Abstract:
:Myotonic dystrophy type 1 (DM1) is an autosomal dominant genetic disease characterized by multi-system involvement. Affected organ system includes skeletal muscle, heart, gastro-intestinal system and the brain. In this review, we evaluate the evidence for alterations in insulin signaling and their relation to clinical DM1 features. We start by summarizing the molecular pathophysiology of DM1. Next, an overview of normal insulin signaling physiology is given, and evidence for alterations herein in DM1 is presented. Clinically, evidence for involvement of insulin signaling pathways in DM1 is based on the increased incidence of insulin resistance seen in clinical practice and recent trial evidence of beneficial effects of metformin on muscle function. Indirectly, further support may be derived from certain CNS derived symptoms characteristic of DM1, such as obsessive-compulsive behavior features, for which links with altered insulin signaling has been demonstrated in other diseases. At the basic scientific level, several pathophysiological mechanisms that operate in DM1 may compromise normal insulin signaling physiology. The evidence presented here reflects the importance of insulin signaling in relation to clinical features of DM1 and justifies further basic scientific and clinical, therapeutically oriented research.
journal_name
Front Neuroljournal_title
Frontiers in neurologyauthors
Nieuwenhuis S,Okkersen K,Widomska J,Blom P,'t Hoen PAC,van Engelen B,Glennon JCdoi
10.3389/fneur.2019.01229subject
Has Abstractpub_date
2019-11-26 00:00:00pages
1229issn
1664-2295journal_volume
10pub_type
杂志文章,评审abstract::Stroke is a leading cause of death and disability worldwide. While various risk factors have been identified, sleep has only been considered a risk factor more recently. Various epidemiologic studies have associated stroke with sleep such as sleep duration, and laboratory and clinical studies have proposed various und...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2017.00392
更新日期:2017-08-08 00:00:00
abstract::Eye trauma and blindness are common in the United States, with an incidence of over 2 million cases/year and 25 million blind adults, respectively. However, literature is surprisingly scarce on the potential confounding effect of eye trauma or blindness on the diagnosis of myasthenia gravis (MG), an autoimmune neuromu...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2014.00112
更新日期:2014-07-01 00:00:00
abstract::Surface electromyography (sEMG) is a non-invasive method, which may be used in France by health practitioners without medical degree, such as physiotherapists, who are taught in Institutes of physiotherapy. However, very few hours are devoted to sEMG teaching in physiotherapist educational programs, especially in a fo...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.584304
更新日期:2020-12-03 00:00:00
abstract::Background: Measuring cognitive functioning is common in traumatic brain injury (TBI) research, but no universally accepted method for combining several neuropsychological test scores into composite, or summary, scores exists. This study examined several possible composite scores for the test battery used in the large...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00670
更新日期:2020-07-17 00:00:00
abstract::Background and Purpose: Stroke-related functional risk scores are used to predict patients' functional outcomes following a stroke event. We evaluate the predictive accuracy of machine-learning algorithms for predicting functional outcomes in acute ischemic stroke patients after endovascular treatment. Methods: Data w...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00889
更新日期:2020-08-25 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::Hearing and balance impairment are the most frequently reported features of infratentorial (classical) superficial siderosis (iSS). There are few comprehensive descriptions of audiovestibular function in iSS and therefore limited understanding of the affected segment(s) of the audiovestibular pathway. In addition, mon...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2020.610819
更新日期:2021-01-14 00:00:00
abstract::Objective: To predict psychological distress at 2 months for patients with mild traumatic brain injury. Method: A prospective cohort study of 162 patients with mild traumatic brain injury (MTBI) admitted consecutively to an outpatient clinic at Haukeland University Hospital, Norway. Demographic data were obtained from...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00639
更新日期:2019-06-18 00:00:00
abstract::Background and objective: The trajectories of long-term and domain-specific cognitive alterations over a decade after stroke are largely unknown. This study aims to investigate the dynamic alterations of domain-specific cognitive performance among young stroke survivors over 10 years after their first stroke. Methods:...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00097
更新日期:2019-02-12 00:00:00
abstract::Introduction: Symptoms following a mild traumatic brain injury (mTBI) usually resolve quickly but may persist past 3 months in up to 15% of children. Mechanisms of mTBI recovery are poorly understood, but may involve alterations in cortical neurophysiology. Transcranial Magnetic Stimulation (TMS) can non-invasively in...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.00451
更新日期:2019-05-17 00:00:00
abstract:INTRODUCTION:Options currently available to individuals with upper limb loss range from prosthetic hands that can perform many movements, but require more cognitive effort to control, to simpler terminal devices with limited functional abilities. We attempted to address this issue by designing a myoelectric control sys...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00007
更新日期:2017-02-01 00:00:00
abstract:Objective:To investigate the correlation between initial response to antiepileptic drugs (AEDs) and long-term outcomes after 3 years in patients with newly diagnosed epilepsy. Methods:This prospective study included 204 patients with newly diagnosed epilepsy, who were followed-up for at least 36 months. The long-term ...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00658
更新日期:2017-12-08 00:00:00
abstract:OBJECTIVE:To provide a systematic review of the clinical and radiological features of lesion-induced central positional nystagmus (CPN) and identify salient characteristics that differentiate central from peripheral positional nystagmus (PN). METHODS:Systematic literature search according to the preferred reporting it...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2017.00141
更新日期:2017-04-20 00:00:00
abstract::Introduction: Few studies have examined the motor, cognitive, and emotional factors involved in effective money management in persons with multiple sclerosis (MS). The aim of this study was to assess money management in persons MS and examine whether cognitive, motor, and emotional processes can predict money manageme...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2019.01128
更新日期:2019-10-23 00:00:00
abstract::Stroke is a leading cause of death and disability, and recombinant tissue plasminogen activator (rtPA) can significantly reduce the long-term impact of acute ischemic stroke (AIS) if given within 3 h of symptom onset. South Carolina is located in the "stroke belt" and has a high rate of stroke and stroke mortality. Ma...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2012.00044
更新日期:2012-03-21 00:00:00
abstract::Background: Glioblastoma (GBM) is the most aggressive type of primary malignant brain tumor. Carmustine is used by intravenous injection or local implantation in the resection cavity for gliomas, including GBMs. However, the therapeutic potential of carmustine is not well-recognized. This analysis aimed to evaluate th...
journal_title:Frontiers in neurology
pub_type:
doi:10.3389/fneur.2020.01036
更新日期:2020-09-17 00:00:00
abstract::Neuroinflammation is one of the most important processes involved in the pathogenesis of Parkinson's disease (PD). The current concept of neuroinflammation comprises an inflammation process, which occurs in the central nervous system due to molecules released from brain-resident and/or blood-derived immune cells. Furt...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2018.00860
更新日期:2018-10-15 00:00:00
abstract::Traumatic brain injury (TBI) is a leading cause of death and disability for children and adolescents in the U.S. and other developed and developing countries. Injury to the immature brain varies greatly from that of the mature, adult brain due to numerous developmental, pre-injury, and injury-related factors that work...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2019.01296
更新日期:2019-12-13 00:00:00
abstract::Objective: Episodic memory is frequently impaired in Multiple Sclerosis (MS), but the cognitive characteristics and neuropsychological processes involved remain controversial. Our aim was to study episodic memory dysfunction in MS, using the LASSI-L, a novel memory-based cognitive stress test that uses a new paradigm ...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00309
更新日期:2020-04-21 00:00:00
abstract:INTRODUCTION:Core cerebrospinal fluid (CSF) biomarkers - Aβ42, Tau, and phosphorylated Tau (pTau) - have been recently incorporated in the revised criteria for Alzheimer's disease (AD). However, their widespread clinical application lacks standardization. Pre-analytical sample handling and storage play an important rol...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2015.00153
更新日期:2015-07-08 00:00:00
abstract::Purpose: Stroke-associated pneumonia (SAP), a common complication in acute ischemic stroke (AIS) patients, is associated with poor prognosis after AIS. Inflammation plays an important role in the development of SAP. In this study, we aimed to explore the association between the monocyte-to-lymphocyte ratio (MLR) and S...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.575809
更新日期:2020-10-06 00:00:00
abstract::The most frequent neurodegenerative diseases (NDs) are Alzheimer's disease (AD), Parkinson's disease (PD), and frontotemporal lobar degeneration associated with protein TDP-43 (FTLD-TDP). Neuropathologically, NDs are characterized by abnormal intracellular and extra-cellular protein deposits and by disease-specific ne...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2011.00042
更新日期:2011-07-11 00:00:00
abstract::We previously identified a distinct mutation pattern in the antibody genes of B cells isolated from cerebrospinal fluid (CSF) that can identify patients who have relapsing-remitting multiple sclerosis (RRMS) and patients with clinically isolated syndromes who will convert to RRMS. This antibody gene signature (AGS) wa...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2014.00166
更新日期:2014-09-16 00:00:00
abstract:Background:Temporal lobe epilepsy (TLE) is one of the most drug-resistant types of epilepsy with about 80% of TLE patients falling into this category. Increasing evidence suggests that neuroinflammation, which has a critical role in the epileptogenesis of TLE, is associated with microglial activation. Therefore, agents...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00661
更新日期:2017-12-14 00:00:00
abstract:Background and Objectives:Benign paroxysmal positional vertigo (BPPV) recurs frequently. This study aims to determine that each patient with BPPV has a predilection for a specific canal and the type of recurred BPPV can be predicted from that observed during the previous attack. Methods:The involved side (right, left,...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2017.00690
更新日期:2017-12-15 00:00:00
abstract::Maternal smoking is a risk factor for both sudden infant death syndrome (SIDS) and sudden intrauterine unexplained death syndrome (SIUDS). Both SIDS and SIUDS are more frequently observed in infants of smoking mothers. The global prevalence of smoking during pregnancy is 1.7% and up to 8.1% of women in Europe smoke du...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.586068
更新日期:2020-10-23 00:00:00
abstract::Disrupted development of oropharyngeal structures as well as cranial nerve and brainstem circuits may lead to feeding and swallowing difficulties in children with 22q11. 2 deletion syndrome (22q11DS). We previously demonstrated aspiration-based dysphagia during early postnatal life in the LgDel mouse model of 22q11DS ...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2020.00004
更新日期:2020-01-31 00:00:00
abstract::Objective: The peak-to-peak amplitude of the p13-n23 wave in cervical vestibular evoked myogenic potential can increase after furosemide administration in patients with Meniere's disease [furosemide-loading VEMP (FVEMP) testing]. The examination is used to test for the presence of endolymphatic hydrops; we investigate...
journal_title:Frontiers in neurology
pub_type: 杂志文章
doi:10.3389/fneur.2018.00636
更新日期:2018-08-03 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::Cognitive dysfunction is a significant non-motor feature of Parkinson's disease, with the risk of dementia increasing with prolonged disease duration. Multiple cognitive domains are affected, and the pathophysiology cannot be explained by dopaminergic loss alone. Sophisticated neuroimaging techniques can detect the na...
journal_title:Frontiers in neurology
pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.00047
更新日期:2020-01-31 00:00:00