Abstract:
:In contrast to the distal sensory response, the somatosensory evoked response (SSER) is detectable in patients with A-alpha sensory fiber diseases such as Friedreich's ataxia. In four patients with this disease a combined histological and electro-physiological investigation of sensory propagation was performed. The sural nerve was analyzed in terms of the various fiber diameters and the distal sensory and somatosensory evoked cortical responses recorded after sural and median nerve (finger) stimulation. A distinct evoked response could be detected with rather low amplitudes and retarded latency times, while in general no distal sensory response was found. The anatomical physiological consequences are discussed.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Colon E,Joosten E,de Weerd Jdoi
10.1007/BF00312980subject
Has Abstractpub_date
1978-12-22 00:00:00pages
273-8issue
4eissn
0340-5354issn
1432-1459journal_volume
219pub_type
杂志文章abstract::In Parkinson's disease (PD), nonmotor symptoms manifest before motor symptoms. In this report, we present a remarkable case of a semiprofessional painter with PD whose painting style dramatically changed from abstract painting to realism before he developed motor, psychiatric, and autonomic nerve disorders. This case ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6271-y
更新日期:2012-05-01 00:00:00
abstract::Spinocerebellar ataxias (SCA) are a genetically heterogeneous group of neurodegenerative diseases characterised by progressive cerebellar ataxia, dysarthria and oculomotor abnormalities. Recently the prodynorphin (PDYN) gene was identified as the cause of SCA23 in four Dutch families displaying progressive gait and li...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6721-1
更新日期:2013-03-01 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:BACKGROUND:Body weight reduction after disease onset is an independent predictor of survival in amyotrophic lateral sclerosis (ALS), but significance of weight variation after diagnosis remains to be established. OBJECTIVE:To investigate weight variation after diagnosis and its prognostic significance in patients with...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09276-2
更新日期:2019-06-01 00:00:00
abstract::We examined 199 consecutive patients who underwent 220 liver transplantations, to define the type, frequency and aetiology of posttransplant neurological complications and their prognostic value. We found neurological complications in 63 patients (32%), mostly involving the central nervous system. The most frequent co...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF02444004
更新日期:1996-02-01 00:00:00
abstract::A man, aged 46, who had been taking Clioquinol in high doses for a long period, developed a characteristic neurological syndrome of subacute myelo-optic neuropathy rather abruptly. Electron microscopical examination of the muscle biopsy, obtained five months after the onset of the disease, revealed severe degenerative...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:
更新日期:1977-05-13 00:00:00
abstract::Acid maltase deficiency is described in non-identical adult twins. The onset of the disease can be traced into late infancy; the clinical picture is one of severe muscular dystrophy; respiratory insuficiency was the cause of death in one case. The autopsy showed the central nervous system, heart and liver to be spared...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313272
更新日期:1976-08-06 00:00:00
abstract::Fatigue is a frequent and often severe symptom in multiple sclerosis. Pathogenic mechanisms proposed for fatigue include the release of proinflammatory cytokines, which is thought to have an important effect on changes in the blood-brain barrier (BBB). To investigate whether fatigue is related to BBB disruption we stu...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s004150050382
更新日期:1999-06-01 00:00:00
abstract::Vanishing white matter disease (VWM) is one of the most prevalent inherited leukoencephalopathies in childhood. Infantile VWM is more severe but less understood than the classic early childhood type. We performed a follow-up study on 14 infantile and 26 childhood patients to delineate the natural history and neuroimag...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8851-6
更新日期:2018-06-01 00:00:00
abstract::Cobrotoxin-binding protein was isolated by affinity chromatography from human thymoma which had been surgically removed from patients with myasthenia gravis. The protein was composed of polypeptides with a molecular mass of 40, 51, 65, and 74 kilodaltons as determined by polyacrylamide gel electrophoresis in the prese...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00618251
更新日期:1987-05-01 00:00:00
abstract::Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an acute neurological disorder affecting children and adults. We aimed to compare the clinical characteristics, treatments, and outcomes between children and adults with anti-NMDAR encephalitis and to assess the probable risk factors. In this observational stu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8282-1
更新日期:2016-12-01 00:00:00
abstract::Recent studies suggest that the risk of cerebral infarction is increased during the puerperium but not during pregnancy itself. Most of the known causes of ischaemic stroke in the young have been reported during pregnancy. In most of these conditions, it is uncertain whether pregnancy is coincidental or plays a role i...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050224
更新日期:1998-06-01 00:00:00
abstract::18FDG-positron emission tomography performed at different stages in the course of subacute sclerosing panencephalitis revealed a changing pattern of metabolic disturbance. In clinical stage II patients the inflammation in the basal ganglia appeared to lead to neuronal excitation accompanied by hypermetabolism. Widespr...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00833917
更新日期:1992-03-01 00:00:00
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 ear...
journal_title:Journal of neurology
pub_type: 历史文章,杂志文章
doi:10.1007/s00415-011-6355-8
更新日期:2012-07-01 00:00:00
abstract:BACKGROUND:Putaminal iron deposition is a histopathological feature of multiple system atrophy (MSA), which is not observed in patients with idiopathic Parkinson's disease (PD). T2*-weighted magnetic resonance imaging (MRI) gradient echo (GE) sequences are sensitive for paramagnetic susceptibility changes and therefore...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0496-1
更新日期:2007-09-01 00:00:00
abstract::Dimethyl fumarate (DMF), fingolimod (FTY) and teriflunomide (TFN) are oral disease-modifying therapies (DMTs) approved for relapsing-remitting multiple sclerosis (RRMS) whose efficacy and tolerability have been separately assessed in phase III trials. Conversely, little evidence exists about their head-to-head compari...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8595-8
更新日期:2017-11-01 00:00:00
abstract::The pharmacokinetics of free and total valproic acid (VPA) in plasma and whole blood after oral administration during steady state was investigated in seven infants (mean age 10.7 months) receiving monotherapy. The VPA concentrations in whole blood closely followed those in plasma but at a reduced level. A positive co...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00315328
更新日期:1991-09-01 00:00:00
abstract::The seasonal occurrence of relapses was analysed retrospectively in a group of 53 patients with treated dermatomyositis (DM) or polymyositis (PM). In DM, the incidence of both myositic and cutaneous relapses was highest in summer whereas in the PM group relapses was more evenly distributed throughout the seasons but l...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150200036
更新日期:2002-04-01 00:00:00
abstract::The relationship between atherosclerosis and cognitive function is less well studied in Chinese populations. In addition, the results among middle-aged adults have been mixed. We aimed to investigate the association of atherosclerosis measured by carotid intima-media thickness (CIMT) and cognitive function in middle-a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8234-9
更新日期:2016-10-01 00:00:00
abstract::Two right-handed patients who exhibited language disability after left frontal infarction are described. The patients spoke fluently and exhibited excellent repetition ability from the onset of infarction without exhibiting any oral apraxia, but had deficits in auditory comprehension, naming, reading and writing. In b...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050180
更新日期:1998-02-01 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::To analyze the in vivo biological effect of anti-interferon beta (IFN-beta) neutralizing antibodies (NABs), blood concentrations of neopterin, beta2microglobulin (Beta2-MG), mRNA-dependent myxovirusresistant protein A (MxA) and dsRNA-dependent protein kinase (PKR) were measured before (predose) and 24 hours after (pos...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-006-0332-7
更新日期:2007-05-01 00:00:00
abstract::A man, aged 63, had an illness which lasted 11 months from onset with pain under the left costal margin which radiated to the epigastrium, until his death from cardiac failure. His symptoms consisted principally of parasthesias and proximal weakness of both upper and lower extremities with atrophy of the shoulder and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314720
更新日期:1978-04-14 00:00:00
abstract:OBJECTIVE:To use arterial spin labelling to investigate differences in perfusion in mild cognitive impairment with Lewy bodies (MCI-LB) compared to Alzheimer type MCI (MCI-AD) and healthy controls. METHODS:We obtained perfusion images on 32 MCI-LB, 30 MCI-AD and 28 healthy subjects of similar age. Perfusion relative t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10271-1
更新日期:2020-10-21 00:00:00
abstract:BACKGROUND AND PURPOSE:Considerable studies have reported inconsistent relationships between ischemic stroke and a large number of factors. These uncertainties may reflect the susceptibility to confounding in observational studies. We aimed to assess genetic correlations and causal relationships between ischemic stroke...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09786-4
更新日期:2020-07-01 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::We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00936890
更新日期:1995-02-01 00:00:00
abstract:UNLABELLED:The objective of this study was to investigate the relationship between electrophysiological recordings and clinical as well as radiological findings in patients suggestive to suffer from a lumbar spinal stenosis (LSS). We hypothesise that the electrophysiological recordings, especially SSEP, indicate a lumb...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0427-1
更新日期:2007-06-01 00:00:00
abstract::Degeneration of central dopaminergic neurons is the most characteristic pathological feature of Parkinson's disease. It remains to be established to what extent these lesions explain the motor, cognitive and affective disorders observed in patients, but there are now some interesting clues to a possible solution. ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00819560
更新日期:1992-01-01 00:00:00
abstract::Acute relapses of multiple sclerosis (MS) are treated with intravenous methylprednisolone (IVMP), which speeds recovery from exacerbation. It is known that IVMP suppresses the immunological activation which occurs during an acute attack of MS. However, the specific target genes affected by this therapy remain obscure....
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0516-y
更新日期:2004-10-01 00:00:00