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Muscle & Nerve

Shree Pandya, Katherine A James, Christina Westfield, Shiny Thomas, Deborah J Fox, Emma Ciafaloni, Richard T Moxley
INTRODUCTION: As the DMD population ages, it is essential that we understand the late stage health profile and provide appropriate care to this emerging population. METHODS: A descriptive study to document the health profile of a cohort of adults with DMD using data from the Muscular Dystrophy Surveillance Tracking and Research network (MD STARnet). Data included information collected from Arizona, Colorado, Iowa, Georgia and 12 counties in western New York on individuals born since January 1982 and followed through December 2012...
March 15, 2018: Muscle & Nerve
Karlien Mul, Chad Heatwole, Katy Eichinger, Nuran Dilek, William B Martens, Baziel G M van Engelen, Rabi Tawil, Jeffrey M Statland
INTRODUCTION: Electrical impedance myography (EIM) is a non-invasive technique for measuring muscle composition and a potential physiological biomarker for facioscapulohumeral muscular dystrophy (FSHD). METHODS: Thirty-two genetically confirmed and clinically affected FSHD participants underwent EIM in 7 muscles bilaterally. Correlations between EIM and baseline clinical measures were used to select EIM parameters of interest in FSHD, and EIM and clinical measures were followed for 1 year...
March 15, 2018: Muscle & Nerve
Jenny L Davies, JaNean K Engelstad, Linde E Gove, Linda K Linbo, Rickey E Carter, Christopher Lynch, Nathan P Staff, Christopher J Klein, P James B Dyck, David N Herrmann, Peter J Dyck
INTRODUCTION: For sequential and somatotopic assessment of small fiber neuropathy (SFN), heat pain (HP) tests of hypoalgesia might be used instead of decreased counts of epidermal nerve fibers (ENFs), but then healthy subject reference values of HP thresholds are needed. METHODS: Using the CASE IVc system, HP thresholds of hypoalgesia were estimated for ten unilateral sites and counts of ENFs for four of them in healthy subjects. RESULTS: In healthy subjects, small but statistically significant differences of both HP thresholds of hypoalgesia and counts of ENFs were observed among tested sites...
March 15, 2018: Muscle & Nerve
Heather Lindstrom, Nigel L Ashworth
INTRODUCTION: This study aims to evaluate the usefulness of electrodiagnostic (EDX) studies in terms of the patient's diagnosis and subsequent management, and to identify patient groups where EDX is particularly useful. METHODS: The records of new patients referred to a single tertiary hospital EDX laboratory during one calendar year were reviewed to determine if EDX studies changed the diagnosis and/or management plan. Logistic regression was used to see if there were any factors that were associated with changed diagnosis or management...
March 13, 2018: Muscle & Nerve
Ted M Burns
No abstract text is available yet for this article.
March 13, 2018: Muscle & Nerve
Bülent Cengiz, Metin Mercan, Reha Kuruoğlu
INTRODUCTION: We studied spinal excitability and its relation to motor unit action potential (MUAP) changes in the thenar and hypothenar muscles in ALS patients. METHODS: Quantitative MUAP and peak ratio interference analyses were performed and cutaneous silent period (CutSP) was measured in the abductor digiti minimi (ADM) and first dorsal interosseous (FDI) muscles of ALS patients and controls. RESULTS: ALS patients revealed more prominently increased duration (p=0...
March 10, 2018: Muscle & Nerve
Crystal Jing Jing Yeo, Milvia Y Pleitez
No abstract text is available yet for this article.
March 10, 2018: Muscle & Nerve
Richard A Lewis
No abstract text is available yet for this article.
March 10, 2018: Muscle & Nerve
Vanessa Baute, Vahakn S Keskinyan, Erica R Sweeney, Kayla D Bowden, Allison Gordon, Janet Hutchens, Michael S Cartwright
INTRODUCTION: Magnet therapy has been proposed as a treatment for neurologic conditions. This trial assessed the feasibility and efficacy of a magnet inserted into a wristband for carpal tunnel syndrome (CTS). METHODS: 22 patients with mild-to-moderate CTS were randomized to wear a high-dose or low-dose "sham" magnetic wristband for 6 weeks. The primary outcome was the symptom severity scale (SSS) of the Boston Carpal Tunnel Questionnaire. Secondary measures were nerve conduction studies (NCS), median nerve ultrasound, and compliance...
March 7, 2018: Muscle & Nerve
William B Stoughton, Jianrong Li, Cindy Balog-Alvarez, Joe N Kornegay
Introduction Duchenne muscular dystrophy (DMD) and golden retriever muscular dystrophy (GRMD) are X-linked disorders caused by mutations in the DMD gene. Autophagy was recently identified as a secondary therapeutic target for DMD. We hypothesized that autophagy would be reduced in GRMD. Methods Autophagic gene and protein expression was assessed in normal and GRMD skeletal muscles and correlated with phenotypic biomarkers. Results Muscles were differentially affected. Autophagy gene levels were lower than normal in the GRMD cranial sartorius (CS) but similar in the vastus lateralis (VL)...
March 7, 2018: Muscle & Nerve
Björn Oskarsson, Dan Moore, Tahseen Mozaffar, John Ravits, Martina Wiedau-Pazos, Nicholas Parziale, Nanette C Joyce, Ross Mandeville, Namita Goyal, Merit E Cudkowicz, Michael Weiss, Robert G Miller, Craig M McDonald
INTRODUCTION: More than 90% of amyotrophic lateral sclerosis (ALS) patients have muscle cramps, and evidence-based treatments have not been available. METHODS: A multicenter double-blind placebo-controlled crossover trial of mexiletine 150 mg twice daily was conducted of ALS patients requesting treatment of symptomatic muscle cramps. RESULTS: Muscle cramp frequency was reduced in 18 of 20 patients; 13 reductions were attributed to treatment (P<...
March 6, 2018: Muscle & Nerve
Ikjae Lee, Hani Kushlaf
INTRODUCTION: The aim of this study was to evaluate needle electromyography (EMG) practice patterns among electromyographers in patients taking novel oral anticoagulants (NOACs). METHODS: A survey questionnaire was sent to members of the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) via email weblink. Anonymous survey responses were collected through an online website. RESULTS: 58 AANEM members responded, 28 (48%) of whom worked at a teaching hospital and 30 (52%) in a private setting...
March 6, 2018: Muscle & Nerve
Ileana M Howard, Marla S Kaufman
Telehealth describes the provision of medical services remotely through technology, and may enhance patient access to specialty care services. Although teleneurology has expanded widely since the introduction of telestroke in 1999, telehealth services for outpatients with neuromuscular or musculoskeletal disorders are less widespread. In this narrative review, we will describe the current technology, applications, outcomes, and limitations of this dynamically growing field. Evidence for telehealth applications related to neuromuscular diseases, palliative care, specialized multidisciplinary services, and musculoskeletal care are reviewed...
March 6, 2018: Muscle & Nerve
Farnaz Sinaei, Atena Khodabakhsh, Kamran Alimoghaddam, Shahriar Nafissi
No abstract text is available yet for this article.
March 6, 2018: Muscle & Nerve
Dong Yoon Park, Seok Kang, Jin Seok Jeong, Joon Shik Yoon
INTRODUCTION: The purpose of this study was to investigate the usefulness of the echogenicity (EI) ratio of the thenar to hypothenar muscle measured using ultrasonography in assessing the severity of carpal tunnel syndrome (CTS). METHODS: Fifty-nine hands of 30 patients electrodiagnostically confirmed as having CTS were classified into 3 subgroups (mild, moderate, and severe). The EI of the thenar and hypothenar muscles was measured with ultrasonography, and the EI ratio was calculated in the patients and 13 normal participants (26 hands)...
March 6, 2018: Muscle & Nerve
Nicole M Engel-Nitz, Audra Boscoe, Ryan Wolbeck, Jonathan Johnson, Nicholas J Silvestri
INTRODUCTION: This study assessed the clinical burden of refractory myasthenia gravis (MG), relative to non-refractory MG. METHODS: Rates of myasthenic crises, exacerbations, inpatient hospitalizations, and emergency room (ER) visits over a one-year period were measured for 403 refractory, 3,811 non-refractory, and 403 non-MG control patients from two administrative health plan databases. RESULTS: Compared to non-refractory patients, a significantly greater percentage of refractory patients had at least one myasthenic crisis (21...
February 27, 2018: Muscle & Nerve
Chad E Glasser, Michael R Gartner, Dawn Wilson, Barry Miller, Matthew L Sherman, Kenneth M Attie
INTRODUCTION: ACE-083 is a locally acting follistatin-based therapeutic that binds myostatin and other muscle regulators and has been shown to increase muscle mass and force in neuromuscular disease mouse models. This first-in-human study examined these effects. METHODS: In this phase 1, randomized, double-blind, placebo-controlled, dose-ranging study in healthy postmenopausal women, ACE-083 (50-200 mg) or placebo was administered unilaterally into rectus femoris (RF) or tibialis anterior (TA) muscles as 1 or 2 doses 3 weeks apart...
February 27, 2018: Muscle & Nerve
Gaia Giovannelli, Giorgia Giacomazzi, Hanne Grosemans, Maurilio Sampaolesi
Introduction - Limb-girdle muscular dystrophy type 2E (LGMD 2E) is caused by mutations in the β-sarcoglycan gene, which is expressed in skeletal, cardiac and smooth muscle. β-sarcoglycan deficient (Sgcb-null) mice develop severe muscular dystrophy and cardiomyopathy with focal areas of necrosis. Methods - We performed morphological (histological and cellular characterization) and functional (isometric tetanic force and fatigue) analyses in dystrophic mice. Comparisons studies were carried out in 1-month-old (clinical onset of the disease) and 7-month-old mice among controls (C57/BL6, Rag2/γc-null), immunocompetent and immunodeficient dystrophic mice (Sgcb-null, Sgcb/Rag2/γc-null mice respectively)...
February 24, 2018: Muscle & Nerve
Kush Kapur, Rebecca S Taylor, Kristin Qi, Janice A Nagy, Jia Li, Benjamin Sanchez, Seward B Rutkove
INTRODUCTION: Electrical impedance can be used to estimate cellular characteristics. We sought to determine whether it could be used to approximate myofiber size using standard prediction modeling approaches. METHODS: Forty-four C57BL/6J wild-type immature mice of varying ages underwent electrical impedance myography (EIM) using a needle electrode array placed in the gastrocnemius. Animals were then euthanized and muscle fixed, stained, and myofiber size quantified...
February 24, 2018: Muscle & Nerve
Radhika Raheja, Keren Regev, Brian C Healy, Maria Antonietta Mazzola, Vanessa Beynon, Felipe von Glehn, Anu Paul, Camilo Diaz-Cruz, Taha Gholipour, Bonnie I Glanz, Pia Kivisakk, Tanuja Chitnis, Howard L Weiner, James D Berry, Roopali Gandhi
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a debilitating neurologic disorder with poor survival rates and no clear biomarkers for disease diagnosis and prognosis. METHODS: We compared serum miRNA expression from patients with ALS to healthy controls and patients with multiple sclerosis and Alzheimer's disease. We also correlated miRNA expression in cross-sectional and longitudinal cohorts of ALS patients with clinical parameters. RESULTS: We identified 7 microRNAs (miR-192-5p, miR-192-3p, miR-1, miR-133a-3p, miR-133b, miR-144-5p and miR-19a-3p) that were upregulated and 6 microRNAs (miR-320c, miR-320a, let-7d-3p, miR-425-5p, miR-320b and miR-139-5p) that were downregulated in ALS patients compared to healthy controls, Alzheimer's disease and multiple sclerosis patients...
February 21, 2018: Muscle & Nerve
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