Sudden Unexpected Death in Epilepsy: Addressing the Challenges
Abstract Epilepsy is associated with a higher rate of premature death than the general population, and the commonest cause of epilepsy mortality is sudden unexpected death in epilepsy (SUDEP). It is difficult to quantify because of the variable reporting of this cause of death. Death occurs due to autonomic deregulation of cardio-respiratory pathways as a result of seizures. Measures to reduce cardio-respiratory dysfunction are discussed together with the importance of seizure control in preventing SUDEP. The role of seizure detection devices, antiepileptic drugs and the importance of providing information about ...
Source: Current Neurology and Neuroscience Reports - October 9, 2014 Category: Neuroscience Source Type: research

Strategies for Streamlining Emergency Stroke Care
This article reviews the most current standards and guidelines related to the flow of stroke care in the prehospital and emergency settings. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - September 25, 2014 Category: Neuroscience Source Type: research

Interdisciplinary Teamwork for the Treatment of People with Parkinson’s Disease and Their Families
Abstract Parkinson’s disease (PD) is a chronic progressive neurodegenerative and multidimensional disease that involves a range of disabling motor and nonmotor symptoms. These symptoms can have a major impact on the quality of life of PD patients. The focus of this article is to stress the importance of the interdisciplinary team intervention approach in the treatment of patients with PD. The team approach uses experts in PD from different health care professions, including a neurologist, a nurse, a speech and language therapist, a physiotherapist, a social worker, a psychiatrist, an occupational therapist,...
Source: Current Neurology and Neuroscience Reports - September 23, 2014 Category: Neuroscience Source Type: research

Tau PET Imaging in Alzheimer’s Disease
Abstract In several neurodegenerative diseases that are collectively called tauopathies, progressive accumulation of tau in the brain is closely associated with neurodegeneration and cognitive impairment. Noninvasive detection of tau protein deposits in the brain would be useful to diagnose tauopathies as well as to track and predict disease progression. Recently, several tau PET tracers including T807, THK-5117, and PBB3 have been developed and succeeded in imaging neurofibrillary pathology in vivo. For use of tau PET as a biomarker of tau pathology in Alzheimer’s disease, PET tracers should have high affi...
Source: Current Neurology and Neuroscience Reports - September 21, 2014 Category: Neuroscience Source Type: research

The Relationship between Amyloid Deposition, Neurodegeneration, and Cognitive Decline in Dementia
Abstract Amyloid imaging has been clinically approved for measuring β amyloid plaque load in patients being evaluated for Alzheimer's disease or other causes of cognitive decline. Here we explore a multidimensional approach to cognitive decline, where we situate amyloid plaque burden among a number of other relevant dimensions, such as aging, volume loss, other proteinopathies such as TDP43 and Lewy bodies, and functional reorganisation of cognitive brain systems. The multidimensional model incorporates a 'pure AD' trajectory, corresponding to e.g. monogenic Alzheimer's disease, but leaves room for other com...
Source: Current Neurology and Neuroscience Reports - September 16, 2014 Category: Neuroscience Source Type: research

Paroxysmal Sympathetic Hyperactivity after Severe Brain Injury
This article aims to outline the evidence base for the management of PSH, as well as to describe an algorithm for management developed at our institution. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - September 16, 2014 Category: Neuroscience Source Type: research

Prion-like Mechanisms in the Pathogenesis of Tauopathies and Synucleinopathies
Abstract Neurodegenerative diseases, including Alzheimer’s disease and Parkinson’s disease, are characterized by the abnormal aggregation of a small number of intracellular proteins, with tau and α-synuclein being the most commonly affected. Until recently, the events leading to aggregate formation were believed to be entirely cell-autonomous, with protein misfolding occurring independently in many cells. It is now believed that protein aggregates form in a small number of brain cells, from which they propagate intercellularly through templated recruitment, reminiscent of the mechanisms by which...
Source: Current Neurology and Neuroscience Reports - September 14, 2014 Category: Neuroscience Source Type: research

Genetic Heterogeneity in Alzheimer Disease and Implications for Treatment Strategies
Abstract Since the original publication describing the illness in 1907, the genetic understanding of Alzheimer’s disease (AD) has advanced such that it is now clear that it is a genetically heterogeneous condition, the subtypes of which may not uniformly respond to a given intervention. It is therefore critical to characterize the clinical and preclinical stages of AD subtypes, including the rare autosomal dominant forms caused by known mutations in the PSEN1, APP, and PSEN2 genes that are being studied in the Dominantly Inherited Alzheimer Network study and its associated secondary prevention trial. Simila...
Source: Current Neurology and Neuroscience Reports - September 14, 2014 Category: Neuroscience Source Type: research

Cerebral Thrombosis and Myeloproliferative Neoplasms
Abstract Myeloproliferative neoplasms (MPN) are acquired clonal disorders characterized by the proliferation of bone marrow myeloid cells. Different somatic mutations have been recently associated with MPN, the most common being JAK-2 V617F. Among MPN, polycythemia vera and essential thrombocythemia are particularly associated with an increased risk to develop thrombotic complications, either arterial or venous. Cerebrovascular events (stroke and transient ischemic attacks) are prevalent, accounting for approximately two-thirds of all events. Also cerebral vein thrombosis can complicate MPN and can be the first m...
Source: Current Neurology and Neuroscience Reports - September 14, 2014 Category: Neuroscience Source Type: research

The Genetics of Primary Familial Brain Calcifications
Abstract Bilateral accumulation of calcium in the brain, most commonly in the basal ganglia, but also in the cerebellum, thalamus, and brainstem can be inherited in an autosomal dominant fashion and is then referred to as primary familial brain calcifications (PFBC). Clinical manifestations include a spectrum of movement disorders and neuropsychiatric abnormalities. In the past 2 years, 3 genes have been identified to cause PFBC, (ie, SLC20A2, PDGFRB, and PDGFB). SCL20A2 encodes the Type III sodium-dependent inorganic phosphate (Pi) transporter 2 (PiT2) and, when mutated, uptake of Pi is severely impaired likely ...
Source: Current Neurology and Neuroscience Reports - September 12, 2014 Category: Neuroscience Source Type: research

Gut Microbiome and Multiple Sclerosis
Abstract The commensal flora that lives in the human gut is a unique ecosystem that has evolved over millennia with human beings. The importance of the microbiota in various bodily functions is gradually becoming more apparent. Besides the gut microbiome playing a role in bowel-related disorders, a role in metabolic and autoimmune disorders is becoming clearer. The gut bacteria play a role in educating the immune system and hence may be a player in the development of multiple sclerosis. We examine the different sources of information linking the gut microbiota to multiple sclerosis and examine the future avenues ...
Source: Current Neurology and Neuroscience Reports - September 10, 2014 Category: Neuroscience Source Type: research

Neurologic Manifestations of Gastrointestinal and Liver Diseases
Abstract Hepatic and gastrointestinal disorders can produce a wide spectrum of neurologic complications both affecting the central nervous system (CNS) and the peripheral nervous system. These manifestations range in severity from coma in acute liver failure and acute pancreatitis, to minor cognitive changes in chronic portosystemic encephalopathy and hepatitis C. Cerebrovascular diseases can complicate hepatitis C infection and inflammatory bowel disease. Demyelinating disorders may co-exist with inflammatory bowel disease. Anti-tumor necrosis factor alpha drugs may induce demyelination. Ataxia may occur in mala...
Source: Current Neurology and Neuroscience Reports - August 30, 2014 Category: Neuroscience Source Type: research

Imaging Frontotemporal Lobar Degeneration
Abstract The term frontotemporal lobar degeneration (FTLD) refers to a group of neurodegenerative disorders that target the frontal and temporal lobes. It accounts for approximately 10 % of pathologically confirmed dementias but has been demonstrated to be as prevalent as Alzheimer’s disease in patients below the age of 65. The 3 major clinical syndromes associated with FTLD include behavioral variant frontotemporal dementia, semantic and nonfluent variants of primary progressive aphasia. The more recently introduced term logopenic variant appears to represent an atypical form of Alzheimer’s dise...
Source: Current Neurology and Neuroscience Reports - August 30, 2014 Category: Neuroscience Source Type: research

Endogenous Retrovirus-K and Nervous System Diseases
Abstract A new appreciation of the microbiome is changing the way we perceive human health and disease. The holobiontic nature of humans is even etched into our DNA in the form of viral symbionts. Empirical evidence for the presence of endogenous retroviruses (ERVs) in the human genome and their activity in homeostatic and pathologic states has accumulated; however, no causal relationship with human disease has been established to date. In this review, we will focus on the role of endogenous retrovirus-K in neurologic disease. Specifically, we will attempt to reconcile the pathologic contribution of ERVK in dispa...
Source: Current Neurology and Neuroscience Reports - August 20, 2014 Category: Neuroscience Source Type: research

Inflammation and Immune System Activation After Traumatic Brain Injury
This article reviews the evidence for inflammation mediating secondary injury after head trauma and outlines potential approaches for immunomodulatory therapies after traumatic brain injury. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 20, 2014 Category: Neuroscience Source Type: research

Disruption of Resting Functional Connectivity in Alzheimer’s Patients and At-Risk Subjects
In this study, we review the current findings in default mode network and other resting state network studies in AD and MCI patients and at-risk subjects as assessed by resting state functional magnetic resonance imaging. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - August 14, 2014 Category: Neuroscience Source Type: research

X-Linked Adrenoleukodystrophy: Pathogenesis and Treatment
Abstract X-linked adrenoleukodystrophy (X-ALD) is a puzzling inborn error of metabolism with a strikingly heterogeneous clinical spectrum. All patients have mutations in the ABCD1 gene and accumulate very long chain fatty acids in all tissues. Virtually all male X-ALD patients develop adrenocortical insufficiency in childhood and progressive myelopathy and peripheral neuropathy in adulthood. A subset of male patients, however, develops a fatal cerebral demyelinating disease, cerebral adrenoleukodystrophy. Female patients also develop progressive myelopathy and peripheral neuropathy, but generally at a later age t...
Source: Current Neurology and Neuroscience Reports - August 13, 2014 Category: Neuroscience Source Type: research

Remyelination and Multiple Sclerosis: Therapeutic Approaches and Challenges
Abstract Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. After acute inflammatory mediated demyelination, some remyelination often occurs, but in chronic demyelinated MS plaques, remyelination frequently fails. Chronically demyelinated axons cause a variety of symptoms and probably are more likely to degenerate, leading to irreversible clinical disability. Oligodendrocyte precursor cells (OPCs) present in the adult brain can proliferate and differentiate to remyelinate lesions. Failure of remyelination in the majority of MS patients is secondary to arrest in OPC dif...
Source: Current Neurology and Neuroscience Reports - August 10, 2014 Category: Neuroscience Source Type: research

Intracranial Cystic Lesions: A Review
Abstract Cysts and cystic-appearing intracranial lesions are common findings with routine cerebral imaging examination. These lesions often represent a challenge in diagnosis. Intracranial cystic lesions have wide pathologic and imaging spectra, of which some require an aggressive and tailored treatment, whereas many others remain asymptomatic and do not require follow-up or intervention. Intracranial cysts can be divided in non-neoplastic lesions that are often of developmental origin but comprise as well infectious cysts and neoplastic lesions that include benign cysts associated with low-grade tumors and cysts...
Source: Current Neurology and Neuroscience Reports - August 9, 2014 Category: Neuroscience Source Type: research

Hypertonic Saline for the Treatment of Intracranial Hypertension
Abstract Intracranial hypertension is caused by brain edema generated by different disorders, the commonest of which is traumatic brain injury. The treatment of brain edema focuses on drawing water out of brain tissue into the intravascular space. This is typically accomplished with osmolar therapy, most commonly mannitol and hypertonic saline. Recent human trials suggest that hypertonic saline may have a more profound and long-lasting effect in reducing intracranial hypertension following traumatic brain injury when compared with mannitol. However, reports suffer from inconsistencies in dose, frequency, concentr...
Source: Current Neurology and Neuroscience Reports - August 1, 2014 Category: Neuroscience Source Type: research

The Role of Olfactory Challenge Tests in Incipient Dementia and Clinical Trial Design
Abstract The brain changes associated with Alzheimer’s disease (AD) develop slowly over many years before the onset of dementia. Biomarkers for AD that allow its detection during this clinically silent phase will be hugely important when disease-modifying treatments that halt or slow its progression become available. Early detection, leading to early treatment, may in some cases avert dementia. Biomarkers aid our understanding of the presymptomatic stages of the disease and enable the identification of individuals with early disease who, by participating in clinical trials of investigational treatments with...
Source: Current Neurology and Neuroscience Reports - July 23, 2014 Category: Neuroscience Source Type: research