Abstract:
:We investigated the muscle strength and motor unit (MU) territory of five patients with postpolio syndrome (PPS), six stable patients with prior poliomyelitis, and five healthy volunteers. The MU territory was assessed by measuring amplitudes of motor unit potentials (MUPs) recorded by the macro EMG technique. The investigations were repeated after 11-20 months (mean 15.6). The macro MUP amplitudes in both patient groups were markedly increased (P = 0.02). However, no statistical difference was found between the two groups in the initial amplitude values. Macro MUP amplitudes obtained on repeated examinations did not differ significantly from the initial macro MUP amplitudes in any of the three groups. In three individual PPS patients, a decline in muscle strength on the follow-up study was documented, providing the diagnosis of post-poliomyelitis muscular atrophy (PPMA). The three PPMA patients had the highest initial macro MUPs. Two of them showed a decrease in macro MUP amplitudes on follow-up. These findings suggest that a later breakdown of oversized MUs may play a role in the pathogenesis of PPMA.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ivanyi B,Ongerboer de Visser BW,Nelemans PJ,de Visser Mdoi
10.1007/BF00920572subject
Has Abstractpub_date
1994-12-01 00:00:00pages
37-40issue
1eissn
0340-5354issn
1432-1459journal_volume
242pub_type
杂志文章abstract::Lipids were extracted from brains of a patient with membranous lipodystrophy (ML) and three normal patients and the neutral lipid and sphingolipid constituents were investigated. The storage of a large amount of free fatty acid was observed in the ML brain, but no cholesterol ester was found. Total lipid, cholesterol ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313947
更新日期:1979-03-22 00:00:00
abstract::In this work, we investigated motor network structure in patients affected by essential tremor (ET) with or without resting tremor, using probabilistic tractography of the cerebello-thalamo-basal ganglia-cortical loop. Twenty-five patients with ET, twenty-two patients with ET associated with resting tremor (rET), and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8553-5
更新日期:2017-09-01 00:00:00
abstract::We studied the diagnostic value of computed tomographic angiography (CTA) in the posterior circulation as a noninvasive substitute for intra-arterial digital subtraction angiography (DSA). We prospectively investigated 103 patients with acute stroke in the posterior circulation. All patients underwent CTA and Doppler ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150070089
更新日期:2000-10-01 00:00:00
abstract:CONTEXT:Polyneuropathy is a common disease and is more prevalent (at least 3 %) in elderly people. However, routine neurological examination of healthy elderly people may show distal sensory loss and absent tendon reflexes, which can obscure the distinction from polyneuropathy. OBJECTIVE:To investigate the relation be...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-005-0064-0
更新日期:2006-05-01 00:00:00
abstract::Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 receptor binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emission tomography and 18F-deoxyglucose and 18F-methylspiperone ([18F]MSP), respectively. There was a severe re...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00920620
更新日期:1994-10-01 00:00:00
abstract:OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ES...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09508-5
更新日期:2019-12-01 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::The association of pathology and neurological deficit with quality of life (QoL) in multiple sclerosis (MS) is not fully understood. In this study, magnetic resonance imaging (MRI) measures of pathology--T1 and T2 lesion volume and ratio; active T2 lesion number; global and regional brain volume and atrophy; magnetiza...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6151-5
更新日期:2012-02-01 00:00:00
abstract::Among the hereditary cerebellar ataxias (CAs), there are at least 36 different forms of autosomal dominant cerebellar ataxia (ADCAs), 20 autosomal recessive cerebellar ataxias (ARCAs), two X-linked ataxias, and several forms of ataxia associated with mitochondrial defects. Despite the steady increase in the number of ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-014-7387-7
更新日期:2014-09-01 00:00:00
abstract::Secondary worsening of stroke symptoms is described in 13-37% of stroke patients, but the exact mechanism of this phenomenon remains unclear. The aim of this study is to find prognostic variables in correlation to secondary worsening of stroke symptoms in patients with lacunar or cortical strokes. In this prospective ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5577-5
更新日期:2010-09-01 00:00:00
abstract::Little is known about the pattern of subacute cognitive domain impairments after ischaemic stroke, nor the temporal evolution of such impairments. Our objective was to investigate the pattern of cognitive impairment in different neuropsychological domains up to a year after ischaemic stroke. We included prospectively ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6625-0
更新日期:2013-01-01 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:BACKGROUND AND AIMS:Most patients with multiple sclerosis presenting with a relapsing-remitting disease course at diagnosis transition to secondary progressive multiple sclerosis (SPMS) 1-2 decades after onset. SPMS is characterized by predominant neurodegeneration and atrophy. These pathogenic hallmarks result in unsa...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-09964-4
更新日期:2020-06-05 00:00:00
abstract:BACKGROUND:Hyposmia can develop with age and in neurodegenerative conditions, including Parkinson's disease (PD). The University of Pennsylvania Smell Identification Test (UPSIT) is a 40-item smell test widely used for assessing hyposmia. However, in a number of situations, such as identifying hyposmic individuals in l...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09340-x
更新日期:2019-08-01 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::There is still uncertainty regarding risk factors for cancer occurrence in patients with myasthenia gravis (MG). The objective of this study is to determine the prevalence of extrathymic neoplasms in patients with MG and the factors associated with tumor occurrence. The archives of four tertiary MG centers were consul...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5091-9
更新日期:2009-08-01 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::Augmented spinal nociception during the "off" phase has been observed early in Parkinson's disease further increasing with disease duration. To find out whether increased spinal nociception represents a premotor feature, experimental pain sensitivity was assessed in idiopathic REM-sleep behavior disorder (IRBD) patien...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8390-y
更新日期:2017-03-01 00:00:00
abstract::The type and frequency of neurological manifestations of Behçet's disease (BD) vary with ethnicity. We analyzed the neurological manifestations of BD in Japanese patients. All patients undergoing treatment at one of the two Yokohama City University hospitals from July 1991 to December 2007 and who fulfilled the Japane...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-010-5454-2
更新日期:2010-06-01 00:00:00
abstract::Forty-four patients (mean age 66, SD 8 years) with either clinical evidence of a focal lacunar syndrome (n = 36) or with disorders of memory or gait (n = 8) in the presence of a lacunar infarct on CT were studied for cognitive functioning and for the presence of white matter lesions on MRI. MR images were assessed by ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF02444014
更新日期:1996-02-01 00:00:00
abstract::We assessed the prevalence of vertebral artery (VA) stenosis or occlusion and its influence on outcome in patients with acute basilar artery occlusion (BAO). We studied 141 patients with acute BAO enrolled in the Basilar Artery International Cooperation Study (BASICS) registry of whom baseline CT angiography (CTA) of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7583-5
更新日期:2015-02-01 00:00:00
abstract::The diagnosis and management of lesions of the brachial roots and of the brachial plexus is improved by appropriate investigation, both in acute and chronic disorders. The choice of investigation should be determined by the clinical problem. Since they are relatively non-invasive, electrophysiological investigations a...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00314416
更新日期:1986-06-01 00:00:00
abstract:OBJECTIVE:To characterize the relation between brain functional connectivity and disability in patients with multiple sclerosis; to investigate the existence of critical values of both disability and functional connectivity corresponding to exhaustion of functional adaptive mechanisms. METHODS:Hundred-and-nineteen pat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9075-5
更新日期:2018-12-01 00:00:00
abstract::Cerebral venous thrombosis (CVT) affects mainly working-aged individuals. Functional recovery after CVT is generally considered good with about 3/4 of patients achieving short-term independence. However, vascular events, long-term functional outcome, and employment after CVT remain poorly investigated. We identified c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7996-9
更新日期:2016-03-01 00:00:00
abstract::Little is known about the relationship between cognitive dysfunctions and the non-motor complex in subjects with newly diagnosed untreated Parkinson's disease (PD). The aim of this study was to explore the association between non-motor symptoms (NMS) and cognitive dysfunctions in an incident cohort of de novo, drug-na...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6407-0
更新日期:2012-09-01 00:00:00
abstract::We describe three patients in whom an isolated sixth nerve palsy was the only clinical symptom or sign of multiple sclerosis (MS). Data were collected prospectively over 6 years on these three patients, who showed no other signs of brainstem dysfunction or prior symptoms; in addition. Retrospective analysis of all pat...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150070114
更新日期:2000-09-01 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::Postpartum lower limb motor and/or sensory deficit is an uncommon obstetrical complication. We aimed to identify its incidence, etiology, and precipitating factors, as well as the neurological prognosis by retrospectively analyzing the successive neurological evaluations, electrophysiological, and MRI data from all th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8388-5
更新日期:2017-03-01 00:00:00
abstract:OBJECTIVE:As metabolic and structural changes in frontotemporal-subcortical pathways have been reported in patients with obsessive-compulsive disorders, we investigated the correlation between complex compulsive behaviour (CCB) and the distribution of atrophy in a group of 90 patients with frontotemporal dementia (FTD)...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150170049
更新日期:2001-11-01 00:00:00
abstract::We aimed to assess the intrasubject reproducibility of a technology-based levodopa (LD) therapeutic monitoring protocol administered in supervised versus unsupervised conditions in patients with Parkinson's disease (PD). The study design was pilot, intrasubject, single center, open and prospective. Twenty patients wer...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-018-8848-1
更新日期:2018-06-01 00:00:00