关键词： emergency medicine   general paediatrics   neurology  

摘要： Aim: Referral wait times for paediatric neurological patients are increasing, leading to an increased burden on the emergency department (ED). The paediatric Rapid Access Neurology (pRAN) clinic was created for paediatric patients who are clinically stable, but require an urgent paediatric neurology consultation. The objectives were to evaluate the pathways of referral, accuracy of referring diagnoses, adherence to clinic appointments, impact of clinic visitation on ED visits and patient satisfaction. Methods: Data were collected from the pRAN clinic from March 2018 until April 2019. Information was obtained from patient charts including the referring and final diagnosis, management plan and the number of visits made to the ED before and after visiting the pRAN clinic. Results: Of the 256 referred patients, 91 met inclusion criteria. The most frequent referral diagnosis was a seizure. Referring physicians and pRAN clinic neurologists differed significantly in the level of diagnostic agreement for patients <2 years of age (P = 0.03;95% confidence interval (CI) -0.294, 0.373). There was a significant reduction in visits to the ED made by patients 3 months after the pRAN appointment compared with before the visit (P < 0.001;95% CI -0.9070, -0.4088). The majority of patients felt that the clinic had high value and were satisfied with their follow-up plan. Conclusion: This pilot study showed that a pRAN clinic can improve the accuracy of neurological diagnoses and management, especially for children <2 years of age. In addition, pRAN clinic patients make fewer subsequent visits to the ED and express high satisfaction with their care.

关键词： caudate nucleus   Huntington's disease   imaging mass spectrometry   lipid   lipidomics   MALDI  

摘要： Huntington's disease (HD) is a fatal disorder associated with germline trinucleotide repeat expansions in the HTT gene and characterised by striatal neurodegeneration. No efficacious interventions are available for HD, highlighting a major unmet medical need. The molecular mechanisms underlying HD are incompletely understood despite its monogenic aetiology. However, direct interactions between HTT and membrane lipids suggest that lipidomic perturbations may be implicated in the neuropathology of HD. In this study, we employed matrix-assisted laser desorption/ionisation imaging mass spectrometry (MALDI-IMS) to generate a comprehensive, unbiased and spatially resolved lipidomic atlas of the caudate nucleus (CN) in human post-mortem tissue from neurologically normal (n = 10) and HD (n = 13) subjects. Fourier transform-ion cyclotron resonance mass spectrometry and liquid chromatography-tandem mass spectrometry were used for lipid assignment. Lipidomic specialisation was observed in the grey and white matter constituents of the CN and these features were highly conserved between subjects. While the majority of lipid species were highly conserved in HD, compared to age-matched controls, CN specimens from HD cases in our cohort spanning a range of neuropathological grades showed a lower focal abundance of the neuroprotective docosahexaenoic and adrenic acids, several cardiolipins, the ganglioside GM1 and glycerophospholipids with long polyunsaturated fatty acyls. HD cases showed a higher focal abundance of several sphingomyelins and glycerophospholipids with shorter monosaturated fatty acyls. Moreover, we demonstrate that MALDI-IMS is tractable as a primary discovery modality comparing heterogeneous human brain tissue, provided that appropriate statistical approaches are adopted. Our findings support further investigation into the potential role of lipidomic aberrations in HD.

关键词： Traumatic brain injury (TBI)   Aquaporin-4 (AQP4)   Glymphatic system   Inflammation  

摘要： Traumatic brain injury (TBI) has been a crucial health problem, with more than 50 million patients worldwide each year. Glymphatic system is a fluid exchange system that relies on the polarized water channel aquaporin-4 (AQP4) at the astrocytes, accounting for the clearance of abnormal proteins and metabolites from brain tissues. However, the dysfunction of glymphatic system and alteration of AQP4 polarization during the progression of TBI remain unclear. AQP4(-/-) and Wild Type (WT) mice were used to establish the TBI mouse model respectively. Brain edema and Evans blue extravasation were conducted 24 h post-injury to evaluate the acute TBI. Morris water maze (MWM) was used to establish the long-term cognitive functions of AQP4(-/-) and WT mice post TBI. Western-blot and qRT-PCR assays were performed to demonstrate protective effects of AQP4 deficiency to blood-brain barrier (BBB) integrity and amyloid-beta clearance. The inflammation of cerebral tissues post TBI was estimated by ELISA assay. AQP4 deficiency alleviated the brain edema and neurological deficit in TBI mice. AQP4-knockout led to improved cognitive outcomes in mice post TBI. The BBB integrity and cerebral amyloid-beta clearance were protected by AQP4 deficiency in TBI mice. AQP4 deficiency ameliorated the TBI-induced inflammation. AQP4 deficiency improved longer-term neurological outcomes in a mouse model of TBI.

关键词： Diagnosis   Etiology   Migraine   Neurological deficit   Seizure   Transient ischemic attack  

摘要： Objective. - Differentiating transient ischemic attack from stroke mimics may be difficult. Besides clinical evaluation and brain imaging, electroencephalography (EEG) may be a useful diagnostic tool. Methods. - We conducted spectral analysis on 67 EEG of patients who had presented a transient neurological deficit (TND) within the previous seven days. Expert clinicians provided the final diagnosis: transient ischemic attack, migraine with aura, focal seizure or "other". We first calculated the relative power of the four EEG frequency bands (delta, theta, alpha and beta), in the whole hemisphere, then, according to the clinical symptoms, in the relevant electrodes of the symptomatic hemisphere. Finally, we calculated the relative power ratio between symptomatic and asymptomatic hemispheres. Results. - Median age was 60.6 years (57% females). The etiological diagnosis was transient ischemic attack (27%), migraine with aura (11%), focal seizures (22%) and "other" (40%). We did not find significant differences in the theta and delta relative power analysis between groups. Over the symptomatic hemisphere only, we found a significant increase of the alpha relative power (p = 0.0026, p < 0.0001, p = 0.0014) in the migraine group compared to transient ischemic attack, migraine and focal seizures groups, and a significant decrease of the beta relative power (p = 0.0034, p = 0.0016, p = 0.0005) compared to the same groups. Conclusions. - Migraine with aura presents a discriminative EEG relative power in comparison to transient neurological deficits of other origins. To further investigate the additive diagnosis value of EEG in other TND, future studies should be performed with an EEG obtained within the first 24 h after the onset of symptoms. Significance. - Spectral EEG analysis discriminates migraine with aura groups from other groups, but not at the individual level. (C) 2021 Elsevier Masson SAS. All rights reserved.

关键词： Cognition   Cognitive dysfunction   Mental disorders   Neurological rehabilitation   Cognitive remediation   Activities of daily living   Social functioning  

摘要： Cognitive impairment occurs across several neuropsychiatric diseases and impede everyday functioning and quality of life. Fully immersive Virtual Reality (VR) aid motivation and engagement and therefore has a potential to help overcome the obstacles in the field of cognitive rehabilitation. The aim of this systematic review is to investigate whether VR can be a useful intervention in cognitive rehabilitation transdiagnostically. We identified nine studies with randomized controlled trials following the PRISMA guidelines in databases Pubmed, Embase and PsychInfo. The trials were all evaluated through Cochrane Collaboration's Risk of Bias. The studies were conducted in patients with mild cognitive impairment (k=4), schizophrenia (k=3), ADHD (k=1), or stroke (k=1) and involved 6-12 weeks of training. Overall, results showed improvement in some domains of cognition, primarily executive function and attention. The studies were pilot studies with 6-34 participants per treatment group. Risk of bias was either high (k=3) or moderate (some concerns) (k=6). Key reasons were suboptimal statistical analyses and lack of clarification on randomization and blinding of participants and assessors. In conclusion, this review found promising evidence for VR cognitive rehabilitation for neuropsychiatric illnesses. However, larger and methodologically stronger studies are warranted to establish the full potential of VR.

关键词： transitional care   neurological disorders   pediatrics  

摘要： Childhood-onset movement disorders represent a heterogenous group of conditions. Given the complexity of these disorders, the transition of care from pediatric to adult medicine is an important consideration. We performed a scoping review of the literature on transitional care in chronic neurological disease, exploring key transitional issues and proposed transitional care models. Our aim was to describe the current knowledge and gaps about the transition process of young adults with chronic neurological disorders, paying special attention to childhood onset movement disorders. A total of 64 articles were included in the qualitative synthesis;56 articles reported on transitional care issues, and 8 articles reported on transitional care models. Only 2 articles included patients with movement disorders. The following 4 main transitional issues were identified following synthesis of the available literature: (1) inadequate preparation for the transition process, (2) inappropriate and inconsistent transition practices, (3) inadequate adult services, and (4) heightened emotional response surrounding transition. Of the reported transitional care models, multidisciplinary ambulatory care was the most common approach. In studies evaluating patient-related outcomes, positive health, educational, and vocational outcomes were found. The available literature provides insights on issues that can arise during transition that should be addressed to improve patient and caregiver comfort and satisfaction with care. Further research is needed to evaluate how transitional care programs affect outcomes and their cost effectiveness. More studies are required to determine the needs and outcomes specific to patients with childhood onset movement disorders. (c) 2020 International Parkinson and Movement Disorder Society

关键词： curriculum   headache medicine   medical education  

摘要： Objective To investigate the current headache medicine education paradigm in allopathic and osteopathic medical schools in the United States and Canada. Background There is a disparity in the number of clinicians specially trained to treat patients with headache disorders and the number of people who have them. Early education and exposure to headache medicine is crucial to address this disparity. However, the current state of headache education within medical schools across the United States and Canada is unknown. Methods The authors created a medical student headache education survey, which is a 20-question REDCap survey that was distributed via email to the neurology clerkship director, curriculum dean, or similar role at each US and Canadian MD or DO conferring medical school. The email listserv was created using the American Academy of Neurology Clerkship Directory, the Association of American Medical Colleges Organization Directory, the American Association of College of Osteopathic Medicine Organization Directory, manual searches of the institutions' websites, and phone calls and emails to administrators as needed. Results Of the 249 individuals contacted, 78 completed the survey, yielding a response rate of 31.3%. Of those responses, 84.6% of respondents (66/78) reported that their institution has at least one mandatory session on headache disorders. Many of these sessions (72.7% (48/78)) occurred during preclinical training, and 74.2% (49/78) occurred as part of the clinical curricula. Of respondents, 44.9% (39/78) reported that their institutions coordinate headache education across training levels (i.e., from preclinical to clinical), and only 17.9% (14/78) coordinate across clinical rotations. The most common topics covered were headache red flags, migraine, pharmacologic management, and differentiating primary versus secondary headache. 65.4% of respondents (51/78) felt that the preclinical headache curriculum prepares their students for the clinical ex

关键词： Cerebellum   tDCS   rTMS   Motor learning   Cerebellar reserve   Motor function recovery  

摘要： Importance The cerebellum plays an important role in motor, cognitive, and affective functions owing to its dense interconnections with basal ganglia and cerebral cortex. This review aimed at summarizing the non-invasive cerebellar stimulation (NICS) approaches used to modulate cerebellar output and treat cerebellar dysfunction in the motor domain. Observation The utility of NICS in the treatment of cerebellar and non-cerebellar neurological diseases (including Parkinson's disease, dementia, cerebellar ataxia, and stroke) is discussed. NICS induces meaningful clinical effects from repeated sessions alone in both cerebellar and non-cerebellar diseases. However, there are no conclusive data on this issue and several concerns need to be still addressed before NICS could be considered a valuable, standard therapeutic tool. Conclusions and relevance Even though some challenges must be overcome to adopt NICS in a wider clinical setting, this tool might become a useful strategy to help patients with lesions in the cerebellum and cerebral areas that are connected with the cerebellum whether one could enhance cerebellar activity with the intention of facilitating the cerebellum and the entire, related network, rather than attempting to facilitate a partially damaged cortical region or inhibiting the homologs' contralateral area. The different outcome of each approach would depend on the residual functional reserve of the cerebellum, which is confirmed as a critical element to be probed preliminary in order to define the best patient-tailored NICS.

关键词： Coronavirus   Neurodegeneration  

摘要： During the recent years, viral epidemic due to coronaviruses, such as SARS (Severe Acute Respiratory Syndrome), Middle East Respiratory Coronavirus Syndrome (MERS), and COVID-19 (coronavirus disese-19), has become a global problem. In addition to causing cardiovascular and respiratory lethal dysfunction, these viruses can cause neurodegeneration leading to neurological disorders. Review of the current scientific literature reveals the multiple neuropathies and neuronal dysfunction associated with these viruses. Here, we review the major findings of these studies and discuss the main neurological sequels and outcomes of coronavirus infections with SARS, MERS, and COVID-19. This article analyzes and discusses the main mechanisms of coronavirus-induced neurodegeneration according to the current experimental and clinical studies. Coronaviruses can damage the nerves directly through endovascular dysfunctions thereby affecting nerve structures and synaptic connections. Coronaviruses can also induce neural cell degeneration indirectly via mitochondrial dysfunction inducing oxidative stress, inflammation, and apoptosis. Thus, coronaviruses can cause neurological disorders by inducing neurovascular dysfunction affecting nerve structures and synaptic connections, and by inducing inflammation, oxidative stress, and apoptosis. While some of these mechanisms are similar to other RNA viruses, the neurotoxic mechanisms of COVID-19, MERS, and SARS-CoV viruses are unknown and need detailed clinical and experimental studies.