Abstract：
:Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably determined in these studies; when neurological parameters were reported, results were often inconclusive, usually because of subject heterogeneity and lack of adequate statistical power. In two of these studies, some patients showed beneficial effects of idebenone on their cardiomyopathy only when the dose was increased, prompting the systematic investigation of higher doses of idebenone. Following a phase 1 dose escalation study, a phase 2 tolerability and efficacy trial with low, intermediate, and high doses of idebenone was conducted. The results suggested that treatment with intermediate- and high-dose idebenone had beneficial effects on neurological symptoms. On the basis of these results, two phase 3 trials have been initiated, one in the United States with young ambulatory patients and one in Europe without limits on age and disease severity.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Schulz JB,Di Prospero NA,Fischbeck Kdoi
10.1007/s00415-009-1008-xsubject
Has Abstractpub_date
2009-03-01 00:00:00pages
42-5eissn
0340-5354issn
1432-1459journal_volume
256 Suppl 1pub_type
杂志文章,评审abstract：:We should like to emphasize the following points: 1. Apathy is defined here as a quantified and observable behavioral syndrome consisting in a quantitative reduction of voluntary (or goal-directed) behaviors; 2. Therefore, apathy occurs when the systems that generate and control voluntary actions are altered; 3. These...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-006-7012-5
更新日期：2006-12-01 00:00:00
abstract：RATIONALE:Phase I/II studies of autologous hematopoietic stem cell transplantation (HSCT) for multiple sclerosis ( MS) were initiated, based on results of experimental transplantation in animal models of multiple sclerosis and clinical observations in patients treated concomitantly for malignant disease. PATIENTS:Eigh...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1007/s00415-002-0800-7
更新日期：2002-08-01 00:00:00
abstract：:Psychiatric complaints are common in Parkinson's disease (PD), and have a significant influence in disease outcome and quality of life. Little attention has been paid to psychiatric symptoms at early stage disease. We aimed to screen a population of early stage PD patients for psychiatric symptoms and to study the rel...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-011-6140-8
更新日期：2012-01-01 00:00:00
abstract：:In 40 rabbits cerebral ischaemia was induced by autologous blood clot emboli injected into the middle cerebral artery. Autologous blood clot formed spontaneously (within 2 h) in a catheter fixed in the internal carotid artery. The effects of embolization were investigated on arterial blood pressure, respiration, local...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314181
更新日期：1988-05-01 00:00:00
abstract：:Cognition and gait appear to be closely related. The chronological interplay between cognitive decline and gait dysfunction is not fully understood. The aim of the present prospective study is investigating whether the dysfunction of specific gait parameters, during specific task and medication conditions, may predict...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-018-8973-x
更新日期：2018-10-01 00:00:00
abstract：:A woman affected by chronic progressive external ophthalmoplegia and muscle mitochondrial DNA deletion was studied by phosphorus magnetic resonance spectroscopy (31P-MRS) prior to and after 1 and 7 months of treatment with oral lipoic acid. Before treatment a decreased phosphocreatine (PCr) content was found in the oc...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00873552
更新日期：1995-07-01 00:00:00
abstract：:Palatal myoclonus is thought to occur after damage to certain brain-stem structures, and with a delay following the causative lesion. A case of palatal myoclonus, probably of epileptic nature, is described. ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00839145
更新日期：1992-04-01 00:00:00
abstract：:A clinical and electrophological study was performed on 30 patients with chronic carbon disulphide poisoning. Although the measurements of motor conduction velocity and of terminal latency were within the normal range in the subclinical stage, estimation of nerve excitability threshold showed distal motor hypoexcitabi...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313208
更新日期：1980-01-01 00:00:00
abstract：:Eighteen patients with dural arteriovenous fistulas or intradural arteriovenous malformations underwent clinical and neurophysiological examination. Bladder disturbances, pain, sensory abnormalities and involvement of both upper and lower motor neurons were commonly observed. Abnormal findings were obtained both in el...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00878524
更新日期：1996-01-01 00:00:00
abstract：:Decreased tongue strength (TS) might herald bulbar involvement in patients with amyotrophic lateral sclerosis (ALS) well before dysarthria or dysphagia occur, and as such might be prognostic of short survival. The purpose of this study was to investigate the prognostic value of a decreased TS, in addition to other pro...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-012-6503-9
更新日期：2012-11-01 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：:Polysomnography (PSG) is considered the gold standard for diagnosis of non-rapid eye movement (NREM) parasomnias, however its diagnostic yield has been rarely reported. We aimed to assess the diagnostic value of polysomnography in different categories of patients with suspected NREM parasomnia and define variables tha...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-014-7578-2
更新日期：2015-02-01 00:00:00
abstract：:The concentration of neopterin was measured in serum samples taken from individuals infected with HTLV-I: 5 from asymptomatic individuals, 1 from a patient with adult T-cell leukaemia and 30 from patients with tropical spastic paraparesis (TSP). In addition, cerebrospinal fluid (CSF) was available from 22 of the TSP p...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00810351
更新日期：1992-05-01 00:00:00
abstract：OBJECTIVE:Widespread metabolic changes are seen in neurodegenerative disease and could be used as biomarkers for diagnosis and disease monitoring. They may also reveal disease mechanisms that could be a target for therapy. In this study we looked for blood-based biomarkers in syndromes associated with frontotemporal lo...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09824-1
更新日期：2020-08-01 00:00:00
abstract：:Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease with an estimated incidence of 1 in 8000 and is the most common form of muscular dystrophy affecting adults. An unstable, untranslated part of the myotonic dystrophy protein kinase gene on the long arm of chromosome 19, composed of CTG repeats, is ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/s004150050220
更新日期：1998-05-01 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：:Unlike most neurodegenerative disorders, individuals at risk from Huntington's disease can be identified prior to the onset of clinical signs of the disease by virtue of it being an autosomal dominant condition. This provides the hypothetical opportunity to delay disease onset and/or slow down the progression of the d...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-015-7700-0
更新日期：2015-08-01 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：:Somatostatin levels were measured in cerebrospinal fluid of patients with Alzheimer's disease, multi-infarct dementia and normal pressure hydrocephalus and compared with levels from a normal control group. All pathological groups showed a statistically significant decrease of somatostatin with respect to the control g...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00319684
更新日期：1991-06-01 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：OBJECTIVE:To investigate whether intracranial arachnoid cysts (AC) compromise visual attention and if so, whether surgical cyst decompression leads to improvement in visual attention performance. METHODS:The experiments were carried out on 27 patients with temporal (n=21) or frontal (n=6) AC, and 27 healthy control su...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/s00415-006-0280-2
更新日期：2007-01-01 00:00:00
abstract：:Interferon beta (IFNbeta) therapy for multiple sclerosis (MS) is associated with a potential for the development of neutralising antibodies (NAbs) that negatively affect therapy. Several factors influence the development of NAbs, such as lack of complete sequence homology with the endogenous IFNbeta sequence, frequenc...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-006-0486-3
更新日期：2007-07-01 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：:5-Hydroxytryptamine (5-HT; serotonin) has long been implicated in the aetiology of migraine but the evidence remains circumstantial and certainly not definitive. Numerous papers have reviewed the background which is briefly outlined here. Although the continued belief in the primary involvement of 5-HT in the genesis ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/BF01642905
更新日期：1991-01-01 00:00:00
abstract：:Based on the observation of bilateral temporoparietal hypoperfusion in Alzheimer's disease (AD), single photon emission computed tomography (SPECT) is advocated by some as a powerful diagnostic tool in the evaluation of demented patients. We studied whether routine brain SPECT in elderly, mildly demented outpatients i...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00868397
更新日期：1995-06-01 00:00:00
abstract：:Discontinuation of natalizumab can lead to severe rebound of disease activity in patients with relapsing-remitting multiple sclerosis (RRMS); nevertheless, the treatment regimen in this clinical situation remains controversial. We report the case of a 25-year-old male patient with RRMS who was clinically stable under ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-017-8622-9
更新日期：2017-12-01 00:00:00
abstract：OBJECTIVES:To evaluate daily life management and functional outcome of Idarucizumab administration in case of emergency situations in patients with Dabigatran treatment. DESIGN:Multicenter observational registry study. SETTING:All hospitals with full neurological departments (n = 6) in Munich, Germany INCLUDED PATIEN...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00415-019-09492-w
更新日期：2019-11-01 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：AIM:To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) spectrum of diseases. METHODS:Comprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-018-8917-5
更新日期：2018-08-01 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
