Abstract:
:The recent discovery of disease specific and pathogenic autoantibodies in neuromyelitis optica (NMO, Devic's disease) has revived the interest in this intriguing yet often devastating condition. While the history of classic multiple sclerosis has been studied extensively, only very little is known so far about the early history of NMO. Here we discuss a now forgotten report by the famous French anatomist and pathologist Antoine Portal (1742-1832), first physician to Louis XVIII and founding and lifelong president of the Académie Nationale de Médecine. Portal's report, which fascinated some of the most renowned 19th century pioneers in the field of neurology but fell into oblivion later, represents the first account of visual loss in a patient with spinal cord inflammation but no brain pathology in the Western literature known so far--published more than 60 years prior to Thomas Clifford Allbutt's much cited note on a patient with myelitis and a "sympathetic eye disorder".
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Jarius S,Wildemann Bdoi
10.1007/s00415-011-6355-8subject
Has Abstractpub_date
2012-07-01 00:00:00pages
1354-7issue
7eissn
0340-5354issn
1432-1459journal_volume
259pub_type
历史文章,杂志文章abstract::Silver syndrome/SPG17 is a motor manifestation of mutations in the BSCL2 gene and usually presents as a complicated form of hereditary spastic paraplegia (HSP). We present clinical data, follow-up, and genetic results of seven patients with Silver syndrome/SPG17 including a family with a variable intrafamilial phenoty...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8301-2
更新日期:2017-01-01 00:00:00
abstract::Fingolimod safety and efficacy data in relapsing-remitting multiple sclerosis (RRMS) are available up to 5 years, from an extension of a randomized, placebo-controlled, double-blind, phase 2 study, at a dose higher (5.0/1.25 mg) than the approved dose of 0.5 mg. The objective of the study is to present the end-of-stud...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-015-7834-0
更新日期:2015-12-01 00:00:00
abstract::In order to identify the precise location of the primary motor area for the diaphragm with respect to the classical motor homunculus, functional magnetic resonance imaging (fMRI) experiments were performed utilizing independent component-cross correlation- sequential epoch (ICS) analysis on a high-field (3.0 Tesla) sy...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0413-4
更新日期:2004-06-01 00:00:00
abstract::Neurological complications following rubella are only rarely encountered. However, in many cases severe neurological impairment may occur, leading to permanent disability. In a recent epidemic of rubella in Israel during the years 1978-1979, 20 patients with severe neurological complications have been seen. We report ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313402
更新日期:1982-01-01 00:00:00
abstract::We report a very rare case of Schwartz-Jampel syndrome associated with von Willebrand's disease. This association might be coincidental because of the different modes of inheritance of the two disorders. However, we speculate that there might be some link between the two disorders, for example in the locus of the affe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314041
更新日期:1985-01-01 00:00:00
abstract:OBJECTIVE:Patient reported outcome measures (PROMs) can provide researchers with a direct view of patients' experiences. They are becoming increasingly important tools for evaluating clinical care and research outcomes. There has been little data on the application of PROMs to amyotrophic lateral sclerosis (ALS) care. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09774-8
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:The frontal assessment battery (FAB) test is a composite tool for assessing executive functions related to the frontal lobe. Neuropsychological and blood-flow studies indicate distinct patterns of deterioration of anterior and posterior cortical function in Alzheimer's disease (AD) and subcortical vascular d...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0251-7
更新日期:2006-11-01 00:00:00
abstract::A 23-year-old woman had progressive right hemiparesis followed by spontaneous clinical remission. The clinical profile and multifocal contrast-enhancing CT lesions in the periventricular region of the cerebrum favored the diagnosis of an acute demyelinating process. Serial examinations showed resolution of CT abnormal...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313759
更新日期:1982-01-01 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::Corticospinal lesions cause impairments in voluntary motor control. Recent findings suggest that some degree of voluntary control may be taken over by a compensatory pathway involving the reticulospinal tract. In humans, evidence for this notion mainly comes from StartReact studies. StartReact is the acceleration of r...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9027-0
更新日期:2018-11-01 00:00:00
abstract::We examined the morphology of spinal accessory motoneurons and immunoreactivity to neurotrophins, brain-derived neurotropic factor (BDNF) and neurotrophin (NT)-3, as well as the presence of reactive astrocytosis in 70 tiptoe walking Yoshimura (twy) mice that develop calcification at C1-C2 vertebral level compressing t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050287
更新日期:1998-12-01 00:00:00
abstract::This review gives an overview of various neuroimmunological diseases in terms of incidence and prevalence rates, age and sex distribution, and the frequency of subtypes, if applicable. The disorders selected for review are inflammatory muscle disorders (polymyositis, dermatomyositis and inclusion body myositis), myast...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-5001-3
更新日期:2006-09-01 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::Nystagmus is a rhythmical rotation of the eyeball. Its characteristics can be defined mathematically by the axis of rotation and the angular velocity around this axis. We analysed the axis of rotation for the nystagmus in benign paroxysmal positional vertigo (BPPV) to elucidate its pathophysiology. Thirteen patients w...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0905-z
更新日期:2002-12-01 00:00:00
abstract::Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7429-1
更新日期:2014-10-01 00:00:00
abstract::Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent sensory and motor neuropathy in individual nerves starting in adolescence or young adulthood, focal conduction abnormalities at entrapment sites on nerve conduction studies, and sausage-like swellings (tomacula) of the myelin...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-019-09319-8
更新日期:2020-08-01 00:00:00
abstract::An IgM antibody cytotoxic to normal human lymphocytes was found in 46.2% (male: 61.5%; female: 30.8%) of sera from chronic schizophrenics by means of a complement-dependent cytotoxicity test. The relationship between this antibody and the immunological manifestations of schizophrenia is discussed. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314296
更新日期:1987-06-01 00:00:00
abstract::The mechanism underlying the motor fluctuations that develop after long-term L-dopa therapy is not fully known. It has been speculated that malabsorption of L-dopa from the small intestine occurs. It was reported that gastric retention in Parkinson's disease (PD) patients with motor fluctuations is increased as compar...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5220-5
更新日期:2009-12-01 00:00:00
abstract:INTRODUCTION:The spinocerebellar ataxias (SCAs), are rare neurodegenerative disorders caused by distinct genetic mutations. Clinically, the SCAs are characterised by progressive ataxia and a variety of other features, including cognitive dysfunction. The latter is consistent with a growing body of evidence supporting a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0680-6
更新日期:2008-03-01 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::We report two adult Japanese sisters with L-2-hydroxy-glutaric aciduria (acidemia), both of whom were much older (aged 57, 47 years old) than previously reported patients (from neonate to 44 years old), and who presented with differing severity. Magnetic resonance imaging revealed typical subcortical white matter lesi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050367
更新日期:1999-05-01 00:00:00
abstract:OBJECTIVE:To determine the response to treatment and the long-term outcome of patients with the antisynthetase syndrome associated with anti-Jo-1-antibodies. PATIENTS AND METHODS:A total of 12 patients with histologically proven myositis and anti-Jo-1-autoantibodies were evaluated over a mean follow-up period of 66.4 ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0449-5
更新日期:2004-07-01 00:00:00
abstract::In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at dise...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09881-6
更新日期:2020-09-01 00:00:00
abstract::Functional changes in the organisation of neuronal circuitries are generally used to explain parkinsonian motor symptoms and levodopa-induced dyskinesias. Based on information from histology and neurophysiological microrecordings of specific basal ganglia nuclei, the most widely accepted scheme is based on a central l...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00007765
更新日期:2000-04-01 00:00:00
abstract::The purpose of this study was to evaluate the contribution of posterior circulation to memory function by comparing memory scores between patients with and without a foetal-type posterior cerebral artery (FTP) during the intracarotid amobarbital procedure (IAP) in epilepsy patients. Patients undergoing bilateral IAP b...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6391-4
更新日期:2012-08-01 00:00:00
abstract::Nerve conduction studies (NCS) and electromyography (EMG), often shortened to 'EMGs', are a useful adjunct to clinical examination of the peripheral nervous system and striated skeletal muscle. NCS provide an efficient and rapid method of quantifying nerve conduction velocity (CV) and the amplitude of both sensory ner...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6497-3
更新日期:2012-07-01 00:00:00
abstract::REM sleep behavior disorder (RBD) is known to be observed more frequently in patients with an α-synucleinopathy such as Parkinson's disease (PD) than in the general population. The precise prevalence of RBD in Japanese PD patients is not known. Therefore, we investigated the prevalence and the clinical characteristics...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-011-6386-1
更新日期:2012-08-01 00:00:00
abstract::Our knowledge of the vestibular sensory system, its functional significance for gaze and posture stabilization, and its capability to ensure accurate spatial orientation perception and spatial navigation has greatly benefitted from experimental approaches using a variety of vertebrate species. This review summarizes t...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-015-7909-y
更新日期:2016-04-01 00:00:00
abstract:OBJECTIVE:To investigate the efficacy of acupuncture on stroke recovery compared to an inert placebo. DESIGN:Placebo-controlled, randomised, clinical trial. SETTING:Post-stroke rehabilitation wards in five NHS hospitals in the UK. SUBJECTS:Patients between 4 and 10 days after their first stroke. INTERVENTIONS AND O...
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/s00415-008-0790-1
更新日期:2008-06-01 00:00:00
abstract::Cerebral ischemia of 15 min was produced in rats by increasing intracranial pressure above the systolic blood pressure level by infusing artificial cerebrospinal fluid into the cisterna magna. Pulmonary edema was prevented by blocking the vasomotor response with a ganglioplegic agent, and reducing the fluid load by pe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00316314
更新日期:1977-12-01 00:00:00