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Total 94 results found since Jan 2013.

Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance
Purpose of review: Sporadic late-onset nemaline myopathy (SLONM) with monoclonal gammopathy of undetermined significance (MGUS) is a rare subacute progressive muscle disease. The prognosis is poor due to severe respiratory insufficiency. Recently, however, autologous stem-cell transplantation following high-dose melphalan has been shown to be effective unless there is delay before the treatment. Therefore, early recognition of the disease is important. This review gives an overview of recent advances in SLONM–MGUS, which could help to understand clinical and pathological features and treatment. Recent findings: Efficacy...
Source: Current Opinion in Neurology - September 1, 2017 Category: Neurology Tags: NERVE, NEURO-MUSCULAR JUNCTION AND MOTOR NEURON DISEASES: Edited by Jean-Marc Leger Source Type: research

Paraneoplastic Anti-3-hydroxy-3-methylglutary-coenzyme A Reductase Antibody-positive Immune-mediated Necrotizing Myopathy in a Patient with Uterine Cancer.
We report the case of a 69-year-old woman with proximal limb muscle weakness, who received post-operative chemotherapy for uterine cancer. Her serum creatinine kinase level was high (10,779 mg/dL) and a muscle biopsy from her left biceps revealed various sizes of muscle fibers accompanied by necrotic and regenerating fibers. She was positive for anti-3 hydroxy-3-methylglutary-coenzyme A reductase (anti-HMGCR) antibodies, but negative for anti-signal recognition particle (anti-SRP) antibodies. She was diagnosed with immune-mediated necrotizing myopathy (IMNM) and treated with prednisolone. Our findings indicate that not onl...
Source: Internal Medicine - July 20, 2017 Category: Internal Medicine Tags: Intern Med Source Type: research

Amyloid Myopathy Presenting with Dysphagia and an Inclusion Body Myositis Phenotype (P2.119)
Conclusions:Muscle involvement was likely present but not recognized at the time of presentation. Dysphagia is not previously described in amyloid myopathy. Features of the patient’s phenotype, including the preferential involvement of his quadriceps, were suggestive of IBM. Amyloidosis should be considered in patients with this pattern of weakness. Though amyloid deposits are a pathological feature of IBM, its pathogenesis is thought to be distinct from that of myopathy in primary amyloidosis.Disclosure: Dr. Jacobson has nothing to disclose. Dr. Gallagher has nothing to disclose. Dr. Feldman has received personal co...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Jacobson, R., Gallagher, G., Feldman, E. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Amyloid Myopathy as an Inclusion Body Myositis Mimic (P2.127)
Conclusions:Amyloid myopathy may clinically mimic IBM and manifest as the initial presentation of primary systemic amyloidosis. In the absence of clinical and electromyographic myotonia, muscle biopsy is of critical importance in the evaluation of patients with Myopathy Pattern 4.Disclosure: Dr. Hamasaki 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. Pasnoor has nothing to disclose. Dr. Statland has received personal compensation for activities with Sarepta, ATYR Br...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Hamasaki, A., Dimachkie, M., Pasnoor, M., Statland, J., Jawdat, O., Barohn, R., Glenn, M., Herbelin, L. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Rare myopathy associated to MGUS, causing heart failure and responding to chemotherapy
Source: Annals of Hematology - January 4, 2017 Category: Hematology Source Type: research

Chemotherapy-Related Neurotoxicity
Abstract Chemotherapy may have detrimental effects on either the central or peripheral nervous system. Central nervous system neurotoxicity resulting from chemotherapy manifests as a wide range of clinical syndromes including acute, subacute, and chronic encephalopathies, posterior reversible encephalopathy, acute cerebellar dysfunction, chronic cognitive impairment, myelopathy, meningitis, and neurovascular syndromes. These clinical entities vary by causative agent, degree of severity, evolution, and timing of occurrence. In the peripheral nervous system, chemotherapy-induced peripheral neuropathy (CIPN) and myopathy are...
Source: Current Neurology and Neuroscience Reports - July 20, 2016 Category: Neuroscience Source Type: research

Mitochondria: Inadvertent targets in chemotherapy-induced skeletal muscle toxicity and wasting?
Abstract Chemotherapy has been associated with increased mitochondrial reactive oxygen species production, mitochondrial dysfunction and skeletal muscle atrophy leading to severe patient clinical complications including skeletal muscle fatigue, insulin resistance and wasting. The exact mechanisms behind this skeletal muscle toxicity are largely unknown, and as such co-therapies to attenuate chemotherapy-induced side effects are lacking. Here, we review the current literature describing the clinical manifestations and molecular origins of chemotherapy-induced myopathy with a focus on the mitochondria as the target ...
Source: Cancer Chemotherapy and Pharmacology - May 10, 2016 Category: Cancer & Oncology Source Type: research

Coenzyme Q-10 in Human Health: Supporting Evidence?
Authors: Saha SP, Whayne TF Abstract Coenzyme Q-10 (CoQ10) is a widely used alternative medication or dietary supplement and one of its roles is as an antioxidant. It naturally functions as a coenzyme and component of oxidative phosphorylation in mitochondria. Decreased levels have been demonstrated in diseased myocardium and in Parkinson disease. Farnesyl pyrophosphate is a critical intermediate for CoQ10 synthesis and blockage of this step may be important in statin myopathy. Deficiency of CoQ10 also has been associated with encephalomyopathy, severe infantile multisystemic disease, cerebellar ataxia, nephrotic s...
Source: Southern Medical Journal - January 16, 2016 Category: Journals (General) Tags: South Med J Source Type: research

Exacerbation of Dermatomyositis with Recurrence of Rectal Cancer: A Case Report
Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by cutaneous and muscle manifestations. The association between DM and malignancy has been well recognized for many years. The clinical course of paraneoplastic DM may be affected by malignancies, although the cause and effect relationship between exacerbation of DM and cancer progression is uncertain. Herein, we report a 44-year-old woman who presented with progressive DM associated with rectal cancer. After curative resection of rectal cancer, DM symptoms resolved. Three months after surgery, blood test surveillance showed elevation of serum ca...
Source: Case Reports in Oncology - November 10, 2015 Category: Cancer & Oncology Source Type: research

Grade IV myositis: A rare complication of docetaxel
Rajeev Saini, D Chandragouda, Vineet Talwar, Sajjan RajpurohitJournal of Cancer Research and Therapeutics 2015 11(3):664-664An extremely rare case of acute inflammatory myopathy during combination chemotherapy with docetaxel and carboplatin for metastatic breast carcinoma in a 62-year-old diabetic female patient is reported. Despite the early clinical partial response of the underlying malignancy to the chemotherapeutic regimen, the patient developed symmetrical, painful, proximal muscle weakness in the lower limbs with peripheral edema after the administration of the third cycle of treatment. The syndrome regressed after ...
Source: Journal of Cancer Research and Therapeutics - October 9, 2015 Category: Cancer & Oncology Authors: Rajeev SainiD ChandragoudaVineet TalwarSajjan Rajpurohit Source Type: research

The Neuromuscular Features of Light Chain Deposition Disease (P5.056)
CONCLUSIONS: Clinicians need to be aware of LCDD myopathy, especially when amyloidosis is suspected and tissue testing is CR-negative. The generalized fibrillations were nearly gone just one week later, suggesting reversible sarcolemmal disruption possibly related to enhanced light chain renal excretion following aggressive hydration. Study Supported by:Disclosure: Dr. Zhang has nothing to disclose. Dr. Ferrante has nothing to disclose. Dr. Ly has nothing to disclose. Dr. Bertorini has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Zhang, L., Ferrante, M., Ly, H., Bertorini, T. Tags: Biomarkers of Neuromuscular Disease Source Type: research

Dermatomyositis in a Patient with Cholangiocarcinoma Detected by an 18F-Fluorodeoxyglucose ...
We report a 66-year-old woman with metastatic cholangiocarcinoma, initially presented with bilateral proximal muscle uptake on PET-CT without clinical muscle symptoms. The initial interpretation of the high muscle uptake was metastasis to the muscles. However, while awaiting for chemotherapy, muscle weakness evolved and rapidly progressed. The level of creatine phosphokinase was significantly elevated. Electromyography revealed moderate myopathy, and a muscle biopsy showed degenerating myofibers with variable sizes. The diagnosis of paraneoplastic dermatomyositis was made. This case highlights that, although rare, paraneop...
Source: Cancer Research and Treatment - March 17, 2015 Category: Cancer & Oncology Tags: Case Report Source Type: research

Bilateral myositis ossificans of the masseter muscle after chemoradiotherapy and critical illness neuropathy-report of a rare entity and review of literature.
Myositis ossificans in the head and neck is a rare heterotropic bone formation within a muscle. Besides fibrodysplasia ossificans progressiva, traumatic and neurogenic forms are described in the literature. We are presenting the case of a 35-year-old female patient with a very rare form of MO of both masseter muscles after 4 weeks of intensive care because of complications (critical illness neuropathy) after chemotherapy. Therefore, special attention should be paid to surgical trauma. As in the present case, radiotherapy, long-time intubation with immobilization and critical myopathy and neuropathy can cause MO with severe...
Source: Head and Neck Oncology - February 13, 2015 Category: Cancer & Oncology Source Type: research

Two concurrent paraneoplastic syndromes-using occam's razor
A 58 year old lady presented with rapidly progressive symmetrical limb weakness over a few weeks. She had a background of WHO-B2 thymoma diagnosed in 2005 with resultant thymectomy and adjuvant radiotherapy, however relapsed with pleural metastases in 2011, requiring chemotherapy for remission. She became known to Neurological services in 2009 with acetylcholine-receptor antibody Myasthenia Gravis(MG) for which she received 6-weekly intravenous immunoglobulin, the last being 2 weeks prior to presentation. Unusually, she sustained no rapid improvement in weakness, had lost weight, and developed dysphagia. She took no myotox...
Source: Journal of Neurology, Neurosurgery and Psychiatry - September 9, 2014 Category: Neurosurgery Authors: Doshi, A., Wells, G., Bodi, I., Barritt, A., Fisniku, L. Tags: Abstracts Source Type: research