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Total 16 results found since Jan 2013.

Two cases of dermatomyositis associated with neuroendocrine tumors
AbstractDermatomyositis is an idiopathic inflammatory myopathy with cutaneous manifestations, which is associated with several types of malignancies, yet it has been rarely linked to neuroendocrine tumors (NETs). Here we report two cases of dermatomyositis associated with NETs of differing primary sites. Case 1: A 46-year-old female presented with a facial rash and proximal muscle weakness of both extremities. Investigations revealed elevated creatine kinase (CK) and positive anti-transcriptional intermediary factor 1- γ antibody (TIF1γ). The patient had been diagnosed with dermatomyositis and underwent a total body CT s...
Source: International Cancer Conference Journal - August 31, 2023 Category: Cancer & Oncology Source Type: research

Critical illness myopathy and trajectory of recovery in acute kidney injury requiring continuous renal replacement therapy: a prospective observational trial protocol
Introduction Acute kidney injury requiring renal replacement therapy (AKI-RRT) is common in the intensive care unit (ICU) and is associated with significant morbidity and mortality. Continuous RRT (CRRT) non-selectively removes large amounts of amino acids from plasma, lowering serum amino acid concentrations and potentially depleting total-body amino acid stores. Therefore, the morbidity and mortality associated with AKI-RRT may be partly mediated through accelerated skeletal muscle atrophy and resulting muscle weakness. However, the impact of AKI-RRT on skeletal muscle mass and function during and following critical illn...
Source: BMJ Open - May 22, 2023 Category: General Medicine Authors: Teixeira, J. P., Griffin, B. R., Pal, C. A., Gonzalez-Seguel, F., Jenkins, N., Jones, B. M., Yoshida, Y., George, N., Israel, H. P., Ghazi, L., Neyra, J. A., Mayer, K. P. Tags: Open access, Intensive care Source Type: research

The Cure VCP Scientific Conference 2021: Molecular and clinical insights into neurodegeneration and myopathy linked to multisystem proteinopathy-1 (MSP-1)
In this report, we summarize the major topics presented at this conference by a range of experts.PMID:35405261 | DOI:10.1016/j.nbd.2022.105722
Source: Neurobiology of Disease - April 11, 2022 Category: Neurology Authors: Michelle A Johnson Jacob A Klickstein Richa Khanna Yunzi Gou Cure VCP Disease Research Consortium Malavika Raman Source Type: research

OptimisAtion of Diagnostic Accuracy in idioPathic inflammaTory myopathies (ADAPT study): a protocol for a prospective diagnostic accuracy study of multimodality testing in patients suspected of a treatable idiopathic inflammatory myopathy
Introduction Idiopathic inflammatory myopathies (IIMs) excluding inclusion body myositis (IBM) are a group of heterogeneous autoimmune disorders characterised by subacute-onset and progressive proximal muscle weakness, which are frequently part of a multisystem autoimmune disorder. Reaching the diagnosis can be challenging, and no gold standard for the diagnosis of IIM exists. Diagnostic modalities include serum creatine kinase activity, muscle imaging (MRI or ultrasound (US)), electromyography (EMG), myositis autoantibody testing and muscle biopsy. Several diagnostic criteria have been developed for IIMs, varying in repor...
Source: BMJ Open - December 13, 2021 Category: General Medicine Authors: Walter, H. A. W., Kamperman, R. G., Raaphorst, J., Verhamme, C., Koelman, J. H. T. M., Potters, W. V., Hemke, R., Smithuis, F. F., Aronica, E., van Leeuwen, E. M. M., Baars, P. A., de Visser, M., van Schaik, I. N., Bossuyt, P. M. M., van der Kooi, A. J. Tags: Open access, Rheumatology Source Type: research

Autoimmune myopathies
Inclusion body myositis (IBM) is the most common acquired myopathy after the age of 45. The slowly progressive and heterogeneous disorder is a challenge for measuring clinical trial efficacy. One current method for measuring progression utilizes the IBM Functional Rating Scale (FRS). We have found that the hand domain scores in the IBM-FRS do not consistently change until there is extreme loss of grip and finger flexor strength. Therefore, we performed a cross-sectional observational study of 83 IBM and 38 control patients recruited at the 2019 Annual Patient Conference of The Myositis Association.
Source: Neuromuscular Disorders - September 28, 2020 Category: Neurology Authors: A. Lin, C. Siener, A. Faino, M. Seiffert, C. Weihl, L. Wang Source Type: research

Neurological Involvement in Primary Systemic Vasculitis
Conclusion Neurological involvement is a common complication of PSV (Table 1), and neurologists play an important role in the identification and diagnosis of PSV patients with otherwise unexplained neurological symptoms as their chief complaint. This article summarizes the neurological manifestations of PSV and hopes to improve neuroscientists' understanding of this broad range of diseases. TABLE 1 Table 1. Common CNS and PNS involvements of primary systemic vasculitis. Author Contributions SZ conceived the article and wrote the manuscript. DY and GT reviewed and edited the manuscript. All authors ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

Corticospinal Tract Integrity and Long-Term Hand Function Prognosis in Patients With Stroke
Conclusions: The present study showed that CST integrity (at 6 months after onset) in patients with chronic stroke was related to functional hand status. In addition, the mid-pons FA value was more predictive of functional restoration of the hand than the FN or FA value at the pontomedullary junction. These results may be useful in predicting the functional restoration of the hand and understanding the functional prognosis of stroke. Introduction Restoration of hand function is one of the most important goals for patients with stroke (1). Thus, techniques that aid in predicting restoration of hand function are also i...
Source: Frontiers in Neurology - April 14, 2019 Category: Neurology Source Type: research

The natural history of nemaline myopathy
This study represents the first longitudinal examination of NM patients, providing a more accurate estimate of the rate of change and a more precise documentation of the clinical phenotype. Participants were evaluated at family conferences in 2009 and 2016. Physical examinations, pulmonary function testing (PFT) and motor function assessment (MFM20) were performed along with collection of medical history.
Source: Neuromuscular Disorders - September 10, 2017 Category: Neurology Authors: K. Amburgey, S. Hewson, C. Hum, M. Glueck, N. Sultanum, M. Girdea, M. Brudno, C. B önnemann, J. Dastgir, R. Amin, O. Mayer, A. Constantinescu, F. Syed, T. Tran, M. Jain, L. Nelson, A. Beggs, C. Genetti, M. Lawlor, J. Dowling Source Type: research

Performance test evaluation of intra-rater and inter-rater reliability among examiners of histopathological findings in myositis subtypes (P2.132)
Conclusions:This study explores intra and inter reliability for diagnosing DM, PM and NM by muscle biopsy specimens. If consistency can be shown in muscle biopsy interpretation by studying intra-rater and inter-rater reliability in fellows studying neuromuscular medicine, this will help to validate the use of muscle biopsy for diagnostic use in medical professionals with limited training.Disclosure: Dr. Kimple has nothing to disclose. Dr Barohn received personal compensation for activities with Grifols & Genzyme as a member of the Speakers Bureau and NuFactor as a consultant. Dr. Dimachkie has received personal compens...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Kimple, D., Barohn, R., Dimachkie, M., Pasnoor, M., Glenn, M., Herbelin, L., Statland, J., Jawdat, O. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Evaluation of Therapeutic Response Between Idiopathic Inflammatory Myopathies and Autoimmune Necrotizing Myopathies at a Tertiary Neuromuscular Center (P1.113)
Conclusions:Gender and age demographics for IIM and ANM patients in our retrospective review were similar to those found in published literature. The results of therapeutic response for both groups will be presented at the conference.Disclosure: Dr. Murphy has nothing to disclose. Dr. Dimachkie has received personal compensation for activities with Pfizer, Depomed, Merck, CSL-Behring, Nufactor, Biomarin, Baxter and Catalyst as a consultant and/or speaker. Dr. Jawdat has nothing to disclose. Dr. Statland has received personal compensation for activities with Sarepta, ATYR Bristol Meyers Squib, Acceleron, Clinical Mind, and ...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Murphy, R., Dimachkie, M., Jawdat, O., Statland, J., Glenn, M., Barohn, R., Herbelin, L., Pasnoor, M. Tags: Myopathies & amp;amp; Myasthenia Gravis I Source Type: research

Resource utilization in a US-based sample of patients with sporadic inclusion body myositis (sIBM)
Sporadic inclusion body myositis (sIBM) is a progressive, idiopathic inflammatory myopathy characterized by dysphagia, and atrophy and weakness of proximal and distal muscles. This cross-sectional study aims to characterize the socioeconomic burden of sIBM patients in the United States. sIBM patients registered for The Myositis Association 2013 and 2014 annual patient conferences completed the Skeletal Muscle Activity and Resource Tool for sIBM (SMART-sIBM). The SMART-sIBM is a measure of self-reported resource utilization designed to characterize direct and out-of-pocket expenses including items not reimbursed by US third party payers.
Source: Neuromuscular Disorders - September 11, 2015 Category: Neurology Authors: C. DeMuro, V. Williams, R. Goldberg, L. Lowes, M. Price, G. Capkun, V. Barghout, B. Tseng Source Type: research

Physical rehabilitation for critical illness myopathy and neuropathy. An abridged version of Cochrane Systematic Review.
CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: There are no published RCTs or quasi-RCTs that examine whether physical rehabilitation interventions improve activities of daily living for people with CIP and CIM. Large RCTs, which are feasible, need to be conducted to explore the role of physical rehabilitation interventions for people with CIP and CIM. This paper is based on a Cochrane Review published in in the Cochrane Database of Systematic Reviews (CDSR) 2015, Issue 3, DOI: 10.1002/14651858.CD010942.pub2. (see www.thecochranelibrary.com for information). Cochrane Reviews are regularly updated as new evidence emerges and...
Source: Physica Medica - July 9, 2015 Category: Physics Authors: Mehrholz J, Pohl M, Kugler J, Burridge J, Mückel S, Elsner B Tags: Eur J Phys Rehabil Med Source Type: research

Editor’s Message and Issue Highlights—March–April 2015
In this study, the authors specifically examined IA among subjects with obsessive-compulsive disorder (OCD). All enrolled patients with OCD alone (n=27) and OCD and IA (n=11) were treated for OCD for a period of one year. At 12 months, only two of the 11 patients with OCD and IA continued to meet the criteria for IA, and the authors concluded that treatment of the underlying disorder, in this case OCD, improved IA, supporting their hypothesis that IA is a symptom of an underlying disorder and not a primary disorder itself. The authors state several limitations to their study that might impact their conclusions and acknowle...
Source: Innovations in Clinical Neuroscience - April 23, 2015 Category: Neuroscience Authors: ICN Online Editor Tags: Editor's Message: Issue Highlights Amir H. Kalali drug development ICNS Innovations in Clinical Neuroscience ISCTM neurology psychiatry Source Type: research

Physical rehabilitation for critical illness myopathy and neuropathy.
CONCLUSIONS: There are no published RCTs or quasi-RCTs that examine whether physical rehabilitation interventions improve activities of daily living for people with CIP and CIM. Large RCTs, which are feasible, need to be conducted to explore the role of physical rehabilitation interventions for people with CIP and CIM. PMID: 25737049 [PubMed - as supplied by publisher]
Source: Cochrane Database of Systematic Reviews - March 4, 2015 Category: Journals (General) Authors: Mehrholz J, Pohl M, Kugler J, Burridge J, Mückel S, Elsner B Tags: Cochrane Database Syst Rev Source Type: research