Abstract:
:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the effect of drugs is still small, this progress cannot only be seen as a success of the present but must also be acknowledged as a basis for future developments. How will future studies be designed? They will have to take into account that the disease presumably has a long preclinical period and they will use a number of novel compounds and treatment strategies which have been shown to be effective in transgenic animal models. This also implies that we are likely to use a combination of therapies and we will try to treat patients early. The latter will be associated with the demand for a novel clinical attitude toward the diagnosis of the disease and the development of novel markers for both the preclinical period and the longitudinal course of the disease.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ludolph ACdoi
10.1007/BF03161074keywords:
subject
Has Abstractpub_date
2000-12-01 00:00:00pages
13-8eissn
0340-5354issn
1432-1459journal_volume
247pub_type
杂志文章,评审abstract:BACKGROUND AND AIMS:Due to common pathophysiological findings of Alzheimer's disease (AD) with diabetes mellitus (DM), insulin has been suggested as a possible treatment of AD or mild cognitive impairment (MCI). A safe alternative of IV insulin is intranasal (IN) insulin. The aim of this systematic review is to investi...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-018-8768-0
更新日期:2018-07-01 00:00:00
abstract::Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients r...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-017-8519-7
更新日期:2017-06-01 00:00:00
abstract::A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00839213
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:Stroke has a major impact on survivors. Our study was designed to describe the mental status and health-related quality of life (HRQoL) in long-term survivors of TIA or minor ischaemic stroke (MIS) and evaluate associations of mental and physical factors with HR-QoL. METHODS:A random sample of the 10-year s...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0480-9
更新日期:2007-08-01 00:00:00
abstract:BACKGROUND:Late-onset Pompe disease (LOPD) is a rare, metabolic disease primarily affecting the musculoskeletal and respiratory systems. Forced vital capacity (FVC) is commonly used to measure pulmonary function; however, associations between FVC and other LOPD outcomes remain unclear. METHODS:A systematic literature ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-019-09401-1
更新日期:2019-09-01 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::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::The short-term benefits of levodopa/carbidopa intestinal gel (LCIG) in patients with advanced Parkinson's disease (PD) are well documented, but the long-term benefits are still uncertain. The aim of this study was to investigate the motor and cognitive outcome of LCIG treatment in advanced PD after a follow-up period ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7235-1
更新日期:2014-03-01 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::Inborn errors of metabolism in 40 children have been investigated by computed tomography to obtain data on the degree of cerebral involvement in neurodegenerative and storage disorders: 20 children had various mucopolysaccharidoses, 8 sphingolipidoses , 3 mucolipidoses, 2 oligosaccharidoses , 3 ceroidlipofuscinoses an...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313718
更新日期:1984-01-01 00:00:00
abstract:OBJECTIVE:A growing number of studies have been conducted on the relationship between anger and hostility and the risk of stroke, and their conclusions are not consistent. Accordingly, we performed a meta-analysis to evaluate the relationship between anger and hostility and the risk of stroke. METHODS:We searched the ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-019-09231-1
更新日期:2019-04-01 00:00:00
abstract::Multiple sclerosis (MS), a chronic inflammatory disorder of the central nervous system (CNS), 2 results in damage to axons and their surrounding myelin sheath. The exact cause of inflammation remains unclear, but an autoimmune response directed against CNS antigens is suspected. MS can affect the brain, optic nerve an...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-004-1504-y
更新日期:2004-09-01 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::Brain metastases occur in 20-40% of patients with cancer and their frequency has increased over time. Lung, breast and skin (melanoma) are the commonest sources of brain metastases, and in up to 15% of patients the primary site remains unknown. After the introduction of MRI, multiple lesions have outnumbered single le...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-002-0870-6
更新日期:2002-10-01 00:00:00
abstract:OBJECTIVE:There exist controversial and discrepant results on the risk of spontaneous abortions and teratogenesis induced by interferon treatment in people with MS.Aim of this study is to evaluate risks of the administration of INFbeta related not only to the foetus, but also to children development up to 12-months dev...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0909-4
更新日期:2008-08-01 00:00:00
abstract::Recent years have seen substantial news and updates in the genetics and diagnosis of schwannomas, even a new hereditary disease with schwannomas; Schwannomatosis has been defined. These developments have consequently led to better evaluation of the incidence of schwannomas. Although there has also been progress in the...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-0347-0
更新日期:2006-12-01 00:00:00
abstract::Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitocho...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-009-5409-7
更新日期:2010-05-01 00:00:00
abstract::Patients with multiple system atrophy (MSA) often have evidence of compromised gastrointestinal motility. Ghrelin is a gut hormone that influences gastrointestinal motility in humans. The aim of this study was to determine whether ghrelin secretion is affected in MSA patients, and to investigate the relation between g...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6944-9
更新日期:2013-08-01 00:00:00
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 stim...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00873973
更新日期:1996-10-01 00:00:00
abstract::Muscle samples obtained in patients with different neuromuscular diseases were stored at 0 degree C for periods of 6-12h and were subsequently frozen for histochemical processing. Compared to reference samples which had been immediately frozen, the individual test sections displayed different degrees of swelling of th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313934
更新日期:1984-01-01 00:00:00
abstract::Three patients developed acute Guillain-Barré syndrome while on steroid treatment. The first patient suffered from ulcerative colitis and developed Guillain-Barré syndrome when the steroid dosage was being tapered. Another patient with long-standing multiple sclerosis received steroids during relapse and the Guillain-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314023
更新日期:1986-08-01 00:00:00
abstract::A major challenge in the diagnosis of disorders of consciousness is the differential diagnosis between the vegetative state (VS) and the minimally conscious state (MCS). Clinically, VS is defined by complete unawareness, whereas MCS is defined by the presence of inconsistent but clearly discernible behavioural signs o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6734-9
更新日期:2013-04-01 00:00:00
abstract::Stavudine (2',3'-didehydro-3'deoxythymidine) is a pyrimidine analogue that may be of great value in combination antiretroviral therapy (ART) for treating patients infected with human immunodeficiency virus type 1 (HIV-1). We assessed potential neurotoxic side effects by comparing peripheral nerve function in patients ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050336
更新日期:1999-03-01 00:00:00
abstract:INTRODUCTION:Patients with blepharospasm in addition to involuntary contraction of the orbicularis oculi muscle also have non-motor symptoms (psychiatric, sleep, cognitive, and ocular). In this paper, we investigated the relationship of non-motor with motor symptoms and the total burden of non-motor symptoms in patient...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09484-w
更新日期:2019-11-01 00:00:00
abstract::Autosomal recessive cerebellar ataxia type 3 (ARCA3) is a rare inherited disorder caused by mutations in the ANO10 gene. The disease is characterized by slowly progressive spastic ataxia variably associated with motor neuron involvement, epilepsy, and cognitive decline. We performed mutational screening in 80 patients...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9141-z
更新日期:2019-02-01 00:00:00
abstract::Ten female patients suffering from catamenial epilepsy were treated with a synthetic analogue of the gonadotrophin releasing hormone (GnRH) in addition to antiepileptic drugs. Three of the patients became seizure free, in four patients seizure frequency decreased and in one patient seizures were of shorter duration. I...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00810354
更新日期:1992-05-01 00:00:00
abstract::Weight loss is a common symptom of Parkinson's disease and is associated with impaired quality of life. Predictors of weight loss have not been studied in large clinical cohorts. We previously observed an association between change in body mass index and change in Unified Parkinson's Disease Rating Scale (UPDRS) motor...
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/s00415-017-8562-4
更新日期:2017-08-01 00:00:00
abstract::The diagnosis of neurosarcoidosis is often difficult; the imaging signs of spinal cord sarcoidosis sometimes mimic those of cervical spondylotic myelopathy, which is common in elderly persons. We examined the characteristics of spinal cord sarcoidosis in Japanese patients with neurosarcoidosis. This case series identi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6080-3
更新日期:2011-12-01 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:BACKGROUND:D-dimer levels are used in several clinical settings, such as in predicting venous thrombosis, cardioembolic stroke and cancer status. In the present study, we investigated the associations between plasma D-dimer levels at admission, clinical characteristics and mortality at discharge in cryptogenic stroke p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8742-x
更新日期:2018-03-01 00:00:00