Abstract:
:Myasthenia gravis (MG) is the most common autoimmune disorder affecting the neuromuscular junction, characterized by skeletal muscle weakness and fatigability. It is caused by autoantibodies targeting proteins of the neuromuscular junction; ~85% of MG patients have autoantibodies against the muscle acetylcholine receptor (AChR-MG), whereas about 5% of MG patients have autoantibodies against the muscle specific kinase (MuSK-MG). In the remaining about 10% of patients no autoantibodies can be found with the classical diagnostics for AChR and MuSK antibodies (seronegative MG, SN-MG). Since serological tests are relatively easy and non-invasive for disease diagnosis, the improvement of methods for the detection of known autoantibodies or the discovery of novel autoantibody specificities to diminish SN-MG and to facilitate differential diagnosis of similar diseases, is crucial. Radioimmunoprecipitation assays (RIPA) are the staple for MG antibody detection, but over the past years, using cell-based assays (CBAs) or improved highly sensitive RIPAs, it has been possible to detect autoantibodies in previously SN-MG patients. This led to the identification of more patients with antibodies to the classical antigens AChR and MuSK and to the third MG autoantigen, the low-density lipoprotein receptor-related protein 4 (LRP4), while antibodies against other extracellular or intracellular targets, such as agrin, Kv1.4 potassium channels, collagen Q, titin, the ryanodine receptor and cortactin have been found in some MG patients. Since the autoantigen targeted determines in part the clinical manifestations, prognosis and response to treatment, serological tests are not only indispensable for initial diagnosis, but also for monitoring treatment efficacy. Importantly, knowing the autoantibody profile of MG patients could allow for more efficient personalized therapeutic approaches. Significant progress has been made over the past years toward the development of antigen-specific therapies, targeting only the specific immune cells or autoantibodies involved in the autoimmune response. In this review, we will present the progress made toward the development of novel sensitive autoantibody detection assays, the identification of new MG autoantigens, and the implications for improved antigen-specific therapeutics. These advancements increase our understanding of MG pathology and improve patient quality of life by providing faster, more accurate diagnosis and better disease management.
journal_name
Front Neuroljournal_title
Frontiers in neurologyauthors
Lazaridis K,Tzartos SJdoi
10.3389/fneur.2020.596981subject
Has Abstractpub_date
2020-11-30 00:00:00pages
596981issn
1664-2295journal_volume
11pub_type
杂志文章,评审abstract::The persistent primitive hypoglossal artery (PPHA) is the second most common persistent carotid-vertebrobasilar anastomosis, with an incidence of 0.027-0.26%. PPHAs change the hemodynamics of the carotid and vertebrobasilar system and may be associated with intracranial vascular anomalies, but basilar bifurcation aneu...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2017.00168
更新日期:2017-04-26 00:00:00
abstract::Background: Listeria rhombencephalitis, infection of the brainstem with Listeria monocytogenes, occurs mainly in humans and farmed ruminants and is associated with high fatality rates. Small ruminants (goats and sheep) are a large animal model due to neuropathological similarities. The purpose of this study was to def...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.518697
更新日期:2020-12-14 00:00:00
abstract:BACKGROUND:Before implementation in clinical practice, biomarker assays need to be thoroughly analytically validated. There is currently a strong interest in implementation of the ratio of amyloid-β peptide 1-42 and 1-40 (Aβ42/Aβ40) in clinical routine. Therefore, in this study, we compared the analytical performance o...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00310
更新日期:2017-07-03 00:00:00
abstract::Introduction: Cerebral autosomal dominant arteriopathy and subcortical infarct leukoencephalopathy (CADASIL) is the most common form of hereditary stroke caused by a mutation in the NOTCH3 gene located on the short arm of chromosome 19. A small number of published reports describe CADASIL patients who were initially d...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2020.00860
更新日期:2020-09-04 00:00:00
abstract::Over the past decade, it was found that relatively simple sphingolipids, such as ceramide, sphingosine, sphingosine-1-phosphate, and glucosylceramide play important roles in neuronal functions by regulating rates of neuronal growth and differentiation. Homeostasis of membrane sphingolipids in neurons and myelin is ess...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.00437
更新日期:2020-05-21 00:00:00
abstract::Significant advances have been made in the understanding of the numerous mechanisms involved in Parkinson's disease (PD) pathogenesis. The identification of PD pathogenic mutations and the use of different animal models have contributed to better elucidate the processes underlying the disease. Here, we report a brief ...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2018.00103
更新日期:2018-03-01 00:00:00
abstract::Psychophysiological investigations of traumatic brain injury (TBI) are being conducted for several reasons, including the objective of learning more about the underlying physiological mechanisms of the pathological processes that can be initiated by a head injury. Additional goals include the development of objective ...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2013.00091
更新日期:2013-07-22 00:00:00
abstract::Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) is a rare outcome of prolonged hemiconvulsion that is followed by diffuse unilateral hemispheric edema, hemiplegia, and ultimately hemiatrophy of the affected hemisphere and epilepsy. Here, we describe the case of a 3-year-old male with a 1;3 translocation leading to a...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2016.00067
更新日期:2016-05-04 00:00:00
abstract::Background: Recent developments in mobile technology have enabled the investigation of human movements and mobility under natural conditions, i.e., in the home environment. Iron accumulation in the basal ganglia is deleterious in Parkinson's disease (i.e., iron accumulation with lower striatal level of dopamine). The ...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2019.00833
更新日期:2019-08-08 00:00:00
abstract::Introduction: Stroke rehabilitation should start early in order to optimize patients' outcomes, but most trials include subacute or chronic patients. Although suggested that early stroke rehabilitation trials face difficulties regarding patient recruitment with corresponding low recruitment rates, no systematically co...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00683
更新日期:2019-07-02 00:00:00
abstract::Purpose: Rapid muscle fatigue limits clinical applications of functional electrical stimulation (FES) for individuals with motor impairments. This study aimed to characterize the sustainability of muscle force elicited with a novel transcutaneous nerve stimulation technique. Method: A hemiplegic chronic stroke survivo...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2018.01061
更新日期:2018-12-04 00:00:00
abstract::This study describes two experiments that were conducted in search for a behavioral paradigm to test for tinnitus in guinea pigs. Conditioning paradigms are available to determine the presence of tinnitus in animals and are based on the assumption that tinnitus impairs their ability to detect silent intervals in conti...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2014.00207
更新日期:2014-10-15 00:00:00
abstract::Robust and reliable stroke lesion segmentation is a crucial step toward employing lesion volume as an independent endpoint for randomized trials. The aim of this work was to develop and evaluate a novel method to segment sub-acute ischemic stroke lesions from fluid-attenuated inversion recovery (FLAIR) magnetic resona...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00541
更新日期:2019-05-24 00:00:00
abstract::X-linked adrenoleukodystrophy (X-ALD) is a rare neurological disorder with a highly complex clinical presentation. Adrenal function, spinal cord, peripheral nerves, and cerebral white matter are commonly affected in adult-onset male patients. Here, we report a family with unusual presentation of X-ALD. The 19-year-old...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2018.00271
更新日期:2018-04-23 00:00:00
abstract::Background: Agoraphobia was described in 1871 as a condition of fear-related alterations in spatial orientation and locomotor control triggered by places or situations that might cause a patient to panic and feel trapped. In contemporary nosology, however, this original concept of agoraphobia was split into two diagno...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00874
更新日期:2019-08-13 00:00:00
abstract::Background: Elderly patients account for 30% of acute ischemic stroke (AIS) but are under-represented in randomized controlled trials of endovascular thrombectomy (EVT). Meta-analysis of "real world" studies evaluating 90-day outcomes in elderly patients ≥80 years have been limited to small numbers undergoing EVT with...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00254
更新日期:2019-03-20 00:00:00
abstract::Tumor necrosis factor-alpha (TNF-α) is a proinflammatory cytokine that plays a prominent role in the nervous system, mediating a range of physiologic and pathologic functions. In the auditory system, elevated levels of TNF-α have been implicated in several types of sensorineural hearing loss, including sensorineural h...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.01353
更新日期:2020-02-10 00:00:00
abstract::Patient registries offer a powerful and practical means of real-world data collection system for rare diseases. Many guidelines have been released to standardize patient registries, although most of them do not address issues specific to rare disease patient registries. In November 2018, the European Medicines Agency ...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.00365
更新日期:2020-05-15 00:00:00
abstract:BACKGROUND:A possible link between 3D movies and headache (HA) has never been a target of specific and systematic investigations. The aim of this study was to investigate the relationship between 3D cinema and HA and to evaluate possible risk factors of developing HA during or after watching a 3D movie. METHODS:This w...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2016.00030
更新日期:2016-03-23 00:00:00
abstract::Neuropathological features in Alzheimer's disease (AD) are amyloid β (Aβ) deposits and neurofibrillary changes. AD is characterized by memory impairment and cognitive dysfunction, with some reports associating these impairments with hyperexcitability caused by Aβ in the medial temporal lobe. Epileptic seizures are kno...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2019.00620
更新日期:2019-06-12 00:00:00
abstract::This study presents the first implementation of functional ultrasound (fUS) imaging of the spinal cord to monitor local hemodynamic response to epidural electrical spinal cord stimulation (SCS) on two small and large animal models. SCS has been successfully applied to control chronic refractory pain and recently was e...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00279
更新日期:2019-03-26 00:00:00
abstract::Background: Using conventional visual evoked potentials (VEPs), migraine patients were found to be hyperresponsive to visual stimulus. Considering that a significant portion of neuronal activity is lost for analysis in the averaging process of conventional VEPs, in this study we investigated visual evoked responses of...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00393
更新日期:2018-05-30 00:00:00
abstract::Functional magnetic resonance imaging studies suggest that different subcortico-cortical circuits control different aspects of Parkinsonian rest tremor. The basal ganglia were proposed to drive tremor onset, and the cerebellum was suggested to be responsible for tremor maintenance ("dimmer-switch" hypothesis). Althoug...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00145
更新日期:2019-03-07 00:00:00
abstract::Background: Better upper limb recovery after stroke could be achieved through tailoring rehabilitation interventions directly at movement deficits. Aim: To identify potential; targets for therapy by synthesizing findings of differences in kinematics and muscle activity between stroke survivors and healthy adults perfo...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00472
更新日期:2018-06-25 00:00:00
abstract::Previous studies have suggested that the corticoreticular tract (CRT) has an important role in motor function almost next to the corticospinal tract (CST) in the human brain. Herein, the CRT is reviewed with regard to its anatomy, function, and recovery mechanisms after injury, with particular focus on previous diffus...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2019.01188
更新日期:2019-11-12 00:00:00
abstract:INTRODUCTION:Limited evidence exists on effective interventions to improve knowledge of preventive medications in patients with chronic diseases, such as stroke. We investigated the effectiveness of a nurse-led intervention, where a component was to improve knowledge of prevention medications, in patients with stroke o...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2016.00205
更新日期:2016-11-18 00:00:00
abstract::Background: Giant vertebrobasilar aneurysms (GVBAs) have an unfavorable natural history if left untreated and often pose a sizeable challenge to endovascular treatment. The aim of this study was to analyze the angiographic and clinical outcomes of GVBAs treated by various endovascular procedures. Methods: Between Janu...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00748
更新日期:2020-08-11 00:00:00
abstract::Most blast-induced traumatic brain injuries (bTBI) are mild in severity and culpable for the lingering and persistent neuropsychological complaints in affected individuals. There is evidence that the prevalence of symptoms post-exposure may be sex-specific. Our laboratory has focused on changes in the monoamine and th...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.540144
更新日期:2020-10-15 00:00:00
abstract::Background: Recent studies have shown that renal disease is associated with magnetic resonance imaging (MRI) markers of cerebral small vessel disease (CSVD), independent of traditional vascular risk factors. Although large artery lesions might be involved in the cerebrorenal association, evidence has been lacking. Met...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00498
更新日期:2018-06-26 00:00:00
abstract::Background: Mild traumatic brain injuries (mTBIs) are a significant social, sport, and military health issue. In spite of advances in the clinical management of these injuries, the underlying pathophysiology is not well-understood. There is a critical need to advance objective biomarkers, allowing the identification a...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00468
更新日期:2018-06-20 00:00:00