Non-Vitamin K Oral Anticoagulants (NOACs) and Their Reversal
AbstractPurpose of ReviewAn 80-year-old man presents with an acute right hemiparesis and National Institutes of Health Stroke Scale (NIHSS) of 25, 14  h after taking dabigatran. Activated partial thromboplastin time (aPTT) is 42.8 s. Arteriogram demonstrates left internal carotid artery thrombosis. What is the appropriate management of this patient with acute ischemic stroke while on a NOAC?Recent FindingsIdarucizumab is a reversal agent approved for dabigatran, and two more reversal agents, andexanet alfa and aripazine, are currently in development for NOACs.SummaryIn this article, we review currently available ...
Source: Current Neurology and Neuroscience Reports - July 22, 2017 Category: Neuroscience Source Type: research

New Genes Causing Hereditary Parkinson ’s Disease or Parkinsonism
This article reviews was to review genes where putative or confirmed pathogenic mutations causing Parkinson ’s disease or Parkinsonism have been identified since 2012, and summarizes the clinical and pathological picture of the associated disease subtypes.Recent FindingsNewly reported genes for dominant Parkinson ’s disease areDNAJC13,CHCHD2, andTMEM230. However, the evidence for a disease-causing role is not conclusive, and further genetic and functional studies are warranted.RIC3 mutations have been reported from one family but not yet encountered in other patients. New genes for autosomal recessive disease i...
Source: Current Neurology and Neuroscience Reports - July 21, 2017 Category: Neuroscience Source Type: research

Clippers
We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - July 19, 2017 Category: Neuroscience Source Type: research

Secondary Stroke Prevention in Cryptogenic Stroke and Embolic Stroke of Undetermined Source (ESUS)
AbstractPurpose of the ReviewThe purpose of the study was to review the literature on cryptogenic stroke and embolic stroke of undetermined stroke (ESUS). Cryptogenic stroke according to TOAST criteria is a stroke which is not due to cardiogenic embolism, small vessel disease with lacunes or large vessel disease of brain supplying arteries. In the context of secondary stroke prevention studies, cryptogenic stroke is not operationally defined.Recent FindingsThe new concept of “embolic stroke of undetermined source” (ESUS) provides an operational definition. ESUS is diagnosed as a non-lacunar stroke on cerebral i...
Source: Current Neurology and Neuroscience Reports - July 13, 2017 Category: Neuroscience Source Type: research

Laser Interstitial Thermal Therapy for Epilepsy
AbstractMRI-guided laser interstitial thermal therapy (MgLiTT) uses a narrow diameter cannula to stereotactically target and heat deeper cerebral structures. This technique produces a precise lesion in the brain with great reliability because the localized tissue temperature change is monitored in real time. Because MgLiTT minimizes injury to surrounding brain, it appears to have a lower risk of affecting normal neurological function, and because it is done through a burr hole, there is less operative risk, less discomfort, and shorter hospitalizations. It is FDA approved for soft tissue ablation and is being increasingly ...
Source: Current Neurology and Neuroscience Reports - July 11, 2017 Category: Neuroscience Source Type: research

Monitoring the Brain After Cardiac Arrest: a New Era
AbstractPurpose of ReviewOf the approximately 350,000 out-of-hospital, and 750,000 after in-hospital cardiac arrest (CA) events in the US annually approximately 5-9% and 20% respectively may achieve return of spontaneous circulation (ROSC) after attempted cardiopulmonary resuscitation (CPR). Up to 2/3 of these initial survivors may go on die in the subsequent 24-72 hours after ROSC due to a combination of (1) on-going cerebral injury, (2) myocardial dysfunction and (3) massive systemic inflammatory response. In order to successfully manage patients more effectively, monitoring methods are needed to aid clinicians in the de...
Source: Current Neurology and Neuroscience Reports - July 1, 2017 Category: Neuroscience Source Type: research

Immunosenescence: the Role of Aging in the Predisposition to Neuro-Infectious Complications Arising from the Treatment of Multiple Sclerosis
AbstractPurpose of ReviewThis review highlights some of the important changes in the immune system that occur in the process of normal aging. Immunosenescence as a concept is directly relevant to the world of neuro-inflammation, as it may be a contributing factor to the risks associated with some of the current immunosuppressive and immunomodulatory therapies used in treating multiple sclerosis (MS) and other inflammatory disorders.Recent FindingsProfound qualitative and quantitative changes occur in the adaptive and innate immunity compartments during aging. These changes may explain why patients of older age are at an in...
Source: Current Neurology and Neuroscience Reports - July 1, 2017 Category: Neuroscience Source Type: research

Dissection of Cervical and Cerebral Arteries
AbstractPurpose of ReviewWe aimed to summarize recent findings in cervical (CeAD) and intracranial artery dissection (IAD) research.Recent FindingsConsidered a disease of the young- and middle-aged, an analysis on the largest CeAD-population to date (n = 2391) revealed that about 1 of 14 CeAD-patients was aged ≥60 years. Distinct genetic variants were associated with CeAD. However, in clinical practice, genetic investigations are not helpful due to the small effect size. Despite the paucity of data from randomized-controlled trials in CeAD-s troke patients, both intravenous thrombolysis and endovascular t...
Source: Current Neurology and Neuroscience Reports - June 30, 2017 Category: Neuroscience Source Type: research

Controversies in Thrombolysis
AbstractPurpose of ReviewThe purpose of the review is to examine recent evidence on the effects of intravenous thrombolysis and identify the remaining uncertainties.Recent FindingsWe review the results of two large trials (the third International Stroke Trial (IST-3) and The Enhanced Control of Hypertension and Thrombolysis Stroke Study (ENCHANTED)) and the publications from the individual patient data analyses of the trials of alteplase conducted by the Stroke Thrombolysis Trialists Collaboration.SummaryDespite about a 2% risk of fatal intracerebral haemorrhage, on average, adult patients of all ages treated with 0.9 &nbs...
Source: Current Neurology and Neuroscience Reports - June 30, 2017 Category: Neuroscience Source Type: research

Current Controversies on Wernicke ’s Area and its Role in Language
AbstractPurpose of ReviewThe aim of the study is to assess historical anatomical and functional definitions of Wernicke ’s area in light of modern lesion and neuroimaging data.Recent Findings“Wernicke’s area” has become an anatomical label usually applied to the left posterior superior temporal gyrus and adjacent supramarginal gyrus. Recent evidence shows that this region is not critical for speech perception or for word comprehension. Rather, it supports retrieval of phonological forms (mental representations of phoneme sequences), which are used for speech output and short-term memory tasks. Focal...
Source: Current Neurology and Neuroscience Reports - June 27, 2017 Category: Neuroscience Source Type: research

Multiple Sclerosis in Latin Americans: Genetic Aspects
AbstractLatin Americans (LA) are a heterogeneous, multiethnic group of individuals who inhabit the continental countries in Latin America (LATAM), Caribbean islands and constitute the largest ethnic minority in the USA. Commonly used terminology and ethno racial classifications to define these groups may not be accurate. Risk for multiple sclerosis (MS) among LA is generally low to medium but frequencies are increasing in the American hemisphere. Genome-Wide Association Studies (GWAS) in LA show diverse variants and genetic proportions among Mestizos, the most representative ethnic population, who themselves are the produc...
Source: Current Neurology and Neuroscience Reports - June 21, 2017 Category: Neuroscience Source Type: research

The Use of Cannabinoids in Treating Dementia
AbstractPurpose of ReviewTo review and summarise the current evidence on the safety and efficacy of using cannabinoids to treat behavioural and neuropsychiatric symptoms of dementia.Recent FindingsTwo randomised controlled trials testing a synthetic form of tetrahydrocannabinol have shown that while well tolerated, there was no significant therapeutic effect, based on changes to scores on the neuropsychiatric inventory (NPI). Case reports and open label trials have indicated that there may be some therapeutic benefit of adding synthetic cannabinoids as an adjunctive therapy to reduce agitation, aberrant motor behaviour and...
Source: Current Neurology and Neuroscience Reports - June 19, 2017 Category: Neuroscience Source Type: research

Current Concepts of Memory Disorder in Epilepsy: Edging Towards a Network Account
AbstractPurpose of ReviewA paradigm shift in contemporary epileptology has been the reframing of both epilepsy and its comorbid memory disorders as the product of diseased brain networks. The current review discusses some of the clinical and theoretical implications that stem from this shift.Recent FindingsSome implications of a network conceptualisation of epilepsy include a need for more widespread cognitive phenotyping in epilepsy; recognition that memory disorders in epilepsy can be multi-determined, including by non-structural factors; deeper consideration given to the neurodevelopmental context in which memory proble...
Source: Current Neurology and Neuroscience Reports - June 19, 2017 Category: Neuroscience Source Type: research

Comparing the Intracarotid Amobarbital Test and Functional MRI for the Presurgical Evaluation of Language in Epilepsy
AbstractSurgery is the treatment of choice for drug-resistant temporal lobe epilepsy (TLE). However, such surgery frequently causes deficits in language function, especially if performed on the dominant hemisphere. In recent years, the intracarotid amobarbital test (IAT) has been gradually replaced by functional magnetic resonance imaging (fMRI) in the preoperative identification of language areas to estimate the risk of postoperative language decline. In this paper, we review the neural substrates for language processing, how language impairment can result both from TLE itself and from surgical attempts to treat it. Subse...
Source: Current Neurology and Neuroscience Reports - June 17, 2017 Category: Neuroscience Source Type: research

An Update on the Approach to the Imaging of Brain Tumors
AbstractPurpose of ReviewNeuroimaging plays a critical role in diagnosis of brain tumors and in assessment of response to therapy. However, challenges remain, including accurately and reproducibly assessing response to therapy, defining endpoints for neuro-oncology trials, providing prognostic information, and differentiating progressive disease from post-therapeutic changes particularly in the setting of antiangiogenic and other novel therapies.Recent FindingsRecent advances in the imaging of brain tumors include application of advanced MRI imaging techniques to assess tumor response to therapy and analysis of imaging fea...
Source: Current Neurology and Neuroscience Reports - May 17, 2017 Category: Neuroscience Source Type: research

Pathophysiology and Treatment of Memory Dysfunction After Traumatic Brain Injury
AbstractMemory is fundamental to everyday life, and cognitive impairments resulting from traumatic brain injury (TBI) have devastating effects on TBI survivors. A contributing component to memory impairments caused by TBI is alteration in the neural circuits associated with memory function. In this review, we aim to bring together experimental findings that characterize behavioral memory deficits and the underlying pathophysiology of memory-involved circuits after TBI. While there is little doubt that TBI causes memory and cognitive dysfunction, it is difficult to conclude which memory phase, i.e., encoding, maintenance, o...
Source: Current Neurology and Neuroscience Reports - May 12, 2017 Category: Neuroscience Source Type: research

Deep Brain Stimulation Emergencies: How the New Technologies Could Modify the Current Scenario
AbstractAfter 25 years of deep brain stimulation (DBS) for the treatment of Parkinson ’s disease, it has become increasingly recognized that a range of postoperative urgent situations and emergencies may occur. In this review we describe the possible scenarios of DBS-related emergencies: perioperative (intraoperative and early postoperative) and postoperative settings and issues fr om suboptimal control of motor and nonmotor symptoms in the early programming phase and during long-term follow-up. We also outline potential advantages in the management of these emergencies offered by the newest devices, emerging technol...
Source: Current Neurology and Neuroscience Reports - May 11, 2017 Category: Neuroscience Source Type: research

The Potential of Cellular- and Viral-Based Immunotherapies for Malignant Glioma –Dendritic Cell Vaccines, Adoptive Cell Transfer, and Oncolytic Viruses
AbstractPurpose of ReviewMalignant gliomas, including glioblastoma and anaplastic astrocytoma, are the most frequent primary brain tumors and present with many treatment challenges. In this review, we discuss the potential of cellular- and viral-based immunotherapies in the treatment of malignant glioma, specifically focusing on dendritic cell vaccines, adoptive cell therapy, and oncolytic viruses.Recent FindingsDiverse cellular- and viral-based strategies have been engineered and optimized to generate either a specific or broad antitumor immune response in malignant glioma. Due to their successes in the preclinical arena,...
Source: Current Neurology and Neuroscience Reports - May 9, 2017 Category: Neuroscience Source Type: research

Neuroimaging in Central Nervous System Infections
AbstractRadiologic imaging is often employed to supplement clinical evaluation in cases of suspected central nervous system (CNS) infection. While computed tomography (CT) is superior for evaluating osseous integrity, demineralization, and erosive changes and may be more readily available at many institutions, magnetic resonance imaging (MRI) has significantly greater sensitivity for evaluating the cerebral parenchyma, cord, and marrow for early changes that have not yet reached the threshold for CT detection. For these reasons, MRI is generally superior to CT for characterizing bacterial, viral, fungal, and parasitic infe...
Source: Current Neurology and Neuroscience Reports - May 2, 2017 Category: Neuroscience Source Type: research

The New Classification of Seizures by the International League Against Epilepsy 2017
AbstractPurpose of ReviewThis review presents the newly developed International League Against Epilepsy (ILAE) 2017 classification of seizure types.Recent FindingsThe fundamental distinction is between seizures that begin focally in one hemisphere of the brain, generalized onset seizures that apparently originate in both hemispheres, and seizures of unknown onset. Focal seizures optionally can be subclassified according to whether awareness (a surrogate marker for consciousness) is intact or impaired. The next level of classification for focal seizures is motor (with subgroups automatisms, atonic, clonic, epileptic spasms,...
Source: Current Neurology and Neuroscience Reports - April 19, 2017 Category: Neuroscience Source Type: research

Update on Chemotherapy-Induced Peripheral Neuropathy
AbstractPurpose of ReviewThe purpose of this study was to briefly discuss chemotherapy-induced peripheral neuropathy (CIPN) and detail the most important and most recent chemotherapeutic agents implicated. This review will examine neuropathy mechanisms, risk factors, and clinical patterns; novel and prospective drugs with similar effects that are less well known to neurologists are discussed.Recent FindingsCIPN is increasingly recognized for its clinical importance and effect on patient quality of life. Identification of risk factors is ongoing and may enable future risk stratification. Newer classes of agents and new memb...
Source: Current Neurology and Neuroscience Reports - April 18, 2017 Category: Neuroscience Source Type: research

Reward Prediction Errors in Drug Addiction and Parkinson ’s Disease: from Neurophysiology to Neuroimaging
AbstractPurpose of ReviewSurprises are important sources of learning. Cognitive scientists often refer to surprises as “reward prediction errors,” a parameter that captures discrepancies between expectations and actual outcomes. Here, we integrate neurophysiological and functional magnetic resonance imaging (fMRI) results addressing the processing of reward prediction errors and how they might be altered in drug addiction and Parkinson’s disease.Recent FindingsBy increasing phasic dopamine responses, drugs might accentuate prediction error signals, causing increases in fMRI activity in mesolimbic areas in...
Source: Current Neurology and Neuroscience Reports - April 17, 2017 Category: Neuroscience Source Type: research

Genetic Testing in Pediatric Epilepsy
This article summarizes the emerging landscape of pediatric epilepsy, highlighting genetic contributions, and reviews approaches to genetic evaluation for pediatric epilepsy in this context.Recent FindingsAdvances in understanding the genetic basis for epilepsy over the last several years have been due in large part to the identification of de novo genetic variation underlying sporadic severe epilepsy in children; the genetic underpinnings of the more common epilepsies remain largely unknown. Next-generation sequencing approaches have been added to the repertoire of clinical tests for the evaluation of pediatric epilepsy, ...
Source: Current Neurology and Neuroscience Reports - April 13, 2017 Category: Neuroscience Source Type: research

Sleep Disordered Breathing in Duchenne Muscular Dystrophy
AbstractThis review aims to explain the inevitable imbalance between respiratory load, drive, and muscular force that occurs in the natural aging of Duchenne muscular dystrophy and that predisposes these patients to sleep disordered breathing (SDB). In DMD, SDB is characterized by oxygen desaturation, apneas, hypercapnia, and hypoventilation during sleep and ultimately develops into respiratory failure during wakefulness. It can be present in all age groups. Young patients risk obstructive apneas because of weight gain, secondary to progressive physical inactivity and prolonged corticosteroid therapy; older patients hypove...
Source: Current Neurology and Neuroscience Reports - April 11, 2017 Category: Neuroscience Source Type: research

A Novel Way to Measure and Predict Development: A Heuristic Approach to Facilitate the Early Detection of Neurodevelopmental Disorders
AbstractPurpose of ReviewSubstantial research exists focusing on the various aspects and domains of early human development. However, there is a clear blind spot in early postnatal development when dealing with neurodevelopmental disorders, especially those that manifest themselves clinically only in late infancy or even in childhood.Recent FindingsThis early developmental period may represent an important timeframe to study these disorders but has historically received far less research attention. We believe that only a comprehensive interdisciplinary approach will enable us to detect and delineate specific parameters for...
Source: Current Neurology and Neuroscience Reports - April 8, 2017 Category: Neuroscience Source Type: research

Cognitive and Behavioral Interventions in Epilepsy
AbstractPurpose of ReviewCognitive and behavioral treatments for epilepsy offer several advantages, as they are relatively low cost, are non-invasive, lack serious side effects, and facilitate patient participation. Their role in the management of epilepsy, however, is unclear. The following manuscript will critically review the efficacy data regarding psychological treatments for seizure reduction.Recent FindingsEncouraging results have been found for the cognitive behavioral therapy-based Reiter/Andrews approach and mindfulness or arousal-based programs (e.g., yoga, meditation, relaxation, and biofeedback). Most studies ...
Source: Current Neurology and Neuroscience Reports - April 5, 2017 Category: Neuroscience Source Type: research

Multiple System Atrophy - State of the Art
AbstractMultiple system atrophy (MSA) is a rare and fatal neurodegenerative disorder that is characterized by a variable combination of parkinsonism, cerebellar impairment, and autonomic dysfunction. Some symptomatic treatments are available while neuroprotection or disease-modification remain unmet treatment needs. The pathologic hallmark is the accumulation of aggregated alpha-synuclein ( α-syn) in oligodendrocytes forming glial cytoplasmic inclusions, which qualifies MSA as synucleinopathy together with Parkinson’s disease and dementia with Lewy bodies. Despite progress in our understanding of the pathogenes...
Source: Current Neurology and Neuroscience Reports - April 4, 2017 Category: Neuroscience Source Type: research

The Spectrum of Vestibular and Ocular Motor Abnormalities in Thiamine Deficiency
AbstractPurposeThe first aim of this review is to summarize recent ocular motor signs in pre-encephalopathy patients with nutritional deficiency at risk of thiamine deficiency. Timely recognition of thiamine depletion in these patients, who may have a normal brain MRI, could lead to appropriate management and prevention of Wernicke ’s encephalopathy (WE) with full recovery. The second aim is to incorporate recent diagnostic testing on the revised WE diagnostic criteria and the identification of patients who may show slow, partial, or no response to treatment.Recent FindingsSelective vulnerability of periventricular g...
Source: Current Neurology and Neuroscience Reports - April 1, 2017 Category: Neuroscience Source Type: research

Reflections on Ethics and Humanity in Pediatric Neurology: the Value of Recognizing Ethical Issues in Common Clinical Practice
We describe what we understand asethics and how and why developments in technology, novel potential interventions, policies, and societal perspectives challenge us to think about and debate ethical issues. Individual patients are not a singular population; each patient has their own unique life situations, culture, goals, and expectations that need to be considered with a good dose of humanity and humility. We believe that using an ethical lens —by which we mean making an explicit effort to identify and consider these issues openly—will help us to achieve this goal in practice, education, and research. (Source:...
Source: Current Neurology and Neuroscience Reports - March 28, 2017 Category: Neuroscience Source Type: research

Post-Traumatic Sleep-Wake Disorders
AbstractAll living organisms that face a traumatic life event are susceptible to sleep-wake disturbances. Stress, which can result in trauma, evokes a high level of physiological arousal associated with sympathetic nervous system activation, during both sleep and wakefulness. Heredity, sex hormones, early losses, developmental factors and intra- and interpersonal conflicts, contribute to the level of baseline physiological arousal, producing either subclinical, clinical or complex clinical traits, acutely and at any time after exposure to a traumatic event. The risk of acute sleep-wake disturbances becoming disorders and s...
Source: Current Neurology and Neuroscience Reports - March 25, 2017 Category: Neuroscience Source Type: research

Targeted Treatment of Brain Metastases
AbstractPurpose of ReviewBrain metastases are the most common intracranial tumors in adults. Historically, the median survival after the diagnosis of brain metastases has been dismal and medical therapies had a limited role in the management of these patients.Recent FindingsThe advent of targeted therapy has ushered in an era of increased hope for patients with brain metastases. The most common malignancies that result in brain metastases —melanoma, lung cancer, and breast cancer, often have actionable mutations, which make them good candidates for targeted systemic therapy. These brain metastases have been shown to ...
Source: Current Neurology and Neuroscience Reports - March 22, 2017 Category: Neuroscience Source Type: research

CNS Demyelination with TNF- α Blockers
AbstractTumor necrosis factor –α (TNF-α) blockers are a popular therapeutic choice in a number of inflammatory diseases. Thus far, five TNF- α blockers have been approved for clinical use (etanercept, infliximab, adalimumab, golimumab. and certolizumab). Despite being considered relatively safe, serious side effects associa ted with immune suppression have been reported, including central and peripheral nervous system (CNS) demyelinating disorders. It is still elusive whether these events are mere coincidence or a side effect of anti-TNF-α use. In this paper, we review the published case repor...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Subtypes of Parkinson ’s Disease: What Do They Tell Us About Disease Progression?
AbstractParkinson ’s disease is a widely heterogeneous disorder with a broad list of motor and nonmotor manifestations. Identifying subtypes of Parkinson’s disease is one of the top clinical and research priorities. This review aims to summarize the most valid conventional and recent subtyping solutions that have been introduced so far and to update our current knowledge with recent discoveries on the association between subtypes and disease progression. We also discuss the challenges of subtyping in the context of Parkinson’s disease, stability of the subtypes over time, and potential clinical implicatio...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Huntington ’s Disease—Update on Treatments
AbstractHuntington ’s disease (HD) is an autosomal dominantly inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, ending in death. Despite the discovery of the underlying genetic mutation more than 20 years ago, treatment remains focused on symptomatic manageme nt. Chorea, the most recognizable symptom, responds to medication that reduces dopaminergic neurotransmission. Psychiatric symptoms such as depression and anxiety may also respond well to symptomatic therapies. Unfortunately, many other symptoms do not respond to current treatments. Furthermore, hig h...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

α-Synuclein and Parkinsonism: Updates and Future Perspectives
AbstractMutations in theSNCA gene, which encodes the α-synuclein protein, were the first discovered genetic causes of familial parkinsonism with Lewy pathology. To date, six differentSNCA missense mutations as well as multiplications are known to cause parkinsonism. For this review, we performed a literature search to identify all published cases ofSNCA-related parkinsonism to provide an updated summary of the clinical and neuropathological features of parkinsonism due toSNCA mutations. Familial parkinsonism associated withSNCA is rare, but α-synuclein aggregation is a core feature of sporadic parkinsonism, inc...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Neuroimaging in Epilepsy
AbstractIn recent years, the field of neuroimaging has undergone dramatic development. Specifically, of importance for clinicians and researchers managing patients with epilepsies, new methods of brain imaging in search of the seizure-producing abnormalities have been implemented, and older methods have undergone additional refinement. Methodology to predict seizure freedom and cognitive outcome has also rapidly progressed. In general, the image data processing methods are very different and more complicated than even a decade ago. In this review, we identify the recent developments in neuroimaging that are aimed at improv...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Management of Elderly Patients with Glioblastoma
AbstractPurpose of ReviewGlioblastoma represents one of the major challenges in neurooncology and approximately half of the patients are 60  years or older. We summarize the particular situation of elderly glioblastoma patients with a focus on therapeutic considerations.Recent FindingsFavorable molecular markers such as mutations in the isocitrate dehydrogenase (IDH) 1 or 2 genes are virtually absent in glioblastomas in elderly patients. Treatment options are similar to the situation in young patients and comprise surgical resection, radiation therapy, and alkylating chemotherapy. The performance status as well as com...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations
This article reviews recent research on the clinical and molecular aspects of the disease.Recent FindingsNew clinical and biomarker tools have been implemented in order to assist in the diagnosis of the disease. In addition, the generation of mouse models, the availability of ‘omics’ data in brain tissue and the use of new seeding techniques shed light on the molecular events in FFI pathogenesis. Biochemical studies in human samples also reveal that neuropathological alterations in vulnerable brain regions underlie severe impairment in key cellular processes such as mitochondrial and protein synthesis machinery...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Circadian Disruption Associated with Alzheimer ’s Disease
AbstractAlzheimer ’s disease (AD) is increasing in prevalence and has a significant impact on caregivers and the healthcare system. One of the many physiologic process affected by AD is the circadian system, with disruption reflected in abnormalities of the sleep-wake cycle. This interaction is bidirectional, with circadian and sleep disruption influencing disease progression. Understanding the bidirectional relationship between AD and circadian disruption may allow for earlier recognition of the potential to develop dementia as well as improved targeted approaches for therapy. Therapies including melatonin a nd brig...
Source: Current Neurology and Neuroscience Reports - March 21, 2017 Category: Neuroscience Source Type: research

Dietary Interventions and Multiple Sclerosis
AbstractMultiple Sclerosis (MS) is a chronic, disabling neurologic disease that has its onset in young adulthood. While the knowledge about underlying pathogenesis of MS has improved significantly over the last few decades, the exact cause still eludes us. Despite the availability of several United States Food and Drug Administration-approved disease-modifying therapies (DMT) for MS in the last two decades, the disease remains disabling for many. DMT use is limited by its partial effectiveness, significant side effects in many cases, and high cost that leads people with MS (PwMS) to look for alternative management options....
Source: Current Neurology and Neuroscience Reports - March 20, 2017 Category: Neuroscience Source Type: research

Neuroinflammation in Neurodegenerative Disorders —a Review
AbstractThe potential for positron emission tomography (PET) to detect neuroinflammation in vivo has sparked a remarkable interest in various disciplines of neuroscience. Early PET radioligands, such as [11C]PK(R)-11195 for the 18-kDa translocator protein (TSPO) and [11C]L-deprenyl for monoamine oxidase B, have been used in studies designed to clarify the role of neuroinflammation in a variety of psychiatric and neurological disorders. Recent years have witnessed the development of several second-generation PET radioligands for TSPO and radioligands to measure endogenous targets that are active in various stages of the inf...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

Neurological and Neurosurgical Emergencies in Patients with Hematological Disorders
AbstractPurpose of ReviewHematologic diseases are blood disorders which can affect different organs, including the central and peripheral nervous systems. Some of them are associated with increased risk of permanent disability and death. This review highlights a selected group of primary and acquired hematologic disorders that can present as neurologic or neurosurgical emergencies.Recent FindingsThere is an increasing recognition of the broad neurologic presentations of hematologic disorders. Diagnostic criteria continue to be revised as we learn more about these diseases. Treatment options are varied depending on the hema...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

Update on the Genetics of Dystonia
We present confirmatory evidence for a role of dystonia genes that had not yet been unequivocally established includingPRKRA,TUBB4A,ANO3, andTAF1. We finally discuss selected novel genes for dystonia such asKMT2B andVAC14 along with the challenges for gene identification in the NGS era and the translational importance of dystonia genetics in clinical practice. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

Integrating the Constructs of Anosognosia and Metacognition: a Review of Recent Findings in Dementia
We present the predominant theoretical models of awareness and summarize both traditional and emerging approaches to assessing awareness from clinical and meta-cognitive perspectives. In this review, we focus primarily on findings from recent studies in anosognosia and meta-cognition in the context of neurodegenerative disease with special emphasis on Alzheimer ’s disease and frontotemporal dementia. Emerging trends in the study of awareness, including examination of the longitudinal course of anosognosia, and investigation of the neural substrates underlying meta-cognitive abilities are addressed. Finally, the pract...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

The Application of Proteomics to Traumatic Brain and Spinal Cord Injuries
AbstractTraumatic brain injury (TBI) and traumatic spinal cord injury (SCI), collectively termed neurotrauma, are two parallel neurological conditions that can cause long-lasting neurological impairment and other comorbidities in patients, while at the same time, can create a high burden to society. To date, there are still no FDA-approved therapeutic interventions for either TBI or SCI. Recent advances in proteomic technologies, including tandem mass spectrometry, as well as imaging mass spectrometry, have enabled new approaches to study the differential proteome in TBI and SCI with the use of either animal disease models...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

Recurrent Gastrointestinal Disturbance: Abdominal Migraine and Cyclic Vomiting Syndrome
AbstractPrimary headache disorders, including migraine, are some of the most common neurological disorders presenting to hospital. Episodic syndromes that may be associated with migraine, including recurrent gastrointestinal disturbances such as abdominal migraine and cyclic vomiting, often pre-date or co-occur with the onset of migraine in a child who is at risk of developing the headache condition. The purpose of this review is to evaluate the two most common episodic syndromes, abdominal migraine and cyclic vomiting syndrome, including their pathophysiology, common presentations, and diagnostic criteria. Differential di...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

PFO and Migraine: Is There a Role for Closure?
AbstractObservational studies suggest that closure of a patent foramen ovale for other indications may reduce or even eliminate migraine attacks, particularly migraine with aura. The first randomized clinical trial of patent foramen ovale (PFO) closure for prevention of migraine, the MIST trial, showed negative results. The results of the other two completed studies in this area have recently been published in the last year. PRIMA and PREMIUM were also both negative for their primary endpoints. The PREMIUM trial did show a reduction in headache days in the migraine with aura subgroup but the final results of this subset an...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

Migraine and Stroke: What ’s the Link? What to Do?
AbstractMigraine and stroke are common, disabling neurologic disorders, with a high socioeconomic burden. A link between them has been proposed years ago, and various theories have been proposed to explain this bidirectional relation. However, the precise causes remain unclear. We briefly summarize existing hypotheses of this correlation seeking for recommendations for stroke prevention in migraineurs, if any exist. Among the strongest suggested theories of migraine –stroke association are cortical spreading depression, endovascular dysfunction, vasoconstriction, neurogenic inflammation, hypercoagulability, increased...
Source: Current Neurology and Neuroscience Reports - March 10, 2017 Category: Neuroscience Source Type: research

New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M
AbstractPurpose of ReviewThis review will discuss the role of several key players in glioma classification and biology, namely isocitrate dehydrogenase 1 and 2 (IDH1/2), alpha thalassemia/mental retardation syndrome X-linked (ATRX), B-Raf (BRAF), telomerase reverse transcriptase (TERT), and H3K27M.Recent FindingsIDH1/2 mutation delineates oligoden-droglioma, astrocytoma, and secondary glioblastoma (GBM) from primary GBM and lower-grade gliomas with biology similar to GBM. Additional mutations including TERT, 1p/19q, and ATRX further guide glioma classification and diagnosis, as well as pointing directions toward individual...
Source: Current Neurology and Neuroscience Reports - March 7, 2017 Category: Neuroscience Source Type: research

Huntington Disease: Linking Pathogenesis to the Development of Experimental Therapeutics
AbstractHuntington disease (HD) is an autosomal dominant neurodegenerative condition caused by a CAG trinucleotide expansion in the huntingtin gene. At present, the HD field is experiencing exciting times with the assessment for the first time in human subjects of interventions aimed at core disease mechanisms. Out of a portfolio of interventions that claim a potential disease-modifying effect in HD, the target huntingtin has more robust validation. In this review, we discuss the spectrum of huntingtin-lowering therapies that are currently being considered. We provide a critical appraisal of the validation of huntingtin as...
Source: Current Neurology and Neuroscience Reports - March 6, 2017 Category: Neuroscience Source Type: research