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-adenine dinucleotide) ratio in the brain. Remarkable deviations from control values were found not only in blood but also in the CSF of patients with different neuromuscular diseases, particularly in Duchenne muscular dystrophy and peroneal muscular atrophy, indicative of disturbance of the carbohydrate metabolism in the central nervous system.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Mechler F,Diószeghy P,Csenkér E,Molnár Ldoi
10.1007/BF00313438subject
Has Abstractpub_date
1981-01-01 00:00:00pages
111-8issue
2eissn
0340-5354issn
1432-1459journal_volume
226pub_type
杂志文章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
abstract::Isolated floccular infarction is extremely rare, and impairments of the vestibulo-ocular reflex (VOR) have not been explored in humans with isolated floccular lesions. The purpose of this study was to examine and report selective impairment of VOR in response to high acceleration using head impulse in a patient with i...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6837-y
更新日期:2013-06-01 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::Laser Doppler is widely used to evaluate sympathetic vasoconstrictor function. Continuous wave (cw)-Doppler of the radial artery may be an alternative but less expensive approach to quantify sympathetically induced resistance changes in the peripheral vascular system. In order to compare the power of both methods, thi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0471-7
更新日期:2004-08-01 00:00:00
abstract::Surface antigens on peripheral blood lymphocytes from myasthenia gravis patients were investigated. The expression of DR+ and CD8+/DR+ T lymphocytes was increased and the expression of CD4+ T cells reduced. Neither thymectomy, clinical condition nor anti-acetylcholine receptor antibody titre correlated with any of the...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00863771
更新日期:1994-02-01 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::In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fl...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-014-7283-1
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Cognitive impairment is a debilitating symptom experienced by ~ 50% of multiple sclerosis patients, with processing speed (PS) and memory most affected. Until now, the field has considered cognition in a binary fashion: patients are designated as impaired or not impaired. This designation is typically arrive...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8747-5
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Sleep disorders can occur in early Parkinson's disease (PD). However, the relationship between different sleep disturbances and their longitudinal evolution has not been fully explored. OBJECTIVE:To describe the frequency, coexistence, and longitudinal change in excessive daytime sleepiness (EDS), insomnia,...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10140-x
更新日期:2021-01-01 00:00:00
abstract:OBJECTIVES:Lumbar spondylosis (LS) is a common spinal degenerative disorder which causes various types of lower urinary tract dysfunction (LUTD). However, it is not certain whether LS may cause urinary retention in elderly women. METHODS:In a period covering the past 3 years, we retrospectively reviewed: a) urodynamic...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0790-3
更新日期:2005-08-01 00:00:00
abstract::Spinocerebellar ataxia (SCA) type 7 is an autosomal dominant disorder characterized by neural loss, mainly in the cerebellum and regions of the brainstem and particularly the inferior olivary complex. This neurodegeneration disease is associated with expansion of unstable CAG repeats within the 5'-translated region of...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150070131
更新日期:2000-08-01 00:00:00
abstract::Dementia is reversible in a minority of patients, and these should be diagnosed but without subjecting the majority with irreversible disease to an excessive set of investigations. Should a battery of ancillary investigations be performed routinely in dementia? Or can these tests be carried out as clinically indicated...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050311
更新日期:1999-02-01 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::Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBSL) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a mitochondrial aspartyl-tRNA synthetase, DARS2. The disease is characterized by progressive spastic ataxia and magnetic resonance imaging (MRI) sh...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6176-9
更新日期:2012-02-01 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::The finding of a mutation in the alpha-synuclein gene in a rare autosomal dominant form of idiopathic Parkinson's disease (IPD), has prompted increased interest in identifying genes that account for the more common sporadic form. A number of association studies have suggested that functional polymorphisms in genes tha...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050258
更新日期:1998-10-01 00:00:00
abstract::The history of our understanding of the pathogenesis and pathophysiology of multiple sclerosis are reviewed in the context of Charcot's contribution. The implications for treatment of the new knowledge gained from studies during life of pathology and pathogenesis (by MRI) and pathophysiology (by evoked potentials) are...
journal_title:Journal of neurology
pub_type: 传,历史文章,杂志文章
doi:10.1007/BF00838443
更新日期:1993-01-01 00:00:00
abstract::We wanted to determine the neurocognitive profile of adult patients with moyamoya disease prior to neurosurgical intervention. The experience of three United States medical centers, Columbia University, University of Illinois at Chicago, and the University of Texas Southwestern Medical Center at Dallas, were combined....
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-009-5424-8
更新日期:2010-05-01 00:00:00
abstract::Migraine is a common debilitating neurological disease characterised by attacks of severe headache with or without preceding aura. Its aetiology remains elusive; however it is clear that an interplay of genetic and environmental components play an important role. Familial hemiplegic migraine (FHM) is a rare and severe...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-007-0641-5
更新日期:2007-12-01 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::Deep brain stimulation of the subthalamic nucleus (STN) is an effective treatment modality for motor symptoms of advanced Parkinson's disease. Recent studies focus on exploring its possible effects on cognition and behavior. We present a case with acute cognitive dysfunction due to misalignment of the electrodes to th...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-009-5103-9
更新日期:2009-08-01 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::The geste antagoniste is a voluntary maneuver that temporarily reduces the severity of dystonic posture or movements. It is a classical feature of focal and particularly cervical dystonia. However, the precise historical aspects of geste antagoniste still remain obscure. The goals of this review were (1) to clarify th...
journal_title:Journal of neurology
pub_type: 历史文章,杂志文章
doi:10.1007/s00415-011-6380-7
更新日期:2012-08-01 00:00:00
abstract:BACKGROUND:Dermic fibroblasts have been proposed as a potential genetic-ALS cellular model. This study aimed to explore whether dermic fibroblasts from patients with sporadic-ALS (sALS) recapitulate alterations typical of ALS motor neurons and exhibit abnormal DNA-damage response. METHODS:Dermic fibroblasts were obtai...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09704-8
更新日期:2020-05-01 00:00:00
abstract::Even though trigeminovestibular connections are well established in animals, mastication-induced dizziness has been described only as a vascular steal phenomenon in humans. We determined induction or modulation of nystagmus in two index patients with mastication-induced vertigo, 12 normal controls, and 52 additional p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7221-7
更新日期:2014-03-01 00:00:00
abstract::Parkinson's disease (PD) is associated with an increased risk of fragility fracture. FRAX and Qfracture are risk calculators that estimate the 10-year risk of hip and major fractures and guide definitive investigation for osteoporosis using dual X-ray absorptiometry (DEXA) imaging. It is unclear which PD patients shou...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7333-8
更新日期:2014-06-01 00:00:00
abstract:BACKGROUND:One of the goals in treating subarachnoid hemorrhage patients is to prevent or minimize vasospasm-induced ischemia. Intracerebral microdialysis is a rapidly developing tool to monitor physiological and pathophysiological changes in chemical processes associated with ischemia. OBJECTIVE:To determine the diag...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-003-1079-z
更新日期:2003-07-01 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::Unilateral mesial temporal lobe epilepsy (MTLE) has been associated with reduced amygdala responsiveness to fearful faces. However, the effect of unilateral MTLE on empathy-related brain responses in extra-amygdalar regions has not been investigated. Using functional magnetic resonance imaging, we measured empathy-rel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7622-2
更新日期:2015-03-01 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