Abstract:
:Ehlers-Danlos syndrome (EDS) is a heterogeneous heritable connective tissue disorder with various neurological manifestations, including chronic pain. The neurological manifestations in EDS are often regarded as being caused by the associated musculoskeletal disorders or polyneuropathy. Here, we present two patients with hypermobile EDS (hEDS), presenting with relapsing central nervous system (CNS) manifestations. Although the two patients showed relapsing signs of CNS manifestations like multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD), they were unique in that they had widespread opioid-dependent chronic pain, which is not consistent with the symptoms of MS/NMOSD. Unexpectedly, the serious pain of unknown origin was remarkably mitigated by plasmapheresis, and magnetic resonance imaging (MRI) examinations conducted for one of the patients were negative. Collectively, we speculate that hEDS may be more susceptible to 'normal-appearing imaging, neuroimmunologically justified, autoimmune-mediated encephalomyelitis (NINJA).' Analysis of the presented cases and an additional three patients with EDS with chronic pain indicates that treatable immune-mediated mechanisms deserve considerations for neurological symptoms observed in hEDS.
journal_name
Ther Adv Neurol Disordjournal_title
Therapeutic advances in neurological disordersauthors
Araki M,Lin Y,Ono H,Sato W,Yamamura Tdoi
10.1177/1756286418793766subject
Has Abstractpub_date
2018-08-18 00:00:00pages
1756286418793766eissn
1756-2856issn
1756-2864pii
10.1177_1756286418793766journal_volume
11pub_type
abstract::Chronic migraine (CM) is a severely disabling neurological condition characterized by episodes of pulsating unilateral or bilateral headache. The United States Food and Drug Administration (FDA) approved onabotulinumtoxinA (Botox®) for the prophylactic treatment of CM in 2010. It has been shown that onabotulinumtoxinA...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285616677005
更新日期:2017-02-01 00:00:00
abstract::Acute stroke is one of the major causes of death and disabilities. Since the 1980s many clinical studies have been conducted to evaluate neuroprotective approaches to treat this important brain vascular event. However, to date the only drug approved (recombinant tissue plasminogen activator [rtPA]) represents a thromb...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285611434926
更新日期:2012-05-01 00:00:00
abstract::Stroke is the fifth leading cause of death and the most frequent cause of disability worldwide. Currently, stroke diagnosis is based on neuroimaging; therefore, the lack of a rapid tool to diagnose stroke is still a major concern. In addition, therapeutic approaches to combat ischemic stroke are still scarce, since th...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286418789340
更新日期:2018-08-06 00:00:00
abstract::The treatment of stroke caused by intracranial vessel occlusion with intravenous recombinant tissue plasminogen activator (rt-PA) was the only evidence-based treatment option for a long time. Nevertheless the response rate was disappointing in large vessel occlusions. Five studies that evaluated the efficacy of mechan...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285617742082
更新日期:2017-11-22 00:00:00
abstract:Background:Lennox-Gastaut syndrome (LGS) is a typical intractable form of epilepsy that most often occurs between the second and sixth year of life. This study aimed to evaluate the clinical efficacy and safety of ketogenic diet therapies (DTs) for LGS with mitochondrial dysfunction. Methods:This was a retrospective s...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286419897813
更新日期:2020-02-06 00:00:00
abstract:Background:Galcanezumab, along with three other monoclonal antibodies targeting the calcitonin gene-related peptide (CGRP) pathway, represents the latest disease-specific and mechanism-based treatment for the prophylaxis of migraine. Galcanezumab shares data also for the prophylaxis of cluster headache. Objective:To p...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286420918088
更新日期:2020-04-28 00:00:00
abstract:BACKGROUND:Efficacy of interferon beta in multiple sclerosis (MS) can be dampened in patients who develop neutralizing antidrug antibodies (NAbs). Peginterferon beta1a is an interferon conjugated with a polyethylene glycol (PEG) moiety. Pegylation increases a drug's half life and exposure, and may also reduce immunogen...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285616633967
更新日期:2016-07-01 00:00:00
abstract::Prolonged-release (PR) fampridine is the only approved medication to improve walking in multiple sclerosis (MS), having been shown to produce a clinically meaningful improvement in walking ability in the subset of MS patients with Expanded Disability Status Scale 4-7. Recent responder subgroup analyses in the phase II...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286418803248
更新日期:2018-10-05 00:00:00
abstract::Spinal muscular atrophy (SMA) is a progressive, recessively inherited neuromuscular disease, characterized by the degeneration of lower motor neurons in the spinal cord and brainstem, which leads to weakness and muscle atrophy. SMA currently represents the most common genetic cause of infant death. SMA is caused by th...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285618754501
更新日期:2018-02-05 00:00:00
abstract::Cluster headache (CH) is a short-lasting unilateral headache associated with ipsilateral craniofacial autonomic manifestations. A positron emission tomography (PET) study has shown that the posterior hypothalamus is activated during CH attacks, suggesting that hypothalamic hyperactivity plays a key role in CH pathophy...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285610370722
更新日期:2010-05-01 00:00:00
abstract::Experimental models of Parkinson's disease (PD) are of great importance for improving the design of future clinical trials. Various neurotoxic models are available, including 6-hydroxydopamine (6-OHDA), 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), paraquat and rotenone. However, no model is considered perfect;...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285611413004
更新日期:2011-11-01 00:00:00
abstract::Brain atrophy, white matter abnormalities, and ventricular enlargement have been described in different neuromuscular diseases (NMDs). We aimed to provide a comprehensive overview of the substantial advancement of brain imaging in neuromuscular diseases by consulting the main libraries (Pubmed, Scopus and Google Schol...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286419845567
更新日期:2019-05-06 00:00:00
abstract::B cells play a central role in the pathogenesis in multiple sclerosis (MS), being involved in the activation of proinflammatory T cells, secretion of proinflammatory cytokines, and production of autoantibodies directed against myelin. Hence, the usage of B-cell-depleting monoclonal antibodies as therapy for autoimmune...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285615601933
更新日期:2016-01-01 00:00:00
abstract::For patients with relapsing-remitting multiple sclerosis (RRMS), there are currently six approved medications that have been shown to alter the natural course of the disease. The approved medications include three beta interferon formulations, glatiramer acetate, natalizumab and mitoxantrone. Treating aggressive forms...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285609344375
更新日期:2009-11-01 00:00:00
abstract::Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl p...
journal_title:Therapeutic advances in neurological disorders
pub_type:
doi:10.1177/1756286420909973
更新日期:2020-06-03 00:00:00
abstract:OBJECTIVES:The cumulative safety and efficacy measures of percutaneous transluminal angioplasty and stenting (PTAS) for secondary stroke prevention in patients with symptomatic intracranial arterial stenosis (sICAS) have not previously been evaluated using a meta-analytical approach. METHODS:We conducted a systematic ...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285616650357
更新日期:2016-09-01 00:00:00
abstract::Brain imaging is increasingly used to support clinicians in diagnosing multiple sclerosis (MS) and monitoring its progression. However, the role of magnetic resonance imaging (MRI) in MS goes far beyond its clinical application. Indeed, advanced imaging techniques have helped to detect different components of MS patho...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286419859722
更新日期:2019-06-27 00:00:00
abstract::Calcitonin gene related peptide (CGRP) monoclonal antibodies (mAbs) have been the first class of specifically developed preventive treatments for migraine. Clinical trials data suggest superiority of the CGRP mAbs to placebo in terms of prevention of migraine symptoms, migraine-specific quality of life and headache re...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756286420927119
更新日期:2020-05-27 00:00:00
abstract::Since the approval in 2010 of fingolimod 0.5 mg (Gilenya; Novartis Pharma AG, Basel, Switzerland) in the USA as an oral therapy for relapsing forms of multiple sclerosis, long-term clinical experience with this therapy has been increasing. This review provides a summary of the cumulative dataset from clinical trials a...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285616628766
更新日期:2016-03-01 00:00:00
abstract:Background:To investigate outcome and toxicity of high-dose systemic methotrexate (HDMTX)-based polychemotherapy and intracerebroventricular (ICV) chemotherapy via an Ommaya reservoir in elderly patients with primary central nervous system lymphoma (PCNSL). Methods:We performed a retrospective analysis on patients ⩾65...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286420951087
更新日期:2020-10-07 00:00:00
abstract::Laquinimod is a novel immunomodulatory agent, in development as a potential disease-modifying treatment for multiple sclerosis (MS). Structurally related to linomide, its pharmacological predecessor, laquinimod combines anti-inflammatory and possibly clinically relevant neuroprotective effects with the convenience of ...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285613499424
更新日期:2013-11-01 00:00:00
abstract::Our inability to identify the invasive margin of glioblastomas hampers attempts to achieve local control. Diffusion tensor imaging (DTI) has been implemented clinically to delineate the margin of the tumor infiltration, its derived anisotropic (q) values can extend beyond the contrast-enhanced area and correlates clos...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286419844664
更新日期:2019-05-14 00:00:00
abstract:BACKGROUND:The aim of this study was to analyse registry data of seizure outcome and adverse events (AEs) for perampanel as add-on therapy in patients with focal epilepsy since its approval in 2012 for adjunctive treatment of focal epilepsy in patients ⩾12 years. METHOD:A retrospective 2-year chart review of all patie...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285616661115
更新日期:2016-11-01 00:00:00
abstract::Alzheimer's dementia (AD) is increasingly being recognized as one of the most important medical and social problems in older people in industrialized and non-industrialized nations. To date, only symptomatic treatments exist for this disease, all trying to counterbalance the neurotransmitter disturbance. Three choline...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285612461679
更新日期:2013-01-01 00:00:00
abstract::Topiramate has been widely utilized worldwide as an effective medication against partial- and generalized-onset seizures. Extended-release topiramate was developed to provide patients with the convenience of once-daily dosing and potentially improved tolerability by reducing serum concentration fluctuation. USL255 is ...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章,评审
doi:10.1177/1756285615578406
更新日期:2015-05-01 00:00:00
abstract:Background:Gastrointestinal (GI) events are common adverse events (AEs) associated with delayed-release dimethyl fumarate (DMF), an approved treatment for relapsing-remitting multiple sclerosis (RRMS). The objective of the TOLERATE study was to evaluate GI tolerability and GI mitigation via symptomatic therapies in pat...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286418768775
更新日期:2018-04-18 00:00:00
abstract:Background:No evidence of disease activity (NEDA) is a composite measurement, incorporating clinical and magnetic resonance imaging (MRI) elements of disease activity to sensitively evaluate the therapeutic efficacy of treatments for relapsing-remitting multiple sclerosis (RRMS). Objective:To assess the NEDA status of...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286418795085
更新日期:2018-08-28 00:00:00
abstract::Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that largely affects optic nerves and spinal cord. Recent studies have identified an elevation of serum anti-aquaporin 4 antibody as a hallmark of NMO. Typical cases of NMO significantly differ from multiple sclerosis (MS) in immunological markers, hi...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285608093978
更新日期:2008-07-01 00:00:00
abstract:Background:The humanized anti-α4 integrin monoclonal antibody natalizumab has proven to be very effective in patients with highly active relapsing-remitting multiple sclerosis (MS), but harbors the risk of progressive multifocal leukoencephalopathy (PML). Recently, new therapeutic options have become available for pati...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756286419887596
更新日期:2019-12-02 00:00:00
abstract::A first step in evaluating the use of computers in language therapy for individuals with aphasia is to establish the treatment as active in small groups prior to large-scale clinical trials. The present study evaluated a comprehensive computer-based language therapy program in a group of eight individuals with chronic...
journal_title:Therapeutic advances in neurological disorders
pub_type: 杂志文章
doi:10.1177/1756285609336548
更新日期:2009-09-01 00:00:00