关键词： Simulation   Mastery learning   Education   Seizures   Status epilepticus  

摘要： Background: Appropriate and timely treatment of status epilepticus (SE) decreases morbidity and mortality. Therefore, skill-based training in the identification and management of SE is crucial for clinicians. Objective: The objective of the study was to develop and evaluate the impact of a simulation-based mastery learning (SBML) curriculum to train neurology residents on the identification and management of SE. Methods: We used pretest-posttest design with a retention test on SE skills for this study. Neurology residents in the second postgraduate year (PGY-2) were eligible to participate in the SE SBML curriculum. Learners completed a baseline-simulated SE skills assessment ( pretest) using a 26 item dichotomous skills checklist. Next, they participated in a didactic session about the identification and management of SE, followed by deliberate skills practice. Subsequently, participants completed another skills assessment (posttest) using the same 26-item checklist. All participants were required to meet or exceed a minimum passing standard (MPS) determined by a panel of 14 SE experts using the Mastery Angoff standard setting method. After meeting the MPS at posttest, participants were reassessed during an unannounced in situ simulation session on the medical wards. We compared pretest with posttest simulated SE skills performance and posttest with reassessment in situ performance. Results: The MPS was set at 88% (23/26) checklist items correct. Sixteen neurology residents participated in the intervention. Participant performance improved from a median of 4423% (Interquartile range (IQR): 34.62-55.77) at pretest to 94.23% (IQR: 92.13-100) at the posttest after SBML (p < .001). There was no significant difference in scores between the posttest and in situ test up to 8 months later (9423%;IQR: 92.31-100 vs. 92.31%;IQR: 88.46-96.15;p = .13). Conclusions: Our SBML curriculum significantly improved residents' SE identification and management skills that were largely re

关键词： Brain lesion   Cryptogenic neurological disease   Diagnostic workup   Neuropathology   Neurosurgery  

摘要： Background: For nonneoplastic neurological diseases, no recommendation exists regarding the place or appropriate timing of brain biopsy. The aim of this study was to evaluate the diagnostic yield and safety of brain biopsies from patients with neurological diseases of unknown etiology. Methods: We performed a retrospective cohort study from January 1, 2008 to December 31, 2018. We analyzed 1847 brain-biopsied patients, including 178 biopsies indicated for neurological diseases of unknown etiology. Specific histological and final diagnosis rates, positive diagnosis-associated factors, complication rate and complication-associated factors were assessed. Results: Specific histological diagnosis and final diagnosis rates were 71.3% and 83.1%, respectively, leading to therapeutic management change(s) for 75.3% of patients. Brain- biopsy-related mortality and permanent neurological morbidity occurred in 1.1% and 0.6% of the patients, respectively. The multivariable logistic-regression model retained (odds ratio [95% CI] only immunodepression (2.2 [1.1-4.7];P = .04) as being independently associated with specific histological diagnosis, while supratentorial biopsy-targeted lesions (4.1 [1.1-15.2];P = .04) were independently associated with a final diagnosis. Biopsies obtained from comatose patients were less contributive to the diagnosis (0.2 [0.05-0.7];P = .01). Prebiopsy platelet count <100 G/L (28.5 [1.8-447];P = .02), hydrocephalus (6.3 [1.2-15.3];P = .02) and targeted lesions <1 cm (4.3 [1.2-15.3];P = .03) were independently associated with brain biopsy-related complications. Conclusion: For highly selected patients with neurological diseases of unknown etiology, brain biopsy has a high diagnostic yield and low frequency of severe complications. We advocate that this procedure be considered early in the diagnosis algorithm of these patients.

关键词： COVID-19   SARS-CoV-2   encephalitis   ADEM  

摘要： Preliminary clinical data indicate that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with neurological and neuropsychiatric illness. Responding to this, a weekly virtual coronavirus disease 19 (COVID-19) neurology multi-disciplinary meeting was established at the National Hospital, Queen Square, in early March 2020 in order to discuss and begin to understand neurological presentations in patients with suspected COVID-19-related neurological disorders. Detailed clinical and paraclinical data were collected from cases where the diagnosis of COVID-19 was confirmed through RNA PCR, or where the diagnosis was probable/possible according to World Health Organization criteria. Of 43 patients, 29 were SARS-CoV-2 PCR positive and definite, eight probable and six possible. Five major categories emerged: (i) encephalopathies (n = 10) with delirium/psychosis and no distinct MRI or CSF abnormalities, and with 9/10 making a full or partial recovery with supportive care only;(ii) inflammatory CNS syndromes (n = 12) including encephalitis (n = 2, para- or post-infectious), acute disseminated encephalomyelitis (n = 9), with haemorrhage in five, necrosis in one, and myelitis in two, and isolated myelitis (n = 1). Of these, 10 were treated with corticosteroids, and three of these patients also received intravenous immunoglobulin;one made a full recovery, 10 of 12 made a partial recovery, and one patient died;(iii) ischaemic strokes (n = 8) associated with a pro-thrombotic state (four with pulmonary thromboembolism), one of whom died;(iv) peripheral neurological disorders (n = 8), seven with Guillain-Barre syndrome, one with brachial plexopathy, six of eight making a partial and ongoing recovery;and (v) five patients with miscellaneous central disorders who did not fit these categories. SARS-CoV-2 infection is associated with a wide spectrum of neurological syndromes affecting the whole neuraxis, including the cerebral vasculature and, in some ca

摘要： Mutations of SOX10 result in a broad range of phenotypes including Waardenburg syndrome (WS types 2 and 4) that can be found in association with peripheral demyelinating neuropathy and/or central dysmyelinating leukodystrophy. Here, we generated induced pluripotent stem cells (iPSCs) from a patient carrying a de novo heterozygous missense mutation in the SOX10 gene (MIM* 602229, NM006941.3c.523C > G;***175Ala) presenting with deafness, depigmentation and progressive neurological impairment. Cells were reprogrammed by non-integrative viral transduction from blood sample, have normal karyotype, express pluripotency markers and are able to differentiate into the three germ cell layers.

关键词： Eugenics   Feebleminded   Medical ethics   Epilepsy   Huntington disease   Friedreich ataxia   Eugenics Record Office   Nuremberg Code  

摘要： Background: In 1936, the American Neurological Association (ANA) published the book "Eugenical Sterilization: A Reorientation of the Problem" in response to what the first author of the book described as a positive reception to a paper presented at the ANA's 1935 annual meeting. The conclusions of the presentation were approved by the organization during the same meeting. As evidenced by the publication of several book reviews in a variety of medical journals, the book garnered some attention. Methods: Reviews of the ANA's book were sought using PubMed, Google Scholar, and Embasa. Also, the book's title was used to search the World Wide Web. Results: The search yielded four reviews, all published in 1937. The reviews make evident a positive opinion of the ANA's book's authors' recommendations including the option for "selective sterilization" of patients with conditions such as Huntington disease, Friedreich ataxia, and epilepsy. In addition, reviewers highlighted the book's authors' assessment that "the feebleminded [breed] docile, servile, useful people who do the dirty work of the race, [as] servants fulfilling a social function." Conclusions: Although the book's authors did not advocate for all-out eugenical sterilization, they did little to counter the popular opinion that patients with certain neurological diseases were a drain on society. In addition, they espoused a positive vision of the feebleminded's role as servants who can do undesirable work. This message was disseminated through book reviews. (C) 2020 Elsevier Inc. All rights reserved.

关键词： Migraine headache   Cardiovascular disease   Stroke   Woman  

摘要： Migraine headache (MH) is a common disorder affecting millions of people in the United States. MH is substantially more prevalent in women compared to men. An association between migraine with or without aura and risk of cardiovascular disease (CVD) has been extensively reported. There are several proposed theories that may explain the pathophysiologic relationship between MH and CVD. This review will summarize the recent literature on this topic and provide an evidence-based perspective regarding the current knowledge and controversies regarding association of MH and CVD. (C) 2019 The Authors. Published by Elsevier Inc.

关键词： Cardiac arrest   Resuscitation   Cardiopulmonary resuscitation   Emergency medical services   Extracorporeal life support  

摘要： Background: The "no flow" interval is the time from out-of-hospital cardiac arrest (OHCA) to cardiopulmonary resuscitation (CPR). Its prognostic value is important to define for prehospital resuscitation decisions, post-resuscitation care and prognostication, and extracorporeal cardiopulmonary resuscitation (ECPR) candidacy assessment. Methods: We examined bystander-witnessed OHCAs without bystander CPR from two Resuscitation Outcomes Consortium datasets. We used modified Poisson regression to model the relationship between the no-flow interval (9-1-1 call to professional resuscitation) and favourable neurological outcome (Modified Rankin Score <= 3) at hospital discharge. Furthermore, we identified the no-flow interval beyond which no patients had a favourable outcome. We analysed a subgroup to simulate ECPR-treated patients (witnessed arrest, age < 65, non-asystole initial rhythm, and >30 min until return of circulation). Results: Of 43,593 cases, we included 7299;616 (8.4%) had favourable neurological outcomes. Increasing no-flow interval was inversely associated with favourable neurological outcomes (adjusted relative risk 0.87, 95% CI 0.85-0.90);the adjusted probability of a favourable neurological outcome decreased by 13% (95% CI 10-15%) per minute. No patients (0/7299, 0%;1-sided 97.5% CI 0-0.051%) had both a no-flow interval >20 min and a favourable neurological outcome. In the hypothetical ECPR group, 0/152 (0%;1-sided 97.5% CI 0-2.4%) had both a no-flow interval >10 min and a favourable neurological outcome. Conclusions: The probability of a favourable neurological outcome in OHCA decreases by 13% for every additional minute of no-flow time until high-quality CPR, with the possibility of favourable outcomes up to 20 min.

关键词： PFBS   Probiotic   Gut-brain axis   Immune system   Neurotoxicity   Zebrafish  

摘要： Perfluorobutanesulfonate (PFBS), an aquatic pollutant of emerging concern, is found to disturb the neural signaling along gut-brain axis, whereas probiotic additives have been applied to improve neuroendocrine function of teleosts. Both PFBS and probiotics can commonly target nervous system. However, whether and how probiotic bacteria can modulate the neurotoxicities of PFBS remain not explored. It is thus necessary to elucidate the probiotic modulation of PFBS neurotoxicity, which can provide implications to the application of probiotic bacteria in aquaculture industry. In the present study, adult zebrafish were exposed to 0, 10 and 100 mu g/L PFBS with or without dietary administration of probiotic Lactobacillus rhamnosus. Interaction between PFBS and probiotic along gut-brain axis was examined, covering three dominant pathways (i.e., neurotransmission, immune response and hypothalamic-pituitary-adrenal (HPA) axis). The results showed that, compared to the single effects, PFBS and probiotic coexposure significantly altered the acetylcholinesterase activity and neurotransmitter profiles in gut and brain of zebrafish, with mild effects on neuronal integrity. Neurotransmitters closely correlated reciprocally in intestines, which, however, was distinct from the correlation profile in brains. In addition, PFBS and probiotic were combined to impact brain health through absorption of bacterial lipopolysaccharides and production of inflammatory cytokines. Relative to neurotransmission and immune signaling, HPA axis was not involved in the neurotoxicological interaction between PFBS and probiotic. Furthermore, it needs to point out that interactive modes between PFBS and probiotic varied a lot, depending on exposure concentrations, sex and toxic indices. Overall, the present study provided the first evidence that probiotic supplement could dynamically modulate the neurotoxicities of PFBS in teleost. (C) 2020 Elsevier Ltd. All rights reserved.

关键词： Cerebellar ataxia   Glutamic acid decarboxylase   Autoantibodies   Vertigo   Diplopia  

摘要： A prompt diagnosis and treatment of patients with autoimmune cerebellar ataxia (CA) with antibodies against glutamic acid decarboxylase (GAD-Abs) may lead to a better prognosis. Herein, we report prodromal transient neurological symptoms that should raise clinical suspicion of CA with GAD-Abs. We initially identified a 70-year-old man who presented a first acute episode of vertigo, diplopia, and ataxia lasting 2 weeks. Two months later, he experienced a similar episode along with new-onset gaze-evoked nystagmus. After 4 months, downbeat nystagmus, left limb dysmetria, and gait ataxia progressively appeared, and an autoimmune CA was diagnosed based on the positivity of GAD-Abs in serum and cerebrospinal fluid (CSF). We searched retrospectively for similar presentations in a cohort of 31 patients diagnosed with CA and GAD-Abs. We found 11 (35.4%) patients (all women, median age 62 years;8/11 [72.7%] with autoimmune comorbidities) with transient neurological symptoms antedating CA onset by a median of 3 months, including vertigo in 9 (81.8%;described as paroxysmal in 8) and fluctuating diplopia in 3 (27.3%) patients. The identification of transient neurological symptoms of unknown etiology, such as paroxysmal vertigo and fluctuating diplopia, should lead to GAD-Abs testing in serum and CSF, especially in patients with autoimmune comorbidities.

关键词： Neurological complications   COVID-19   Altered mental status   Length of stay   Mortality   African Americans  

摘要： People with Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection, COVID-19, can have neurological problems including headache, anosmia, dysgeusia, altered mental status (AMS), ischemic stroke with or without large vessel occlusion, and Guillen-Barre Syndrome. Louisiana was one of the states hit hardest by the pandemic with just over 57,000 laboratory-confirmed cases of COVID-19 by the end of June 2020. We reviewed the electronic medical records (EMR) of patients hospitalized during the peak of the pandemic, March 1st through March 31st, to document the type and frequency of neurological problems seen in patients with COVID-19 at presentation to the emergency room. Secondary aims were to determine: 1) the frequency of neurological complaints during the hospital stay;2) whether the presence of any neurological complaint at presentation or any of the individual types of neurological complaints at admission predicted three separate outcomes: death, length of hospital stay, or the need for intubation;and 3) if the presence of any neurological complaint or any of the individual types of neurological complaints developed during hospital stay predicted the previous three outcomes. A large proportion of our sample (80 %) was African American and had hypertension (79 %). Out of 250 patients, 56 (22 %) patients died, and 72 (29 %) patients required intubation. Thirty-four (14 %) had a neurological chief complaint at presentation;the most common neurological chief complaints in the entire sample were altered mental status (AMS) (8 %), headache (2 %), and syncope (2 %). We used a competing risk model to determine whether neurological symptoms at presentation or during hospital stay were predictors of prolonged hospital stay and death. To establish whether neurological symptoms were associated with higher odds of intubation, we used logistic regression. Age was the only significant demographic predictor of death and hospital stay. The HR (95 %CI) for remaining i