***, and medR chi iv from inception to November 11, 2020. Eligibility Criteria Inclusion criteria were a) Adult population (> 18 year old);b) Positive PCR test for SARS-CoV-2 with active COVID-19 disease;c) ECMO therapy due to COVID-19 ARDS;and d) Neurological events and outcome described while on ECMO support. We excluded articles when no details of neurologic events were available. Results 1,322 patients from 12 case series and retrospective cohort studies were included in our study. The median age was 49.2, and 75% (n=985) of the patients were male. Diabetes mellitus and dyslipidaemia were the most common comorbidities (24% and 20%, respectively). Most (95%, n=1,241) patients were on venovenous ECMO with a median P:F ratio at the time of ECMO cannulation of 69.1. The prevalence of intracranial haemorrhage (ICH), ischaemic stroke, and hypoxic ischaemic brain injury (HIBI) was 5.9% (n=78), 1.1% (n=15), and 0.3% (n=4), respectively. The overall mortality of the 1,296 ECMO patients in the 10 studies that reported death was 36% (n=477), and the mortality of the subset of patients who had a neurological event was 92%. Conclusions Neurological injury is a concern for COVID-19 patients who receive ECMO. Further research is required to explore how neuromonitoring protocols can inform tailored anticoagulation management and improv

关键词： baby motion analysis   early diagnosis   neurodevelopmental disorders   machine learning   deep learning  

摘要： Neurodevelopmental disorders (NDD) are impairments of the growth and development of the brain and/or central nervous system. In the light of clinical findings on early diagnosis of NDD and prompted by recent advances in hardware and software technologies, several researchers tried to introduce automatic systems to analyse the baby's movement, even in cribs. Traditional technologies for automatic baby motion analysis leverage contact sensors. Alternatively, remotely acquired video data (e.g., RGB or depth) can be used, with or without active/passive markers positioned on the body. Markerless approaches are easier to set up and maintain (without any human intervention) and they work well on non-collaborative users, making them the most suitable technologies for clinical applications involving children. On the other hand, they require complex computational strategies for extracting knowledge from data, and then, they strongly depend on advances in computer vision and machine learning, which are among the most expanding areas of research. As a consequence, also markerless video-based analysis of movements in children for NDD has been rapidly expanding but, to the best of our knowledge, there is not yet a survey paper providing a broad overview of how recent scientific developments impacted it. This paper tries to fill this gap and it lists specifically designed data acquisition tools and publicly available datasets as well. Besides, it gives a glimpse of the most promising techniques in computer vision, machine learning and pattern recognition which could be profitably exploited for children motion analysis in videos.

关键词： targeted temperature management   hypothermia   extracorporeal cardiopulmonary resuscitation   neurological outcome   survival outcome  

摘要： Purpose: Targeted temperature management (TTM) is a standard of care in patients after cardiac arrest for neuroprotection. Currently, the effectiveness and efficacy of TTM after extracorporeal cardiopulmonary resuscitation (ECPR) is unknown. We aimed to compare neurological and survival outcomes between TTM vs non-TTM in patients undergoing ECPR for refractory cardiac arrest. Methods: We searched PubMed and 5 other databases for randomized controlled trials and observational studies reporting neurological outcomes or survival in adult patients undergoing ECPR with or without TTM. Good neurological outcome was defined as cerebral performance category Results: We included 35 studies (n = 2,643) with the median age of 56 years (interquartile range [IQR]: 52-59). The median time from collapse to ECMO cannulation was 58 minutes (IQR: 49-82) and the median ECMO duration was 3 days (IQR: 2.0-4.1). Of 2,643, 1,329 (50.3%) patients received TTM and 1,314 (49.7%) did not. There was no difference in the frequency of good neurological outcome at any time between TTM (29%, 95% confidence interval [CI]: 23%-36%) vs. without TTM (19%, 95% CI: 9%-31%) in patients with ECPR (P = 0.09). Similarly, there was no difference in overall survival between patients with TTM (30%, 95% CI: 22%-39%) vs. without TTM (24%, 95% CI: 14%-34%) (P = 0.31). A cumulative meta-analysis by publication year showed improved neurological and survival outcomes over time. Conclusions: Among ECPR patients, survival and neurological outcome were not different between those with TTM vs. without TTM. Our study suggests that neurological and survival outcome are improving over time as ECPR therapy is more widely used. Our results were limited by the heterogeneity of included studies and further research with granular temperature data is necessary to assess the benefit and risk of TTM in ECPR population.