摘要： A functional neurologic disorder is a prevalent and disabling condition at the intersection of neurology and psychiatry [1] with a current PubMed search of the term Functional Neurology (FN) listing 44,939 articles [2], 2618 Systematic Reviews [3] and 191 Meta-Analysis [4]. A PubMed search of my name and FN finds one publication addressing persistent vegetative state as a pejorative term [5] and another addressing the metrological performance of instruments used in clinical evaluation of balance [6]. However, Demortier and Leboeuf-Yde reviewed 121 of my publications through October, 2018 to measure the effect/benefit of treatment/intervention using a FN approach when I have not written anything on the subject.

关键词： Robotic gait training   Rehabilitation   Gait   Stroke   Spinal cord injury   Assist-as-needed  

摘要： Background In clinical practice, therapists choose the amount of assistance for robot-assisted training. This can result in outcomes that are influenced by subjective decisions and tuning of training parameters can be time-consuming. Therefore, various algorithms to automatically tune the assistance have been developed. However, the assistance applied by these algorithms has not been directly compared to manually-tuned assistance yet. In this study, we focused on subtask-based assistance and compared automatically-tuned (AT) robotic assistance with manually-tuned (MT) robotic assistance. Methods Ten people with neurological disorders (six stroke, four spinal cord injury) walked in the LOPES II gait trainer with AT and MT assistance. In both cases, assistance was adjusted separately for various subtasks of walking (in this study defined as control of: weight shift, lateral foot placement, trailing and leading limb angle, prepositioning, stability during stance, foot clearance). For the MT approach, robotic assistance was tuned by an experienced therapist and for the AT approach an algorithm that adjusted the assistance based on performances for the different subtasks was used. Time needed to tune the assistance, assistance levels and deviations from reference trajectories were compared between both approaches. In addition, participants evaluated safety, comfort, effect and amount of assistance for the AT and MT approach. Results For the AT algorithm, stable assistance levels were reached quicker than for the MT approach. Considerable differences in the assistance per subtask provided by the two approaches were found. The amount of assistance was more often higher for the MT approach than for the AT approach. Despite this, the largest deviations from the reference trajectories were found for the MT algorithm. Participants did not clearly prefer one approach over the other regarding safety, comfort, effect and amount of assistance. Conclusion Automatic tuning had the f

关键词： Combined malonic and methylmalonic aciduria   ACSF3   mtFASII   Metabolic flexibility   Brain energy metabolism  

摘要： Combined malonic and methylmalonic aciduria (CMAMMA) is an inborn error of metabolism which has been proposed being a benign condition. However, older patients may present with neurological manifestations such as seizures, memory problems, psychiatric problems and/ or cognitive decline. In fibroblasts from CMAMMA patients we have recently demonstrated a dysregulation of energy metabolism with increased dependency on beta-oxidation for energy production. Because of the inability of the brain to rely efficiently on this pathway to retrieve the required energy to a great extent, we hypothesize an alternative disease-causing mechanism that does not only include the accumulation of the metabolites malonic and methylmalonic acids. Here, we suggest a novel hypothesis on the possible pathophysiological mechanism responsible for the development of neurological symptoms in the long-run.

关键词： ubiquitination   ubiquitin ligases   seizure   epilepsy   degradation   proteasome  

摘要： Ubiquitination is a post-translational modification that can dynamically alter the function, degradation and transport of a protein, as well as its interaction with other proteins, and activity of an enzyme. Dysfunctional ubiquitination is detrimental to normal cellular functions, and can result in severe diseases. Over the last decade, although much research has focused on deciphering the role of the ubiquitination/ubiquitin proteasome system (UPS) in the onset and progression of various neurological disorders, the specific relationship between ubiquitination and various epilepsies has not been carefully reviewed. As an increasing amount of research has revealed the roles of ubiquitination in the trafficking of ion channels and the turn-over of synaptic receptors, it is crucial to take a deeper look into ubiquitination-associated epilepsy. Here, we review the role of ubiquitination in maintaining normal cellular activities in neurons and recent findings on the dysregulation of ubiquitination in epilepsy. We particularly focus on rare neurological disorders with comorbid epilepsy in the hope of drawing more attention to this area. Through categorizing epilepsy-associated E3 ubiquitin ligases and their substrates and discussing ubiquitination-related rare neurological disorders, we summarize where the field stands at the moment and what directions we should consider in the future. (C) 2019 IBRO. Published by Elsevier Ltd. All rights reserved.

关键词： ABNS   Education   Residency education   USMLE step 2  

摘要： Objectives: The neurosurgery residency match is becoming increasingly competitive, with numerous factors being considered as part of the application. We aim to determine whether USMLE Step 2 scores were a significant predictor of neurosurgery board performance. Patients and methods: Residents who entered a neurological surgery residency program at a single academic institution during 2000-2017 provided scores for all ABNS attempts, USMLE Step 1 and Step 2 scores. Data were deidentified and analyzed for correlation and regression. Pearson's correlation coefficients were determined. Results: USMLE Step 1, Step 2, and maximum ABNS scores were all normally distributed. Step 1 and Step 2 scores were less variable than ABNS scores. USMLE Step 2 and residents' best ABNS written examination scores were not correlated (Pearson Correlation of 0.228 with a 2-tailed significance of 0.272). No outliers were present. When comparing USMLE Step 2 scores with year in residency at which residents scored over 300 on the ABNS written examination, Pearson correlation was -0.500 (p = .015). A simple linear regression was calculated using Step 2 scores to predict the passing year of ABNS written examination (F(1,14) = 6.984, p = .015, R-2 = 0.25). Conclusion: Although other studies have found correlations between USMLE Step 2 scores and performance before graduating medical school and during residency for other specialties, this is the first study comparing USMLE Step 2 scores with the ABNS written examination scores of neurosurgical residents. Our data showed that USMLE Step 2 was not a reliable predictor of ABNS written examination scores.

关键词： Cardiac arrest   Automated external defibrillator   Cardiopulmonary resuscitation   Public policy  

摘要： Background: Randomized controlled trials or observational studies showed that the use of public-access automated external defibrillator (AED) was effective for patients with out-of-hospital cardiac arrest (OHCA). However, it is unclear whether public-access AED use is effective for all patients with OHCA irrespective of first documented rhythm. We aimed to evaluate the effect of public-access AED use for OHCA patients considering first documented rhythm (shockable or non-shockable) in public locations. Methods: From the Utstein-style registry in Osaka City, Japan, we obtained information on adult patients with OHCA of medical origin in public locations before emergency-medical-service personnel arrival between 2011 and 2015. Primary outcomewas 1-month survivalwith favorable neurological outcome. Multivariable logistic regression analysiswas performed to assess the association between the public-access AED pad application and favorable neurological outcome after OHCA by using one-to-one propensity score matching analysis. Results: Among 1743 eligible patients, a total of 336 (19.3%) patients received public-access AED pad application. The proportion of patients who survived 1-month with favorable neurological outcome was significantly higher in the pad application group than in the non-pad application group (29.8% vs. 9.7%;adjusted odds ratio [AOR], 2.85;95% confidence interval [CI], 1.73-4.68, AOR after propensity score matching, 2.46;95% CI, 1.29-4.68). In a subgroup analysis, the AORs of patients with shockable or non-shockable rhythms were 3.36 (95% CI, 1.786.35) and 2.38 (95% CI, 0.89-6.34), respectively. Conclusions: Public-access AED pad application was associated with better outcome among patients with OHCA of medical origin in public locations irrespective of first documented rhythm. (C) 2019 Elsevier B.V. All rights reserved.

关键词： Antiganglioside antibodies   Gangliosides   Guillain-Barre syndrome   Polyradiculoneuropathy   Nodo-paranodopathies  

摘要： Gangliosides are sialylated glycosphingolipids, highly abundant in our nervous system. Antibodies targeting gangliosides are usually developed as a consequence of molecular mimicry following infections. Antiganglioside antibodies are implicated in many neurological disorders such as acute and chronic polyradiculoneuropathies which includes different variants of Guillain-Barre syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy. Presence of such antibodies in paraneoplastic peripheral neuropathy, neurodegenerative disorders, multiple sclerosis, myasthenia gravis and amyotrophic lateral sclerosis have also been reported. Recent evidence supports a role of antiganglioside antibodies in the pathogenesis of acute vestibular syndrome. Binding of antibodies to gangliosides on axonal membranes, nodes of Ranvier, myelin sheath components, Schwann cells, neuromuscular junctions or other neural cell surfaces may elicit inflammatory damage through complement-dependent and independent mechanisms, resulting in nerve conduction blocks and subsequent axonal degeneration. Gangliosides are essential for proper cell signaling, transduction and influences neuroplasticity, all of which are affected by autoimmune mediated damage. Better insight into the pathophysiological role of antiganglioside antibodies in different neurological diseases may improve their utility as diagnostic and prognostic biomarkers.

摘要： Use of bionic overground exoskeletons to assist with neurological rehabilitation is becoming increasingly prevalent and has important implications for physiotherapists and their patients. Yet, there is a paucity of research about the impact of integrating this technology on physiotherapists' work. The purpose of this study was to explore how the training and implementation of using the Ekso robotic exoskeleton with patients affects physiotherapists' work. An exploratory qualitative study of three physiotherapists working at a neurological rehabilitation centre in Eastern Canada was conducted using one-on-one semistructured interviews in July 2017. Audio recordings were transcribed verbatim, and data was coded and analyzed using thematic analysis. Six themes emerged from the data: developing organizational capacity;ethical use of technology;benefits of the equipment;challenges of the equipment;cognitive workload;and the technological environment. The results suggest that the adoption and integration of bionic exoskeletons into rehabilitation practice is not as simple as training physiotherapists and giving them the device. More research is needed to understand the increased cognitive demands of working with patients using technologically advanced exoskeletons within a dynamic, technology-rich healthcare environment, while managing patient expectations and ethical use.

关键词： vasculitis   autoinflammatory diseases   hypertension   anti-TNF  

摘要： A 9.5-year-old boy was referred with a 2-year history of recurrent fever, myalgia, abdominal pain and various neurological manifestations associated with increased acute phase reactants and IgG level. During the recent episode, severe hypertension and right-sided hemiparesis developed and angiography demonstrated irregularities and stenosis in renal and mesenteric artery branches. Although these manifestations were consistent with polyarteritis nodosa (PAN), the consanguinity of his parents, a cousin with similar clinical features and early disease onset led to suspicion of deficiency of adenosine deaminase type 2 (DADA2) diseases. DADA2 was established by demonstration of decreased ADA2 enzyme activity and a homozygous G47R mutation in the CECR1 gene. The diagnosis of DADA2 is challenging because of the overlapping manifestations with PAN and other periodic fever syndromes. DADA2 should be considered in the differential diagnosis of PAN. Raised IgG levels (usually low in DADA2) should be sought in future cases.

关键词： parainfluenza virus 5   virus discovery   encephalitis   neurological disease   cattle   genome analysis   in situ hybridization   simian virus 5   bovine astrovirus   bovine herpesvirus  

摘要： The etiology of viral encephalitis in cattle often remains unresolved, posing a potential risk for animal and human health. In metagenomics studies of cattle with bovine non-suppurative encephalitis, parainfluenza virus 5 (PIV5) was identified in three brain samples. Interestingly, in two of these animals, bovine herpesvirus 6 and bovine astrovirus CH13 were additionally found. We investigated the role of PIV5 in bovine non-suppurative encephalitis and further characterized the three cases. With traditional sequencing methods, we completed the three PIV5 genomes, which were compared to one another. However, in comparison to already described PIV5 strains, unique features were revealed, like an 81 nucleotide longer open reading frame encoding the small hydrophobic (SH) protein. With in situ techniques, we demonstrated PIV5 antigen and RNA in one animal and found a broad cell tropism of PIV5 in the brain. Comparative quantitative analyses revealed a high viral load of PIV5 in the in situ positive animal and therefore, we propose that PIV5 was probably the cause of the disease. With this study, we clearly show that PIV5 is capable of naturally infecting different brain cell types in cattle in vivo and therefore it is a probable cause of encephalitis and neurological disease in cattle.