Abstract:
PURPOSE OF REVIEW:To summarize advances in our understanding of the clinical phenotypes, genetics, and molecular pathophysiology of the periodic paralyses, the nondystrophic myotonias, and other muscle channelopathies. RECENT FINDINGS:The number of pathogenic mutations causing periodic paralysis, nondystrophic myotonias, and ryanodinopathies continues to grow with the advent of exon hierarchy analysis strategies for genetic screening and better understanding and recognition of disease phenotypes. Recent studies have expanded and clarified the role of gating pore current in channelopathy pathogenesis. It has been shown that the gating pore current can account for the molecular and phenotypic diseases observed in the muscle sodium channelopathies, and, given that homologous residues are affected in mutations of calcium channels, it is possible that pore leak represents a pathomechanism applicable to many channel diseases. Improvements in treatment of the muscle channelopathies are on the horizon. A randomized controlled trial has been initiated for the study of mexiletine in nondystrophic myotonias. The class IC antiarrhythmia drug flecainide has been shown to depress ventricular ectopy and improve exercise capacity in patients with Andersen-Tawil syndrome. SUMMARY:Recent studies have expanded our understanding of gating pore current as a disease-causing mechanism in the muscle channelopathies and have allowed new correlations to be drawn between disease genotype and phenotype.
journal_name
Curr Opin Neuroljournal_title
Current opinion in neurologyauthors
Platt D,Griggs Rdoi
10.1097/WCO.0b013e32832efa8fsubject
Has Abstractpub_date
2009-10-01 00:00:00pages
524-31issue
5eissn
1350-7540issn
1473-6551journal_volume
22pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:The successful motor rehabilitation of stroke, traumatic brain-injured and spinal cord-injured patients requires an intensive and task-specific therapy approach. Budget constraints limit a hand-to-hand therapy approach, so that intelligent machines may offer a solution to promote motor recovery and ob...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000102630.16692.38
更新日期:2003-12-01 00:00:00
abstract:PURPOSE OF REVIEW:In recent years, advances in the understanding of the regulatory mechanisms of the immune system has led to the development of new approaches for cancer treatment. Currently, immune checkpoint inhibitors are the first successful examples of this approach and several agents that target cytotoxic lympho...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000391
更新日期:2016-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Myasthenic syndromes are distinct disorders at the neuromuscular junction, most often with well characterized autoimmune or genetic pathology. New aspects of the dysfunctions give insight into the normal neuromuscular function in addition to giving therapeutic clues and tailoring the therapy to the pa...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e3283572588
更新日期:2012-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Although cluster headache has traditionally been thought of as a vascular headache disorder, its periodicity suggests an involvement of central areas such as the hypothalamus. This review covers the past 3 years, which have seen remarkable progress in understanding the pathophysiology of circadian hea...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000073934.19076.0e
更新日期:2003-06-01 00:00:00
abstract::New reports of the incidence, prevalence and mortality of Parkinson's disease and parkinsonism are reviewed. Evidence for occupational and lifestyle risk factors and protective factors is updated. Recent genetic epidemiology studies of parkinsonism in twins and families, and studies that investigated imaging and physi...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200008000-00010
更新日期:2000-08-01 00:00:00
abstract:PURPOSE OF REVIEW:This review summarizes key findings of the current literature on functional neuroimaging in migraine and describes how these studies have changed our view of the disorder. RECENT FINDINGS:Recent studies have started not only to investigate the global cerebral activation pattern during migraine attack...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e3283532ca3
更新日期:2012-06-01 00:00:00
abstract:PURPOSE OF REVIEW:This review aims to describe new features on the epidemiology of encephalitis world-wide. As this neurological presentation is most frequently related to transmitted viruses, surveillance of encephalitis is of major importance to detect their emergence or re-emergence. RECENT FINDINGS:Rabies causes o...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000097
更新日期:2014-06-01 00:00:00
abstract::Slowly, advances are being made in the understanding and treatment of primary childhood brain tumors. The addition of chemotherapy has resulted in better survival for children with medulloblastoma. For other tumor types, therapeutic advances have been slower. High-dose chemotherapy is increasingly being employed to tr...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199712000-00005
更新日期:1997-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Patients with acute life-threatening neuromuscular disease require close cooperation between neuromuscular and intensive care specialists to achieve the best possible outcomes. The problems encountered by these patients are different from those in traditional neuromuscular practice, and neurologists c...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000480
更新日期:2017-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Neurosteroids are a family of compounds synthesized directly in the brain by transforming cholesterol into pregnenolone, which is then converted to compounds such as allopregnanolone and allotetrahydrodeoxycorticosterone. In view of their ability to modulate neurotransmission, neurosteroids may influe...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e32833735cf
更新日期:2010-04-01 00:00:00
abstract:PURPOSE OF REVIEW:Hyperglycemia is frequent in patients with cerebrovascular disease. This review article aims to summarize the recent evidence from observational studies that examined the adverse cerebrovascular effects of dysglycemic states as well as interventional studies assessing intensive management strategies f...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e3283418fed
更新日期:2011-02-01 00:00:00
abstract:PURPOSE OF REVIEW:A stroke mimic is a situation in which a diagnosis of stroke at admission is not confirmed, and a stroke chameleon is a situation in which a stroke is revealed by clinical symptoms that are not usual in stroke. The objective of this review is to identify the most frequent clinical situations in which ...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000620
更新日期:2019-02-01 00:00:00
abstract::A long series of studies beginning with the pioneering experiments of Wiesel and Hubel [1] has shown that correct visual experience is crucial for the development of the visual system. Recent results suggest that neurotrophic factors of the nerve growth factor family might have a relevant role in mediating the effects...
journal_title:Current opinion in neurology
pub_type: 杂志文章
doi:10.1097/00019052-199604000-00012
更新日期:1996-04-01 00:00:00
abstract:PURPOSE OF REVIEW:Neuroimaging in motor neuron disease (MND) has traditionally been seen as an academic tool with limited direct relevance to individualized patient care. This has changed radically in recent years as computational imaging has emerged as a viable clinical tool with true biomarker potential. This transit...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000569
更新日期:2018-08-01 00:00:00
abstract:PURPOSE OF REVIEW:The role of chemotherapy in low-grade glioma has been redefined with the long-term follow-up of the RTOG 9802, which investigated adjuvant procarbazine, CCNU, and vincristine (PCV) chemotherapy in addition to radiotherapy, and the results of EORTC trial 22033 in a similar patient population that compa...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000257
更新日期:2015-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Traumatic brain injury (TBI) is a leading cause of death and disability in children. Prognostication of outcome following TBI is challenging in this population and likely requires complex, multimodal models to achieve clinically relevant accuracy. This review highlights injury characteristics, physiol...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000504
更新日期:2017-12-01 00:00:00
abstract:PURPOSE OF REVIEW:The correct classification of non-Alzheimer's dementia is crucial to study disease mechanisms, predict disease progression and test disease-specific treatments. Brain atrophy assessment with morphometric MRI is currently the gold standard for in-vivo localization of neurodegeneration. Structural and f...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000571
更新日期:2018-08-01 00:00:00
abstract::Magnetic resonance spectroscopy provides noninvasive insight into the regional and global biochemical alterations that are concomitants of the dynamic processes that underlie the evolution of fundamental pathologic changes in multiple sclerosis. These include now well-recognized alterations of neuronal biochemical mar...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200206000-00004
更新日期:2002-06-01 00:00:00
abstract:PURPOSE OF REVIEW:Gliomas represent a disparate group of malignancies with varying clinical outcomes despite a tremendous amount of time, effort, and resources dedicated to their management and understanding. The most aggressive entity, glioblastoma, has a dismal prognosis with poor local control despite intense local ...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000760
更新日期:2019-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Uveomeningoencephalitides, commonly known as Vogt-Koyanagi-Harada (VKH) disease, are characterized by chronic bilateral granulomatous panuveitis involving the central nervous, auditory and integumentary systems. Visual prognosis is generally favourable, but outcomes in patients with VKH disease may va...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000169753.31321.4e
更新日期:2005-06-01 00:00:00
abstract::Desmin-related myopathies are marked by accumulation of desmin, which is often familial and associated with cardiomyopathy. When multifocal this excess is characterized by inclusions such as cytoplasmic or spheroid bodies, when disseminated the excess is called granulofilamentous material. Excess of desmin might repre...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199710000-00012
更新日期:1997-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Convergent evidence from a number of neuroscience disciplines supports the hypothesis that Alzheimer's disease and other neurodegenerative disorders progress along brain networks. This review considers the role of neuroimaging in strengthening the case for network-based neurodegeneration and elucidati...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e32835a26b3
更新日期:2012-12-01 00:00:00
abstract::Dermatomyositis and polymyositis are treatable disorders of skeletal muscle. Despite their clinical similarities, they appear to have fundamentally different autoimmune origins. Inclusion body myositis, from its origins 30 years ago, has emerged as the commonest acquired myopathy of the elderly. Despite inflammatory c...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200110000-00007
更新日期:2001-10-01 00:00:00
abstract:PURPOSE OF REVIEW:The aim is to systematically and critically review the relationship between migraine and estrogen, the predominant female sex hormone, with a focus on studies published in the last 18 months. RECENT FINDINGS:Recent functional MRI (fMRI) studies of the brain support the existence of anatomical and fun...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000091
更新日期:2014-06-01 00:00:00
abstract::A relatively small number of studies were published on the intra-arterial therapy of stroke in the past year. Those reports, however, do offer strong evidence for the feasibility and safety of this therapeutic approach. Partly on the basis of these data it has become evident that the intra-arterial thrombolytic therap...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200002000-00010
更新日期:2000-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Medulloblastoma is no more a unique disease. Clinical and biologic classification used so far are challenged by molecular classification(s). Following the consensus article that described four molecular groups of medulloblastoma in 2012, several articles in 2017 provided more relevant classifications ...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000626
更新日期:2018-12-01 00:00:00
abstract:PURPOSE OF REVIEW:This review will summarize new neuroimaging techniques, particularly MRI and PET imaging, that can be used to assess brain tumor growth and angiogenesis. RECENT FINDINGS:Glioma tumor vasculature is abnormal, and advances in MRI now permit visualization of the hemodynamic properties of gliomas includi...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e328318402a
更新日期:2008-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Clinical MRI is of paramount importance for multiple sclerosis diagnosis but lacks the specificity to investigate the pathogenic mechanisms underlying disease onset and progression. The application of advanced MR sequences allows the characterization of diverse and complex pathological mechanisms, gra...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000559
更新日期:2018-06-01 00:00:00
abstract:PURPOSE OF REVIEW:To describe the background, principles and implementation of the Stroke Action Plan for Europe 2018--2030 (SAP-E) in the perspective of the current COVID-19 pandemic. RECENT FINDINGS:SAP-E sets targets for the implementation of evidence-based preventive actions and stroke services to 2030. A recent m...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000893
更新日期:2021-02-01 00:00:00
abstract:PURPOSE OF REVIEW:To summarize the currently known side effects of the approved therapies of multiple sclerosis and to suggest monitoring procedures. RECENT FINDINGS:The progress in the treatment of multiple sclerosis with new very effective therapies is accompanied by a number of side effects. Some of these have alre...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000824
更新日期:2020-06-01 00:00:00