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Total 48 results found since Jan 2013.
Oral cyclophosphamide in treatment of patients with refractory idiopathic inflammatory myopathies: a retrospective observational study
We report the first cohort study of PO CYC use in IIM patients with severe , treatment refractory disease. Further trials are needed to verify these results as well as to evaluate long-term safety outcomes.
Source: Clinical Rheumatology - July 3, 2018 Category: Rheumatology Source Type: research
Case of Anti–Single Recognition Particle–Mediated Necrotizing Myopathy After Influenza Vaccination
We reported a 28-year-old healthy woman presented with subacute onset ascending muscle weakness 2 weeks after an annual influenza vaccination. Cerebral Spinal Fluid study showed normal cell counts with elevated protein and nerve conduction study showed reduced diffuse compound muscle action potential amplitudes suggesting a diagnosis of Guillain–Barré syndrome. Despite treatment using intravenous immunoglobulin, her condition continued to get worse with new bulbar and respiratory muscle weakness. Eventually, the diagnosis of anti–single recognition particle–mediated necrotizing myopathy was made based on elevated cr...
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Case Review Source Type: research
Sporadic late-onset nemaline myopathy: clinical, pathology and imaging findings in a single center cohort
AbstractSporadic late-onset nemaline myopathy (SLONM) is a rare acquired myopathy characterized by rapid-onset proximal weakness in late adulthood, and the presence of nemaline bodies on muscle biopsy. In recent years, several therapeutic interventions, including immunomodulating agents and autologous stem cell transplantation, have shown variable degrees of efficacy in different patients, but no consensus has been reached to allow an effective tailoring of treatments in this severe disease. We performed a retrospective evaluation of clinical, pathological, laboratory, muscle MRI, and follow-up data of SLONM patients diagn...
Source: Journal of Neurology - January 22, 2018 Category: Neurology Source Type: research
Dysphagia secondary to focal inflammatory myopathy and consequent dorsiflexion of the tongue in a dog
A 14‐month‐old female pitbull terrier mix was presented for evaluation of dysphagia of 8 months’ duration secondary to intermittent dorsiflexion of the tongue apex. Physical and neurological examinations were unremarkable with the exception of the dorsiflexed tongue. Serum creatine kinase activity was increased (703 IU/L, reference interval: 55 to 257 IU/L), and electromyography of the tongue demonstrated areas of fibrillation potentials. Histopathology of the tongue showed myopathic changes with excessive variability in myofibre size and endomysial fibrosis. Cytochemical stains verified mixed mononuclear cells ...
Source: The Journal of Small Animal Practice - January 1, 2018 Category: Veterinary Research Authors: P. C. Str øm, S. L. Marks, J. A. Rivera, G. D. Shelton Tags: CASE REPORT Source Type: research
How can we effectively address the paraneoplastic dermatomyositis: Diagnosis, risk factors and treatment options.
CONCLUSIONS: Timely diagnosis coupled with a treatment plan focused on muscular endurance and improvement of skin lesions and other symptoms offer a favorable response to therapy along with the achievement of a higher quality of life for these patients.
PMID: 28952230 [PubMed - in process]
Source: Journal of B.U.ON. - September 29, 2017 Category: Cancer & Oncology Tags: J BUON Source Type: research
Immune Myopathy With Perimysial Pathology Associated With Interstitial Lung Disease and Anti-EJ Antibodies
Conclusions: Our case adds to the growing spectrum of inflammatory myopathies and highlights the importance of performing a comprehensive, multisystem workup.
Source: Journal of Clinical Neuromuscular Disease - May 24, 2017 Category: Neurology Tags: Case Review Source Type: research
Monoclonal gammopathy with both nemaline myopathy and amyloid myopathy
We report a monoclonal gammopathy patient with nemaline and amyloid myopathy.• Muscle biopsy revealed interstitial amyloid deposition and clusters of rods.• Immunohistochemistry revealed sarcolemmal immunoreaction for the kappa light chain.• The patient responded well to intravenous immuno globulin (IVIG) and prednisone.
Source: Neuromuscular Disorders - May 11, 2017 Category: Neurology Authors: Min Wang, Lin Lei, Hai Chen, Li Di, Mi Pang, Yan Lu, Lu Lu, Xin-Ming Shen, Yuwei Da Source Type: research
Tanshinol Alleviates Osteoporosis and Myopathy in Glucocorticoid-Treated Rats
In this study, we aimed to investigate whether tanshinol has potential therapeutic effects against glucocorticoid-induced osteoporosis and glucocorticoid-induced myopathy. Ninety-six female Sprague-Dawley rats were randomly assigned to five groups: a control group, a model group, and three model groups treated with 25 or 50 mg/kg of tanshinol, or calcitriol. All model groups received prednisone acetate for 90 days to induce glucocorticoid-induced osteoporosis. Afterwards, all animals underwent a surgical procedure to induce bone defects at the right proximal tibia. Prednisone treatment was stopped after surgery, but tans...
Source: Planta Medica - April 20, 2017 Category: Drugs & Pharmacology Authors: Chen, Guanghua Zhang, Xinle Lin, Han Huang, Guizhi Chen, Yahui Cui, Liao Tags: Original Papers Source Type: research
Neuromuscular complications occurring in solid tumor cohort treated with PD-1 inhibitors (P6.176)
Conclusions:Immune mediated neuromuscular adverse events from PD-1 inhibitor therapy are diverse, can affect any part of the nervous system and have an unpredictable time of onset. Although relatively rare, they are likely to be encountered with increasing frequency as PD-1 inhibitors become widely used. Clinicians need to keep a high suspicion for these disorders. The neurological deficits can evolve rapidly and may be life threatening. With prompt recognition and treatment, neurological outcomes are generally favorable.Disclosure: Dr. Kao has nothing to disclose. Dr. Liao has nothing to disclose. Dr. Markovic has nothing...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Kao, J., Liao, B., Markovic, S. N., Naddaf, E., Staff, N., Klein, C., Hammack, J., Sandroni, P., Finnes, H., Mauermann, M. Tags: Neurologic Complications of Cancer Source Type: research
A Case of new onset antibody-positive myasthenia gravis in a patient treated with pembrolizumab for melanoma. (P2.122)
Conclusions:Nivolumab and Ipilimumab have been associated with several cases of MG. Pembrolizumab was associated with MG in 3 published cases of patients with melanoma. Two patients were previously diagnosed with MG and had exacerbations after pembrolizumab. They responded well to a combination of IVIG, plasmapharesis and prednisone. The third patient had a new onset antibody-negative MG but failed to respond to the same treatments. Our patient had new onset antibody-positive MG.Disclosure: Dr. Alnahhas has nothing to disclose. Dr. Vota has nothing to disclose. Dr. Wong has nothing to disclose.
Source: Neurology - April 17, 2017 Category: Neurology Authors: Alnahhas, I., Vota, S., Wong, J. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research
Clinical Dermatomyositis Associated with Anti-HMG-CoA Reductase Antibody Positive Immune Mediated Necrotizing Myopathy: A Case Report (P2.125)
Conclusions:Statins have been previously linked to clinical dermatomyositis. Here we report the first known case of anti-HMGCR antibody confirmed immune mediated necrotizing myopathy with clinical features of dermatomyositis, yet pathologic findings consistent with necrotizing myopathy.Disclosure: Dr. Lavian has nothing to disclose. Dr. Mozaffar has received personal compensation for activities with Sanofi Genzyme, Grifols, Amicus, Biomarin, Idera Pharmaceuticals and Ultragenyx. Dr. Mozaffar has received research support from Cytokinetics, Alexion, Amicus, Biomarin, Grifols, GlaxoSmithKline, Ultragenyx, and Novartis. Dr. G...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Lavian, M., Mozaffar, T., Goyal, N. 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
First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial
This study is registered with ClinicalTrials.gov, number NCT00784589. Findings Between May 10, 2010, and Dec 7, 2012, we enrolled 91 patients and randomly assigned 90 to treatment (90 were analysed; 1 patient withdrew consent before the random assignment). At month 24, 41 (89%) of 46 patients assigned to rituximab plus short-term prednisone were in complete remission off-therapy versus 15 (34%) of 44 assigned to prednisone alone (absolute difference 55 percentage points, 95% CI 38·4–71·7; p<0·0001. This difference corresponded to a relative risk of success of 2·61 (95% CI 1·71–3·99, p<0·0001), corr...
Source: The Lancet - March 23, 2017 Category: Journals (General) Source Type: research
Necrotising myopathy associated with anti-signal recognition particle (anti-SRP) antibody.
CONCLUSIONS: Unlike the cases described in the literature, there was a high frequency of extra-muscular symptoms in the patients studied. Moreover, one fifth of patients had previous exposure to statin use. There was a high relapse rates, but with good clinical and laboratory recovery, especially with pulse therapy regimen of methylprednisolone and/or IVIg.
PMID: 28281460 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - March 12, 2017 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
Pneumocystis jiroveci pneumonia in rheumatic disease: a 20-year single-centre experience.
CONCLUSIONS: PJP portends high mortality yet is a largely preventable complication of rheumatic disease treatment. Consideration to initiate prophylaxis should be made for patients exceeding the daily 20 mg prednisone threshold, and those receiving cyclophosphamide.
PMID: 28134084 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - February 1, 2017 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
