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Ashok Kumar, Anshul Goel, Mehul Lapsiwala, Suman Singhal
Systemic rheumatoid vasculitis (SRV) can develop in rheumatoid arthritis of long duration and high disease activity. It most commonly manifests as cutaneous vasculitis and mononeuritis multiplex. This can involve any organ of the body and carries very high mortality. We report a case of a young male who had rheumatoid arthritis for the past 15 years and became refractory to standard drugs and anti-TNF agents. He subsequently developed SRV, which started as mononeuritis multiplex. Disease progressed to result in gangrene of hands and feet despite receiving intravenous cyclophosphamide...
November 2016: Oxford Medical Case Reports
Eoin Mulroy, Sarah Gleeson, Michael James Furlong
Ketoacidosis is an important but under-recognized complication of neuromuscular disease, in particular spinal muscular atrophy. This easily treatable condition is largely overlooked in best practice guidelines, and lack of awareness contributes to adverse outcomes in this patient population. Neuromyopathy associated ketosis should be considered in all patients with severe muscle wasting presenting with an elevated anion gap metabolic ketoacidosis. Treatment is simple, effective, and should be instituted early...
August 30, 2016: Journal of Neuromuscular Diseases
D J Lewis-Smith, J Duff, A Pyle, H Griffin, T Polvikoski, D Birchall, R Horvath, P F Chinnery
OBJECTIVE: To identify the cause of isolated distal weakness in a family with both neuropathic and myopathic features on EMG and muscle histology. METHODS: Case study with exome sequencing in 2 affected individuals, bioinformatic prioritization of genetic variants, and segregation analysis of the likely causal mutation. Functional studies included Western blot analysis of the candidate protein before and after heat shock treatment of primary skin fibroblasts. RESULTS: A novel HSPB1 variant (c...
December 2016: Neurology. Genetics
Yi-Chia Su, Chih-Chien Wu
A 54-year-old woman presented at the emergency department after experiencing lower limb weakness and bilateral ankle pain for 2 days. She had a history of type 2 diabetes mellitus, diabetes mellitus nephropathy with chronic kidney disease, and chronic gouty arthritis. She had received 0.6 mg colchicine orally once or twice daily for 8 months. Four days prior to her emergency department visit, she was discharged from our nephrology ward, where she had been admitted because of a urinary tract infection. During hospitalization, she was treated with intravenous cefazolin for 7 days...
December 2015: Drug Safety—Case Reports
Shweta S Duggal, Chirag Shah
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Neetu Ramrakhiani
No abstract text is available yet for this article.
July 2016: Neurology India
Christina Marciniak, Ashwin Babu, Leda Ghannad, Richard Burnstine, Susan Keeshin
An 83-year-old man with multiple medical problems, including gout, pseudogout, and renal insufficiency, presented with more than a year of proximal weakness. He had an extensive previous medical workup, including a normal creatinine kinase. His weakness persisted despite endurance and strength training. Electrodiagnostic findings were consistent with a myopathy, although without abnormal spontaneous activity and a length-dependent neuropathy. On the basis of these findings, colchicine was discontinued. The patient experienced marked symptomatic improvement within a week...
March 10, 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Lakshmi Vas, Sushama Phanse, Renuka Pai
UNLABELLED: We present a new perspective of neuromyopathy in pancreatic cancer pain (PCP) referral to bodywall; proposal of new rationale to include ultrasound guided dry needling (USGDN) of body wall muscles as an effective adjunct to neurolytic coeliac plexus block (NCPB) or splanchnic nerve radiofrequency ablation (SRF) for comprehensive interventional management. METHODS: PCP response to SRF in 2 patients and NCPB in 3 patients was documented on numerical rating scale (NRS) on post procedure days 3 and 15...
January 2016: Indian Journal of Palliative Care
Djillali Annane
Neuromuscular blocking agents and corticosteroids are widely used in medicine and in particular in the intensive care unit (ICU). Neuromuscular blockade is commonly used to ease tracheal intubation, to optimize mechanical ventilation and oxygenation in acute respiratory disorders such as status asthmaticus and acute respiratory distress syndrome (ARDS), to prevent shivering during therapeutic hypothermia, and also in patients with elevated intracranial pressure. In the ICU, patients with sepsis, ARDS, community-acquired pneumonia, exacerbation of chronic obstructive pulmonary disease, severe asthma, or trauma may receive corticosteroids...
February 2016: Seminars in Respiratory and Critical Care Medicine
P Rocheteau, L Chatre, D Briand, M Mebarki, G Jouvion, J Bardon, C Crochemore, P Serrani, P P Lecci, M Latil, B Matot, P G Carlier, N Latronico, C Huchet, A Lafoux, T Sharshar, M Ricchetti, F Chrétien
Sepsis, or systemic inflammatory response syndrome, is the major cause of critical illness resulting in admission to intensive care units. Sepsis is caused by severe infection and is associated with mortality in 60% of cases. Morbidity due to sepsis is complicated by neuromyopathy, and patients face long-term disability due to muscle weakness, energetic dysfunction, proteolysis and muscle wasting. These processes are triggered by pro-inflammatory cytokines and metabolic imbalances and are aggravated by malnutrition and drugs...
December 15, 2015: Nature Communications
Sergio Bagnato, Chiara Minà, Antonino Sant'Angelo, Cristina Boccagni, Caterina Prestandrea, Alessandra Caronia, Francesco Clemenza, Giuseppe Galardi
Neuropathies may affect heart reinnervation and functional outcome after heart transplantation (HT). In this study, neurological evaluations, standard nerve conduction studies, and electromyography were performed in 32 HT candidates without a previous history of neuromuscular disorder. Ten patients underwent HT and were revaluated 3 months later. We found that before HT 10 (31.3%) patients had sensorimotor polyneuropathy (18.8%) or sensory polyneuropathy (12.5%). After HT, the percentage of patients with a neuromuscular disorder increased to 70%, most of them showing new or worsening neuropathies or neuromyopathies...
March 2016: Neurological Sciences
Samar Medani, Catherine Wall
Colchicine is an approved agent in the management and prophylaxis of gout and familial Mediterranean fever but its therapeutic value is limited by its narrow therapeutic index. Multisystem toxicity is uncommonly reported; and is often associated with renal impairment and/or specific drug interactions. We report two cases of colchicine toxicity marked by severe neuromyopathy in a diabetic with stage 4 chronic kidney disease (CKD) and a renal transplant recipient. Both patients presented with diarrhea, acute on chronic kidney injury and progressive muscle weakness while on colchicine for several weeks or longer...
August 2016: Clinical Nephrology
Jingying Ye
The sleep breathing disorders (SDB) include obstructive sleep apnea (OSA), central sleep apnea disorders, sleep related hypoventilation disorders, and sleep related hypoxemia disorder in international classification of sleep diseases 3rd edition (ICSD-3). Latest diagnosis criteria are introduced. Treatments, which target to Individual contributors, should be applied. Thus identification of the phenotype in patients with OSA is important. The methods of evaluation patients' arousal thresholds, loop gain as well as neuromyopathy in clinical setting are reported...
March 2015: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Margherita Capasso, Maria Vittoria De Angelis, Antonio Di Muzio, Francesca Anzellotti, Laura Bonanni, Astrid Thomas, Marco Onofrj
Akinetic crisis (AC) is a life-threatening complication of parkinsonism characterized by an acute severe akinetic-hypertonic state, consciousness disturbance, hyperthermia, and muscle enzymes elevation. Injectable dopaminomimetic drugs, high-dose methylprednisolone, and dantrolene are advocated as putative specific treatments. The course of the illness is frequently complicated by infections, pulmonary embolism, renal failure, disseminated intravascular coagulation, and cardiac arrhythmias. Critical illness neuromyopathy (CINM) is an acquired neuromuscular disorder characterized by flaccid quadriparesis and muscle enzyme elevation, often occurring in intensive care units and primarily associated with inactivity, sepsis, multiorgan failure, neuromuscular blocking agents, and steroid treatment...
July 2015: Medicine (Baltimore)
Waqar Waheed, Joshua Nickerson, Abiy B Ambaye, Mohamed-Ali Babi, Rup Tandan
OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized idiopathic systemic disorder associated with elevated serum IgG4 level and tissue infiltration by IgG4-positive plasma cells. Multiple neurological manifestations, including peripheral neuropathy, have been described in IgG4-RD. Our objective is to discuss a case report and review of the literature, which would expand the spectrum of IgG4-RD. METHODS: We describe the clinical features and biopsy findings in a patient with IgG4-RD who presented with features suggestive of neuromyopathy in the setting of recurrent pleural effusion and weight loss...
June 2015: Journal of Clinical Neuromuscular Disease
Frédéric Pène, Hafid Ait-Oufella, Fabio Silvio Taccone, Guillaume Monneret, Tarek Sharshar, Fabienne Tamion, Jean-Paul Mira
Experimental research has always been the cornerstone of pathophysiological and therapeutic advances in critical care medicine, where clinical observations and basic research mutually fed each other in a so-called translational approach. The objective of this review is to address the different aspects of translational research in the field of critical care medicine. We herein highlighted some demonstrative examples including the animal-to-human approach to study host-pathogen interactions, the human-to-animal approach for sepsis-induced immunosuppression, the still restrictive human approach to study critical illness-related neuromyopathy, and the technological developments to assess the microcirculatory changes in critically ill patients...
2015: Annals of Intensive Care
Dezső David, Lígia S Almeida, Maristella Maggi, Carlos Araújo, Stefan Imreh, Giovanna Valentini, György Fekete, Irén Haltrich
Carriers of cytogenetically similar, apparently balanced familial chromosome translocations not always exhibit the putative translocation-associated disease phenotype. Additional genetic defects, such as genomic imbalance at breakpoint regions or elsewhere in the genome, have been reported as the most plausible explanation.By means of comprehensive molecular and functional analyses, additional to careful dissection of the t(3;14)(q26.33;q12) breakpoints, we unveil a novel X-linked PGK1 mutation and examine the contribution of these to the extremely severe clinical phenotype characterized by hemolytic anemia and neuromyopathy...
2015: JIMD Reports
Haran Yogasundaram, Brendan N Putko, Julia Tien, D Ian Paterson, Bibiana Cujec, Jennifer Ringrose, Gavin Y Oudit
Drug-induced heart and vascular disease remains an important health burden. Hydroxychloroquine and its predecessor chloroquine are medications commonly used in the treatment of systemic lupus erythematosus, rheumatoid arthritis, and other connective tissue disorders. Hydroxychloroquine interferes with malarial metabolites, confers immunomodulatory effects, and also affects lysosomal function. Clinical monitoring and early recognition of toxicity is an important management strategy in patients who undergo long-term treatment with hydroxychloroquine...
December 2014: Canadian Journal of Cardiology
Lakshmi Vas, Renuka Pai, Nishigandha Khandagale, Manorama Pattnaik
We report a new technique for pulsed radiofrequency (PRF) of the entire nerve supply of the knee as an option in treating osteoarthritis (OA) of knee. We targeted both sensory and motor nerves supplying all the structures around the knee: joint, muscles, and skin to address the entire nociception and stiffness leading to peripheral and central sensitization in osteoarthritis. Ten patients with pain, stiffness, and loss of function in both knees were treated with ultrasonography (USG) guided PRF of saphenous, tibial, and common peroneal nerves along with subsartorial, peripatellar, and popliteal plexuses...
November 2014: Pain Physician
K L Woolson, A Forbes, L Vine, L Beynon, L McElhinney, V Panayi, J G Hunter, R G Madden, T Glasgow, A Kotecha, H C Dalton, L Mihailescu, U Warshow, H S Hussaini, J Palmer, B N Mclean, B Haywood, R P Bendall, H R Dalton
BACKGROUND: Autochthonous (locally acquired) hepatitis E is increasingly recognised in developed countries, and is thought to be a porcine zoonosis. A range of extra-hepatic manifestations of hepatitis E infection have been described, but have never been systematically studied. AIM: To report the extra-hepatic manifestations of hepatitis E virus. METHODS: Retrospective review of data of 106 cases of autochthonous hepatitis E (acute n = 105, chronic n = 1)...
December 2014: Alimentary Pharmacology & Therapeutics
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