Abstract:
:Multiple system atrophy (MSA) is a rare late onset neurodegenerative disorder which presents with autonomic failure and a complicated motor syndrome including atypical parkinsonism, ataxia and pyramidal signs. MSA is a glial alpha-synucleinopathy with rapid progression and currently poor therapeutic management. This paper reviews the clinical features, natural history and novel diagnostic criteria for MSA as well as contemporary knowledge on pathogenesis based on evidence from neuropathological studies and experimental models. An outline of the rationale for managing symptomatic deterioration in MSA is provided together with a summary of novel experimental therapeutic approaches to decrease disease progression.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Wenning GK,Stefanova Ndoi
10.1007/s00415-009-5173-8subject
Has Abstractpub_date
2009-11-01 00:00:00pages
1791-808issue
11eissn
0340-5354issn
1432-1459journal_volume
256pub_type
杂志文章,评审abstract::Applying microarray technology to identify new diagnostic and prognostic markers in peripheral blood cells (PBC) after therapeutic intervention opens great perspectives regarding patient subclassification. Three recombinant products of the pleiotropic agent interferon beta (rIFN-beta) are available for disease modifyi...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-008-6010-1
更新日期:2008-12-01 00:00:00
abstract:: ...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-018-9066-6
更新日期:2018-12-01 00:00:00
abstract::The diagnosis of natalizumab-associated progressive multifocal leukoencephalopathy (PML) in multiple sclerosis patients in an asymptomatic stage is crucial since it is associated with better clinical outcome measures. Current diagnostic criteria on PML diagnosis in asymptomatic patients require the detection of JC vir...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7336-5
更新日期:2014-06-01 00:00:00
abstract::The gut with its variety of microbiota may serve as an etiological origin of diseases. Gut microbes may also play a role in the pathogenesis of diseases beyond their simple nutritional maintenance and support. For example, gut protein aggregation, possibly aided by microbes as well as nasal influences, might be linked...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8588-7
更新日期:2017-09-01 00:00:00
abstract::We recently reported findings that loss of cortical acetylcholinesterase (AChE) activity is greater in parkinsonian dementia than in Alzheimer's disease (AD). In this study we determined cognitive correlates of in vivo cortical AChE activity in patients with parkinsonian dementia (PDem, n = 11), Parkinson's disease wi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0971-0
更新日期:2006-02-01 00:00:00
abstract::Polysomnography (PSG) is considered the gold standard for diagnosis of non-rapid eye movement (NREM) parasomnias, however its diagnostic yield has been rarely reported. We aimed to assess the diagnostic value of polysomnography in different categories of patients with suspected NREM parasomnia and define variables tha...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7578-2
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Rehabilitation seems to promote brain plasticity, but objective measures of efficacy are lacking and there is a limited understanding of the mechanisms underlying functional recovery. OBJECTIVE:To study functional and structural brain changes induced by gait rehabilitation. METHODS:We enrolled MS inpatient...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-018-8859-y
更新日期:2018-06-01 00:00:00
abstract:OBJECTIVES:Recent retrospective studies found sleep disorders, including obstructive sleep apnea and its symptoms to occur more often in patients following aneurysmal subarachnoid hemorrhage, but studies investigating the incidence of subarachnoid hemorrhage in patients with diagnosed obstructive sleep apnea [OSA] comp...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09265-5
更新日期:2019-06-01 00:00:00
abstract:OBJECTIVE:To determine the prevalence and risk factors for paroxysmal atrial fibrillation (PAF) diagnosis in non- cryptogenic ischemic stroke (CIS) patients. METHODS:In this pilot-prospective cohort study of non-CIS patients from September 2014 to September 2017, 53 patients were enrolled. 51/53 patients were implante...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-018-8982-9
更新日期:2018-10-01 00:00:00
abstract::The cause of idiopathic intracranial hypertension (IIH) remains unknown, and no consensus exists on how patients should be monitored and treated. Acetazolamide is a common treatment but has never been examined in a randomised controlled trial. The objectives of this pilot trial are to prospectively evaluate the use of...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-010-5861-4
更新日期:2011-05-01 00:00:00
abstract::A recently developed technique is used to study the permeation of water through membranes of human erythrocyte ghosts. The method, based on the difference of the indices of refraction of H2O and D2O, is briefly described. Comparative measurements on erythrocyte ghosts from normal donors and from patients with Huntingt...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313655
更新日期:1984-01-01 00:00:00
abstract::Both neurologists and urologists might encounter patients with acute urinary retention due to benign inflammatory nervous diseases. Based on the mechanism of urinary retention, these disorders can be divided into two subgroups: disorders of the peripheral nervous system (e.g., sacral herpes) or the central nervous sys...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0189-9
更新日期:2006-08-01 00:00:00
abstract::Recent studies reported mutations in the gene encoding the proline-rich transmembrane protein 2 (PRRT2) to be causative for paroxysmal kinesigenic dyskinesia (PKD), PKD combined with infantile seizures (ICCA), and benign familial infantile seizures (BFIS). PRRT2 is a presynaptic protein which seems to play an importan...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-012-6777-y
更新日期:2013-05-01 00:00:00
abstract::Glucocorticoids are the standard of care for multiple sclerosis (MS) relapses, but the most desirable route of administration is still matter of debate. The aim of the study was to compare the efficacy and safety of oral versus intravenous steroids for treatment of acute relapses in patients with MS. Randomized or qua...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-017-8505-0
更新日期:2017-08-01 00:00:00
abstract::Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy and usually follows an autosomal dominant trait. Clinically, FSHD affects facial muscles and proximal upper limb and girdle muscles, but may present with variable clinical phenotypes even within the same family. Most genetically ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5471-1
更新日期:2010-07-01 00:00:00
abstract:OBJECTIVE:To establish differences in basal ganglia and thalamic volume between preclinical carriers and non-carriers of the Huntington's disease (HD) gene and to link the volume to motor, cognitive and behavioural characteristics in carriers. METHODS:Sixteen HD gene carriers without overt clinical motor signs and 14 ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0050-4
更新日期:2008-11-01 00:00:00
abstract:: ...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-018-9103-5
更新日期:2019-01-01 00:00:00
abstract::The polymorphism of the HLA class II genes was investigated in 97 patients with multiple sclerosis (MS) in Hokkaido, the northernmost main island of Japan. Of these, 80 patients were classified as having conventional MS and 17 as having opticospinal MS (OS-MS). Our findings confirmed a previous report that the DPB 1*0...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050558
更新日期:2000-03-01 00:00:00
abstract::Small retrospective case series suggest that decompressive hemicraniectomy can be life saving in patients with cerebral venous thrombosis (CVT) and impending brain herniation. Prospective studies of consecutive cases are lacking. Thus, a single centre, prospective study was performed. In 2006 we adapted our protocol f...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6307-3
更新日期:2012-06-01 00:00:00
abstract::Differentiation of herpes simplex virus (HSV) types 1 and 2 in cerebrospinal fluid of 17 patients with serologically diagnosed HSV encephalitis and meningitis or acute limbic encephalitis was determined by stringent hybridization of polymerase chain reaction--amplified DNAs. Ten of 17 patients were positive; six with ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00873514
更新日期:1994-08-01 00:00:00
abstract:BACKGROUND:The vegetative state, also known as the unresponsive wakefulness syndrome, is one of the worst possible outcomes of acquired brain injury and confronts rehabilitation specialists with various challenges. Emergence to (minimal) consciousness is classically considered unlikely beyond 3-6 months after non-traum...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09542-3
更新日期:2019-12-01 00:00:00
abstract:OBJECTIVE:Determine toxicity and efficacy of autologous hematopoietic stem cell transplantation (HSCT) for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are dependent on intravenous immunoglobulins or plasmapheresis. METHODS:Unselected peripheral blood stem cells were re-infused on...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10010-6
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy. METHODS:We performed a 9-year (2009-2017) population-based epi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09544-1
更新日期:2020-01-01 00:00:00
abstract:INTRODUCTION:Ataxic polyneuropathy, which occurs in endemic form in an area in southwest Nigeria, is attributed to exposure to cyanide from cassava foods. Exposure to cyanide from cassava is, however, not exclusive to this endemic area. In this study, the occurrence of ataxic polyneuropathy was compared in two communit...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0779-0
更新日期:2002-08-01 00:00:00
abstract::A 56-year-old woman with coeliac disease developed myoclonus of cortical origin and palatal myoclonus with lesions of subcortical white matter on magnetic resonance imaging. Myoclonus can thus be a prominent feature of coeliac disease encephalopathy. A slight vitamin E deficiency was found but does not satisfactorily ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314464
更新日期:1989-07-01 00:00:00
abstract::Cerebral perfusion patterns in 18 cases with vascular dementia of Binswanger type (VDBT) (8 moderate and 10 severe cases) were compared with 25 cases with senile dementia of Alzheimer type (SDAT) (16 moderate and 9 severe cases) and 14 controls by single photon emission computed tomography using N-isopropyl-p-123I iod...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00319853
更新日期:1991-10-01 00:00:00
abstract::We discuss two cases receiving different anti-tumornecrosis-factor alpha antagonists (anti-TNF-alpha); one for psoriatic arthritis (PA) and the other for ankylosing spondylitis (AS). Due to neurological symptoms cerebral magnetic resonance imaging (MRI) was performed and cerebral lesions were detected. Our interpretat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-6020-z
更新日期:2008-12-01 00:00:00
abstract::Parkinson's disease (PD) is a neurodegenerative disease caused by both genetic and environmental factors. Sirtuins are highly-conserved, NAD-dependent class III deacetylases that regulate a variety of cellular functions. Most of the known sirtuins have been involved in animal models of neurodegenerative disorders, suc...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6970-7
更新日期:2013-09-01 00:00:00
abstract::In all the sera of four patients with meningoradiculitis of Bannwarth admitted to the neurological department of the University Clinic Grosshadern in Munich in 1983, we found antibodies against Ixodes dammini spirochetes. In three patients antibodies were also present in the CSF. In one patient, we isolated spirochete...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313682
更新日期:1984-01-01 00:00:00
abstract::Neuromyelitis optica (NMO) is an inflammatory/demyelinating disorder predominantly affecting the optic nerves and spinal cord. Recent findings showed an underlying humoral abnormality in NMO, characterized by a serum antibody against aquaporin-4 (Aqp-4-Ab). In this study, we evaluated the Aqp-4-Ab status among Turkish...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5780-4
更新日期:2011-03-01 00:00:00