Abstract:
OBJECTIVES:Though much information exists about the diagnosis, treatment, and epidemiology of myasthenia gravis (MG), a comprehensive data registry and biorepository is critical to better understand disease mechanisms, treatment outcomes, and the impact of treatment strategies. We aimed to design and implement the "Exploring Outcomes and Characteristics of Myasthenia Gravis (EXPLORE-MG) Registry" to address these knowledge gaps. METHODS:A web-based, non-interventional, longitudinal, observational disease and outcomes registry was developed; incorporating NIH recommended common data elements for the study of MG. Individuals diagnosed with MG based on prespecified criteria were eligible to participate. The registry was further strengthened by a complementary biorepository. An interim analysis was completed on registry data collected through data-lock in 2017. RESULTS:A total of 232 MG patients, followed at the Yale MG Clinic from 2011 to 2017, were enrolled, which included 2142 total visit entries. Of the 232 MG patients (mean age 60 years, range 17-99; female:male, 1.04:1), 165 were acetylcholine receptor antibody-positive, 20 were muscle-specific kinase antibody-positive, and 47 were seronegative. This cohort consisted of 64 patients with ocular disease, 168 patients with generalized disease, and 65 patients post-thymectomy, including 20 with thymoma-associated MG. CONCLUSIONS:Identification of key clinical features that may predict treatment responsiveness or provide insight into patient outcomes is essential to improve patient care. As current research focuses on the development of patient-tailored, targeted-treatment regimens, this registry can help provide important clinical and epidemiological data from a large contemporary patient cohort with long-term follow-up. REGISTRATION:ClinicalTrials.gov Identifier: NCT03792659.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Anil R,Kumar A,Alaparthi S,Sharma A,Nye JL,Roy B,O'Connor KC,Nowak RJdoi
10.1016/j.jns.2020.116830subject
Has Abstractpub_date
2020-07-15 00:00:00pages
116830eissn
0022-510Xissn
1878-5883pii
S0022-510X(20)30166-0journal_volume
414pub_type
杂志文章abstract::Over two decades, a 42-year old woman experienced the gradual onset of choreic involuntary movements, dystonia, and tics. Decreased caudate nucleus metabolism on 2-deoxyglucose PET scan and a heterozygous 49-CAG repeat expansion within the HTT gene established the diagnosis of HD, although no other family history was ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.08.034
更新日期:2012-12-15 00:00:00
abstract::Cutaneous reflex (CR) was measured in 15 patients with acute lacunar stroke and in 15 age- and sex-matched normal subjects. EMG activities were recorded from the first dorsal interosseous muscle with surface electrodes, rectified and averaged. Intensity of the stimulation on the index finger was four times the sensory...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00142-7
更新日期:1998-07-15 00:00:00
abstract:OBJECTIVES:Tumor necrosis factor-alpha inhibitors (TNFα-I) are biological agents used in the treatment of rheumatologic disorders. TNFα-I have been associated with demyelinating disorders mimicking multiple sclerosis. The goal of this report is to illustrate cases of myelopathy which developed during the use of TNFα-I....
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2017.01.023
更新日期:2017-02-15 00:00:00
abstract:OBJECTIVE:Characteristic sleep disturbance in patients with Parkinson's disease (PD) was evaluated using a subjective questionnaire called the PD sleep scale (PDSS). In this study we sought to examine the relationship between the results from the PDSS with those from the Pittsburgh Sleep Quality Index (PSQI), the Epwor...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.09.015
更新日期:2009-12-15 00:00:00
abstract::Drivers with cognitive impairment are at increased odds for vehicular crashes. Rear-end collisions (REC) are among the most common crash types. We tested REC avoidance in 61 drivers with mild Alzheimer's disease (AD) and 115 elderly controls using a high-fidelity interactive driving simulator. After a segment of uneve...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.08.011
更新日期:2006-12-21 00:00:00
abstract::MS is a disease that patients can suffer from over several decades. The effects of ageing are therefore likely to have a bearing on the natural history of the disease and the manner in which it is treated. In this review we consider how age affects remyelination, a spontaneously occurring regenerative process that fol...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2008.04.027
更新日期:2008-11-15 00:00:00
abstract:INTRODUCTION:Reports regarding outcome of Guillain-Barré syndrome (GBS) are usually limited to relatively short follow-up. We assessed the occurrence of fatigue and correlated it to clinical measures in patients who had suffered from GBS over 20 years previously. METHODS:We contacted 24 patients with GBS requiring in-...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.01.024
更新日期:2012-05-15 00:00:00
abstract::We studied neurite regeneration in MRL-lpr/lpr mice, a murine model of systemic lupus erythematosus, using a culture system to investigate the influences of immunological abnormalities on neurons. The regeneration of cultured dorsal root ganglion (DRG) neurons from MRL-lpr/lpr mice was delayed compared with control MR...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)05216-1
更新日期:1997-07-01 00:00:00
abstract:BACKGROUND:Endocrine dysfunction is known to occur in various infectious diseases of the brain. The neuroendocrine dysfunction is not well studied in patients of Tuberculous meningitis (TBM). In this study, we aimed at knowing pattern of endocrine dysfunction in newly diagnosed patients of tuberculous meningitis, struc...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2017.06.015
更新日期:2017-08-15 00:00:00
abstract::A method is described which characterises the conduction deficit of a nerve in terms of the distribution of the refractory periods of transmission (RPTs) of its constituent fibres. The RPT is a particularly sensitive index of conduction deficit and measures the ability of a nerve fibre to conduct pairs of closely spac...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(80)90200-2
更新日期:1980-11-01 00:00:00
abstract::Out of 66 patients who were diagnosed as suffering from polymyalgia rheumatica (PMR; n = 40), temporal arteritis (AT; n = 14) or both (n = 12) in a 6.5 year period (incidence 3.4/100,000 per year), 9 died and 49 were followed up for an average period of 28 months. Exacerbations of the illness (n = 24) and complication...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/0022-510x(92)90105-t
更新日期:1992-08-01 00:00:00
abstract::We describe a case of a 50-year-old man with bilateral shoulder girdle weakness caused by anterior subdural hygroma secondary to a previous spontaneous CSF leak. The CSF leak occurred and resolved 16 years prior to presenting with a 6-year progressive painless, asymmetric proximal muscle weakness involving both upper ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.029
更新日期:2011-06-15 00:00:00
abstract:PURPOSE:To prospectively evaluate the usage of intra-voxel incoherent motion (IVIM) magnetic resonance imaging (MRI) parameters in grading and differentiating histological subtypes of meningiomas and in comparing their ability with the traditional diffusion-weighted imaging (DWI) parameter (apparent diffusion coefficie...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.11.037
更新日期:2017-01-15 00:00:00
abstract::Traumatic brain injury (TBI) is a major cause of disability or death worldwide, especially in the young. Thus, effective medication with few side effects needs to be developed. This work aimed to explore the potential benefits of formononetin (FN) on TBI rodent model and to discuss the regarding mechanism. These findi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.12.027
更新日期:2014-03-15 00:00:00
abstract::Cranial dystonia is normally considered as a pure movement disorder. Sensory symptoms have not received much attention, but we found ill-defined pain, discomfort, distortion of sensory modalities, 'phantom' kinetic or postural sensations in the orofacial areas subsequently involved by the dyskinesia in all of 11 conse...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(93)90318-s
更新日期:1993-06-01 00:00:00
abstract::A procedure for placing a crush lesion in the sciatic and tibial nerve of the rat based on anatomical landmarks is described. These crush lesions are used to study the process of regeneration of peripheral nervous tissue and the beneficial effects of melanocortins on speed and quality of nerve regeneration. A new prec...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(86)90109-7
更新日期:1986-07-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Patent foramen ovale (PFO) can be diagnosed by contrast-enhanced transcranial Doppler (cTCD), but no definitive criteria exist to correlate the results obtained from cTCD with diagnoses of PFO obtained by contrast-enhanced transesophageal echocardiography (cTEE). The aim of the present study was ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.02.003
更新日期:2010-06-15 00:00:00
abstract:OBJECTIVES:Central autonomic impairment is frequent in ischemic stroke at acute or chronic stages. The mechanism by which these symptoms occur in patients with ischemic stroke has not been elucidated. This study sought to investigate cardiovascular autonomic function in patients with different subtypes of post-acute is...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.11.036
更新日期:2014-02-15 00:00:00
abstract::The mechanisms primarily responsible for the degenerative processes occurring in dystrophic skeletal muscle remain unresolved. The identification of the mechanisms that lead to the complete sparing of extraocular muscle in dystrophinopathies is of particular interest. A number of studies have provided evidence to sugg...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:
更新日期:1996-08-01 00:00:00
abstract:OBJECTIVE:Examine the relative importance of several risk factors for progression, in a large sample of MS patients. METHODS:Using a retrospective design in a single university-based MS treatment center, we studied 207 patients with relapsing remitting MS diagnosed, treated at our center, evaluated within one year of ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.02.008
更新日期:2010-05-15 00:00:00
abstract::The primary angiitis of the central nervous system (PACNS) is a rare and potentially fatal form of vasculitis with unknown etiology. Headache and encephalopathy are the most frequent symptoms. Neuroimaging plays an important role in the diagnosis, but the pattern of abnormal findings is not specific. In some cases bra...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.05.014
更新日期:2011-08-15 00:00:00
abstract:OBJECTIVE:GM1 ganglioside has been suggested as a treatment for Parkinson's disease (PD), potentially having symptomatic and disease modifying effects. The current pilot imaging study was performed to examine effects of GM1 on dopamine transporter binding, as a surrogate measure of disease progression, studied longitud...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/j.jns.2015.06.028
更新日期:2015-09-15 00:00:00
abstract:BACKGROUND:The epidemiology and pathophysiology of iNPH remain unclear. We aimed to investigate the incidence of iNPH in elderly inhabitants of the community, and to identify how ventriculomegaly develops on brain MRIs and how symptoms develop in iNPH patients. METHODS:In 2000, 350 inhabitants, all 70-year-olds living...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.01.033
更新日期:2014-04-15 00:00:00
abstract::Multiple sclerosis is a chronic inflammatory disease of the white and grey matter which results in irrevocable axonal and neuronal damage. Grey matter injury is widespread and reflects disability to a greater extent than do white matter lesions. Growing understanding of the immunopathology of multiple sclerosis is lea...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/S0022-510X(11)70003-X
更新日期:2011-12-01 00:00:00
abstract::The ability of galantamine (Reminyl) to inhibit the aggregation and toxicity of the beta-amyloid peptide (Abeta) was investigated. Galantamine showed concentration-dependent inhibition of aggregation of both Abeta 1-40 and Abeta 1-42, as determined by an ELISA method. Electron microscope studies of Abeta 1-40 incubate...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.01.024
更新日期:2009-05-15 00:00:00
abstract::The understanding of the genetic basis of neurological disorders has grown rapidly in the last two decades. Despite the genomic heterogeneity within African populations, large-scale candidate gene or linkage and exome studies are lacking. However, current knowledge on neurogenetics in African populations is limited an...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2016.05.006
更新日期:2016-07-15 00:00:00
abstract:OBJECTIVES:MRI assessments were correlated with serial Combined Mini-Mental Cognitive Capacity Screening Examinations (CMC). Vascular-MCI (VMCI), Neurodegenerative MCI (NMCI) and Parkinson-Lewy body MCI (PLB-MCI) were compared during conversions to dementia. Mild cognitive impairments (MCI) are identifiable prodromes f...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.01.016
更新日期:2007-06-15 00:00:00
abstract::Human leptomeningeal (arachnoid and pia mater) cells in culture have been demonstrated in replicated studies to express typical neuronal proteins such as neurofilament protein and neuron-specific enolase. In addition, they can express glial fibrillary acidic protein. The present study examines the possibility that neu...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)00189-x
更新日期:1996-12-01 00:00:00
abstract::This paper describes 2 brothers with increasingly severe exercise-induced muscle pain and stiffness, beginning in adolescence. Histochemical studies showed that myoadenylate deaminase activity was absent in the propositus, but present in his younger brother. Biochemical examination of muscle homogenates confirmed thes...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(82)90086-7
更新日期:1982-01-01 00:00:00
abstract::Naturally occurring botulinum toxin (BoNT) serotypes have different pharmacological features of therapeutic and aesthetic interest. This phase 1, double-blind, placebo-controlled study (EudraCT: 2016-002609-20) assessed safety, tolerability and pharmacodynamics (PD) of the first recombinant BoNT serotype E (rBoNT-E) v...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jns.2019.116516
更新日期:2019-12-15 00:00:00