Abstract:
:Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after high-dose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Jaspert A,Claus D,Grehl H,Neundörfer Bdoi
10.1007/BF00873973subject
Has Abstractpub_date
1996-10-01 00:00:00pages
684-92issue
10eissn
0340-5354issn
1432-1459journal_volume
243pub_type
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::The long-duration response (LDR) to levodopa is an important component of the therapeutic response to the drug in Parkinson's disease (PD). Some characteristics are peculiar: it is independent from peripheral pharmacokinetics of levodopa, but it is dependent on the intervals between doses and on the size of each dose....
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-010-5731-0
更新日期:2010-11-01 00:00:00
abstract::Fatigue is a common and frequently disabling symptom of multiple sclerosis (MS). The aim of this study was to develop the Fatigue index Kliniken Schmieder (FKS) for detecting motor fatigue in patients with MS using kinematic gait analysis. The FKS relies on the chaos theoretical term "attractor", which, if unchanged, ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7415-7
更新日期:2014-09-01 00:00:00
abstract::Windmill nystagmus is characterized by a clock-like rotation of the beating direction of a jerk nystagmus suggesting separate horizontal and vertical oscillators, usually 90° out of phase. We report oculographic characteristics in three patients with variants of windmill nystagmus in whom the common denominator was pr...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8152-x
更新日期:2016-07-01 00:00:00
abstract:OBJECTIVE:To evaluate the role of MR morphometry in the characterization of cerebral microangiopathy (CMA) in relation to clinical and neuropsychological impairment. SUBJECTS AND METHODS:3D MR images of 27 patients and 27 age-matched controls were morphometrically analysed for regional thickness. The normalized values...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0671-9
更新日期:2005-04-01 00:00:00
abstract::Myasthenia gravis patients usually present with fluctuating, asymmetrical stress-dependent weakness in the absence of other neurological disturbances. In weak patients psychopathological disturbances are frequently reported and misdiagnosed as a psychiatric disorder. The question to what extent psychiatric symptoms ar...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-007-2022-5
更新日期:2007-05-01 00:00:00
abstract::To further evaluate (1) transcranial sonography (TCS) for (pre)clinical diagnosis of Parkinson's disease (PD) and (2) to examine asymptomatic carriers of Parkin mutations we investigated substantia nigra (SN) hyperechogenicity in PD patients and unaffected subjects with and without Parkin mutations. The area (aSN) of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-007-0567-y
更新日期:2007-10-01 00:00:00
abstract::The age-related structural changes of the human hippocampus are not entirely understood. The goal of the present investigation was to understand better the nature of age-related hippocampal changes by a comparative MR-analysis of four complementary aspects of hippocampal integrity: total volume, metabolite concentrati...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0540-y
更新日期:2004-11-01 00:00:00
abstract::Neuromyelitis optica (NMO) is an uncommon, demyelinating disease that causes long-term disability in adults. Though much has recently been learned about its pathogenesis, there are still only a few studies regarding the epidemiology of NMO. The aim of the study was to describe the epidemiology of NMO among adults in t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6926-y
更新日期:2013-08-01 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::Internuclear ophthalmoplegia (INO) indicates a lesion involving the medial longitudinal fasciculus (MLF) that interconnects the abducens nucleus and medial rectus subnucleus of the oculomotor nuclear complex. In fact, rostral-caudal localization value of the INO is often limited except when it accompanies symptoms and...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8088-1
更新日期:2016-05-01 00:00:00
abstract::Uric acid (UA) is reduced in multiple sclerosis (MS), and possibly relates to MS outcomes, with lower UA levels in subjects experiencing a relapse or presenting higher disability scores. The present retrospective longitudinal study evaluated UA variations in MS, in relation to clinical relapses, disability progression...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7666-y
更新日期:2015-01-01 00:00:00
abstract::Bilateral paramedian thalamic infarctions are usually associated with impaired consciousness, oculomotor disturbances and neuropsychological changes. A 44-year-old healthy woman presented with amaurosis fugax of the right eye immediately after a Valsalva maneuver. Neurological examination, in particular visual acuity,...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-009-5030-9
更新日期:2009-06-01 00:00:00
abstract::Auto-oxidation of levodopa generates toxic metabolites, such as free radicals, semiquinones and quinones. In vitro, levodopa is a powerful toxin that is lethal to cultures of neurones. This raises the concern that levodopa may also be toxic in vivo, and that chronic treatment with levodopa could induce further damage ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00022914
更新日期:2000-04-01 00:00:00
abstract::Rodent oligodendrocytes have a unique susceptibility among glia to the lytic effects of complement, due in part to a deficiency in CD59 (protectin), a key surface inhibitor of the complement membrane attack complex (MAC). The possibility that shedding of CD59 by human oligodendrocytes contributes to complement-mediate...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00873519
更新日期:1994-08-01 00:00:00
abstract::Movement-associated cortical potentials (MP) associated with thumb-opposition or fist-clenching were studied in 20 patients with unilateral cerebral lesions and in 8 healthy subjects. MP was abnormal in 16 patients. The most frequent abnormality was an attenuation of the N component or readiness potential, recorded on...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312541
更新日期:1975-07-02 00:00:00
abstract:BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that still lacks reliable diagnostic biomarkers. This study aims to evaluate the diagnostic and prognostic potential of CSF total Tau (t-Tau), phospho-Tau (p-Tau) and p-Tau/t-Tau ratio in ALS patients using CSF neurofilament light (NFL)...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9008-3
更新日期:2018-10-01 00:00:00
abstract::Two sexually active female patients presented with acute meningitis. The CSF abnormalities were severe and persistent. In spite of the absence of genital lesions, serological studies revealed a primary infection by herpes simplex virus type 2. An immunoblot study revealed intrathecal synthesis of anti-herpes antibodie...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314743
更新日期:1990-08-01 00:00:00
abstract::The SOX10 transcription factor is involved in development of neural crest derivatives and fate determination in glial cells. SOX10 mutations have been found in patients with intestinal aganglionosis and depigmentation with deafness (Waardenburg-Hirschsprung). Associated neurological signs have been reported in some ca...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150170159
更新日期:2001-06-01 00:00:00
abstract::To investigate the role of endogenous opioid peptides in the pathophysiology of cerebral ischaemia, the CSF levels of immunoreactive beta-endorphin and leu-enkephalin in 16 patients with cerebral infarction were measured. Both the CSF beta-endorphin and leu-enkephalin levels in the acute stage of cerebral infarction w...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314282
更新日期:1987-06-01 00:00:00
abstract::We have investigated how the abnormal head posture and motility in spasmodic torticollis interferes with ecological movements such as combined eye-to-foot whole-body reorientations to visual targets. Eight mildly affected patients and 10 controls voluntarily rotated eyes and body in response to illuminated targets of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6937-8
更新日期:2013-08-01 00:00:00
abstract::Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-012-6790-1
更新日期:2013-05-01 00:00:00
abstract::This meta-analysis aims to compare percutaneous transluminal angioplasty (PTA) to medical treatment (MT) for symptomatic vertebral artery stenosis (SVAS) treatment. We searched PubMed, Springer, Google Scholar, Clinical Trials, Cochrane Central, Chinese National Knowledge Infrastructure, and China Biological Medicine ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-016-8267-0
更新日期:2017-05-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150200069
更新日期:2002-05-01 00:00:00
abstract::Parkinson's disease (PD) is preceded by a premotor phase of unknown duration. Dopaminergic degeneration during this phase may lead to subtle cognitive and behavioural changes, such as decreased novelty seeking. Consequently, premotor subjects might be most comfortable in jobs that do not require optimal dopamine level...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7828-y
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVE:To characterize the clinical symptoms and magnetic resonance imaging (MRI) findings of unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES). METHODS:This is a case report and systematic review of the literature to identify cases of unilateral cortical FLAM...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09440-8
更新日期:2019-10-01 00:00:00
abstract::The concentrations of glucose, pyruvate and lactate were determined in the blood and lumbar CSF of 63 patients with neuromuscular disorders and 40 controls. The glucose/lactate and lactate/pyruvate ratios were also calculated, in addition to the cytoplasmic NADH/NAD+ (nicotinamide-adenine dinucleotide-H/nicotinamide-a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313438
更新日期:1981-01-01 00:00:00
abstract:AIM:To assess: (1) the prevalence of convexity subarachnoid hemorrhage (cSAH) in infective endocarditis (IE); (2) its relationship with IE features; (3) the associated lesions; (4) whether cSAH is a predictor of future hemorrhage; (5) whether cSAH could cause cortical superficial siderosis (cSS). METHODS:We retrospect...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09953-7
更新日期:2020-10-01 00:00:00
abstract::The pathophysiology of the striatum and cerebral cortex were studied from the pharmacological aspect. Investigation of the dopamine content in the cerebral cortex revealed that the premotor and motor area showed the highest level (61+/-6.2 ng/g). Intravenous injection of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (M...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/pl00007779
更新日期:2000-09-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:The number of neurological second opinions (SO) and tertiary referrals (TR) is increasing. The main purpose of this study was to assess whether a day-care admission made a meaningful contribution to standard neurological outpatient care, for a wide range of second opinions and tertiary referral...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0019-3
更新日期:2008-11-01 00:00:00