Genetics of Frontotemporal Dementia
AbstractFrontotemporal dementia (FTD) is the second most common cause of dementia following Alzheimer ’s disease (AD). Between 20 and 50% of cases are familial. Mutations inMAPT,GRN andC9orf72 are found in 60% of familial FTD cases.C9orf72 mutations are the most common and account for 25%. Rarer mutations (
Source: Current Neurology and Neuroscience Reports - November 22, 2016 Category: Neuroscience Source Type: research

Mental Health Comorbidity in MS: Depression, Anxiety, and Bipolar Disorder
AbstractAmong individuals with multiple sclerosis (MS), mental health comorbidities play a significant role in contributing to secondary disability and detracting from quality of life. This review examines current evidence surrounding three mental health issues of particular relevance to MS: depression, anxiety, and bipolar disorder. We review what is known of the prevalence, correlates, screening mechanisms, and current treatment of each issue and provide recommendations for future areas of research. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - November 15, 2016 Category: Neuroscience Source Type: research

Does the Type of Multisystem Atrophy, Parkinsonism, or Cerebellar Ataxia Impact on the Nature of Sleep Disorders?
AbstractMultiple system atrophy (MSA) is a neurodegenerative disease characterized by a combination of autonomic failure, parkinsonism, and/or cerebellar ataxia. The cause of MSA is unknown, but neuropathologically the disease is characterized by widespread α-synuclein-positive glial cytoplasmic inclusions and striatonigral and/or olivopontocerebellar neurodegeneration. Two motor phenotypes have been clinically identified: parkinsonian (MSA-P) and cerebellar (MSA-C). In order to elucidate if in addition to the motor abnormalities there are other signi ficant differences between these two phenotypes, we performed a re...
Source: Current Neurology and Neuroscience Reports - November 14, 2016 Category: Neuroscience Source Type: research

mTOR Inhibitors in Children: Current Indications and Future Directions in Neurology
AbstractThe mammalian/mechanistic target of rapamycin (mTOR) pathway is a key signaling pathway that has been implicated in genetic epilepsy syndromes, neurodegenerative diseases, and conditions associated with autism spectrum disorder and cognitive impairment. The mTOR pathway has become an exciting treatment target for these various disorders, with mTOR inhibitors such as rapamycin being studied for their potential therapeutic applications. In particular, tuberous sclerosis complex (TSC) is a genetic disorder resulting from overactivation of the mTOR pathway, and pharmacologic therapy with mTOR inhibitors has emerged as ...
Source: Current Neurology and Neuroscience Reports - November 4, 2016 Category: Neuroscience Source Type: research

Neurogenetics in Child Neurology: Redefining a Discipline in the Twenty-first Century
AbstractIncreasing knowledge on genetic etiology of pediatric neurologic disorders is affecting the practice of the specialty. I reviewed here the history of pediatric neurologic disorder classification and the role of genetics in the process. I also discussed the concept of clinical neurogenetics, with its role in clinical practice, education, and research. Finally, I propose a flexible model for clinical neurogenetics in child neurology in the twenty-first century. In combination with disorder-specific clinical programs, clinical neurogenetics can become a home for complex clinical issues, repository of genetic diagnosti...
Source: Current Neurology and Neuroscience Reports - November 4, 2016 Category: Neuroscience Source Type: research

Bacterial Endocarditis and Cerebrovascular Disease
AbstractCerebrovascular complications of endocarditis occur in 25 –70% of patients with infective endocarditis. The cornerstone of treatment is early initiation of antibiotic treatment, which significantly reduces the risk of embolization after 1 week of treatment. In general, thrombolysis and anticoagulation of these patients should be avoided, while antiplate let therapy may be considered in those with other indications. Endovascular treatment of acute septic emboli is uncertain, but a few case reports have demonstrated benefit. Other complications of infective endocarditis include intracerebral hemorrhage, wh...
Source: Current Neurology and Neuroscience Reports - November 4, 2016 Category: Neuroscience Source Type: research

Headaches: a Review of the Role of Dietary Factors
AbstractDietary triggers are commonly reported by patients with a variety of headaches, particularly those with migraines. The presence of any specific dietary trigger in migraine patients varies from 10 to 64  % depending on study population and methodology. Some foods trigger headache within an hour while others develop within 12 h post ingestion. Alcohol (especially red wine and beer), chocolate, caffeine, dairy products such as aged cheese, food preservatives with nitrates and nitrites, monosodium g lutamate (MSG), and artificial sweeteners such as aspartame have all been studied as migraine triggers in the p...
Source: Current Neurology and Neuroscience Reports - October 6, 2016 Category: Neuroscience Source Type: research

Post-Concussive Syndrome: a Focus on Post-Traumatic Headache and Related Cognitive, Psychiatric, and Sleep Issues
AbstractPurpose of ReviewPost-traumatic headache (PTH) is a secondary headache disorder following traumatic brain injury. We sought to examine the recent literature on PTH and associated cognitive, psychiatric, and sleep conditions to understand the latest findings about the associated conditions and available screening tools, and to understand the available treatment options for PTH.Recent FindingsUp to one third of PTH patients may have depression and about one quarter may have insomnia. Anxiety and cognitive issues are also common. While there are few studies examining abortive medications for PTH, recent studies of pre...
Source: Current Neurology and Neuroscience Reports - October 5, 2016 Category: Neuroscience Source Type: research

Communication, Leadership, and Decision-Making in the Neuro-ICU
AbstractThe Neuro-ICU is a multidisciplinary location that presents peculiar challenges and opportunities for patients with life-threatening neurological disease. Communication skills are essential in supporting caregivers and other embedded providers (e.g., neurosurgeons, advanced practice providers, nurses, pharmacists), through leadership. Limitations to prognostication complicate how decisions are made on behalf of non-communicative patients. Cognitive dysfunction and durable reductions in health-related quality of life are difficult to predict, and the diagnosis of brain death may be challenging and confounded by medi...
Source: Current Neurology and Neuroscience Reports - September 26, 2016 Category: Neuroscience Source Type: research

Hypothalamic Dysfunction and Multiple Sclerosis: Implications for Fatigue and Weight Dysregulation
AbstractSigns and symptoms of multiple sclerosis are usually attributed to demyelinating lesions in the spinal cord or cerebral cortex. The hypothalamus is a region that is often overlooked yet controls many important homeostatic functions, including those that are perturbed in multiple sclerosis. In this review we discuss how hypothalamic dysfunction may contribute to signs and symptoms in people with multiple sclerosis. While dysfunction of the hypothalamic-pituitary-adrenal axis is common in multiple sclerosis, the effects and mechanisms of this dysfunction are not well understood. We discuss three hypothalamic mechanis...
Source: Current Neurology and Neuroscience Reports - September 24, 2016 Category: Neuroscience Source Type: research

Toward a Mechanistic Understanding of Epileptic Networks
We present lines of investigation that may usefully p robe these interactions and thus serve to advance our understanding of the long-range effects of epileptiform activity. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - September 24, 2016 Category: Neuroscience Source Type: research

Treatment of Epilepsy in the Elderly
AbstractThe treatment of epilepsy in older individuals is an increasingly important topic in neurology and an area that all treating neurologists should have familiarity with. As the population ages, the number of patients over 65 who present with new-onset epilepsy will increase, as will the complexity of their comorbid medical and neurological disorders. In older patients, seizures are often unwitnessed, or present with atypical symptoms, making the diagnosis more challenging. Additionally, there are relatively limited data to guide the use of anti-epileptic medications and other treatments in this patient population. El...
Source: Current Neurology and Neuroscience Reports - September 15, 2016 Category: Neuroscience Source Type: research

Prevention of Epilepsy: Issues and Innovations
AbstractEpilepsy is a common brain disease and preventing epilepsy is a very relevant public health concern and an urgent unmet need. Although 40  % of all epilepsy cases are thought to have acquired causes, there is a roadblock for successful prevention. Efforts to protect the brain from epileptogenic insults are severely hampered by our lack of biomarkers to identify the few percent at high risk meriting treatment among those exposed. Prev enting brain injury has been moderately effective from around birth to middle age; however, the strategy has failed to stop a substantial increase over the last decades in symptom...
Source: Current Neurology and Neuroscience Reports - September 15, 2016 Category: Neuroscience Source Type: research

Transcriptional Dynamics at Brain Enhancers: from Functional Specialization to Neurodegeneration
AbstractOver the last decade, the noncoding part of the genome has been shown to harbour thousands ofcis-regulatory elements, such as enhancers, that activate well-defined gene expression programs. Driven by the development of numerous techniques, many of these elements are now identified in multiple tissues and cell types, and their characteristics as well as importance in development and disease are becoming increasingly clear. Here, we provide an overview of the insights that were gained from the analysis of noncoding gene regulatory elements in the brain and describe their potential contribution to cell type specializa...
Source: Current Neurology and Neuroscience Reports - September 14, 2016 Category: Neuroscience Source Type: research

Post-Craniotomy Pain Management: Beyond Opioids
AbstractCraniotomy pain may be severe and is often undertreated. Pain management following craniotomy is a balancing act of achieving adequate analgesia but avoiding sedation, respiratory depression, hypercapnia, nausea and vomiting, and hypertension. Opioids are a first-line analgesic therapy; however, concern that opioid-related adverse effects (sedation, respiratory depression) may interfere with neurologic assessment and increase intracranial pressure has limited use of these drugs for intracranial surgery. Non-opioid analgesics avoid these effects and may be useful as part of a multimodal regimen for post-craniotomy p...
Source: Current Neurology and Neuroscience Reports - September 7, 2016 Category: Neuroscience Source Type: research

Recent Advances in Neonatal Seizures
This article strives to review and summarize selected recent literature and topics contributing to a greater understanding of the diagnosis and treatments of neonatal seizures that have emerged in the past several years.Recent FindingsContinuous EEG is recommended as the gold standard for neonatal seizure monitoring as it can provide additional information that may stratify patients by etiology, as well as identify at-risk groups of newborns for neuromonitoring. Investigations are moving beyond traditional antiepileptic agents in search of treatments with better efficacy and with less concern for developmental effects. Tar...
Source: Current Neurology and Neuroscience Reports - September 5, 2016 Category: Neuroscience Source Type: research

Connectivity Changes in Parkinson ’s Disease
AbstractParkinson ’s disease (PD) is a chronic and progressive movement disorder of the central nervous system characterized by widespread alterations in several non-motor aspects such as mood, sleep, olfactory, and cognition in addition to motor dysfunctions. Advanced neuroimaging using functional connectivity rec onstruction of the human brain has provided a vast knowledge on the pathophysiological mechanisms underlying this disorder, but this, however, does not cover the overall inter-/intra-individual variability of PD phenotypes. The present review is aimed at discussing to what extent the evidence provid ed by ...
Source: Current Neurology and Neuroscience Reports - August 27, 2016 Category: Neuroscience Source Type: research

Diagnostic Criteria, Classification and Treatment Goals in Multiple Sclerosis: The Chronicles of Time and Space
AbstractMultiple sclerosis (MS) is one of the most diverse human diseases. Since its first description by Charcot in the nineteenth century, the diagnostic criteria, clinical course classification, and treatment goals for MS have been constantly revised and updated to improve diagnostic accuracy, physician communication, and clinical trial design. These changes have improved the clinical outcomes and quality of life for patients with the disease. Recent technological and research breakthroughs will almost certainly further change how we diagnose, classify, and treat MS in the future. In this review, we summarize the key ev...
Source: Current Neurology and Neuroscience Reports - August 22, 2016 Category: Neuroscience Source Type: research

Visual Cortex Plasticity Following Peripheral Damage To The Visual System: fMRI Evidence
AbstractOver the last two decades, functional magnetic resonance imaging (fMRI) has become a powerful research method to investigate cortical visual plasticity. Abnormal fMRI response patterns have been occasionally detected in the visually deprived cortex of patients with bilateral retinal diseases. Controversy remains whether these observations indicate structural reorganization of the visual cortex or unmasking of previously silent cortico-cortical connections. In optic nerve diseases, there is weak evidence showing that early visual cortex seems to lack reorganization, while higher-order visual areas undergo plastic ch...
Source: Current Neurology and Neuroscience Reports - August 20, 2016 Category: Neuroscience Source Type: research

The Impact of Dementia on Family Caregivers: What Is Research Teaching Us?
AbstractDementia family caregiving has been the focus of research for decades. Much has been learned about the negative impact of caregiving as well as characteristics that may be protective. This paper explores themes in caregiving pertinent to clinicians and researchers working with dementia family caregivers: the psychological, subjective, and physical outcomes of caregiving, ways in which dementia alters relationships between the patient and caregiver, and strategies for improving outcomes for caregivers. Suggestions for next steps in research and clinical care are made. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 19, 2016 Category: Neuroscience Source Type: research

Psychiatric and Cognitive Effects of Deep Brain Stimulation for Parkinson ’s Disease
AbstractDeep brain stimulation (DBS) is effective for Parkinson ’s disease (PD), dystonia, and essential tremor (ET). While motor benefits are well documented, cognitive and psychiatric side effects from the subthalamic nucleus (STN) and globus pallidus interna (GPi) DBS for PD are increasingly recognized. Underlying disease, medications, microlesions, and pos t-surgical stimulation likely all contribute to non-motor symptoms (NMS). (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 18, 2016 Category: Neuroscience Source Type: research

ICU Management of the Potential Organ Donor: State of the Art
AbstractEnd-organ failure is associated with high mortality and morbidity, in addition to increased health care costs. Organ transplantation is the only definitive treatment that can improve survival and quality of life in such patients; however, due to the persistent mismatch between organ supply and demand, waiting lists continue to grow across the world. Careful intensive care management of the potential organ donor with goal-directed therapy has the potential to optimize organ function and improve donation yield. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 6, 2016 Category: Neuroscience Source Type: research

Alemtuzumab for Multiple Sclerosis
AbstractAlemtuzumab is a humanised anti-CD52 monoclonal antibody approved for use in active, relapsing multiple sclerosis (MS). Administration results in a rapid depletion of circulating lymphocytes with a subsequent beneficial immune reconstitution. Early open-label experience and recent clinical trials have demonstrated a dramatic effect on relapse rates as well as a positive effect on radiological disease outcomes and disability measures. Despite a mechanism of action that results in profound lymphopaenia, opportunistic infections are rarely seen and no excess association with malignancy has been identified. However, ac...
Source: Current Neurology and Neuroscience Reports - August 2, 2016 Category: Neuroscience Source Type: research

Sleep Disturbances in Frontotemporal Dementia
AbstractSleep disorders appear to be frequent comorbidities in patients with frontotemporal dementia (FTD). Insomnia and excessive daytime sleepiness commonly occur in patients with FTD and significantly contribute to caregiver burden and burnout. Sleep is severely fragmented in FTD patients, likely secondary to behavioral disturbances, other primary sleep disorders such as sleep disordered breathing and restless leg syndrome, and neurodegeneration of nuclei involved in sleep and wakefulness. Treatment of primary sleep disorders may improve excessive daytime sleepiness and sleep quality and may improve daytime cognitive fu...
Source: Current Neurology and Neuroscience Reports - August 2, 2016 Category: Neuroscience Source Type: research

Impact of Infection on Stroke Morbidity and Outcomes
AbstractEach year, millions of persons worldwide are disabled by stroke. The burden of stroke is expected to increase as a consequence of growth in our elderly population. Outcome is dependent upon limitation of secondary medical processes in the acute setting that lead to deterioration and increased long-term disability. The prevalence of infection after stroke is greater that seen in other medical conditions with similar acuity and its impact upon morbidity and mortality is substantial. Physical impairment and immune modulation are chief determinants in rate of infection after stroke. Each of these factors has been a tar...
Source: Current Neurology and Neuroscience Reports - August 2, 2016 Category: Neuroscience Source Type: research

Pharmacological Treatment of Drug-Resistant Epilepsy in Adults: a Practical Guide
Abstract More than 30  % of adults with epilepsy do not fully control on the currently available antiepileptic drugs (AEDs). For these and many other patients, combinations of agents, often possessing different mechanisms of actions, are employed with the aim of achieving seizure freedom or the best available prognosis in terms of reduced seizure numbers and severity. This review discusses my own approach to optimising outcomes in as many of these patients as possible by adjusting the drug burden using a combination of two, three or sometimes four or more AEDs. Modes of drug action are reviewed and practical strat eg...
Source: Current Neurology and Neuroscience Reports - July 21, 2016 Category: Neuroscience Source Type: research

Chemotherapy-Related Neurotoxicity
Abstract Chemotherapy may have detrimental effects on either the central or peripheral nervous system. Central nervous system neurotoxicity resulting from chemotherapy manifests as a wide range of clinical syndromes including acute, subacute, and chronic encephalopathies, posterior reversible encephalopathy, acute cerebellar dysfunction, chronic cognitive impairment, myelopathy, meningitis, and neurovascular syndromes. These clinical entities vary by causative agent, degree of severity, evolution, and timing of occurrence. In the peripheral nervous system, chemotherapy-induced peripheral neuropathy (CIPN) and myopathy are...
Source: Current Neurology and Neuroscience Reports - July 21, 2016 Category: Neuroscience Source Type: research

Driving and Epilepsy: a Review of Important Issues
Abstract Driving restrictions in people with epilepsy (PWE) is a highly contentious topic. The fundamental difficulty lies in achieving a balance between safety and practicality. The aim of this review is to provide an overview, history, and rationale behind current laws regarding driving restriction in PWE. We also discuss recent findings that may be helpful to practitioners during individual discussions with PWE including seizure recurrence risk after first seizure, recurrent seizure, and anticonvulsant with drawl and driving restrictions in patients with psychogenic non-epileptic seizures (PNES). (Source: Current Neuro...
Source: Current Neurology and Neuroscience Reports - July 21, 2016 Category: Neuroscience Source Type: research

A Neuropsychological Perspective on Abstract Word Representation: From Theory to Treatment of Acquired Language Disorders
Abstract Natural languages are rife with words that describe feelings, introspective states, and social constructs (e.g., liberty, persuasion) that cannot be directly observed through the senses. Effective communication demands linguistic competence with such abstract words. In clinical neurological settings, abstract words are especially vulnerable to the effects of stroke and neurodegenerative conditions such as Alzheimer ’s disease. A parallel literature in cognitive neuroscience suggests that abstract and concrete words are at least partially neuroanatomically dissociable. Much remains to be learned about the na...
Source: Current Neurology and Neuroscience Reports - July 21, 2016 Category: Neuroscience Source Type: research

Hypothermia for Increased Intracranial Pressure: Is It Dead?
Abstract Mild to moderate therapeutic hypothermia (HT) has been used to alleviate intracranial hypertension in traumatic brain injury (TBI). Its main contribution is thought to be via reduction in cerebral metabolic requirement leading both to favorable oxygen/metabolic delivery-demand ratios as well as a reduction of cerebral blood volume resulting in decreased ICP. Nevertheless, HT is a clinically complex, labor-intensive procedure with numerous potential adverse effects. Furthermore, randomized controlled trials suggest either no effect or harm. These facts challenge the role of HT in TBI. We address this challenge by ...
Source: Current Neurology and Neuroscience Reports - July 21, 2016 Category: Neuroscience Source Type: research

Anosognosia in Dementia
This article reviews recent research on anosognosia in dementia, including methods of assessing anosognosia, its prevalence and developmental course in dementia, its occurrence in different forms of dementia, neuroimaging findings, and hypothesized component mechanisms. The results suggest that anosognosia is eventually exhibited by nearly all persons with dementia. Its occurrence is robustly associated with common dementia-related pathologies and damage to memory and self-referential brain networks and their interconnections. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - July 20, 2016 Category: Neuroscience Source Type: research

Neuropsychological Effects of Cerebral Amyloid Angiopathy
This article reviews recent literature which confirms that CAA independently contributes to cognitive impairment by potentiating the neurodegeneration of Alzheimer’s disease, by predisposing to microhemorrhagic and microischemic injury to the brain parenchyma, and by interfering with the autoregulation of CNS blood flow. In this review, we discuss the clinical presentation of cerebral amyloid angiopathy, with a focus on the neuropsychological manifestations of this vasculopathy. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - June 30, 2016 Category: Neuroscience Source Type: research

A Cognitive Overview of Limb Apraxia
Abstract Since the first studies on limb apraxia carried out by Hugo Liepmann more than a century ago, research interests focused on the way humans process manual gestures by assessing gesture production after patients suffered neurologic deficits. Recent reviews centered their attention on deficits in gesture imitation or processing object-related gestures, namely pantomimes and transitive gestures, thereby neglecting communicative/intransitive gestures. This review will attempt to reconcile limb apraxia in its entirety. To this end, the existing cognitive models of praxis processing that have been designed to a...
Source: Current Neurology and Neuroscience Reports - June 27, 2016 Category: Neuroscience Source Type: research

Pure Autonomic Failure
Abstract Pure autonomic failure (PAF) is a rare sporadic neurodegenerative autonomic disorder characterized by slowly progressive pan autonomic failure without other features of neurologic dysfunctions. The main clinical symptoms result from neurogenic orthostatic hypotension and urinary and gastrointestinal autonomic dysfunctions. Autonomic failure in PAF is caused by neuronal degeneration of pre- and postganglionic sympathetic and parasympathetic neurons in the thoracic spinal cord and paravertebral autonomic ganglia. The presence of Lewy bodies and α-synuclein deposits in these neural structures suggests...
Source: Current Neurology and Neuroscience Reports - June 23, 2016 Category: Neuroscience Source Type: research

Alien Hand Syndrome
Abstract Alien hand syndrome (AHS) is a rare disorder of involuntary limb movement together with a sense of loss of limb ownership. It most commonly affects the hand, but can occur in the leg. The anterior (frontal, callosal) and posterior variants are recognized, with distinguishing clinical features and anatomical lesions. Initial descriptions were attributed to stroke and neurosurgical operations, but neurodegenerative causes are now recognized as most common. Structural and functional imaging and clinical studies have implicated the supplementary motor area, pre-supplementary motor area, and their network con...
Source: Current Neurology and Neuroscience Reports - June 17, 2016 Category: Neuroscience Source Type: research

The Impact of Psychoactive Drugs on Seizures and Antiepileptic Drugs
Abstract Psychiatric comorbidities are very common in patients with epilepsy, and in fact, a bidirectional relationship between epilepsy and some psychiatric disorders have been identified. However, despite their high prevalence, these comorbidities are not routinely recognized or adequately treated causing a significant burden for these patients. Atypical presentations of some of these psychiatric comorbidities in epilepsy, the concern that some psychotropic drugs may lower seizure threshold worsening frequency of seizures, possibility of many drug-drug interactions, and the negative impact of some antiepileptic...
Source: Current Neurology and Neuroscience Reports - June 17, 2016 Category: Neuroscience Source Type: research

The Utility of Cerebral Blood Flow Assessment in TBI
This article reviews the history of CBF measurements in TBI as well as the role of CBF in pathologies associated with TBI, such as cerebral autoregulation, hyperemia, and cortical spreading depression. The limitations of various CBF monitors are reviewed in order to better understand their role in TBI management. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - June 17, 2016 Category: Neuroscience Source Type: research

Cognitive and Functional Consequence of Cardiac Arrest
Abstract Cardiac arrest is associated with high morbidity and mortality. Better-quality bystander cardiopulmonary resuscitation training, cardiocerebral resuscitation principles, and intensive post-resuscitation hospital care have improved survival. However, cognitive and functional impairment after cardiac arrest remain areas of concern. Research focus has shifted beyond prognostication in the immediate post-arrest period to identification of mechanisms for long-term brain injury and implementation of promising protocols to reduce neuronal injury. These include therapeutic temperature management (TTM), as well a...
Source: Current Neurology and Neuroscience Reports - June 16, 2016 Category: Neuroscience Source Type: research

Isolated Abducens Nerve Palsy: Update on Evaluation and Diagnosis
Abstract Abducens nerve palsy is a common clinical finding in neurology practice. In many instances, the origin is obvious and management straightforward; however, the list of possible etiologies and mimics is vast and diverse and diagnostic decisions can be challenging and even controversial. This is especially true when the abducens nerve is affected in isolation, since in the current era of cost-effective medicine, it is critical to accurately diagnose etiologies that may lead to major morbidity or mortality with efficiency. Topics for highlighted updates in this review include management of isolated abducens ...
Source: Current Neurology and Neuroscience Reports - June 15, 2016 Category: Neuroscience Source Type: research

Paediatric Multiple Sclerosis: Update on Diagnostic Criteria, Imaging, Histopathology and Treatment Choices
Abstract Paediatric multiple sclerosis (MS) represents less than 5 % of the MS population, but patients with paediatric-onset disease reach permanent disability at a younger age than adult-onset patients. Accurate diagnosis at presentation and optimal long-term treatment are vital to mitigate ongoing neuroinflammation and irreversible neurodegeneration. However, it may be difficult to early differentiate paediatric MS from acute disseminated encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD), as they often have atypical presentation that differs from that of adult-onset MS. The purp...
Source: Current Neurology and Neuroscience Reports - June 6, 2016 Category: Neuroscience Source Type: research

Neurological Complications of Pregnancy
Abstract Physiologic alterations during pregnancy create an environment for the occurrence of disease states that are either unique to pregnancy, occur more frequently in pregnancy, or require special management considerations that may be different from the nonpregnancy state. In the realm of cerebrovascular disease, preeclampsia, eclampsia, reversible cerebral vasoconstriction syndrome, sources of cardiogenic embolization including peripartum cardiomyopathy, cerebral venous thrombosis, pituitary apoplexy, subarachnoid hemorrhage, intracerebral hemorrhage, and special considerations for anticoagulation during pre...
Source: Current Neurology and Neuroscience Reports - May 26, 2016 Category: Neuroscience Source Type: research

Facioscapulohumeral Dystrophy
Abstract Facioscapulohumeral muscular dystrophy (FSHD) is a clinically recognizable and relatively common muscular dystrophy. It is inherited mostly as an autosomal dominant disease or in a minority of cases, in a digenic pattern. The disease manifestation is variable and most likely dependent on genetic and epigenetic factors. We review the history, epidemiology, clinical presentation, and genetics of the disease, present the recently elucidated molecular pathogenesis, discuss the pathology and the possible consequence of the inflammation seen in the muscle biopsies, and consider future treatments. (Source: Curr...
Source: Current Neurology and Neuroscience Reports - May 23, 2016 Category: Neuroscience Source Type: research

Migraine: What Imaging Reveals
Abstract Although migraine symptomatology is well-defined, our understanding of migraine pathophysiology is incomplete. Structural and functional brain imaging can contribute to a greater understanding of migraine pathophysiology. Recent neuroimaging studies demonstrate that migraine is associated with structural and functional alterations of brain regions commonly implicated in pain processing. This review summarizes recent brain structural and functional imaging findings in migraine and highlights those that are associated with characteristics such as the presence or absence of aura, associated cognitive dysfun...
Source: Current Neurology and Neuroscience Reports - May 16, 2016 Category: Neuroscience Source Type: research

New Techniques and Progress in Epilepsy Surgery
Abstract While open surgical resection for medically refractory epilepsy remains the gold standard in current neurosurgical practice, modern techniques have targeted areas for improvement over open surgical resection. This review focuses on how a variety of these new techniques are attempting to address these various limitations. Stereotactic electroencephalography offers the possibility of localizing deep epileptic foci, improving upon subdural grid placement which limits localization to neocortical regions. Laser interstitial thermal therapy (LITT) and stereotactic radiosurgery can minimally or non-invasiv...
Source: Current Neurology and Neuroscience Reports - May 16, 2016 Category: Neuroscience Source Type: research

Melatonin for Sleep Disorders in Patients with Neurodegenerative Diseases
Abstract In patients with neurodegenerative diseases, sleep disorders are common; they impair the quality of life for patients and caregivers and are associated with poorer clinical outcomes. Melatonin has circadian, hypnotic, and free radical-scavenging effects, and preclinical data suggest benefits of melatonin on neurodegeneration. However, randomized, controlled trials of melatonin in patients with neurodegenerative diseases have not shown strong effects. Trials in Alzheimer’s patients demonstrate a lack of benefit on sleep quantity. Subjective measures of sleep quality are mixed, with possible symptoma...
Source: Current Neurology and Neuroscience Reports - May 14, 2016 Category: Neuroscience Source Type: research

Neurologic Complications in Treated HIV-1 Infection
Abstract Effective combination antiretroviral therapy has transformed HIV infection into a chronic disease, with HIV-infected individuals living longer and reaching older age. Neurological disease remains common in treated HIV, however, due in part to ongoing inflammation and immune activation that persist in chronic infection. In this review, we highlight recent developments in our understanding of several clinically relevant neurologic complications that can occur in HIV infection despite treatment, including HIV-associated neurocognitive disorders, symptomatic CSF escape, cerebrovascular disease, and periphera...
Source: Current Neurology and Neuroscience Reports - May 12, 2016 Category: Neuroscience Source Type: research

Muscle-Specific Receptor Tyrosine Kinase (MuSK) Myasthenia Gravis
Abstract Autoimmune myasthenia gravis (MG) is the prototypic, antibody-mediated neuromuscular disease and is characterized by a decrease in the number of functional acetylcholine receptors (AChR) within the muscle end plate zone of the neuromuscular junction (NMJ). Although the pathophysiology of AChR-mediated myasthenia gravis has been extensively studied over the last 40 years since its original description by Patrick and Lindstrom (Science 180:871–872, 1973), less is known about the much more recently described muscle-specific kinase (MuSK) antibody-mediated MG. MuSK-MG has features clinically disti...
Source: Current Neurology and Neuroscience Reports - May 12, 2016 Category: Neuroscience Source Type: research

Kleine-Levin Syndrome
This article reviews common presenting symptoms, differential diagnosis, diagnostic workup, and potential treatment options. Current updates on functional imaging studies and long-term neuropsychological studies are reviewed. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - May 2, 2016 Category: Neuroscience Source Type: research

Advances of Molecular Imaging in Epilepsy
This article reviews the development of several PET radioligands and their application in studying the molecular mechanisms of epilepsy. Over the last decade, tracers binding to serotonin and γ-aminobutyric acid (GABA) receptors have been used to delineate the location of the epileptic focus. PET studies have examined the role of opioids, cannabinoids, acetylcholine, and dopamine in modulating neuronal hyperexcitability and seizure termination. In vivo analyses of drug transporters, e.g., P-glycoprotein, have increased our understanding of pharmacoresistance that could inform new therapeutic strategies. Finally, PET ...
Source: Current Neurology and Neuroscience Reports - April 25, 2016 Category: Neuroscience Source Type: research

ALS: Recent Developments from Genetics Studies
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal disorder that is characterized by a progressive degeneration of the upper and lower motor neurons. Most cases appear to be sporadic, but 5–10 % of cases have a family history of the disease. High-throughput DNA sequencing and related genomic capture tools are methodological advances which have rapidly contributed to an acceleration in the discovery of genetic risk factors for both familial and sporadic ALS. It is interesting to note that as the number of ALS genes grows, many of the proteins they encode are in shared intracellular processes. This ...
Source: Current Neurology and Neuroscience Reports - April 25, 2016 Category: Neuroscience Source Type: research