Abstract:
:The greatest unmet need in multiple sclerosis (MS) are treatments that delay, prevent or reverse progression. One of the most tractable strategies to achieve this is to therapeutically enhance endogenous remyelination; doing so restores nerve conduction and prevents neurodegeneration. The biology of remyelination-centred on the activation, migration, proliferation and differentiation of oligodendrocyte progenitors-has been increasingly clearly defined and druggable targets have now been identified in preclinical work leading to early phase clinical trials. With some phase 2 studies reporting efficacy, the prospect of licensed remyelinating treatments in MS looks increasingly likely. However, there remain many unanswered questions and recent research has revealed a further dimension of complexity to this process that has refined our view of the barriers to remyelination in humans. In this review, we describe the process of remyelination, why this fails in MS, and the latest research that has given new insights into this process. We also discuss the translation of this research into clinical trials, highlighting the treatments that have been tested to date, and the different methods of detecting remyelination in people.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Cunniffe N,Coles Adoi
10.1007/s00415-019-09421-xsubject
Has Abstractpub_date
2021-01-01 00:00:00pages
30-44issue
1eissn
0340-5354issn
1432-1459pii
10.1007/s00415-019-09421-xjournal_volume
268pub_type
杂志文章,评审abstract::In this work, we investigated motor network structure in patients affected by essential tremor (ET) with or without resting tremor, using probabilistic tractography of the cerebello-thalamo-basal ganglia-cortical loop. Twenty-five patients with ET, twenty-two patients with ET associated with resting tremor (rET), and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8553-5
更新日期:2017-09-01 00:00:00
abstract:OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ES...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09508-5
更新日期:2019-12-01 00:00:00
abstract:OBJECTIVE:The variability of the severity and regional distribution of pathological process in basal ganglia (BG) and brainstem-cerebellar systems results in clinical heterogeneity and represents the motor subtype of multiple system atrophy (MSA). This study aimed to quantify spatial patterns of multimodal MRI abnormal...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8874-z
更新日期:2018-07-01 00:00:00
abstract::Natalizumab (TYSABRI(®)), a specific α4-integrin antagonist, is approved as a second-line treatment of relapsing-remitting MS (RRMS) patients who fail therapy with interferons or as first-line treatment of patients with highly active relapsing-remitting disease. Since the market introduction of natalizumab as a monoth...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-011-6116-8
更新日期:2011-11-01 00:00:00
abstract:OBJECTIVE:To assess the changes of muscle-related biomarkers at the early stage of amyotrophic lateral sclerosis, and to confirm these findings in an experimental animal model. METHODS:Thirty-nine subjects with sporadic amyotrophic lateral sclerosis and 20 healthy controls were enrolled and longitudinally evaluated. W...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09507-6
更新日期:2019-12-01 00:00:00
abstract::Epileptic syndromes are distinctive disorders with specific features, which when taken together, permit a specific diagnosis. There is actually a debate on that medial temporal lobe epilepsy with hippocampal sclerosis is an epileptic syndrome. To address this issue, we searched for discriminative semiological features...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8441-z
更新日期:2017-05-01 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:OBJECTIVE:To characterize the clinical symptoms and magnetic resonance imaging (MRI) findings of unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES). METHODS:This is a case report and systematic review of the literature to identify cases of unilateral cortical FLAM...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09440-8
更新日期:2019-10-01 00:00:00
abstract::The goal of this study is to define the clinical features, pathogenesis and key investigation findings of fasciculation anxiety syndrome in clinicians (FASICS). Twenty consecutive clinicians presenting with fasciculations were prospectively assessed with serial clinical and neurophysiological evaluations. Clinicians w...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-6856-8
更新日期:2013-07-01 00:00:00
abstract::A 40-year-old man suffered for 5 years from a progressive proximal myopathy mimicking an atypical limb-girdle dystrophy. A "myopathic" pattern with myotonic and pseudomyotonic discharges was determined by electromyography. Enzyme histochemical and ultrastructural investigations of muscle and liver biopsies pointed to ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312870
更新日期:1976-01-01 00:00:00
abstract:BACKGROUND:Since the detection of autoantibodies against neuronal surface antigens, autoimmune encephalitis (AE) has been more frequently diagnosed, especially in patients with symptoms typical of limbic encephalitis, such as seizures, short-term memory deficits, or psychosis. However, the clinical spectrum of AE may b...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-018-9105-3
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:The cerebellum is a predilection site of pathology in progressive multiple sclerosis (PMS) patients, contributing to cognitive deficits. Aim of this study was to investigate lobular cerebellar functional connectivity (FC) in PMS patients in relation to cognition. METHODS:In this cross-sectional study, resti...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8985-6
更新日期:2018-10-01 00:00:00
abstract::Global aphasia is described as a unitary syndrome which is characterized by a severe impairment of all linguistic capabilities. Speech production is extremely limited and consists of stereotyped phrases, recurring utterances or a few isolated words which are usually neologistically distorted. The patients are unable t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF02430346
更新日期:1977-01-13 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::Gilles de la Tourette's syndrome (GTS) and restless legs syndrome (RLS) are two different neurological disorders with common features such as involuntary movements. In both disorders a disturbance of the dopaminergic system has been considered among other possible mechanisms. Since periodic leg movements (PLMS) during...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150050136
更新日期:1997-08-01 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:BACKGROUND:Anti-epileptic drug (AED) prophylaxis in the first-seven days post-traumatic brain injury (TBI) is known to reduce seizure frequency acutely. AED efficacy is equivalent; therefore, choice of AED may rest with their side-effects. We hypothesise that AEDs that impair balance will prolong recovery, shown by a l...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09769-5
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:To date, insertable cardiac monitors (ICMs) are the most effective method for the detection of occult atrial fibrillation (AF) in cryptogenic stroke. The overall detection rate after 12 months, however, is low and ranges between 12.4 and 33.3%, even if clinical predictors are considered. Ischemic stroke pati...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09524-5
更新日期:2019-12-01 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::We assessed the prevalence of vertebral artery (VA) stenosis or occlusion and its influence on outcome in patients with acute basilar artery occlusion (BAO). We studied 141 patients with acute BAO enrolled in the Basilar Artery International Cooperation Study (BASICS) registry of whom baseline CT angiography (CTA) of ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7583-5
更新日期:2015-02-01 00:00:00
abstract::Altered cerebral vasoreactivity (CVR) has been implicated in migraine. To test this hypothesis, we studied CVR as measured by transcranial Doppler ultrasound (TCD) in 11 migraineurs and 12 healthy controls of similar age. Mean flow velocities (MFV) in the middle cerebral artery (MCA) were recorded during a cognitive a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00867591
更新日期:1992-07-01 00:00:00
abstract::Recent genetic studies suggest a Sardinian type of amyotrophic lateral sclerosis (ALS). Thus, ALS incidence, prevalence and survival were investigated in a large population of Sardinians aimed to disclose population-specific patterns and their temporal changes. This is a population-based incidence and prevalence study...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6681-5
更新日期:2013-02-01 00:00:00
abstract::The spinocerebellar ataxias (SCAs) with autosomal dominant inheritance are a clinically and genetically heterogeneous group of neurological disorders with overlapping as well as highly variable phenotypes primarily affecting the cerebellum. To date, 28 different loci have been identified. Nine SCAs are caused by repea...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5209-0
更新日期:2009-11-01 00:00:00
abstract::Three families are described which include members with "typical" Friedreich's disease (FD) and others who are ataxic but do not satisfy all the diagnostic criteria for that disease. In family A two patients have an early-onset, rapidly progressive FD, while two others have a late-onset, more benign form. In families ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00319681
更新日期:1991-06-01 00:00:00
abstract:BACKGROUND:Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare early onset neurodegenerative disease that typically results in ataxia, upper motor neuron dysfunction and sensorimotor peripheral neuropathy. Dysarthria and dysphagia are anecdotally described as key features of ARSACS but the natu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8950-4
更新日期:2018-09-01 00:00:00
abstract:OBJECTIVE:To determine the prevalence and characteristics of the cricopharyngeal bar (CPB), defined as marked protrusion with lacking relaxation and stricture of the upper esophageal sphincter on videofluoroscopy, in patients with inclusion body myositis (IBM). METHODS:We conducted a case-control study of comprehensiv...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10241-7
更新日期:2020-09-26 00:00:00
abstract::Headache attributed to ingestion of a cold stimulus (ICHD-3 beta 4.5.1) is also known as ice cream headache (ICH). This cross-sectional epidemiological study included 283 students (10-14-year-olds) attending a grammar school in Germany, their parents (n = 401), and 41 teachers. A self-administered questionnaire was us...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8098-z
更新日期:2016-06-01 00:00:00
abstract::The clinical features of 31 cases of syringomyelic syndrome, confirmed by metrizamide CT myelography (CTM) or magnetic resonance imaging (MRI), were analysed. Bilateral suspended sensory disturbance, previously regarded as a characteristic feature, was encountered in only 2 cases (6.5%). Thirteen patients (41.9%) show...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314193
更新日期:1987-10-01 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:BACKGROUND:Recently, therapeutic attempts to control motor choreatic hyperkinesia of Huntington's disease (HD) by means of pallidal deep brain stimulation (Gp-DBS) were successful. With respect to the clinical effects of Gp-DBS in juvenile hypokinetic-rigid HD (jHD; Westphal variant), only one single-case has been repo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8880-1
更新日期:2018-07-01 00:00:00