Abstract:
PURPOSE OF REVIEW:This article discusses recent advances in the understanding of clinical and genetic aspects of primary ataxias, including congenital, autosomal recessive, autosomal dominant, episodic, X-linked, and mitochondrial ataxias, as well as idiopathic degenerative and secondary ataxias. RECENT FINDINGS:Many important observations have been published in recent years in connection with primary ataxias, particularly new loci and genes. The most commonly inherited ataxias may present with typical and atypical phenotypes. In the group of idiopathic degenerative ataxias, genes have been found in patients with multiple system atrophy type C. Secondary ataxias represent an important group of sporadic, cerebellar, and afferent/sensory ataxias. SUMMARY:Knowledge of primary ataxias has been growing rapidly in recent years. Here we review different forms of primary ataxia, including inherited forms, which are subdivided into congenital, autosomal recessive cerebellar ataxias, autosomal dominant cerebellar ataxias, episodic ataxias, X-linked ataxias, and mitochondrial ataxias, as well as sporadic ataxias and idiopathic degenerative ataxias. Secondary or acquired ataxias are also reviewed and the most common causes are discussed.
journal_name
Curr Opin Neuroljournal_title
Current opinion in neurologyauthors
Teive HA,Ashizawa Tdoi
10.1097/WCO.0000000000000227subject
Has Abstractpub_date
2015-08-01 00:00:00pages
413-22issue
4eissn
1350-7540issn
1473-6551journal_volume
28pub_type
杂志文章,评审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 ato...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e32834f038c
更新日期:2012-02-01 00:00:00
abstract:PURPOSE OF REVIEW:To review the most relevant developments in the understanding of headache in idiopathic intracranial hypertension (IIH). RECENT FINDINGS:The phenotype of the typical IIH headache is diverging from the historical thinking of a raised intracranial pressure headache, with the majority being classified a...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000651
更新日期:2019-02-01 00:00:00
abstract:PURPOSE OF REVIEW:Functional neuro-imaging studies of aphasic stroke offer the potential for a better understanding of the neuronal mechanisms that sustain language recovery. Conclusions, however, have been hampered by a set of unexpected challenges related to experimental design and interpretation. In this review of s...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000168081.76859.c1
更新日期:2005-08-01 00:00:00
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
更新日期:2001-04-01 00:00:00
abstract:PURPOSE OF REVIEW:Patients with asymptomatic carotid stenosis are at increased vascular risk but optimal treatment is controversial. We reviewed the current evidence for medical and surgical intervention in these patients. RECENT FINDINGS:Optimal medical treatment is the most important aspect of management of patients...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e328012da60
更新日期:2007-02-01 00:00:00
abstract:PURPOSE OF REVIEW:The aim is to review sleep disturbances in different movement disorders, mainly Parkinson's disease, and highlight current concepts on merging the boundaries between movement disorders and dissociative states of being. RECENT FINDINGS:Since the observation that rapid eye movement sleep behavior disor...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e3283632cef
更新日期:2013-08-01 00:00:00
abstract:PURPOSE OF REVIEW:This review on traumatic brain injury consolidates the substantial current literature available on the pathophysiology, mechanisms, developments, and their subsequent effects on outcome. In particular, it tries to conceptualize why our greatly improved understanding of pathophysiology and neurobiology...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200412000-00011
更新日期:2004-12-01 00:00:00
abstract:PURPOSE OF REVIEW:This review focuses on recent advances in the diagnostic approaches and the underlying pathophysiological mechanisms of Charcot-Marie-Tooth (CMT) disease. We also discuss the emerging therapies for this hereditary neuropathy. RECENT FINDINGS:To date, numerous genes are implicated in CMT, and new gene...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e328364c04b
更新日期:2013-10-01 00:00:00
abstract::The review intends to draw the attention of researchers working in retinal degenerations on the fact that classical apoptosis, for example, apoptosis triggering caspase activation, may not be the main pathway of cellular demise in this tissue.Former work already showed the difficulty of proving the activation of apopt...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000272
更新日期:2016-02-01 00:00:00
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
更新日期:1999-04-01 00:00:00
abstract:PURPOSE OF REVIEW:This review describe the role and advancements of MRI in the diagnosis and monitoring of progressive multifocal leukoencephalopathy (PML) in multiple sclerosis patients treated with natalizumab. RECENT FINDINGS:MRI is the most sensitive paraclinical tool in the detection of PML lesions in natalizumab...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000099
更新日期:2014-06-01 00:00:00
abstract::Neural circuits comprised of dopamine-containing and dopamine-receptive neurons are altered functionally after the repeated intermittent administration of cocaine and its withdrawal. The characterization of these neuroadaptive changes at various times after repeated exposure to cocaine is important because they may re...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199712000-00017
更新日期:1997-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Gadolinium-enhancement depicts blood-brain barrier disruption associated with new inflammatory MRI lesions in multiple sclerosis (MS) and is widely used for diagnosis and therapeutic monitoring. However, earlier and more specific markers of inflammation are urgently needed. RECENT FINDINGS:Susceptibi...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000326
更新日期:2016-06-01 00:00:00
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
更新日期:2011-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Trigeminal autonomic cephalgias (TACs) are characterized by frequent, short-lasting headache attacks with ipsilateral facial autonomic features. They include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. The...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/wco.0b013e32832b4bb3
更新日期:2009-06-01 00:00:00
abstract:PURPOSE OF REVIEW:The present review will outline neuroprotective and neurotoxic effects of central nervous system (CNS) infiltrating T cells during viral infections. Evidence demonstrating differential roles for antiviral effector and resident memory T-cell subsets in virologic control and immunopathology in the CNS w...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000825
更新日期:2020-06-01 00:00:00
abstract:PURPOSE OF REVIEW:Campylobacter jejuni is the most frequent agent of antecedent infection in an axonal variant of Guillain-Barré syndrome, acute motor axonal neuropathy, and anti-GM1 or anti-GD1a IgG antibody is also associated with acute motor axonal neuropathy. Molecular mimicry has been found between human GM1 gangl...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000174604.42272.2d
更新日期:2005-10-01 00:00:00
abstract::Multiple sclerosis is a common neurological disorder that represents a significant source of disability. B cells have recently emerged as a novel therapeutic target for multiple sclerosis. The natural development of B cells is characterized by an antigen-independent phase that occurs in the bone marrow and an antigen-...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/01.wco.0000313358.53553.c7
更新日期:2008-04-01 00:00:00
abstract::In the past few years evidence that atherosclerotic disease of the aortic arch is probably an underestimate source of emboli, particularly moving thrombi in the lumen, has accumulated. Pathological studies, and more recently transesophageal echocardiographic studies, have shown that atherosclerotic disease of the aort...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199702000-00013
更新日期:1997-02-01 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:PURPOSE OF REVIEW:This is an update on skeletal muscle sodium channelopathies since knowledge in the field have dramatically increased in the past years. RECENT FINDING:The relationship between two phenotypes and SCN4A has been confirmed with additional cases that remain extremely rare: severe neonatal episodic laryng...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000238
更新日期:2015-10-01 00:00:00
abstract::Deletion of the 1.5 Mb tract on chromosome 17p11.2-12 that is duplicated in Charcot-Marie-Tooth disease type 1A is commonly associated with hereditary neuropathy with liability to pressure palsies. The deletion, which originates from an unequal meiotic crossover involving two homologous repeats, causes underexpression...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199610000-00006
更新日期:1996-10-01 00:00:00
abstract:PURPOSE OF REVIEW:Cognitive impairments are common in children with epilepsy. They may already be present before the onset of epilepsy or occur - and even progress - during its course. Many variables contribute to cognitive dysfunction. Those that can be targeted to prevent (further) cognitive impairment will be highli...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000424
更新日期:2017-04-01 00:00:00
abstract::The motoneuron has a strong ability to regenerate after injury. However, the problems of nerve cell survival after a proximal axotomy, difficulty in axonal elongation after intraspinal lesion and the lack of target specificity during nerve fibre regrowth interfere with a good functional restitution. Current research h...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-200012000-00012
更新日期:2000-12-01 00:00:00
abstract::Although reactive oxygen species are natural metabolic products, they can be toxic to cells and are implicated in some neurodegenerative disease. Cytosolic Cu, Zn superoxide dismutase normally defends against damage by reactive oxygen species; however, mutant forms of the enzyme might instead contribute to damage of m...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/00019052-199612000-00016
更新日期:1996-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Frontotemporal dementia (FTD) is a clinically, pathologically and genetically heterogeneous disorder. Mutations in a number of genes are associated with FTD, although until recently only two [progranulin (GRN) and microtubule-associated protein tau (MAPT)] were known to be major causes of the disease....
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0b013e32834cd442
更新日期:2011-12-01 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:We present an overview of recent concepts in mechanisms underlying cognitive decline associated with brain aging and neurodegeneration from the perspective of MRI. RECENT FINDINGS:Recent findings challenge the established link between neuroimaging biomarkers of neurodegeneration and age-related or di...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000029
更新日期:2013-12-01 00:00:00
abstract:PURPOSE OF REVIEW:Cerebral amyloid angiopathy (CAA) is diagnosed primarily as a cause of lobar intracerebral hemorrhages (ICH) in elderly patients. With improving MRI techniques, however, the role of CAA in causing other symptoms has become clear. Recognizing the full clinical spectrum of CAA is important for diagnosis...
journal_title:Current opinion in neurology
pub_type: 杂志文章,评审
doi:10.1097/WCO.0000000000000510
更新日期:2018-02-01 00:00:00
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
更新日期:2016-08-01 00:00:00