Abstract:
:Natalizumab, an anti-alpha4 integrin antibody, significantly reduces the number of visibly enhancing multiple sclerosis (MS) lesions. In this substudy of a 2-year trial of natalizumab monotherapy versus placebo, contrast-enhanced imaging investigated for subtle blood brain barrier (BBB) leakage in relapsing remitting (RRMS) patients, and whether such leakage is modified by natalizumab. After 24 weeks on treatment, 40 patients from 3 centres (27 on natalizumab and 13 on placebo) were studied. T1 weighted images were obtained before and at set timepoints up to 46 minutes after gadolinium (Gd)-DTPA (0.3 mmol/kg to 18 patients, 0.15 mmol/kg to 22). Paired regions of interest were placed around non-enhancing lesions and contralateral normal appearing white matter (NAWM). BBB leakage was inferred through post-Gd T1 weighted signal intensity (SI) change. SI change was greater in T2 non-enhancing lesions than paired NAWM at all timepoints (P<0.005), indicating BBB leakage in lesions. No significant difference in inferred BBB leakage was observed between treatment arms as measured by SI change of lesions (P>0.05 for all timepoints, joint test P=0.24), or in SI change of NAWM (joint test P=0.37). T1 hypointense and isointense lesions exhibited similar SI changes (joint test P=0.12). There is evidence of a subtle BBB leakage within visibly non-enhancing lesions in RRMS that was not modified by alpha4 integrin blockade in this substudy cohort.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Soon D,Altmann DR,Fernando KT,Giovannoni G,Barkhof F,Polman CH,O'Connor P,Gray B,Panzara M,Miller DHdoi
10.1007/s00415-006-0356-zsubject
Has Abstractpub_date
2007-03-01 00:00:00pages
306-14issue
3eissn
0340-5354issn
1432-1459journal_volume
254pub_type
临床试验,杂志文章,多中心研究,随机对照试验abstract::The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a major...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-016-8377-8
更新日期:2017-08-01 00:00:00
abstract:BACKGROUND:Despite advances in the field, diagnosis and management of the wide spectrum of neurological events post allogeneic hematopoietic cell transplantation (alloHCT) remain challenging. Therefore, we investigated their incidence, diagnosis, management and long-term prognosis in alloHCT recipients. METHODS:We ret...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09372-3
更新日期:2019-08-01 00:00:00
abstract::We report a case of central European tick-borne encephalitis with cervical myelitis presenting clinically as a lower motor neuron syndrome of the upper limbs with proximal asymmetrical pareses and atrophies. There were no sensory deficits nor signs of lesions of the spinal pathways or signs of encephalitis or meningit...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050537
更新日期:1999-12-01 00:00:00
abstract:BACKGROUND:Nusinersen recently became available as the first treatment for Spinal Muscular Atrophy (SMA) and data on its effectiveness and safety in adult SMA patients are still scarce. METHODS:We evaluated the effectiveness and safety of nusinersen treatment during 14 months in 16 adult patients with SMA types 3 and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10223-9
更新日期:2020-09-15 00:00:00
abstract::Vitamin B(12) (VitB(12), cobalamin) deficiency has been associated with various neuropsychiatric conditions, such as peripheral neuropathy, subacute combined degeneration, affective disorders, and cognitive impairment. Current assays analyze vitamin B(12), of which only a small percentage is metabolically active. Meas...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5764-4
更新日期:2011-03-01 00:00:00
abstract::Three cases of Fahr's syndrome are described. All patients had disturbances of calcium metabolism and had had a meningoencephalitis in childhood. It is suggested that gliovascular changes, induced by cerebral inflammation, can later facilitate the occurrence of calcification of the striopallidodentate system when abno...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313985
更新日期:1986-02-01 00:00:00
abstract::The neutrotrophins stimulate survival and differentiation of a range of target neurons. A wealth of evidence suggests that central cholinergic neurons depend on nerve growth factor (NGF) for trophic support. Grafts of NGF-producing cells rescue axotomized basal forebrain cholinergic neurons and reduce cholinergic cell...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00939231
更新日期:1994-12-01 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:BACKGROUND:Classifying and diagnosing peripheral vestibular disorders based on their symptoms is challenging due to possible symptom overlap or atypical clinical presentation. To improve the diagnostic trajectory, gadolinium-based contrast-enhanced magnetic resonance imaging of the inner ear is nowadays frequently used...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-10278-8
更新日期:2020-12-01 00:00:00
abstract::The International Cooperative Ataxia Rating Scale (ICARS) is a 100-point semiquantitative scale designed primarily to assess cerebellar dysfunction. However, little is known of the metric properties of this scale. We assessed the ICARS by rating the severity of cerebellar dysfunction in 27 patients with spinocerebella...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0310-0
更新日期:2007-06-01 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
abstract::Disturbances of balance, gait and posture are a hallmark of parkinsonian syndromes. Recognition of these axial features can provide important and often early clues to the nature of the underlying disorder, and, therefore, help to disentangle Parkinson's disease from vascular parkinsonism and various forms of atypical ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-019-09382-1
更新日期:2020-11-01 00:00:00
abstract::A patient with chronic relapsing inflammatory demyelinating polyneuropathy was successfully treated with plasma exchanges and cyclosporin A (CsA). Dynamometric measurements of hand force during the time of CsA treatment showed a highly significant correlation between hand force and CsA blood levels. The largest influe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314752
更新日期:1990-08-01 00:00:00
abstract::Absence of AMP-deaminase was demonstrated by histochemical and biochemical methods in a muscle biopsy of a 25-year-old woman with facial and limb girdle myopathy. Venous ammonia failed to rise after ischaemic exercise. This patient further contributes to the variety of clinical pictures associated with AMP-deaminase d...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313744
更新日期:1981-01-01 00:00:00
abstract::Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the de...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313709
更新日期:1985-01-01 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::Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-011-6278-4
更新日期:2012-06-01 00:00:00
abstract::From a family with essential familial myoclonus, 150 members in eight generations were studied. Twenty-five of them suffered from myoclonus of varying severity. The findings in routine examinations of blood, urine and cerebrospinal fluid, EEG and skull radiographs were normal. Therapeutic trials did not produce lastin...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313626
更新日期:1983-01-01 00:00:00
abstract::Suicide is a leading cause of death in Huntington's disease (HD), following pneumonia. Up to one-fifth of individuals with HD report suicidal ideation. Identifying the risk factors of suicidal ideation in this clinical population is thus pivotal. Here, we review the literature on prevalence rates and risk factors of s...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-018-9013-6
更新日期:2018-11-01 00:00:00
abstract::There has been a resurgence in the use of functional neurosurgery for Parkinson's disease. An important factor that has played a role in this development is the recent understanding of the functional anatomy of the basal ganglia including a knowledge of the changes in the activities of neurons in the internal segment ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF03161079
更新日期:1999-09-01 00:00:00
abstract:OBJECTIVE:Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9082-6
更新日期:2018-12-01 00:00:00
abstract::The various clinical features of multiple system atrophy (MSA) make the diagnosis of the disease difficult, especially in its early stages, when signs of differentiated neuroanatomical system involvement have not yet appeared. Mortality studies may be affected by the variability of the diagnostic criteria and selectio...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00868999
更新日期:1996-05-01 00:00:00
abstract::Disease management is defined as any medical or pharmaceutical intervention designed to improve both outcomes for the patient and overall cost-effectiveness of the health plan. Disease management focuses on the patient throughout the entire course of the disease, involving both health providers and third-party payers....
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00014806
更新日期:1998-08-01 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:OBJECTIVE:The main aim was to explore the changes in hand-grip strength in patients with Duchenne muscular dystrophy (DMD) aged 5-29 years. Secondary aims were to test the effect of mutation, ambulatory status and glucocorticoid use on grip strength and its changes over time and to compute the number of subjects needed...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09800-9
更新日期:2020-07-01 00:00:00
abstract:BACKGROUND:The symptomatic treatment of myotonia and myalgia in patients with dystrophic and non-dystrophic myotonias is often not satisfactory. Some patients anecdotally report symptoms' relief through consumption of cannabis. METHODS:A combination of cannabidiol and tetrahydrocannabinol (CBD/THC) was prescribed as c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09593-6
更新日期:2020-02-01 00:00:00
abstract::Long-term treatment studies with any antiparkinsonian drug are rather limited. Especially, double-blind, randomized and multicenter studies do not exist except for some rare exceptions. Nonetheless, such studies are mandatory to prove certain therapy regimens. This overview reports on the comparison between dopamine a...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00007768
更新日期:2000-09-01 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::Rapid-eye-movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by complex motor activity associated with dreaming during REM sleep. RBD may be idiopathic or associated with various neurological diseases involving the brainstem. The association of RBD and limbic system impairment was unclear. We r...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-009-5067-9
更新日期:2009-07-01 00:00:00
abstract::We report the effects of intravenous gamma-globulin (IVGG) in severe Guillain-Barré syndrome (GBS) in a small open study. Seven patients were given IVGG (0.4 g/kg/day) for 5 consecutive days. At the start of treatment all had progressing limb weakness and none could stand unaided. Within 24 h all but one patient start...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00838447
更新日期:1993-01-01 00:00:00