Advances in Biomarker Research in Parkinson’s Disease
Abstract Parkinson’s disease (PD) is the second most common neurodegenerative disease, and the numbers are projected to double in the next two decades with the increase in the aging population. An important focus of current research is to develop interventions to slow the progression of the disease. However, prerequisites to it include the development of reliable biomarkers for early diagnosis which would identify at-risk groups and disease progression. In this review, we present updated evidence of already known clinical biomarkers (such as hyposmia and rapid eye movement (REM) sleep behavior disorder (RBD...
Source: Current Neurology and Neuroscience Reports - December 29, 2015 Category: Neuroscience Source Type: research

Cluster Headache: Special Considerations for Treatment of Female Patients of Reproductive Age and Pediatric Patients
The objective of this article is to explore the current literature pertaining to special considerations in cluster headache management, including treatment of pregnant or breastfeeding patients and pediatric patients. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - December 28, 2015 Category: Neuroscience Source Type: research

Administration of Uric Acid in the Emergency Treatment of Acute Ischemic Stroke
Abstract Oxidative stress is one of the main mechanisms implicated in the pathophysiology of inflammatory and neurodegenerative diseases of the central nervous system (CNS). Uric acid (UA) is the end product of purine catabolism in humans, and it is the main endogenous antioxidant in blood. Low circulating UA levels have been associated with an increased prevalence and worse clinical course of several neurodegenerative and inflammatory diseases of the CNS, including Parkinson’s disease and multiple sclerosis. Moreover, the exogenous administration of UA exerts robust neuroprotective properties in experiment...
Source: Current Neurology and Neuroscience Reports - December 28, 2015 Category: Neuroscience Source Type: research

Management of Patients with an Asymptomatic Carotid Stenosis—Medical Management, Endovascular Treatment, or Carotid Endarterectomy?
Abstract Patients with asymptomatic carotid stenosis all warrant intensive medical therapy; they are at a higher risk of myocardial infarction than of stroke. With modern intensive medical therapy, the annual risk of ipsilateral is now ∼0.5 %. Justifying carotid intervention on the basis of the results of historic trials with a medical arm, extrapolated to modern trials with lower interventional risks but no medical arm is not legitimate. Most patients (∼90 %) with asymptomatic carotid stenosis would be better served by intensive medical therapy than by either stenting or endarterectomy. The few...
Source: Current Neurology and Neuroscience Reports - December 28, 2015 Category: Neuroscience Source Type: research

Understanding and Promoting Resiliency in Patients with Chronic Headache
Abstract Even among patients with the same type and severity of headache, there is considerable variability in functional outcomes. Some individuals are resilient, able to thrive despite pain, whereas others find that pain is an overwhelming burden that comes to define their lives. A substantial body of evidence suggests that patients’ cognitive, emotional, and behavioral coping responses to their pain play a significant role in determining their long-term health. Resilient pain responses, which are shaped by both qualities of the individual and his/her social environment, can be learned and thus hold promi...
Source: Current Neurology and Neuroscience Reports - December 28, 2015 Category: Neuroscience Source Type: research

Infection and Stroke: an Update on Recent Progress
Abstract The role of infection in cerebrovascular disease is complex and remains incompletely understood. Over the last 5 years, investigators have made notable inroads in untangling this thorny topic. In this review, we examine these recent developments, concentrating on four aspects of the relationship between infection and stroke. We first discuss specific infectious agents as direct causes of stroke, focusing on recent work implicating herpesviruses and HIV in cerebral vasculopathy. We then discuss systemic infection of any type as a stroke trigger, focusing on the relationship of infection to timing of ...
Source: Current Neurology and Neuroscience Reports - December 16, 2015 Category: Neuroscience Source Type: research

Update on Cysticercosis Epileptogenesis: the Role of the Hippocampus
Abstract Neurocysticercosis (NCC) is the most common helminthic infection of the nervous system and a frequent cause of reactive seizures and epilepsy worldwide. In many cases, multiple episodes of focal seizures related to an identifiable parenchymal brain cyst (and likely attributable to local damage) continue for years after the cyst resolves. However, cases where seizure semiology, interictal EEG abnormalities, and parasites location do not correlate raise concerns about the causal relationship between NCC and either reactive seizures or epilepsy, as well as the epileptogenic potential of parasites. Neurosurg...
Source: Current Neurology and Neuroscience Reports - December 10, 2015 Category: Neuroscience Source Type: research

Neurological Complications of Bariatric Surgery
Abstract Obesity has attained pandemic proportions, and bariatric surgery is increasingly being employed resulting in turn to more neurological complications which must be recognized and managed. Neurological complications may result from mechanical or inflammatory mechanisms but primarily result from micro-nutritional deficiencies. Vitamin B12, thiamine, and copper constitute the most frequent deficiencies. Neurological complications may occur at reasonably predictable times after bariatric surgery and are associated with the type of surgery used. During the early post-operative period, compressive or stretch pe...
Source: Current Neurology and Neuroscience Reports - October 22, 2015 Category: Neuroscience Source Type: research

Clinical Relevance of Orthostatic Hypotension in Neurodegenerative Disease
Abstract The autonomic nervous system appears to be uniquely susceptible to degeneration in disorders of α-synuclein pathology. Clinically, autonomic dysfunction in these disorders manifests as neurogenic orthostatic hypotension (nOH), a condition that results in substantial morbidity and mortality. nOH results from pathology affecting either the central autonomic pathways or peripheral autonomic nerve fibers. Determining the localization and pathophysiology of nOH is critical in effectively managing this disorder and selecting appropriate treatment options. In this review, we discuss the pathophysiology of...
Source: Current Neurology and Neuroscience Reports - October 20, 2015 Category: Neuroscience Source Type: research

Prevention of Stroke in Rheumatoid Arthritis
Abstract The risk of cerebrovascular disease is increased among rheumatoid arthritis (RA) patients and remains an underserved area of medical need. Only a minor proportion of RA patients achieve suitable stroke prevention. Classical cardiovascular risk factors appear to be under-diagnosed and undertreated among patients with RA. Reducing the inflammatory burden is also necessary to lower the cardiovascular risk. An adequate control of disease activity and cerebrovascular risk assessment using national guidelines should be recommended for all patients with RA. For patients with a documented history of cerebrovascu...
Source: Current Neurology and Neuroscience Reports - October 20, 2015 Category: Neuroscience Source Type: research

Atypical Optic Neuritis
Abstract Classic demyelinative optic neuritis is associated with multiple sclerosis and typically carries a good prognosis for visual recovery. This disorder is well characterized with respect to its presentation and clinical features by baseline data obtained through the optic neuritis treatment trial and numerous other studies. Atypical optic neuritis entails clinical manifestations that deviate from this classic pattern of features. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features to consider include lack of pain, si...
Source: Current Neurology and Neuroscience Reports - October 14, 2015 Category: Neuroscience Source Type: research

Using Administrative Data to Examine Health Disparities and Outcomes in Neurological Diseases of the Elderly
Abstract The fields of neurodegenerative disease and dementia research have grown considerably in the last several decades. Due to tremendous efforts of basic and clinical research scientists, we know a great deal about dementia risk factors and have multiple treatment options. Clinician recognition of cognitive impairment has increased considerably, national policies which support screening for and documenting cognitive dysfunction now exist, and public awareness of neurodegenerative disease has never been greater. These conditions promote (and demand) the growth of translational epidemiology and health ser...
Source: Current Neurology and Neuroscience Reports - October 1, 2015 Category: Neuroscience Source Type: research

Seizure Prediction: Science Fiction or Soon to Become Reality?
Abstract This review highlights recent developments in the field of epileptic seizure prediction. We argue that seizure prediction is possible; however, most previous attempts have used data with an insufficient amount of information to solve the problem. The review discusses four methods for gaining more information above standard clinical electrophysiological recordings. We first discuss developments in obtaining long-term data that enables better characterisation of signal features and trends. Then, we discuss the usage of electrical stimulation to probe neural circuits to obtain robust information regarding e...
Source: Current Neurology and Neuroscience Reports - September 24, 2015 Category: Neuroscience Source Type: research

Super-Refractory Status Epilepticus
Abstract Although the vast majority of patients with status epilepticus (SE) respond fairly well to the first- or second-line anti-epileptics, a minority require anesthetic agents to put the seizures under control. An even smaller number of patients do not even respond to those and constitute the subgroup of super-refractory SE. Because of the small numbers, there are no definitive studies regarding its etiology, pathophysiology, and treatment, and those are still based on expert opinions. Encephalitides, either infectious, autoimmune, or paraneoplastic may be the main etiological factors. Induced pharmacological...
Source: Current Neurology and Neuroscience Reports - September 24, 2015 Category: Neuroscience Source Type: research

Brain Stimulation and the Role of the Right Hemisphere in Aphasia Recovery
This article summarizes the current evidence on noninvasive brain stimulation methods for aphasia and the neuroscientific considerations surrounding treatments using right hemisphere inhibition. Suggestions are provided for further investigation and for clinicians whose patients ask about brain stimulation treatments for aphasia. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - September 22, 2015 Category: Neuroscience Source Type: research

Metabolic Myoglobinuria
Abstract One large group of hereditary myopathies characterized by recurrent myoglobinuria, almost invariably triggered by exercise, comprises metabolic disorders of two main fuels, glycogen and long-chain fatty acids, or mitochondrial diseases of the respiratory chain. Differential diagnosis is required to distinguish the three conditions, although all cause a crisis of muscle energy. Muscle biopsy may be useful when performed well after the episode of rhabdomyolysis. Molecular genetics is increasingly the diagnostic test of choice to discover the underlying genetic basis. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 30, 2015 Category: Neuroscience Source Type: research

Using Patient-Derived Induced Pluripotent Stem Cells to Model and Treat Epilepsies
Abstract Human induced pluripotent stem cells (iPSCs) are transforming the fields of disease modeling and precision therapy. For the treatment of neurological disorders, iPSCs introduce the possibility for targeted cell-based therapies by deriving patient-specific neural tissue in vitro that may ultimately be used for transplantation. We review iPSC technologies and their applications that have already advanced our understanding of neurological disorders, focusing on the epilepsies. We also discuss the application of powerful new tools such as genome editing and multi-well, multi-electrode array recording platfor...
Source: Current Neurology and Neuroscience Reports - August 30, 2015 Category: Neuroscience Source Type: research

Genetic Discoveries Drive Molecular Analyses and Targeted Therapeutic Options in the Epilepsies
Abstract Epilepsy is a serious neurological disease with substantial genetic contribution. We have recently made major advances in understanding the genetics and etiology of the epilepsies. However, current antiepileptic drugs are ineffective in nearly one third of patients. Most of these drugs were developed without knowledge of the underlying causes of the epilepsy to be treated; thus, it seems reasonable to assume that further improvements require a deeper understanding of epilepsy pathophysiology. Although once the rate-limiting step, gene discovery is now occurring at an unprecedented rapid rate, especially ...
Source: Current Neurology and Neuroscience Reports - August 30, 2015 Category: Neuroscience Source Type: research

Update on TBI and Cognitive Impairment in Military Veterans
Abstract Traumatic brain injury (TBI) is a common cause of morbidity and mortality in military life. Interest in military TBI has increased recently due to the conflicts in Iraq and Afghanistan. Certain types of TBI are relatively unique to the military, the most prominent being blast-related TBI. Blast-related mild TBI has been of particular concern in veterans from the most recent conflicts although controversy remains concerning its separation from post-traumatic stress disorder. TBI is also a risk factor for the later development of neurodegenerative diseases in which cognitive impairment is prominent putting...
Source: Current Neurology and Neuroscience Reports - August 24, 2015 Category: Neuroscience Source Type: research

Treatment of Super-Refractory Status Epilepticus
Abstract Super-refractory status epilepticus (SRSE) is a devastating neurological condition with limited treatment options. We conducted an extensive literature search to identify and summarize the therapeutic options for SRSE. The search mainly resulted in case reports of various pharmacologic and non-pharmacologic treatments. The success rate of each of the following agents, ketamine, inhaled anesthetics, intravenous immunoglobulin G (IVIG), IV steroids, ketogenic diet, hypothermia, electroconvulsive therapy (ECT), transcranial magnetic stimulation (TMS), and vagal nerve stimulation (VNS), are discussed in grea...
Source: Current Neurology and Neuroscience Reports - August 24, 2015 Category: Neuroscience Source Type: research

Marijuana Use in Epilepsy: The Myth and the Reality
Abstract Marijuana has been utilized as a medicinal plant to treat a variety of conditions for nearly five millennia. Over the past few years, there has been an unprecedented interest in using cannabis extracts to treat epilepsy, spurred on by a few refractory pediatric cases featured in the media that had an almost miraculous response to cannabidiol-enriched marijuana extracts. This review attempts to answer the most important questions a clinician may have regarding the use of marijuana in epilepsy. First, we review the preclinical and human evidences for the anticonvulsant properties of the different cannabino...
Source: Current Neurology and Neuroscience Reports - August 24, 2015 Category: Neuroscience Source Type: research

Update on Hippocampal Sclerosis
Abstract The diagnostic hallmarks of hippocampal sclerosis (HS) are severe volume loss of the hippocampus, severe neuronal loss, and reactive gliosis involving primarily two especially vulnerable fields, CA1 and the subiculum. Occasionally, HS may be the only neuropathological change detected in older individuals with dementia and is known as pure HS. In the majority of cases, HS occurs in the setting of other degenerative changes, usually Alzheimer’s disease (AD). In these cases, it is classified as combined HS. Although a clinical profile for HS has been identified, its similarities with AD make the diagn...
Source: Current Neurology and Neuroscience Reports - August 24, 2015 Category: Neuroscience Source Type: research

Axons to Exons: the Molecular Diagnosis of Rare Neurological Diseases by Next-Generation Sequencing
Abstract Neurological disorders secondary to single gene mutations are an extremely heterogeneous group of diseases, individually rare, and often associated with progressive and severe disability. Given the degree of both clinical and genetic heterogeneity, next-generation sequencing (NGS) has become an important diagnostic tool. Multi-gene panel testing based on NGS is now prominently used, while whole-exome sequencing and whole-genome sequencing are emerging to facilitate the molecular diagnosis for many genetic neurological diseases. Although single-gene testing remains an important first tier test for disorde...
Source: Current Neurology and Neuroscience Reports - August 21, 2015 Category: Neuroscience Source Type: research

The Neurobiological Grounding of Persistent Stuttering: from Structure to Function
Abstract Neuroimaging and transcranial magnetic stimulation provide insights into the neuronal mechanisms underlying speech disfluencies in chronic persistent stuttering. In the present paper, the goal is not to provide an exhaustive review of existing literature, but rather to highlight robust findings. We, therefore, conducted a meta-analysis of diffusion tensor imaging studies which have recently implicated disrupted white matter connectivity in stuttering. A reduction of fractional anisotropy in persistent stuttering has been reported at several different loci. Our meta-analysis revealed consistent deficits i...
Source: Current Neurology and Neuroscience Reports - July 30, 2015 Category: Neuroscience Source Type: research

Benefits of Exercise Training in Multiple Sclerosis
Abstract Exercise training represents a behavioral approach for safely managing many of the functional, symptomatic, and quality of life consequences of multiple sclerosis (MS). This topical review paper summarizes evidence from literature reviews and meta-analyses, supplemented by recent individual studies, indicating that exercise training can yield small but important improvements in walking, balance, cognition, fatigue, depression, and quality of life in MS. The paper highlights limitations of research on exercise training and its consequences and future research directions and provides an overview for promot...
Source: Current Neurology and Neuroscience Reports - July 30, 2015 Category: Neuroscience Source Type: research

Immune-Mediated Neurological Disorders
This article reviews recent developments in our understanding on the pathophysiology, clinical presentations, and diagnoses of selected immune-mediated neurological disorders. It also provides a brief summary of current theories on autoimmunity and the role that certain environmental factors play in the development of immune-mediated neurological disorders. Recently recognized biomarkers might play a pathogenetic role or simply serve as a diagnostic tool. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - July 23, 2015 Category: Neuroscience Source Type: research

Postural Tachycardia Syndrome: Beyond Orthostatic Intolerance
Abstract Postural tachycardia syndrome (POTS) is a form of chronic orthostatic intolerance for which the hallmark physiological trait is an excessive increase in heart rate with assumption of upright posture. The orthostatic tachycardia occurs in the absence of orthostatic hypotension and is associated with a>6-month history of symptoms that are relieved by recumbence. The heart rate abnormality and orthostatic symptoms should not be caused by medications that impair autonomic regulation or by debilitating disorders that can cause tachycardia. POTS is a “final common pathway” for a number of overla...
Source: Current Neurology and Neuroscience Reports - July 22, 2015 Category: Neuroscience Source Type: research

Application of “Omics” Technologies for Diagnosis and Pathogenesis of Neurological Infections
Abstract Infections of the human nervous system have substantial morbidity and mortality but also represent among the most challenging of all neurological diseases because of the difficulty in establishing a diagnosis and implementing effective therapies. Neurological infections lead to altered expression levels of a wide range of host- and pathogen-derived biomolecules both within and outside of the nervous system. Quantitative analyses of these biomolecular perturbations have been traditionally performed using “classical” molecular or analytical methods, which evaluate one or few genes or their prod...
Source: Current Neurology and Neuroscience Reports - July 21, 2015 Category: Neuroscience Source Type: research

Cognitive Profile of C9orf72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis
Abstract This review article focuses on the cognitive profile associated with the C9orf72 gene with GGGGCC (G4C2) hexanucleotide repeat expansions that is commonly found in both familial and sporadic forms of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) in order to aid clinicians in the screening process. In this growing clinical continuum between FTD and ALS, understanding and recognizing a neurocognitive profile is important for diagnosis. Key features of this profile include executive dysfunction with memory impairment and language deficits as the disease progresses. Behaviorally, pati...
Source: Current Neurology and Neuroscience Reports - July 21, 2015 Category: Neuroscience Source Type: research

A Clinical Approach to the Differential Diagnosis of Multiple Sclerosis
Abstract The diagnostic criteria for multiple sclerosis (MS) rely on clinical, paraclinical, and radiographic findings of limited specificity. Many disorders mimic MS, and the decision of when to investigate an alternative diagnosis can be challenging. Reliance on extensive ancillary testing to exclude potential mimics, however, is unnecessary in most cases. Rather, recognition and rigorous interpretation of “classic” clinical and radiographic features of MS are often sufficient to establish the diagnosis. Misinterpretation of the clinical and radiographic diagnostic criteria for MS in the setting of ...
Source: Current Neurology and Neuroscience Reports - June 26, 2015 Category: Neuroscience Source Type: research

Does Stroke Imaging Provide Insights into the Neural Basis of Cognition?
Abstract Since the advent of in vivo imaging, first with CT, and then MRI, structural neuroimaging in patients has been widely used as a tool to explore the neural correlates of a wide variety of cognitive functions. Findings from studies using this methodology have formed a core component of current accounts of cognition, but there are a number of problematic issues related to inferring cognitive functions from structural imaging data in stroke and more generally, lesion-based neuropsychology as a whole. This review addresses these concerns in the context of spatial neglect, a common disorder most frequently enc...
Source: Current Neurology and Neuroscience Reports - June 23, 2015 Category: Neuroscience Source Type: research

The Inflammatory Form of Cerebral Amyloid Angiopathy or “Cerebral Amyloid Angiopathy-Related Inflammation” (CAARI)
Abstract Cerebral amyloid angiopathy-related inflammation (CAARI) is a recently recognized syndrome of reversible encephalopathy seen in a subset of patients with cerebral amyloid angiopathy (CAA). CAA is a disorder of the elderly in which amyloid peptides are deposited in the walls of cerebral arteries, leading to microhemorrhages, macrohemorrhages, and eventually dementia. In a few cases, the amyloid deposition is accompanied by inflammation or edema. The clinical syndrome of CAARI is distinguished by subacute neurobehavioral symptoms, headaches, seizures, and stroke-like signs, contrasting the acute intracrani...
Source: Current Neurology and Neuroscience Reports - June 23, 2015 Category: Neuroscience Source Type: research

Erratum to: New Genes for Focal Epilepsies with Speech and Language Disorders
(Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - June 23, 2015 Category: Neuroscience Source Type: research

How Relevant Are Imaging Findings in Animal Models of Movement Disorders to Human Disease?
Abstract The combination of novel imaging techniques with the use of small animal models of disease is often used in attempt to understand disease mechanisms, design potential clinical biomarkers and therapeutic interventions, and develop novel methods with translatability to human clinical conditions. However, it is clear that most animal models are deficient when compared to the complexity of human diseases: they cannot sufficiently replicate all the features of multisystem disorders. Furthermore, some practical differences may affect the use or interpretation of animal imaging to model human conditions such as...
Source: Current Neurology and Neuroscience Reports - June 21, 2015 Category: Neuroscience Source Type: research

TMS as a Tool for Examining Cognitive Processing
Abstract Transcranial magnetic stimulation (TMS) is a non-invasive method where an externally placed, rapidly changing magnetic field causes induction of weak electric currents that lead to changes in neuronal polarization and activity. TMS is a modality that has emerged as a unique tool in the study of functional neuroscience for several reasons. TMS can be used to selectively activate or inhibit specific cortical structures, leading to transient perturbations in their function. Systematic study of these perturbations has been employed to determine the function of specific cortical structures and to investigate ...
Source: Current Neurology and Neuroscience Reports - June 20, 2015 Category: Neuroscience Source Type: research

Neural Mechanisms Underlying Musical Pitch Perception and Clinical Applications Including Developmental Dyslexia
Abstract Music production and perception invoke a complex set of cognitive functions that rely on the integration of sensorimotor, cognitive, and emotional pathways. Pitch is a fundamental perceptual attribute of sound and a building block for both music and speech. Although the cerebral processing of pitch is not completely understood, recent advances in imaging and electrophysiology have provided insight into the functional and anatomical pathways of pitch processing. This review examines the current understanding of pitch processing and behavioral and neural variations that give rise to difficulties in pitch p...
Source: Current Neurology and Neuroscience Reports - June 20, 2015 Category: Neuroscience Source Type: research

Neurologic Complications of Chronic Kidney Disease
Abstract Chronic kidney disease (CKD) is an increasing problem worldwide and is now being recognized as a global health burden particularly for cardiovascular and cerebrovascular events. The incidence of stroke increases in the presence of CKD with a 3-fold increased rate reported in ESRD. Atrial fibrillation (AF) increases the risk of stroke in CKD. There is conflicting observational evidence regarding benefit of anticoagulation in CKD for prevention of stroke in AF as risk of bleeding is high. Overall, anticoagulant in CKD may be beneficial in appropriate patients with meticulous monitoring of international nor...
Source: Current Neurology and Neuroscience Reports - June 18, 2015 Category: Neuroscience Source Type: research

Update in Aphasia Research
Abstract The sequelae of post-stroke aphasia are considerable, with implications at the societal and personal levels. An understanding of the mechanisms of recovery of cognitive and language processes after stroke and the factors associated with increased risk of post-stroke language and cognitive deficits is vital in providing optimal care of individuals with aphasia and in counseling to their families and caregivers. Advances in neuroimaging facilitate the identification of dysfunctional or damaged brain tissue responsible for these cognitive/language deficits and contribute insights regarding the functional ne...
Source: Current Neurology and Neuroscience Reports - June 16, 2015 Category: Neuroscience Source Type: research

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy
Abstract Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the acquired demyelinating neuropathies and is considered to be immune mediated. Diagnosis is typically based on clinical history, neurologic examination, electrophysiologic studies, CSF studies, and pathologic examination. Early diagnosis and treatment is important to prevent irreversible axonal loss and optimize improvement in function. The first-line agents for treatment are intravenous immunoglobulin (IVIg), corticosteroids, and plasmapheresis, which have all been demonstrated to be effective in controlled studies. Studies have not sh...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

What Does Imaging Reveal About the Pathology of Amyotrophic Lateral Sclerosis?
Abstract Amyotrophic lateral sclerosis (ALS) is now recognised to be a heterogeneous neurodegenerative syndrome of the motor system and its frontotemporal cortical connections. The development and application of structural and functional imaging over the last three decades, in particular magnetic resonance imaging (MRI), has allowed traditional post mortem histopathological and emerging molecular findings in ALS to be placed in a clinical context. Cerebral grey and white matter structural MRI changes are increasingly being understood in terms of brain connectivity, providing insights into the advancing degenerati...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Neuromuscular Issues in Systemic Disease
Abstract The neuromuscular system can be involved in several systemic conditions. Clinical manifestations can appear at onset or throughout the course of the disease process. New investigational methods, including imaging of peripheral nerves, new laboratory tests, and antibodies, are available. In addition to symptomatic therapies, specific treatment options, such as for familial amyloid neuropathy and Fabry’s disease, are becoming increasingly available. Pathomechanisms vary depending on the underlying disease process. In addition to metabolic, hormonal, immune, and antibody-mediated mechanisms, in some g...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Developments in the Role of Transcranial Sonography for the Differential Diagnosis of Parkinsonism
Abstract In the last two decades transcranial sonography (TCS) has developed as a valuable, supplementary tool in the diagnosis and differential diagnosis of movement disorders. In this review, we highlight recent evidence supporting TCS as a reliable method in the differential diagnosis of parkinsonism, combining substantia nigra (SN), basal ganglia and ventricular system findings. Moreover, several studies support SN hyperechogenicity as one of most important risk factors for Parkinson’s disease (PD). The advantages of TCS include short investigation time, low cost and lack of radiation. Principal limitat...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

The Neurology of Solid Organ Transplantation
Abstract Transplantation is the rescue treatment for end-stage organ failure with more than 110,000 solid organs transplantations performed worldwide annually. Recent advances in transplantation procedures and posttransplantation management have improved long-term survival and quality of life of transplant recipients, shifting the focus from acute perioperative critical care needs toward long-term chronic medical problems. Neurologic complications affect up to 30–60 % of solid organ transplant recipients. Common etiologies include opportunistic infections and toxicities of antirejection medications, an...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

The Genetics of the Epilepsies
Abstract While genetic causes of epilepsy have been hypothesized from the time of Hippocrates, the advent of new genetic technologies has played a tremendous role in elucidating a growing number of specific genetic causes for the epilepsies. This progress has contributed vastly to our recognition of the epilepsies as a diverse group of disorders, the genetic mechanisms of which are heterogeneous. Genotype-phenotype correlation, however, is not always clear. Nonetheless, the developments in genetic diagnosis raise the promise of a future of personalized medicine. Multiple genetic tests are now available, but there...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

The Relationship Between Atrophy and Hypometabolism: Is It Regionally Dependent in Dementias?
Abstract Neuronal failure leading to dementia in neurodegenerative diseases is evidenced in vivo by functional and structural changes in the brain such as reductions of glucose consumption and volume of grey matter. The earliest phase of cognitive decline and presymptomatic stages of these diseases are heralded by specific patterns of hypometabolism, even in the absence of atrophy, which are currently considered as diagnostic biomarkers. Atrophy is less consistently found as an initial marker of these diseases and is invariably present in moderate to severe stages with a disease-related topography. The relationsh...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

LRRK2 Pathways Leading to Neurodegeneration
Abstract Mutations in LRRK2 are associated with inherited Parkinson’s disease (PD) in a large number of families, and the genetic locus containing the LRRK2 gene contains a risk factor for sporadic PD. The LRRK2 protein contains several domains that suggest a role in cellular signaling, including a kinase domain. It is also clear that LRRK2 interacts, either physically or genetically, with several other important proteins implicated in PD, suggesting that LRRK2 may be a central player in the pathways that underlie parkinsonism. As such, LRRK2 has been proposed to be a plausible target for therapeutic interv...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Psychotic Symptoms in Frontotemporal Dementia
Abstract Although psychotic features have long been recognized in association with frontotemporal dementia (FTD), recent genetic discoveries enabling further subtyping of FTD have revealed that psychotic symptoms are frequent in some forms of FTD. Hallucinations and delusions can even precede onset of other cognitive or behavioural symptoms in patients with FTD. In this review, we explore the frequency and types of psychotic symptoms reported in patients with FTD, as well as in other neuropsychiatric disorders, to aid practitioners’ consideration of these features in the diagnosis of FTD and related disorde...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Retinal Vascular Changes are a Marker for Cerebral Vascular Diseases
Abstract The retinal circulation is a potential marker of cerebral vascular disease because it shares origin and drainage with the intracranial circulation and because it can be directly visualized using ophthalmoscopy. Cross-sectional and cohort studies have demonstrated associations between chronic retinal and cerebral vascular disease, acute retinal and cerebral vascular disease, and chronic retinal vascular disease and acute cerebral vascular disease. In particular, certain qualitative features of retinopathy, retinal artery occlusion, and increased retinal vein caliber are associated with concurrent and futu...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Neuroscience of Aphasia Recovery: the Concept of Neural Multifunctionality
Abstract Aphasia therapy, while demonstrably successful, has been limited by its primary focus on language, with relatively less attention paid to nonlinguistic factors (cognitive, affective, praxic) that play a major role in recovery from aphasia. Neuroscientific studies of the past 15–20 years have opened a breach in the wall of traditional clinico-anatomical teachings on aphasia. It is not an exaggeration to talk of a paradigm shift. The term “neural multifunctionality” denotes a complex web of neural networks supporting both linguistic and nonlinguistic functions in constant and dynamic...
Source: Current Neurology and Neuroscience Reports - May 26, 2015 Category: Neuroscience Source Type: research

Capillary Dysfunction: Its Detection and Causative Role in Dementias and Stroke
Abstract In acute ischemic stroke, critical hypoperfusion is a frequent cause of hypoxic tissue injury: As cerebral blood flow (CBF) falls below the ischemic threshold of 20 mL/100 mL/min, neurological symptoms develop and hypoxic tissue injury evolves within minutes or hours unless the oxygen supply is restored. But is ischemia the only hemodynamic source of hypoxic tissue injury? Reanalyses of the equations we traditionally use to describe the relation between CBF and tissue oxygenation suggest that capillary flow patterns are crucial for the efficient extraction of oxygen: without close cap...
Source: Current Neurology and Neuroscience Reports - May 9, 2015 Category: Neuroscience Source Type: research