Abstract:
:We measured changes in brain magnetization transfer ratio (MTR) as a potential indicator of myelin density in brain tissue of patients with relapsing-remitting multiple sclerosis (RRMS) treated with delayed-release dimethyl fumarate (DMF) in the Phase 3 DEFINE study. DEFINE was a randomized, double-blind, placebo-controlled study in which patients with RRMS were randomized 1:1:1 to 2 years of treatment with delayed-release DMF 240 mg twice daily (BID) or three times daily (TID) or placebo. MTR was analyzed in whole brain and normal-appearing brain tissue (NABT) at baseline, week 24, 1 year, and 2 years in a subset of patients. MTR data from 392 patients were analyzed. Mean percentage reduction from baseline to 2 years in median whole brain MTR was -0.386% in the placebo group vs increases of 0.129% (p = 0.0027) and 0.096% (p = 0.0051) in the delayed-release DMF BID and TID groups, respectively. Similarly, mean percentage reduction from baseline in median NABT MTR was -0.392% with placebo vs increases of 0.190% (p = 0.0006) and 0.115% (p = 0.0029) with delayed-release DMF BID and TID, respectively. Post hoc analysis of data from patients with no new or enlarging T2 lesions (n = 147), or who experienced no relapses (n = 238), yielded similar results. In this analysis, increases in MTR in brain tissue most likely reflect increases in myelin density in response to delayed-release DMF. These data in patients with RRMS are consistent with preclinical studies that indicate a potential for cytoprotection and remyelination with delayed-release DMF treatment.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Arnold DL,Gold R,Kappos L,Bar-Or A,Giovannoni G,Selmaj K,Yang M,Zhang R,Stephan M,Sheikh SI,Dawson KTdoi
10.1007/s00415-014-7504-7subject
Has Abstractpub_date
2014-12-01 00:00:00pages
2429-37issue
12eissn
0340-5354issn
1432-1459journal_volume
261pub_type
杂志文章,随机对照试验abstract:BACKGROUND:Trans-active response DNA-binding protein of 43 kDa (TDP-43) can be detected in up to 63% of autopsy-confirmed Lewy body disease (LBD) cases. It is unclear whether TDP-43 is associated with a decreased likelihood of a clinical diagnosis of probable dementia with Lewy bodies (pDLB) during life. METHODS:In an...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09718-2
更新日期:2020-05-01 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 clinicopathological findings of a 50-year-old man, who developed cervicothoracic syringomyelia at the age of 25 are presented. He was given radiation therapy at the age of 33. At the age of 57 he developed a foramen jugulare syndrome on the left, caused by a low grade leiomyosarcoma. Etiologically, the most attrac...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313150
更新日期:1980-01-01 00:00:00
abstract:OBJECTIVE:Determine toxicity and efficacy of autologous hematopoietic stem cell transplantation (HSCT) for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are dependent on intravenous immunoglobulins or plasmapheresis. METHODS:Unselected peripheral blood stem cells were re-infused on...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10010-6
更新日期:2020-11-01 00:00:00
abstract::Hirayama's disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. We report clinical and magnetic resonance imaging findings in nine patients with Hirayama's disease. Cervical imaging of seven pat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050514
更新日期:1999-11-01 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::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::A retrospective study was performed to investigate seasonal variation in stroke incidence and to evaluate the hypothesis that cold might be a risk factor. Data were obtained from the central registry of the Hospital de S. João, Porto, Portugal, concerning 4048 patients consecutively admitted for cerebrovascular diseas...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00919594
更新日期:1995-03-01 00:00:00
abstract::The effect of furosemide (Lasix) therapy on a standardized experimental cerebral edema, induced in rats by applying a cooling stamp to the right side of the skull over the right coronal suture by means of a stereotactic instrument, was examined. The hemispherically separated water and electrolyte contents of the brain...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00705536
更新日期:1979-05-02 00:00:00
abstract:OBJECTIVE:Spinal and bulbar muscular atrophy (SBMA) is caused by an abnormal expansion of the CAG repeat in the androgen receptor gene. This study aimed to systematically phenotype a German SBMA cohort (n = 80) based on laboratory markers for neuromuscular, metabolic, and endocrine status, and thus provide a basis for ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8790-2
更新日期:2018-05-01 00:00:00
abstract::In Parkinson's disease (PD), nonmotor symptoms manifest before motor symptoms. In this report, we present a remarkable case of a semiprofessional painter with PD whose painting style dramatically changed from abstract painting to realism before he developed motor, psychiatric, and autonomic nerve disorders. This case ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6271-y
更新日期:2012-05-01 00:00:00
abstract::Intravenous immunoglobulins (IVIg) are effective for treating chronic inflammatory demyelinating polyneuropathy (CIDP), although treatment needs are variable and need to be individualized. Dose and frequency requirements are not currently predictable in advance. In Guillain-Barré syndrome, IVIg interpatient pharmacoki...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6938-7
更新日期:2013-08-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Current demographic changes indicate that more people will be care-dependent due to increasing life expectancy. Little is known about impact of preexisting dependency on stroke outcome after endovascular treatment (EVT). METHODS:We compared prospectively collected baseline and outcome data of pr...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10172-3
更新日期:2020-08-31 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:OBJECTIVE:Patients with classic ataxia-telangiectasia (A-T) generally die in the second or third decade of life. Clinical descriptions of A-T tend to focus on the symptoms at presentation. However, during the course of the disease, other symptoms and complications emerge. As long-term survivors with classic A-T develop...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09641-1
更新日期:2020-03-01 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::Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-1021-4
更新日期:2003-04-01 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::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: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:BACKGROUND:Putaminal iron deposition is a histopathological feature of multiple system atrophy (MSA), which is not observed in patients with idiopathic Parkinson's disease (PD). T2*-weighted magnetic resonance imaging (MRI) gradient echo (GE) sequences are sensitive for paramagnetic susceptibility changes and therefore...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0496-1
更新日期:2007-09-01 00:00:00
abstract::Muscle glucose-6-phosphate dehydrogenase (G6PD) deficiency is described in four clinically heterogeneous patients: an athlete who developed myoglobinuria after physical exercise; a 7-year-old, mildly mentally retarded boy, who had episodes of dark urine and high creatine kinase; and two brothers of Sardinian origin, t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314498
更新日期:1989-05-01 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::Clinical data and the serum and cerebrospinal fluid (CSF) findings of 71 patients with Guillain-Barré syndrome (GBS), 7 with Fisher syndrome and 24 with chronic inflammatory polyradiculoneuropathy (CIP), were analysed. Isoelectric focusing of serum and CSF together with different formulae and diagrams were applied to ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314019
更新日期:1986-08-01 00:00:00
abstract::The primary cause of neurological syndromes with antibodies against glutamic acid decarboxylase 65 (GAD65-Ab) is unknown, but genetic predisposition may exist as it is suggested by the co-occurrence in patients and their relatives of other organ-specific autoimmune diseases, notably type 1 diabetes mellitus (T1DM), an...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09782-8
更新日期:2020-07-01 00:00:00
abstract::The computed tomography, magnetic resonance imaging and angiographic findings are described in a patient with Klippel-Trenaunay syndrome, who also had a cerebral haemorrhage from an arteriovenous malformation. The resulting aphasia disappeared completely after resorption of the haemorrhage. In this syndrome, the occur...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313992
更新日期:1986-02-01 00:00:00
abstract::Left-handedness is most often genetic, but may also follow early, localised damage to the developing brain. This so-called "pathological" left-handedness syndrome is often associated with right hemisphere speech dominance. To find out whether verbal laterality or handedness were affected by congenital, intracranial ar...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-0941-3
更新日期:2003-01-01 00:00:00
abstract::There have been few reports concerning the characteristics of cerebral infarction associated with migraine (CIAM), and especially about the subsequent fate of these patients. We studied 14 patients (9 female) with CIAM. In all these patients the onset of cerebral infarction was accompanied by a unilateral throbbing he...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00886872
更新日期:1996-07-01 00:00:00
abstract::Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7429-1
更新日期:2014-10-01 00:00:00
abstract::Alemtuzumab is an anti-CD52 monoclonal antibody recently licensed for use in relapsing-remitting multiple sclerosis. Here, we report our experience of its use in neuromyelitis optica (NMO) spectrum disorders. A retrospective case review of patients treated with alemtuzumab in Cambridge, UK, was conducted to identify t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7925-y
更新日期:2016-01-01 00:00:00