Cochlear gene therapy.

Abstract:

PURPOSE OF REVIEW:This review highlights recent advances in cochlear gene therapy over the past several years. Cochlear gene therapy has undergone tremendous advances over the past decade. Beginning with some groundbreaking work in 2005 documenting hair cell regeneration using virally mediated delivery of the mouse atonal 1 gene, gene therapy is now being explored as a possible treatment for a variety of causes of hearing loss. RECENT FINDINGS:Recent advances in cochlear gene therapy include improved methods of gene delivery with a better delineation of viral vectors that are suitable for this purpose, additional improvements in hair cell regeneration, and directed research toward autoimmune hearing loss, ototoxicity, spiral ganglion survival, and genetic forms of hearing loss. SUMMARY:If successful, cochlear gene therapy will dramatically alter our ability to treat a variety of forms of acquired and genetic hearing loss.

journal_name

Curr Opin Neurol

authors

Lustig LR,Akil O

doi

10.1097/WCO.0b013e32834f038c

subject

Has Abstract

pub_date

2012-02-01 00:00:00

pages

57-60

issue

1

eissn

1350-7540

issn

1473-6551

journal_volume

25

pub_type

杂志文章,评审
  • Factors directly affecting impulse transmission in inflammatory demyelinating disease: recent advances in our understanding.

    abstract::Demyelination and inflammation both contribute to the neurological deficits characteristic of multiple sclerosis and Guillain-Barré syndrome. Conduction deficits attributable to demyelination are well known, but it is becoming clear that factors such as nitric oxide, endocaine, cytokines, and antiganglioside antibodie...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-200106000-00005

    authors: Smith KJ,Hall SM

    更新日期:2001-06-01 00:00:00

  • Cervicogenic causes of vertigo.

    abstract:PURPOSE OF REVIEW:Herein we discuss the recent literature concerning cervicogenic vertigo including vertigo associated with rotational vertebral artery syndrome, as well as whiplash and degenerative disturbances of the cervical spine. We conclude with a summary of progress regarding diagnostic methods for cervicogenic ...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000161

    authors: Hain TC

    更新日期:2015-02-01 00:00:00

  • Genetics of the epilepsies.

    abstract::Epilepsies, like other common diseases, have complex inheritance, and molecular genetic studies in such conditions are difficult. There has been recent success in identifying the molecular basis of certain epilepsies, particularly in those syndromes with autosomal dominant inheritance. All four genes discovered to dat...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-199904000-00008

    authors: Berkovic SF,Scheffer IE

    更新日期:1999-04-01 00:00:00

  • Structural human brain networks: hot topics in diffusion tractography.

    abstract:PURPOSE OF REVIEW:After more than 10 years of methodological developments and clinical applications, diffusion imaging tractography has reached a crossroad. Although the method is still in its infancy, the time has come to address some important questions. Can tractography reproduce reliably known anatomy or describe n...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e328355d544

    authors: Dell'Acqua F,Catani M

    更新日期:2012-08-01 00:00:00

  • Neuropathology of variants of progressive supranuclear palsy.

    abstract:PURPOSE OF REVIEW:Neurodegenerative tauopathies, of which progressive supranuclear palsy (PSP) is one of the most common, are clinically heterogeneous, reflecting differences in distribution and biochemical composition of tau pathology. This review highlights the range of clinical and pathologic presentations of PSP an...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e32833be924

    authors: Dickson DW,Ahmed Z,Algom AA,Tsuboi Y,Josephs KA

    更新日期:2010-08-01 00:00:00

  • Diffusion tensor magnetic resonance imaging tractography in cognitive disorders.

    abstract:PURPOSE OF REVIEW:The advent of novel techniques for tracing connections in vivo, such as diffusion tensor magnetic resonance imaging tractography, allows us, for the first time in the human brain, to study the microstructural integrity of white matter fibres and perform virtual dissections of large scale neurocognitiv...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/01.wco.0000247610.44106.3f

    authors: Catani M

    更新日期:2006-12-01 00:00:00

  • Defective glycosylation in congenital muscular dystrophies.

    abstract:PURPOSE OF REVIEW:The recent identification of mutations in five genes coding for proteins with putative or demonstrated glycosyltransferase activity has shed light on a novel mechanism responsible for muscular dystrophy. Abnormal glycosylation of alpha-dystroglycan appears to be a common finding in all these condition...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-200404000-00020

    authors: Muntoni F,Brockington M,Torelli S,Brown SC

    更新日期:2004-04-01 00:00:00

  • The changing concepts in the neuropathology of acquired demyelinating central nervous system disorders.

    abstract:PURPOSE OF REVIEW:Research on multiple sclerosis (MS) pathogenesis and therapy is to a large extent driven by results obtained in experimental autoimmune encephalomyelitis (EAE). This approach provided deep insights into the mechanism of brain inflammation and immune mediated tissue injury and, thus, most of our curren...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000685

    authors: Lassmann H

    更新日期:2019-06-01 00:00:00

  • Intra-arterial thrombolysis in acute stroke.

    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

    authors: Abou-Chebl A,Furlan AJ

    更新日期:2000-02-01 00:00:00

  • Treatment of the migraine attack.

    abstract::Migraine is an episodic headache disorder that occurs with or without aura and is often accompanied by other symptoms. Treatment must address these associated symptoms as well as the headache, and it can involve either acute or preventive therapy alone or a combination of both. Acute therapy treats the individual atta...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-199406000-00014

    authors: Silberstein SD

    更新日期:1994-06-01 00:00:00

  • The influence of anxiety on ocular motor control and gaze.

    abstract:PURPOSE OF REVIEW:The influence of anxiety on ocular motor control and gaze has received less research attention than its effects on postural control and locomotion. This review summarizes research on trait anxiety, state anxiety, anxiety disorders, ocular motor reflexes, and gaze. It applies these findings to clinical...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000055

    authors: Staab JP

    更新日期:2014-02-01 00:00:00

  • Neurodegeneration with brain iron accumulation.

    abstract:PURPOSE OF REVIEW:Recent years have witnessed the discoveries of several genes causing neurodegeneration with brain iron accumulation (NBIA) and subsequently their novel classification scheme was suggested. The first results of treatments with modern chelating drugs are also being published. RECENT FINDINGS:Most recen...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e3283550cac

    authors: Dusek P,Schneider SA

    更新日期:2012-08-01 00:00:00

  • Diabetic Myopathy: current molecular understanding of this novel neuromuscular disorder.

    abstract:PURPOSE OF REVIEW:Here we summarize the evidence from human studies of the impairments to the structural, functional, and metabolic capacities in skeletal muscle in those with type 1 diabetes (T1D) - a condition known as diabetic myopathy. Given the importance of skeletal muscle for blood lipid and glucose management, ...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000479

    authors: Monaco CMF,Perry CGR,Hawke TJ

    更新日期:2017-10-01 00:00:00

  • Advances in memory research: single-neuron recordings from the human medial temporal lobe aid our understanding of declarative memory.

    abstract:PURPOSE OF REVIEW:To gain a complete understanding of how the brain functions, both in illness and good health, data from multiple levels of analysis must be integrated. Technical advances have made direct recordings of neuronal activity deep inside the human brain tractable, providing a rare glimpse into cellular proc...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e3283168e03

    authors: Viskontas IV

    更新日期:2008-12-01 00:00:00

  • Learning and memory: recent findings.

    abstract::Recent findings from neuroimaging, event-related potential and lesion investigations reflect a rapidly emerging view that the memory system is widely distributed throughout the cortex. It is clear that the pattern of cortical involvement during encoding and retrieval of memories is critically dependent on the nature a...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-200108000-00004

    authors: Bright P,Kopelman MD

    更新日期:2001-08-01 00:00:00

  • Central nervous system infections in the systemic vasculitides.

    abstract:PURPOSE OF REVIEW:Central nervous system infections are not uncommon in systemic vasculitides. Being vigilant of central nervous system infections as an alternative consideration, instead of systemic vasculitides per se, could prove to be lifesaving in certain circumstances. RECENT FINDINGS:Common pathogens, such as t...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e3283013983

    authors: Ye S,Yang CD

    更新日期:2008-06-01 00:00:00

  • Cognitive abilities and behaviour of children exposed to antiepileptic drugs in utero.

    abstract:PURPOSE OF REVIEW:The last two decades have witnessed a growing concern over the treatment of epilepsy in women of childbearing age, with an increased risk of major congenital malformations and possible cognitive difficulties associated with certain antiepileptic drugs. The aim here is to review the literature regardin...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e3283292401

    authors: Bromley RL,Baker GA,Meador KJ

    更新日期:2009-04-01 00:00:00

  • The clinical and pathologic hallmarks of progressive supranuclear palsy.

    abstract::This article reviews recent studies evaluating the clinicopathologic markers of progressive supranuclear palsy, which have helped establish standardized clinical and pathologic diagnostic criteria. Although these criteria increase diagnostic accuracy, the clinical and pathologic overlap between progressive supranuclea...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-199708000-00011

    authors: Litvan I

    更新日期:1997-08-01 00:00:00

  • Surgical treatment of Parkinson's disease.

    abstract::Ablative surgery and deep brain stimulation for Parkinson's disease can be performed in the thalamus, the pallidum and the subthalamic nucleus. The efficacy and safety of unilateral pallidotomy is well established. Deep brain stimulation has a lower morbidity and is preferred for bilateral surgery. The subthalamic nuc...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-199908000-00008

    authors: Krack P,Hamel W,Mehdorn HM,Deuschl G

    更新日期:1999-08-01 00:00:00

  • The pupil.

    abstract:PURPOSE OF REVIEW:To give an overview on topics of pupillary function selected according to their relevance in clinical neurology. Mainly publications of the last 3 years have been considered. RECENT FINDINGS:The discovery of photosensitive retinal ganglion cells not serving vision, but serving circadian rhythm and th...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章

    doi:10.1097/WCO.0b013e3282f39173

    authors: Wilhelm H

    更新日期:2008-02-01 00:00:00

  • Treatment of painful neuropathy.

    abstract:PURPOSE OF REVIEW:With the aging of the population, treatment of painful neuropathies is becoming more and more important for neurological practice. This short review highlights recent findings and current problems. RECENT FINDINGS:In addition to tricyclic antidepressants and gabapentin, the reliability of which is es...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e328285dfd6

    authors: Cruccu G

    更新日期:2007-10-01 00:00:00

  • Migraine: imaging the aura.

    abstract::We currently conceive of a migraine attack as originating in the brain. Triggers of an attack initiate a depolarizing neuroelectric and metabolic event likened to the spreading depression of Leao. This event activates the headache and associated features of the attack by mechanisms that remain to be determined, but ap...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-200006000-00007

    authors: Aurora SK,Welch KM

    更新日期:2000-06-01 00:00:00

  • Inflammation and ischaemic stroke.

    abstract:PURPOSE OF REVIEW:Inflammation is implicated in ischaemic stroke as a general cardiovascular risk factor, a possible immediate trigger, a component (and possible exacerbating factor) of the response to tissue injury, a marker of future risk, and as a therapeutic target. Each aspect is reviewed. RECENT FINDINGS:Evidenc...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e32813ba151

    authors: Muir KW,Tyrrell P,Sattar N,Warburton E

    更新日期:2007-06-01 00:00:00

  • Advances in ultra-high field MRI for the clinical management of patients with brain tumors.

    abstract:PURPOSE OF REVIEW:The last 5 years have seen the number of ultra-high field (UHF; 7 T and beyond) MRI scanners nearly double. Benefits include improved specificity, better sensitivity for signal-starved compounds, and the ability to detect, quantify, and monitor tumor activity and treatment effects. This is especially ...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e32834cd495

    authors: Lupo JM,Li Y,Hess CP,Nelson SJ

    更新日期:2011-12-01 00:00:00

  • Pseudoprogression and pseudoresponse in the treatment of gliomas.

    abstract:PURPOSE OF REVIEW:Treatment response of brain tumours is typically evaluated with gadolinium-enhanced MRI using the Macdonald criteria. These criteria depend on changes in the area of enhancement. However, gadolinium enhancement of brain tumours primarily reflects impairment of the blood-brain barrier. RECENT FINDINGS...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0b013e328332363e

    authors: Brandsma D,van den Bent MJ

    更新日期:2009-12-01 00:00:00

  • Biomarkers in amyotrophic lateral sclerosis: a review of new developments.

    abstract:PURPOSE OF REVIEW:This review draws together the most recent findings in ALS biomarker research from biochemical, imaging and neurophysiology techniques. RECENT FINDINGS:The potential of circulating RNA is highlighted, including new retrieval techniques. With ongoing genetic clinical trials, the need for pharmacodynam...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章

    doi:10.1097/WCO.0000000000000854

    authors: Verber N,Shaw PJ

    更新日期:2020-10-01 00:00:00

  • Tremor entities and their classification: an update.

    abstract:PURPOSE OF REVIEW:This review focuses on important new findings in the field of tremor and illustrates the consequences for the current definition and classification of tremor. RECENT FINDINGS:Since 1998 when the consensus criteria for tremor were proposed, new variants of tremors and new diagnostic methods were disco...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000211

    authors: Gövert F,Deuschl G

    更新日期:2015-08-01 00:00:00

  • MRI monitoring of spinal cord changes in patients with multiple sclerosis.

    abstract:PURPOSE OF REVIEW:We analyze recent data on technical aspects, clinical indications, and imaging features of spinal cord MRI in multiple sclerosis, and on the value of this examination for assessing the type and extension of spinal cord damage, and for predicting prognosis in patients with this disease. RECENT FINDING...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/WCO.0000000000000343

    authors: Rovira A,de Stefano N

    更新日期:2016-08-01 00:00:00

  • Assessing the severity of seizures and epilepsy: which scales are valid?

    abstract::A review of the literature on methods of seizure or epilepsy severity assessments resulted in tabulation of the seizure rating scales known as US Department of Veterans Affairs (VA), Chalfont-National Hospital, Liverpool, Hague, and others. Each of the scales reviewed has some advantages, but none of them appears to b...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/00019052-200104000-00015

    authors: Cramer JA

    更新日期:2001-04-01 00:00:00

  • Genetic dissection of the common epilepsies.

    abstract:PURPOSE OF REVIEW:Only two functionally validated susceptibility genes, CACNA1H and GABRD, have so far been identified in the common epilepsies using a candidate gene approach. The difficulty with the alternative statistical approach, where none of the suggested candidates has been functionally validated, may partly be...

    journal_title:Current opinion in neurology

    pub_type: 杂志文章,评审

    doi:10.1097/01.wco.0000218232.66054.46

    authors: Tan NC,Mulley JC,Scheffer IE

    更新日期:2006-04-01 00:00:00