NEURO

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Purpose of review A variety of high-efficacy disease-modifying therapies (DMTs) are available for the treatment of multiple sclerosis (MS). After evaluation and approval by regulatory agencies, DMTs are likely to be administered to patients whose characteristics differ from those enrolled in clinical trials. This may contribute to the emergence of unexpected adverse events observed in the real-world setting. Higher age may be a relevant factor that could change the benefit–risk balance of DMTs, as it may associate with lower efficiency and higher frequency of adverse events. Recent findings The absolute and relative number of patients with MS who reach the age of 55 and higher increases. Growing evidence demonstrates lower efficacy of DMTs in older persons with MS. Specific risks during DMTs for MS, such as the risk of developing progressive multifocal leukoencephalopathy (PML) or the outcome following PML, have been associated with age. It is hypothesized that age-related and therapy-

Purpose of review Research on multiple sclerosis (MS) pathogenesis and therapy is to a large extent driven by results obtained in experimental autoimmune encephalomyelitis (EAE). This approach provided deep insights into the mechanism of brain inflammation and immune mediated tissue injury and, thus, most of our currently established therapies for MS patients have been developed with profound contributions of experimental autoimmune research. Recent data, which are summarized in this review article, however, show important differences between EAE and MS. Recent findings EAE models perfectly reproduce a disease, now called myelin oligodendrocyte glycoprotein (MOG) antibody-associated inflammatory demyelinating disease, which, however, is different from classical MS. In MS, the inflammatory reaction in the brain is dominated by CD8+ T-lymphocyte and CD20+ B cells. Demyelination in MS appears to be triggered by soluble factors, produced by T cells and/or B cells, which are different from

Purpose of review This review provides a brief update of new research findings on the changing epidemiology, disease course, and prognosis of multiple sclerosis (MS). Recent findings Evidence not only continues to support the female predominance in incidence and prevalence of the disease but also supports an increase in incidence of MS in geographic areas that were previously considered to be low incidence for the disease. Summary An increased interest in population-based registries and databases will provide more valid epidemiological measures and observational studies conducted in well-defined study populations. Such studies are crucial for an accurate description of both changing prognosis of MS and differential characteristics of the various MS phenotypes. from Current Opinion in Neurology - Current Issue http://bit.ly/2WfCKzV

Purpose of review The purpose of this review is to describe the new 2017 revisions of the McDonald diagnostic criteria for multiple sclerosis and review first experiences in their application to different patient populations. Recent findings The 2017 revisions agreed on by an international expert panel, as the precursors, define criteria needed to fulfill dissemination in time and space in the clinically isolated syndrome after exclusion of alternative diagnoses. One major change is the inclusion of cerebrospinal fluid (CSF) oligoclonal bands as evidence of dissemination in time in a patient with dissemination in space gathered by clinical or magnetic resonance examination. The distinction between asymptomatic and symptomatic lesions in counting for evidence of dissemination in space or time in supra, infratentorial, and spinal cord syndrome has been abandoned. Finally, cortical lesions can be used to demonstrate dissemination in space. Major differential diagnoses, in particular, the

Purpose of review Focal white matter lesions are the defining pathological and imaging hallmark of the multiple sclerosis. Until recently, elucidation of the pathophysiology of lesion formation, progression and repair has relied on point neuropathological observations. Here, we review current and emerging concepts of the MRI-defined multiple sclerosis lesion phenotype, advanced longitudinal imaging techniques that permit in-vivo exploration of dynamic microstructural change within lesions and emerging MRI measures of lesion repair. Recent findings Novel MRI techniques have elucidated dynamic features of the active multiple sclerosis lesion, defined imaging surrogates for chronic active lesions and revealed progressive microstructural change within chronic inactive lesions. Lesion-related anterograde, retrograde and trans-synaptic neurodegenerative mechanisms are being unravelled in vivo through MRI. An array of myelin-imaging techniques have emerged and in some cases have already been

Purpose of review Multiple sclerosis (MS) and related autoimmune disorders of the central nervous system such as neuromyelitis optica spectrum disorders (NMOSD) are characterized by chronic disability resulting from autoimmune neuroinflammation, with demyelination, astrocyte damage, impaired axonal transmission and neuroaxonal loss. Novel therapeutics stopping or reversing the progression of disability are still urgently warranted. This review addresses research on optic neuritis in preclinical experimental models and their translation to clinical trials. Recent findings Optic neuritis can be used as paradigm for an MS relapse which can serve to evaluate the efficacy of novel therapeutics in clinical trials with a reasonable duration and cohort size. The advantage is the linear structure of the visual pathway allowing the assessment of visual function and retinal structure as highly sensitive outcome parameters. Experimental autoimmune encephalomyelitis is an inducible, inflammatory an

Purpose of review Recent years have seen the approval of more than 15 disease-modifying drugs for multiple sclerosis (MS), mainly for its relapsing-remitting form (RRMS). The focus of the MS clinical trials is moving toward clinical trials aimed at progressive patients or based on putatively neuroprotective compounds. Here we reviewed the challenges of this paradigm shift. Recent findings Progressive MS and neuroprotective drugs trials will both need a change in patients’ enrollment criteria, outcome selection, and clinical trials design. Published ocrelizumab Primary Progressive MS data, as well as translational neuroimaging and clinical research suggest that MRI markers of inflammation could be used to enrich progressive MS trials population, albeit with the risk of overestimating the relevance of antiinflammatory therapeutic effects in this population and that conventional MRI-based metrics need to be complemented with volumetric and multiparametric approaches to disease severity qu

Purpose of review To critically assess the current landscape of disease-modifying agents for multiple sclerosis (MS). Treatment algorithms will be discussed and studies for new agents in late development or recently approved are analyzed in terms of their impact on current treatment strategies. Recent findings A real-world study from Wales suggests that early initiation of highly effective therapy may provide more benefit that an escalation approach in relapsing MS. A study from the MSBase dataset found evidence that early treatment with highly effective therapies decreased the risk of developing secondary progressive MS. Ocrelizumab is highly efficacious in relapsing MS and in a group of patients with primary progressive MS. Another CD20 directed mAb, ofatumumab, is in phase 3. A large study examining extended interval dosing of natalizumab in an attempt to decrease the risk of developing progressive multifocal leukoencephalopathy is underway. Cladribine and alemtuzumab may work by im

Purpose of review Medications for relapsing multiple sclerosis (MS) effectively reduce relapse rate, mitigate disability progression and improve MRI measures of inflammation. However, they have virtually no impact on remyelination which is the major mechanism preventing MS-associated neurodegeneration. Stimulating the generation of myelin-(re)producing cells is therefore a central focus of current MS research and a yet unmet clinical need. Here, we present and evaluate key scientific studies from the field of (therapeutic) remyelination research covering the past 1.5 years. Recent findings On the one hand, recent research in the field of remyelination has strongly focused on repurposing drugs that are already approved for other indications by the Food and Drug Administration or the European Medicines Agency. On the other hand, emerging agents such as the mAbs opicinumab and GNbAC1 target entirely new and unconventional pathways. Some of them have already been tested in clinical trials

Purpose of review The diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) has been revised in the past 20 years and pathological and therapeutic data have been accumulated. This review provides an overview of evolution and broadening of the concept of NMOSD. Recent findings NMOSD encompassing brain syndrome as well as optic neuritis and acute myelitis is now classified into aquaporine-4 (AQP)-antibody-seropositive and aquaporine-4 (AQP)-antibody-seronegative diseases, detecting more patients earlier than before. Seronegative NMOSD includes cases of myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive disease with its unique clinical spectrum somewhat different from AQP4-antibody-seropositive NMOSD. Pathologically, NMOSD includes AQP4-antibody-seropositive autoimmune astrocytopathic disease and MOG-antibody-seropositive inflammatory demyelinating disease. Double seronegative group needs further research. Therapeutic options of NMOSD has taken shape and firs

Purpose of review Migraine is a prevalent and extremely disabling brain disorder, with an impact on the individual, family, workplace and society. This review focuses on describing Calcitonin Gene Related Peptide Monoclonal Antibodies (CGRP-mABs) efficacy on improving the quality of life (QoL) and decreasing the disability and impact of migraine measured with patient related outcomes (PROs), on patients who participated in clinical trials with erenumab, fremanezumab, galcanezumab and eptinezumab. The goal is to better reflect the effect of these preventive migraine treatments in the daily life of our patients. Recent findings CGRP-mABs have been approved by the Food and Drug Administration's (FDA) and European Medicines Agency (EMA) as the first migraine specific treatment for the prevention of migraine. In clinical trials, CGRP-mABs have proven to achieve their primary endpoint which is to reduce the number of headache days, and also have shown to have an impact on disability, QoL

Purpose of review Migraine is a common, highly disabling disorder. Its treatment involves acute and preventive therapy. Many of available preventive medications are not well tolerated, which results in poor compliance and limited effectiveness. Cannabinoids have been proposed for the treatment of migraine but their efficacy and tolerability are controversial. Recent findings Cannabinoids modulate functions and activity of signaling pathways that have a key role in pain control. Growing preclinical evidence and initial clinical findings suggest that modulation of the endocannabinoid system, via endogenous or exogenous cannabinoids may be relevant for migraine via multiple mechanisms. Summary The endocannabinoid system qualifies as an interesting area of research worth exploration in the quest for therapeutic targets for the treatment of migraine. from Current Opinion in Neurology - Current Issue http://bit.ly/2DI0fuf

Purpose of review Migraine is a primary headache disorder and one of the most common and disabling neurological diseases worldwide. Genome-wide association studies have identified ≈40 genetic loci associated with migraine. How these and other genetic findings are used to expand our knowledge on the pathophysiological mechanism of common migraine and rare migraine variants will be discussed. Recent findings The genetic load, based on common polygenic variation, is higher in familial migraine cases than in nonfamilial cases, and higher for migraine with aura and hemiplegic migraine. Migraine shares common genetic variant risks with depression. Specific clinical features of common migraine seem to be determined by genetic factors. A stronger family history of migraine is associated with lower age-at-onset, higher frequency and number of medication days and the migraine with aura subtype. Mild hemiplegic migraine is likely caused by complex polygenic interaction of multiple gene variants a

Purpose of review The current article appraises the recent developments in idiopathic intracranial hypertension (IIH), with particular attention to novel therapeutic avenues and advanced clinical assessment and monitoring with optical coherence tomography and telemetric intracranial pressure devices. Recent findings The incidence of IIH is increasing. The first consensus guidelines for IIH have been published detailing investigation and management algorithms for adult IIH. Improved understanding, clinical assessment and monitoring are emerging with the use of optical coherence tomography. Intracranial pressure telemetry is providing unique insights into the physiology of raised intracranial pressure in IIH. There are now an increasing number of ongoing clinical trials evaluating weight loss methods and novel targeted therapies, such as 11ß-HSD1 inhibition and Glucagon-like peptide 1 (GLP-1) receptor agonists. Summary Several studies are evaluating new therapies for IIH. Monitoring tech

Purpose of review Cluster headache stands among the worst debilitating pain conditions. Available treatments for cluster headache have often disabling side effects, are not tolerated, or are ineffective. The management of drug-refractory chronic forms is challenging. New treatments are warranted and reported here. Recent findings In cluster headache acute treatment, delivery systems like Demand Valve Oxygen or nonrebreather-type masks could enhance the effectiveness of inhaled oxygen therapy. Noninvasive vagus nerve stimulation relieves cluster headache pain at short-term in episodic patients. Sphenopalatine ganglion stimulation combines acute and preventive properties in subsets of patients and is of interest in selected refractory chronic forms. In cluster headache prevention, ‘hypothalamic’ deep brain stimulation is being refined using slightly different stereotactic coordinates or lower risk methods like endoventricular stimulation. Anti-CGRP monoclonal antibodies provide interesti

Purpose of review The trigeminal autonomic reflex is a physiological reflex with an important protective function which also plays a role in pathophysiological conditions, such as primary headache. It is not understood whether the autonomic symptoms in trigeminal autonomic cephalalgias and migraine are the consequence of severe trigeminal discharge or indeed directly driven by central generators as part of the pathophysiology, underlying these syndromes. Recent findings Modulating this reflex, and particularly the parasympathetic reflex arc, has been shown to be effective in treating headache. Among these modulators, left noninvasive vagal nerve stimulation has been shown to bilaterally inhibit the parasympathetic output of the reflex. Furthermore, the peripheral activation of the reflex, resulting in parasympathetic discharge, is not sufficient to provoke headache attacks in cluster headache patients, suggesting a central modulation. Summary Here, we review the anatomy and physiology

Purpose of review Cluster headache is by many regarded as a males’ disorder that is often accompanied by an unhealthy lifestyle. We aimed to study the influence of sex and lifestyle factors on clinical presentation, the diagnostic process and management. Recent findings Overall, the clinical presentation of cluster headache in both sexes was similar; however, chronic cluster headache may occur more frequently in women than in men. Misdiagnosis was most prevalent in women and more women than men were not correctly diagnosed until seen in a highly specialized center. In relation to lifestyle, smoking prevalence remains very high and some studies suggest that obesity and use of illegal drugs may be pronounced as well. In contrast to previous beliefs, recent findings on alcohol consumption report a lower use in patients than in controls. Overall, men and to some extent chronic patients were more prone to some unhealthy lifestyle factors than women and episodic patients. Summary Despite an

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Purpose of review To describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Recent findings Affected patients present with symptoms of one or more of meningitis (headache and neck ache), encephalitis (delirium, tremor, seizures, or psychiatric symptoms), and myelitis (sensory symptoms and weakness). Optic disc papillitis (blurred vision) is common. CNS inflammation is evident in characteristic T1 postgadolinium enhancement of GFAP-enriched CNS regions, and lymphocytic cerebrospinal fluid (CSF) white cell count elevation. CSF is more reliable than serum for GFAP-immunoglobulin G (IgG) testing. Ovarian teratoma commonly coexists, particularly among patients with accompanying N-methyl-D-aspartate receptor or aquaporin-4 autoimmunity. Parainfectious autoimmunity is suspected in some other patients, though the culprit organism is rarely verified. Pathophysiologic relevance of

Purpose of review The clinical interest for auto-antibodies against myelin oligodendrocyte glycoprotein (MOG) has recently reemerged, with the use of more specific detection methods. Large national cohorts have allowed characterizing a more precise clinical spectrum delineated by the presence of human MOG-antibodies. Recent findings In adults with MOG-antibodies, optic neuritis is the most frequent clinical presentation, with features different from multiple sclerosis (MS), including bilateral involvement and predilection for the anterior part of the optic nerve. Myelitis and brainstem syndrome are also frequent, and may clinically mimic neuromyelitis optica spectrum disorders (NMOSD). Despite the frequently severe clinical presentation, most of patients recover quickly after steroids initiation. Other less typical presentations include encephalitis with seizures, cranial nerve involvement, and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroid

Purpose of review To review the clinical findings, differential diagnosis, treatment and outcome of pseudotumoral demyelinating lesions including tumefactive demyelination and Baló's concentric sclerosis. Recent findings MRI findings, such as dynamic restricted diffusion changes at the edge of pseudotumoral lesions help to discriminate atypical demyelination from key differential diagnoses, and together with histopathological data, indicate that tissue hypoxia may be important aetiologically. CT-PET imaging can help to distinguish pseudotumoral lesions from high-grade tumours. Although most patients with pseudotumoral lesions have or later develop multiple sclerosis, a proportion will experience a monophasic course or be diagnosed with neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination or acute disseminated encephalomyelitis (ADEM). Many patients with pseudotumoral demyelinating lesions have a favourable prog

Purpose of review Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality. Recent findings Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease. Recent guidelines have helped to clarify criteria for diagnosing neurosarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment depends on the presentation and distribution, extensiveness, and severity of neurosarcoidosis. As regards evidence-based treatment, only a few randomized controlled trials have been done. Hence, several aspects of (neuro)sarcoidosis management are not fully addressed by the current literature. Summary Significant advances have been made in the

Purpose of review The current review develops the clinical presentations of nonparaneoplastic autoimmune cerebellar ataxia (ACA) and analyzes the association with autoantibodies. Recent findings Emerging evidence suggests that autoimmunity is involved in a significant proportion of sporadic ataxia cases. Moreover, numerous autoantibodies have recently been described in association with sporadic cerebellar ataxia, improving diagnosis and patient categorization. Summary Nonparaneoplastic ACA encompasses postinfectious acute cerebellar ataxia, opsoclonus-myoclonus-ataxia syndrome, and pure cerebellar ataxia with or without autoantibodies. There is still confusion about how to diagnose and classify the patients, and retrospective data suggest that these very rare entities are in fact largely underrecognized. Numerous autoantibodies have been found associated with sporadic ataxia, improving diagnosis accuracy, and patient categorization. However, although anti-glutamate decarboxylase isotyp

Purpose of review Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease. Recent findings Apart from sleep problems, the most frequent neurological symptoms in anti-IgLON5 disease are bulbar dysfunction and gait abnormalities. Other symptoms include movement disorders like chorea or abnormal orofacial movements, oculomotor abnormalities, cognitive impairment, and symptoms of nervous system hyperexcitability. All these symptoms can present in different combinations and severity leading to distinct clinical phenotypes beyond the sleep disorder:

Purpose of review Immune checkpoint inhibitors (ICPI) and chimeric antigen receptor T cells (CAR-T) represent novel therapies recently approved to treat a number of human cancers. As both approaches modulate the immune system, they can generate a number of immune-related adverse events (irAEs), including a large spectrum of novel neurological toxicities. These are of special interest given their potential severity and risk of compromising further oncologic treatment. We aim to provide a comprehensive review of the literature and discuss their optimal management. Recent findings In contrast to irAEs involving other organs, neurological complications of ICPI are uncommon, may present throughout the course of treatment and involve the peripheral and central nervous system, including polyneuropathy, myositis, myasthenia gravis, demyelinating polyradiculopathy, myelitis, encephalitis and others. If started early, ICPI-related neurologic irAEs are usually responsive to steroids. In contrast,

Small changes in a single olfactory receptor gene can affect how strong, or how pleasant, a person finds a particular odor. from Neuroscience News Updates http://bit.ly/2Wfhsmf

Mimicking effects of the ketogenic diet with a drug called 2-DG reduced cell excitation and epileptic activity in mouse models of post-traumatic epilepsy. 2-DG may have the potential to restore network function following TBI, reducing the risk of epilepsy associated with head injuries. The findings may have positive implications for people who developed post-traumatic epilepsy, suggesting a change in diet could help alleviate some symptoms. from Neuroscience News Updates http://bit.ly/2J5mEVW

Veterans with comorbid mild traumatic brain injury and PTSD had significantly greater amygdala volume compared to those with mTBI alone. from Neuroscience News Updates http://bit.ly/2IRzLLc

Mouse models and human brain tissue studies reveal microglia react the amyloid beta earlier in older females. Findings may provide avenues for the development of new drugs to help treat the neurodegenerative disease. from Neuroscience News Updates http://bit.ly/2J6g8y0

Nocebo, the negative counterpart of the placebo phenomenon results in the induction of adverse events (AEs) following the administration of an inert substance. Nocebo has been demonstrated to be associated with low treatment compliance in clinical trials, thus affecting treatment outcomes. This study sought to determine the prevalence of nocebo in cerebellar ataxia. from Journal of the Neurological Sciences http://bit.ly/2VJpwi9

Purpose: Carbon dioxide (CO2) is a potent cerebral vasomotor agent. Despite reduction in CO2 levels (hypocapnia) being described in several acute diseases, there is no clear data on baseline CO2 values in acute stroke. The aim of the study was to systematically assess CO2 levels in acute stroke. Material and methods: Four online databases, Web of Science, MEDLINE, EMBASE and CENTRAL, were searched for articles that described either partial pressure of arterial CO2 (PaCO2) and end-tidal CO2 (EtCO2) in acute stroke. from Journal of the Neurological Sciences http://bit.ly/2Lc4Zi1

The purpose of this study was to elucidate the association of cerebral large artery disease (CLAD) with cerebral blood flow (CBF) in the posterior cerebral artery (PCA) territory and cognitive performance. from Journal of the Neurological Sciences http://bit.ly/2VJpqqN

Inhibiting VIP interneurons disrupts medial prefrontal cortex representations of anxiety-related information which drive avoidance behaviors. from Neuroscience News Updates http://bit.ly/2ZLbcVj

Researchers discovered changes in thousands of genes induced by the tumor microenvironment. The study may help in identifying new biomarkers for the early detection, and subsequent treatment, of brain cancers. from Neuroscience News Updates http://bit.ly/2URVbcy

Please give a warm welcome to Savannah Leitheiser our newest Clinical Service Rep and Dr. Vanlalrinchanni who is a Post doctoral associate in Dr. Rincon Limas’ lab. Savannah Leitheiser Dr. Vanlalrichanni from Department of Neurology http://bit.ly/2IPYNKv

Congratulations to Aparna Wagle-Shukla ,  Anand Rajan ,  Zakia Jabarkheel ,  Nikolaus McFarland on the publication of “Prevalence estimates and clinical characterization for tremor and dystonia in Progressive Supranuclear Palsy.”  This article was published in the April 9, edition of Neurology.   Abstract Objective:  Determine the prevalence rate and clinical characteristics of tremor and dystonia in patients with progressive supranuclear palsy (PSP). Background:  Tremor and dystonia are known to occur in PSP and can sometimes present together. However, there is scant literature on the prevalence and the clinical characteristics of these features, which may help with diagnosis of PSP and development of better-targeted therapies. Design/Methods:  We conducted a retrospective analysis of 217 PSP patients. Diagnosis of PSP was established using recent criteria recommended by the Movement Disorders Society. Data for patients exhibiting tremor, dystonia or both were extracted. Dem

LATE, a form of dementia that appears in the oldest-old is often mistaken for Alzheimer's disease, but the brain pathology is very different. The protein TDP-43 appears to play a significant role in the development of LATE. The neurodegenerative disease may progress more gradually than Alzheimer's, but when combined with Alzheimer's disease (a common combination), appears to cause a more rapid decline than either would alone. from Neuroscience News Updates http://bit.ly/2GLQmwi

In pregnant women and mice, the daily schedule shifts a few hours forward during the first trimester. Activity levels also dip during this time. The advance of the daily schedule stabilizes, before gradually returning to normal during later stages of pregnancy. from Neuroscience News Updates http://bit.ly/2vtX5pp

How VIP interneurons modulate network-level activity to regulate behavior is not understood. Lee et al. show that in the elevated plus maze, prefrontal VIP neurons promote open arm avoidance by disinhibiting theta-frequency communication that transmits anxiety-related information across hippocampal-prefrontal networks. from journals http://bit.ly/2DQF1dZ

Combining electrophysiology, atomic force microscopy, and X-ray crystallography, Dawe et al. reveal that nanoscale mobility of the apo state and TARP stoichiometry coordinate the responsiveness of alternatively spliced AMPA receptors to neurotransmitter, allosteric anions, and TARP auxiliary proteins. from journals http://bit.ly/2GRuCz4

D'Arcy Carter provides insight into her experiences with synesthesia. from Neuroscience News Updates http://bit.ly/2PANkyW

The secretory pathway delivery of cannabinoid type 1 receptor is axonally based, and surface expressed CB1R is more stable in axons than dendrites. from Neuroscience News Updates http://bit.ly/2URIz54

A new study in mice reveals a possible link between the use of SSRI antidepressants during pregnancy and an increased risk of autism-like symptoms in offspring. Offspring exposed to fluoxetine (Prozac) in utero were more likely to exhibit impaired neurotransmission caused by an overactive serotonin receptor in the prefrontal cortex, an area of the brain implicated in modulating social behaviors. However, treating the mice with a compound that blocks the receptor alleviated the behavioral problems and improved working memory. from Neuroscience News Updates http://bit.ly/2URs9K6

Study offers new insights into the mechanisms behind thought control and suggests individual differences play a role in our ability to control our thoughts. from Neuroscience News Updates http://bit.ly/2PGjbyu

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Poor olfaction is correlated with higher long-term mortality in older adults, particularly in those with good health at baseline. Dementia, Parkinson's disease and weight loss may be possible drivers for the higher mortality rates, but around 70% of cases are unexplained. from Neuroscience News Updates http://bit.ly/2V6DHya

Medication-overuse headache detoxification reduces headache disability-the Akershus study of chronic headache Kristoffersen ES, Grande RB, Aaseth K, Russell MB, Lundqvist C Eur J Neurol 2018; 25:1140-1147. Abstract BACKGROUND: Medication-overuse headache (MOH) is a chronic headache (≥15 days/month) associated with overuse of acute headache medication. The objective was to investigate headache-related disability before and after self-detoxification […] via Is detoxification effective for medication overuse headache? — Neurochecklists Blog from The Neurology Lounge http://bit.ly/2Lfo0Qv

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Hippocampal replay selectively enhances memory of highly rewarded locations in a familiar context. from Neuroscience News Updates http://bit.ly/2PC7ysn

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Publication bias is a well-known issue among scientists and clinicians. Research waste is very much in focus among funders and the scientific community, and one of the factors contributing to this wastage is the nonpublication of negative data; that is, data that do not support the initial hypothesis. Through Neurology Null Hypothesis, the American Academy of Neurology (AAN), Neurology , and AAN members are enhancing research culture and opportunities for researchers to reach scientists and clinicians with their findings. Together we can all promote a more balanced and complete publication culture. from Neurology recent issues http://bit.ly/2XYricD

Nonsteroidal anti-inflammatory drugs (NSAIDs) have long been hypothesized to play a protective role in Alzheimer disease (AD). This hypothesis is supported by a large number of cohort studies; a recent meta-analysis of 16 studies demonstrated that current or former NSAID use was associated with a reduced relative risk (RR) of AD (RR 0.81; 95% confidence interval 0.70–0.94). 1 A role for NSAIDs in AD is consistent with the increasing evidence that inflammatory activity plays a crucial role in AD pathogenesis. 2,3 In AD, protein complexes of β-amyloid and hyperphosporylated tau interact with microglia and astrocytes and result in the secretion of cytokines and chemokines, markers of inflammatory activation. Despite the observational epidemiologic data on the protective effect of NSAIDs and the evidence for a biologically plausible role of anti-inflammatory treatment, a large multiyear randomized placebo-controlled trial with naproxen and celecoxib (Alzheimer's Disease Anti-inflamma

Although the incidence is declining, subarachnoid hemorrhage (SAH) continues to exact a substantial toll on society, with an estimated 20,000 aneurysmal SAHs (aSAHs) a year in the United States. 1 Outcomes have improved, but the mortality remains 35%, and half of the survivors cannot return to their previous level of functioning. 2 The most important prognostic factors for outcome are the neurologic condition of the patient on admission to hospital, the patient’s age, and preexisting hypertension. 3 aSAH has a unique biphasic course, with initial or early brain injury (reflected in the neurologic condition) and a delayed phase of brain injury called delayed cerebral ischemia (DCI). Subarachnoid blood and specifically the erythrocytes and their main content, hemoglobin, appear to mediate DCI in humans. Extravascular free hemoglobin and iron released from it, whether into the CSF or the brain, are highly inflammatory and cytotoxic oxidants. 4 The body has developed ways to mitigate t

The American Academy of Neurology (AAN) has a long history of leadership in using principles of evidence-based medicine (EBM) to improve outcomes for our neurologic patients. Starting in 1990, concomitant with the publication of an Institute of Medicine call for development of evidence-based clinical practice guidelines (CPGs), 1 the AAN began developing CPGs. This body of work has resulted in the publication of 90 CPGs. The second stage of evolution in EBM to practice efforts led, beginning in 2010, to the AAN’s development of tools to help us measure processes and outcomes associated with high-quality care: clinical quality measures. Many quality measures are derived from high-quality CPGs. This body of work now encompasses 15 diseases and 139 quality measures. 2 The third stage of the AAN EBM/quality effort led to the rollout in 2016 of the Axon Registry®, 3 a prospective data collection tool that systematically collects and aggregates data for review and analysis. The data, larg

Gretchen Birbeck, MD, MPH from Neurology recent issues http://bit.ly/2XVDzya

A 29-year-old man began haloperidol therapy (1.5 mg/d) for severe agitation due to mental stress. He developed jaw opening dystonia at 1 month, and paroxysmal stridor and dyspnea at rest occurred at 5 months. A fiberscope revealed both involuntary tongue base retraction and airway obstruction (video), suggesting diagnosis of drug-induced oromandibular dystonia. Haloperidol was discontinued. At follow-up 1 year following cessation of haloperidol, he reported gradual disappearance of tongue base retraction within 1 month and the jaw opening dystonia within 4 month. Although tongue base retraction is an uncommon form of oromadibular dystonia, 1,2 it is a serious complication of therapy. from Neurology recent issues http://bit.ly/2XTEsrc

This issue contains a Pearls & Oy-sters article of a case of toxic-metabolic encephalopathy due to bismuth toxicity from prolonged topical application. One Teaching NeuroImages case shows myoclonus as the presenting feature of Wilson disease and the second shows a rare case of Jacobsen syndrome. The Teaching Video NeuroImages case discusses myoclonus as the presenting feature of Wilson disease. from Neurology recent issues http://bit.ly/2GRB7Dd

A 23-year-old man was found at a train station with convulsions. He was treated with benzodiazepines in the field and transferred to the nearest emergency department, where he was intubated. This episode had been preceded by a 2-week history of headaches, nausea, confusion, and staring spells that precluded the patient from going to work. His mother also noted that his eye started to appear "droopy" several days prior to presentation. She stated that he had no recent travel, sick contacts, or tick or insect bites. He was recently prescribed and had been taking acetaminophen/butalbital/caffeine for symptomatic relief of his headaches but was not taking any other medications. He was admitted to an outside hospital and subsequently transferred to our facility. from Neurology recent issues http://bit.ly/2XVDwT0

In the article "Deep brain stimulation improves restless legs syndrome in patients with Parkinson disease," Dr. Klepitskaya et al. reported sustained improvement in symptoms of restless legs syndrome (RLS) over 2 years in 22 patients with Parkinson disease (PD) who underwent subthalamic nucleus (STN) deep brain stimulation (DBS), despite a decrease in dopaminergic treatment. Drs. Marques et al. point out that the article misinterpreted the results of their previous study, which showed an emergence of RLS in patients with PD with a higher dose of dopamine agonists and a lower decrease in dopaminergic treatment after STN DBS. from Neurology recent issues http://bit.ly/2GQV9xH

Several studies assessed the effect of deep brain stimulation (DBS) of the subthalamic nucleus (STN) on restless legs syndrome (RLS) symptoms in patients with Parkinson disease (PD) with conflicting results. 1–5 As underlined by Klepitskaya et al., 1 one crucial variable is dopaminergic therapy, as emergence of RLS after STN DBS in patients with PD has been reported after abrupt decrease of dopaminergic treatment. 3 Klepitskaya et al. argued that, in a previous study we conducted, 5 patients with emergence of RLS after STN DBS took a higher dose and had a more significant reduction of dopamine agonists compared to those without emergence with RLS. Yet, we actually reported the opposite, showing an emergence of RLS in patients with PD with a higher dose of dopamine agonists postoperatively and with a lesser decrease of dopamine agonists after STN DBS. 5 from Neurology recent issues http://bit.ly/2XVDvhU

I thank Marques et al. for the interest in our study, 1 the clarification, and the thoughtful comments. Their study reported that patients with Parkinson disease (PD) with postoperative emergence of restless legs syndrome (RLS) had higher preoperative and postoperative dopamine agonist (DA) doses, and had a lower percentage of DA reduction after deep brain stimulation (DBS), 2 not "more significant reduction" as misinterpreted from our article. 1 This underscores the controversy surrounding this issue since there are reports of RLS emergence after DBS in patients with PD with greater reduction of dopaminergic therapy, 3 less reductions, 2 and no correlation with degree of medication reduction. 1 We thank Marques et al. for bringing attention to the complexity of this topic, and the call for further research in this area that might shed light on the effects of DBS on RLS symptoms and the mechanisms of RLS in general. from Neurology recent issues http://bit.ly/2GQVaSh

In the article "Deep brain stimulation improves restless legs syndrome in patients with Parkinson disease," Dr. Klepitskaya et al. reported sustained improvement in symptoms of restless legs syndrome (RLS) over 2 years in 22 patients with Parkinson disease (PD) who underwent subthalamic nucleus (STN) deep brain stimulation (DBS), despite a decrease in dopaminergic treatment. Drs. Marques et al. point out that the article misinterpreted the results of their previous study, which showed an emergence of RLS in patients with PD with a higher dose of dopamine agonists and a lower decrease in dopaminergic treatment after STN DBS. from Neurology recent issues http://bit.ly/2XWTFYn

I was surprised that the Mystery Case by Lindegger et al. 1 was published in the Resident & Fellow Section. Although there is clear temporal crescent sparing, there are also smaller—but equally clear—residual nasal crescents in both the superior and inferior fields that cross the vertical meridian. The case seems to teach that the anterior most portion of the calcarine cortex has not only peripheral temporal fibers, but also fibers serving a peripheral nasal crescent. from Neurology recent issues http://bit.ly/2GQtZqF

My coauthors and I appreciate Dr. Rosenberg's comments on our article, 1 and we agree in principle. The scope of the case report did not allow for the elaboration of the additional teaching points that Dr. Rosenberg elucidates, namely that there may be a contribution by the so-called peripheral nasal crescent, and that central sparing is typically very congruous. The visual field published is a copy of the exact visual field as it was obtained in the acute clinical setting. 1 It depicts precisely what was extracted from the patient shortly after the original CNS insult by an experienced perimetrist. The patient was not cognitively entirely fit at the time due to the insult itself, which limits the precision of perimetry. However, it demonstrates the main principles offered as teaching points in the short communication. Regarding the comment pertaining to the notch, it is difficult to demonstrate such a finding even by experienced perimetrists, let alone in patients coming out of

from Neurology recent issues http://bit.ly/2GQtYD7

An 8-year-old girl, with no relevant developmental or family history, presented with progressive orolingual and limb dystonia since 3 years of age. MRI brain showed bilateral posterior putamen volume loss and hyperintensity. Susceptibility-weighted images showed globus pallidus blooming in characteristic wishbone pattern with medial and lateral parts forming the forked ends (figure). MRI spine showed features of dysostosis with platyspondyly and vertebral beaking. β-Galactosidase assay was low and genetic workup revealed compound heterozygous pathogenic mutation in GLB1 gene. Combination of putaminal finding and wishbone pattern of iron deposition is highly diagnostic of late onset/type 3 GM1 gangliosidosis 1,2 and helps differentiate from neurodegeneration with brain iron accumulation. from Neurology recent issues http://bit.ly/2GRVTms

A 56-year-old immunocompetent woman presented with severe headaches and encephalopathy. Brain MRI showed numerous punctate infarcts throughout the cerebral and cerebellar hemispheres (figure). White blood cell count was 185,000, of which 97% were eosinophils. Infectious and neoplastic evaluations, including CSF and bone marrow analysis, were unremarkable. The course was complicated by eosinophilic pneumonia, myocardial infarction, and deep venous thrombosis. Endocardial fibrosis was absent. Idiopathic hypereosinophilic syndrome (IHES) was diagnosed. IHES is a leukoproliferative disorder causing multiorgan damage. Diagnosis requires marked peripheral eosinophilia and exclusion of infection, hematologic malignancy, and vasculitis. Infarcts in IHES occur due to microvascular occlusion in watershed regions. 1,2 from Neurology recent issues http://bit.ly/2XUqLbn

Experts worry that the new criteria for certification of thrombectomy-capable stroke centers are insufficient to ensure quality of care. from Front page feed http://bit.ly/2PDAS1j

A prospective case controlled study looked at the relationship of retinal blood flow and vessel density to early onset Alzheimer disease and mild cognitive impairment. from Front page feed http://bit.ly/2vsPxTT

Listening to music was associated with improvements in memory and cognition while participating in mindfulness improved mood in patients who recently suffered an ischemic stroke. Researchers believe combining the two approaches may enhance both cognition and mood in patients early post-stroke. from Neuroscience News Updates http://bit.ly/2WdSSlB

Autism diagnosis becomes stable starting at 14 months of age, researchers report. The accurate diagnosis of ASD, four months earlier than previously believed, leads to more opportunities for early interventions. from Neuroscience News Updates http://bit.ly/2GHquSq

Firing rate set-point regulation has puzzled researchers for decades. Our findings show that mitochondrial DHODH meets the criteria of a bone fide regulator of activity set points and suggest lowering firing set point as a new strategy to treat epilepsy. from journals http://bit.ly/2JazItl

Humans combine multisensory information sharing a common cause while avoiding distraction from irrelevant sources. Cao et al. show how this flexible sensory inference is guided by prefrontal cortex and unfolds as sequential neural computations comprising functionally distinct multisensory representations. from journals http://bit.ly/2IMQ4sv

Researchers have identified a new autoimmune disease that causes muscle pain and weakness. Large-histiocyte-related immune myopathy (LHIM), generates similar results to the condition rhabdomyolysis in blood chemistry samples. LHIM can effectively be treated with anti-inflammatory drugs to help reduce symptoms. from Neuroscience News Updates http://bit.ly/2XY0n0z

Deficiencies in the SHANK3 gene have been linked to sleep disruptions in both mouse models and people with ASD. Researchers found patients with Phelan-McDermid syndrome, a genetic disorder associated with autism, report trouble falling, and staying asleep. In mouse models, animals lacking the SHANK3 gene had a reduction in deep sleep quality and spent more time awake when other mice were sleeping. from Neuroscience News Updates http://bit.ly/2GPRBM5

In Leonora Carrington’s novel The Hearing Trumpet , a woman, Carmella, revels in sending letters to strangers ... The post The Return of the Surrealist Women appeared first on Public Books . from Public Books http://bit.ly/2XRb5pp

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Author: Dale Purves ISBN: 9780190880163 Binding: Hardcover Publication Date: 29 April 2019 Price: $49.95 from journals http://bit.ly/2GEsOJQ

The Mobile Universal Lexicon Evaluation System (MULES) is a test of rapid picture naming under investigation. Measures of rapid automatic naming (RAN) have been used for over 50 years to capture aspects of vision and cognition. MULES was designed as a series of 54 grouped color photographs (fruits, random objects, animals) that integrates saccades, color perception and contextual object identification. We examined MULES performance in youth, collegiate and professional athletes at pre-season baseline and at the sidelines following concussion. from Journal of the Neurological Sciences http://bit.ly/2PIlBN7

Study adds to a growing body of evidence that stress in the workplace, coupled with impaired sleep, increases the risk of cardiovascular disease and coronary mortality, especially in hypersensitive workers. from Neuroscience News Updates http://bit.ly/2ZFQ1UG

Regardless of socioeconomic status, children who were overweight by age 7 had a higher risk of developing emotional problems by age 11, researchers report. from Neuroscience News Updates http://bit.ly/2Pz3UiI

CT1 cells connect around 1400 areas in the fly brain. Each cell area works like a separate neuron, allowing CT1 to access information from the fly's eye and support local motion detection. from Neuroscience News Updates http://bit.ly/2La1KYu