关键词： Epileptic spasms   Hypsarrhythmia   Infantile spasms   Treatment  

摘要： Justification West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. Process An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval. Recommendations Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.

关键词： Free-living   Gait analysis   Instrumentation   Wearable technology  

摘要： Gait assessment is an essential tool for clinical applications not only to diagnose different neurological conditions but also to monitor disease progression as it contributes to the understanding of underlying deficits. There are established methods and models for data collection and interpretation of gait assessment within different pathologies. This narrative review aims to depict the evolution of gait assessment from observation and rating scales to wearable sensors and laboratory technologies and provide limitations and possible future directions in the field of gait assessment. In this context, we first present an extensive review of current clinical outcomes and gait models. Then, we demonstrate commercially available wearable technologies with their technical capabilities along with their use in gait assessment studies for various neurological conditions. In the next sections, a descriptive knowledge for existing inertial and EMG based algorithms and a sign based guide that shows the outcomes of previous neurological gait assessment studies are presented. Finally, we state a discussion for the use of wearables in gait assessment and speculate the possible research directions by revealing the limitations and knowledge gaps in the literature. (C) 2020 IPEM. Published by Elsevier Ltd. All rights reserved.

关键词： All Health Services Research   All Practice Management   Quality Measurement  

关键词： chronic fatigue syndrome   COVID-19   functional neurological disorder   Long Covid   neurological services  

摘要： Aims: In response to the rapid spread of COVID-19, this paper provides health professionals with better accessibility to available evidence, summarising findings from a systematic overview of systematic reviews of the neurological symptoms seen in patients with COVID-19. Implications of so-called 'Long Covid' on neurological services and primary care and similarities with other neurological disorders are discussed. Methods: Firstly, a systematic overview of current reviews of neurological symptoms of COVID-19 was conducted. Secondly, the implications of these findings are discussed in relation to the potential effect on neurological services and the similarities in the experience of patients with COVID-19 and those with other neurological disorders. A total of 45 systematic reviews were identified within seven databases, published between 11 April 2020 and 15 October 2020, following a search in June 2020, updated on 20 October 2020. Results: The results indicated that COVID-19 exhibits two types of neurological symptoms;life-threatening symptoms such as Guillain-Barre Syndrome (GBS) and encephalitis, and less devastating symptoms such as fatigue and myalgia. Many of these so-called lesser symptoms appear to be emerging as longer-term for some sufferers and have been recently labelled Long Covid. When compared, these less devastating symptoms are very similar to other neurological conditions such as chronic fatigue syndrome (CFS) and functional neurological disorder (FND). Conclusion: Implications for neurological healthcare services in the United Kingdom (UK) may include longer waiting times and a need for more resources (including more qualified health professionals). There is also a possible change-effect on health professionals' perceptions of other neurological conditions such as CFS and FND. Future research is recommended to explore changes in health professionals' perceptions of neurological symptoms because of COVID-19.

关键词： Neuroglobin   Mitochondria   Oxidative stress   ATP   Apoptosis   Neurodegenerative diseases  

摘要： Neuroglobin is the third member of the globin family to be identified in 2000 in neurons of both human and mouse nervous systems. Neuroglobin is an oxygen-binding globin found in neurons within the central nervous system as well as in peripheral neurons, that produces a protective effect against hypoxic/ischemic damage induced by promoting oxygen availability within the mitochondria. Numerous investigations have demonstrated that impaired neuroglobin functioning is implicated in the pathogenesis of multiple neurodegenerative disorders. Several in vitro and animal studies have reported the potential of neuroglobin upregulation in improving the neuroprotection through modulation of mitochondrial functions, such as ATP production, clearing reactive oxygen species (ROS), promoting the dynamics of mitochondria, and controlling apoptosis. Neuroglobin acts as a stress-inducible globin, which has been associated hypoxic/ischemic insults where it acts to protect the heart and brain, providing a wide range of applicability in the treatment of human disorders. This review article discusses normal physiological functions of neuroglobin in mitochondria-associated pathways, as well as outlining how dysregulation of neumglobin is associated with the pathogenesis of neurodegenerative disorders.

关键词： COVID-19   SARS-CoV-2   Cerebrospinal fluid   Neurofilament light protein   Total Tau protein   Oligoclonal bands  

摘要： Objectives: To analyze the cerebrospinal fluid (CSF) of patients with SARS-CoV-2 infection and neurological manifestations to provide evidence for the understanding of mechanisms associated with central nervous system (CNS) involvement in COVID-19. Methods: Patients (n = 58) were grouped according to their main neurological presentation: headache (n = 14);encephalopathy (n = 24);inflammatory neurological diseases, including meningoencephalitis (n = 4), acute myelitis (n = 3), meningitis (n = 2), acute disseminated encephalomyelitis (ADEM) (n = 2), encephalitis (n = 2), and neuromyelitis optica (n = 1);and Guillain-Barre syndrome (n = 6). Data regarding age, sex, cerebrovascular disease, and intracranial pressure were evaluated in combination with CSF profiles defined by cell counts, total protein and glucose levels, concentration of total Tau and neurofilament light chain (NfL) proteins, oligoclonal band patterns, and detection of SARS-CoV-2 RNA. Results: CSF of patients with inflammatory neurological diseases was characterized by pleocytosis and elevated total protein and NfL levels. Patients with encephalopathy were mostly older men (mean age of 61.0 17.6 years) with evidence of cerebrovascular disease. SARS-CoV-2 RNA in CSF was detected in 2 of 58 cases: a patient with refractory headache, and another patient who developed ADEM four days after onset of COVID-19 symptoms. Three patients presented intrathecal IgG synthesis, and four had identical oligoclonal bands in CSF and serum, indicating systemic inflammation. Conclusion: Patients with neurological manifestations associated with COVID-19 had diverse CSF profiles, even within the same clinical condition. Our findings indicate a possible contribution of viral replication on triggering CNS infiltration by immune cells and the subsequent inflammation promoting neuronal injury. (C) 2020 The Author(s). Published by Elsevier Ltd on behalf of International Society for Infectious Diseases.

关键词： Cardiac arrest   Gastric regurgitation   Neurological outcome  

摘要： Hypoxic-ischemic brain injury can affect and disturb the autonomous nervous system (ANS), which regulates various visceral systems including the gastro-intestinal and emetic system. The present study aimed to analyze the predictive value of gastric regurgitation (GReg) for neurological outcome in out-of-hospital cardiac arrest (OHCA) survivors. In this prospective, single-center study, 79 OHCA survivors treated at a university-affiliated tertiary care centre were included and GReg was measured at the first day after successful cardiopulmonary resuscitation. Neurological outcome was assessed by the Cerebral Performance Categories score at discharge. Seventy-six percent of the study population had a poor neurological outcome. GReg was found to be associated with poor neurological outcome with an adjusted OR of 5.37 (95% CI 1.41-20.46;p = 0.01). The area under the ROC curve for GReg was 0.69 (95% CI, 0.56-0.81) for poor neurological outcome. GReg on the first day after OHCA is an early, strong and independent predictor for poor neurological outcome in comatose OHCA survivors. These results are particularly compelling because measurement of GReg is inexpensive and routinely performed in critical care units.

关键词： bereavement   child   death   grandparents   pediatric palliative care  

摘要： Purpose Pediatric palliative care focuses mainly on the children suffering from a life-limiting disease, but always includes parents and siblings. However, grandparents are also often highly involved in caring for the child and require additional attention. Therefore, the aim of this study was to investigate the experiences of grandparents during the end-of-life care and after the death of a grandchild. Design and Methods A qualitative approach using semi-structured interviews was chosen. Fifteen grandparents of 10 children who had died of neurological or oncological diseases were interviewed. Participants were recruited among the families cared by the pediatric palliative care team of a children's hospital in northern Switzerland. Grandparents were interviewed at least 1 year after the death of the grandchild. The data was analyzed employing reconstructive interview analysis. Results Regardless of the child's diagnosis and circumstances of death, the participants described how the child's death had a major impact on them and their entire family. Grandparents felt obligated to support the family and constantly be a source of support for the parents. They bore a heavy psychological burden as they cared and mourned not only for their dying grandchild but also for their own daughter or son. Grandparents struggled with their ability to communicate about disease and death. They tried to process and make sense of their loss by remembering the deceased child. Practice Implications These findings emphasize the importance of identifying and understanding grandparents' suffering. Pediatric palliative care teams can achieve this by actively making contact with grandparents, taking their concerns seriously and demonstrating appreciation for their role in supporting the family.

关键词： Eponym   History of neurology   Twentieth century   Evidence-based medicine  

摘要： Eponyms are common in neurology, but their use is controversial. Recent studies have demonstrated increasing eponym use over time in the scientific literature, but it is unclear whether this is a result of authors choosing to use eponyms more frequently, or is merely a product of increasing rates of scientific publication. Our goal was to explore trends in decision-making pertaining to eponym usage. We identified cases where an eponym and a corresponding non-eponymous term existed, and assessed temporal trends in the relative usage of these terms using Google's n-gram viewer for each decade from 1900-2019. Relative to corresponding non-eponymous terms, the use of eponyms increased across the 20th century, peaking in the decade from 1980 to 1989, before sharply declining after the turn of the 21st century. This indicates that when faced with a choice between using an eponym and non-eponymous term, contemporary authors increasingly chose the non-eponymous term. This recent trend may reflect increased awareness of the limitations of eponyms, greater attention to the personal and political lives of namesakes, and a cultural shift toward viewing scientific advances as the result of collective and collaborative efforts rather than the solitary achievements of eminent individuals.

关键词： Injury mechanism   Sport-related concussion   Repetitive head impact   Tissue response   Clinical outcome   Cumulative exposure   Measurement metrics   Brain trauma profiling   Risk mitigation  

摘要： As awareness on the short-term and long-term consequences of sports-related concussions and repetitive head impacts continues to grow, so too does the necessity to establish biomechanical measures of risk that inform public policy and risk mitigation strategies. A more precise exposure metric is central to establishing relationships among the traumatic experience, risk, and ultimately clinical outcomes. Accurate exposure metrics provide a means to support evidence-informed decisions accelerating public policy mandating brain trauma management through sport modification and safer play.