Abstract：
:Corticobasal degeneration (CBD) was first described by Rebeiz et al. in 1967, and was called corticodentatonigral degeneration with neuronal achromasia [1]. Since then, our knowledge of the clinical features and underlying tau pathology has grown tremendously. Clinical antemortem diagnosis of CBD pathology remains challenging and has led to the development of revised diagnostic criteria. As various clinical phenotypes may have CBD pathology, accurate prevalence studies are lacking. Recently, pooled prevalence of fronto-temporal lobar degeneration, PSP and CBS was reported as 10.6 per 100,000 [2]. Although rare, CBD is an important disease to understand because it provides a model of a specific proteinopathy (tauopathy) and, therefore, opportunity to study pathophysiology of tauopathies and efficacy of tau-directed therapies. In the past few years, identification of tau specific ligands has advanced neuroimaging of tauopathies such as CBD and progressive supranuclear palsy. However, clinical prediction of CBD pathology remains challenging and an active are of research. In this review, we highlight key emerging issues in CBD pathophysiology, genetics and novel neuroimaging techniques with tau ligands.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ali F,Josephs KAdoi
10.1007/s00415-017-8644-3subject
Has Abstractpub_date
2018-02-01 00:00:00pages
439-445issue
2eissn
0340-5354issn
1432-1459pii
10.1007/s00415-017-8644-3journal_volume
265pub_type
历史文章,杂志文章abstract：OBJECTIVES:Sporadic (SSP) and hereditary spastic paraplegias (HSP) are clinically and genetically heterogeneous disorders, which are characterised by a slowly progressive spastic paraparesis. Initial symptoms and the rate of progression are variable even among members of the same family. Spastic paraparesis is the majo...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0366-7
更新日期：2004-05-01 00:00:00
abstract：BACKGROUND AND PURPOSE:The efficacy and safety of mechanical thrombectomy (MT) for M2 segment occlusion of middle cerebral artery in patients with acute ischemic stroke (AIS) was investigated. METHODS:We searched PubMed and EMBASE from inception to 16 April 2019 for relevant studies, calculated the pool relative risks...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-020-09710-w
更新日期：2020-01-22 00:00:00
abstract：:We conducted an open-labeled clinical trial of interferon beta-1b (IFNB) treatment in 20 patients with primary progressive multiple sclerosis (PPMS) and longitudinally monitored autoantibodies against double-stranded DNA (dsDNA), thyroid peroxidase (TPO),myelin basic protein (MBP), myelin oligodendrocyte glycoprotein ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/s00415-004-0580-3
更新日期：2004-12-01 00:00:00
abstract：:Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a common autoimmune encephalitis presenting with psychosis, dyskinesias, autonomic dysfunction and seizures. The underlying autoantibodies against the NR1 subunit are directly pathogenic by disrupting synaptic NMDAR currents. However, antibody titers correlate...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-018-9042-1
更新日期：2018-11-01 00:00:00
abstract：:Intrathecal synthesis of immunoglobulin G (IgG) specific to Treponema pallidum, ssp. pallidum, or to the human immunodeficiency virus 1 (HIV-1) was investigated in patients with contemporary treponemal and HIV-1 infections. Using a T. pallidum and an HIV-1 IgG enzyme-linked immunosorbent assay, specific antibody units...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314359
更新日期：1988-03-01 00:00:00
abstract：:Evidence of a significant improvement of IFNB-1b in clinical severity in the older population with RRMS has not been established so far. The aim of this exploratory post hoc analysis of the 250 mcg IFNB-1b group of the BEYOND study is to compare the efficacy and safety of older versus younger patients using a cut-off ...
journal_title：Journal of neurology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00415-013-6888-0
更新日期：2013-07-01 00:00:00
abstract：:Familial paroxysmal exercise-induced dyskinesia (PED) is a rare movement disorder that is mostly caused by mutations in the solute carrier family 2, member 1 (SLC2A1) gene and inherited in an autosomal dominant manner. Clinical, laboratory, and genetic studies were performed in three family members. The proband's symp...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-014-7441-5
更新日期：2014-10-01 00:00:00
abstract：:Presently, there are more than 50 possible indications for the application of botulinum toxin. In some indications the use of botulinum toxin has already been approved; others are about to be approved. For most of these indications a sufficient number of studies have been published. For rare indications, an insufficie...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/pl00007819
更新日期：2001-04-01 00:00:00
abstract：:Recent genetic studies suggest a Sardinian type of amyotrophic lateral sclerosis (ALS). Thus, ALS incidence, prevalence and survival were investigated in a large population of Sardinians aimed to disclose population-specific patterns and their temporal changes. This is a population-based incidence and prevalence study...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-012-6681-5
更新日期：2013-02-01 00:00:00
abstract：:Patients with multiple system atrophy (MSA) often have evidence of compromised gastrointestinal motility. Ghrelin is a gut hormone that influences gastrointestinal motility in humans. The aim of this study was to determine whether ghrelin secretion is affected in MSA patients, and to investigate the relation between g...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6944-9
更新日期：2013-08-01 00:00:00
abstract：INTRODUCTION:Mounting evidence supports the existence of an important feedforward cycle between sleep and neurodegeneration, wherein neurodegenerative diseases cause sleep and circadian abnormalities, which in turn exacerbate and accelerate neurodegeneration. If so, sleep therapies bear important potential to slow prog...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10334-3
更新日期：2020-12-23 00:00:00
abstract：:Parkinson's disease (PD) is a neurodegenerative disorder of the aging population with unknown etiopathogenesis. It is assumed that the underlying pathobiochemical processes comprise multifactorial and multigenetic disturbances leading to a progressive and devastating disorder without remission. Subtypes exist suggesti...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/pl00007777
更新日期：2000-09-01 00:00:00
abstract：BACKGROUND:Spontaneous cervical artery dissections (sCAD) are often preceded by infections. However, existing data about inflammatory parameters remained inconsistent. Remarkably, concurrent information about the coagulation system, whose affection seems also reasonable to cause ischaemic events, are still lacking in s...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-017-8696-4
更新日期：2018-02-01 00:00:00
abstract：:The short-term benefits of levodopa/carbidopa intestinal gel (LCIG) in patients with advanced Parkinson's disease (PD) are well documented, but the long-term benefits are still uncertain. The aim of this study was to investigate the motor and cognitive outcome of LCIG treatment in advanced PD after a follow-up period ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-7235-1
更新日期：2014-03-01 00:00:00
abstract：:We report on a 14-year-old boy with congenital isolated hemifacial hyperplasia. Hemifacial hypertrophy most likely represents a minor form of congenital hemihypertrophy. MRI of the soft tissue is particularly suitable to support the diagnosis and reveal associated bony asymmetries. ...
journal_title：Journal of neurology
pub_type： 信件
doi：10.1007/s00415-009-5148-9
更新日期：2009-09-01 00:00:00
abstract：:Exercise therapy (ET) can be beneficial in disabled multiple sclerosis (MS) patients. Intermittent transcranial magnetic theta burst stimulation (iTBS) induces long-term excitability changes of the cerebral cortex and may ameliorate spasticity in MS. We investigated whether the combination of iTBS and a program of ET ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/s00415-011-5924-1
更新日期：2011-07-01 00:00:00
abstract：:Multi-modality evoked potentials and computed cranial tomography (CT) were performed in ten patients with Wilson's disease to determine if any of these studies would correlate reliably with neurologic status. While all four patients with CT abnormality had neurologic signs, two additional patients with neurologic find...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314035
更新日期：1985-01-01 00:00:00
abstract：:Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-003-1021-4
更新日期：2003-04-01 00:00:00
abstract：:The greatest unmet need in multiple sclerosis (MS) are treatments that delay, prevent or reverse progression. One of the most tractable strategies to achieve this is to therapeutically enhance endogenous remyelination; doing so restores nerve conduction and prevents neurodegeneration. The biology of remyelination-cent...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-019-09421-x
更新日期：2021-01-01 00:00:00
abstract：:A woman affected by chronic progressive external ophthalmoplegia and muscle mitochondrial DNA deletion was studied by phosphorus magnetic resonance spectroscopy (31P-MRS) prior to and after 1 and 7 months of treatment with oral lipoic acid. Before treatment a decreased phosphocreatine (PCr) content was found in the oc...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00873552
更新日期：1995-07-01 00:00:00
abstract：OBJECTIVE:We report a clinical description of pure sensory stroke based on data collected from a prospective acute stroke registry. METHODS:From 2500 acute stroke patients included in a hospital-based prospective stroke registry over a 12-year period, 99 were identified as having pure sensory stroke. RESULTS:Pure sen...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-005-0622-5
更新日期：2005-02-01 00:00:00
abstract：:Carbon monoxide (CO) intoxication leads to acute and chronic neurological deficits, but little is known about the specific noxious mechanisms. (1)H magnetic resonance spectroscopy (MRS) may allow insight into the pathophysiology of CO poisoning by monitoring neurochemical disturbances, yet only limited information is ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-009-5057-y
更新日期：2009-06-01 00:00:00
abstract：:Glycogen storage disease type II (GSDII) is a lysosomal storage disorder caused by acid alpha-1,4-glucosidase deficiency and associated with recessive mutations in its coding gene GAA. Few studies have provided so far a detailed phenotypical characterization in late onset GSDII (LO-GSDII) patients. Genotype-phenotype ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-013-7137-2
更新日期：2014-01-01 00:00:00
abstract：:A night-time polygraphic sleep recording with continuous HbSaO2 monitoring was performed in 11 chair-bound Duchenne muscular dystrophy patients with severe restrictive lung disease but with blood gas values within normal limits when awake. No abnormalities of sleep pattern were detected. Nocturnal sleep did not have s...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314896
更新日期：1989-10-01 00:00:00
abstract：:The Richards-Rundle syndrome (RRS) is characterized by hearing loss, mental deterioration, ataxia, primary hypogonadism and autosomal recessive transmission. In a sibship of six members we found two sisters with RRS together with baldness, impaired GH and PRL secretion after stimulation and different degrees of impair...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313645
更新日期：1984-01-01 00:00:00
abstract：:Decreased tongue strength (TS) might herald bulbar involvement in patients with amyotrophic lateral sclerosis (ALS) well before dysarthria or dysphagia occur, and as such might be prognostic of short survival. The purpose of this study was to investigate the prognostic value of a decreased TS, in addition to other pro...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-012-6503-9
更新日期：2012-11-01 00:00:00
abstract：OBJECTIVE:To assess the association of Medial Temporal lobe Atrophy (MTA) and White Matter Hyperintensities (WMHs) with gray matter perfusion in Mild Cognitive Impairment (MCI). METHODS:56 MCI patients (age = 69.3 +/- 7.0, 32 females) underwent brain MR scan and (99m)Tc ECD SPECT. We evaluated MTA according to Schelte...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-006-0498-z
更新日期：2007-08-01 00:00:00
abstract：:Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients r...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00415-017-8519-7
更新日期：2017-06-01 00:00:00
abstract：BACKGROUND AND PURPOSE:Dysphagia is estimated to occur in up to 50% of the stroke neurorehabilitation population. Those patients with severe neurogenic oropharyngeal dysphagia (NOD) may receive feeding gastrostomy tubes (FGT) if noninvasive therapies prove ineffective in eliminating aspiration or sustaining adequate nu...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/s00415-005-0906-9
更新日期：2005-12-01 00:00:00
abstract：:We describe three patients with a late presentation of congenital muscular torticollis to emphasize that this non-dystonic type of torticollis has to be considered in the differential diagnosis of cervical dystonia, even in adult patients. Surgery is the treatment of choice. Postoperatively, two of our patients had a ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00868411
更新日期：1996-04-01 00:00:00
