DOCSLIB.ORG

Opmaak 1 5/10/11 12:33 Pagina 563

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Opmaak 1 5/10/11 12:33 Pagina 563 finsterer-_Opmaak 1 5/10/11 12:33 Pagina 563 Acta Orthop. Belg. , 2011, 77 , 563-582 REVIEW ARTICLE Orthopaedic abnormalities in primary myopathies Josef FiNStErEr , Walter StrOBl From Krankenanstalt Rudolfstiftung and Orthopaedic Hospital Speising, Vienna, Austria Orthopaedic abnormalities are frequently recognised Keywords : myopathy ; muscular dystrophy ; neuro - in patients with myopathy but are hardly systemati - muscular disorder ; orthopaedic disorders ; surgery. cally reviewed with regard to type of myopathy, type of orthopaedic problem, and orthopaedic manage - ment. this review aims to summarize recent findings and current knowledge about orthopaedic abnormal - List Of abbreviatiOns ities in these patients, their frequency, and possible therapeutic interventions. AMC Arthrogryposis multiplex congenita a MeDLine search for the combination of specific BMD Becker muscular dystrophy terms was carried out and appropriate articles CCD Central core disease were reviewed for the type of myopathy, types of CMD Congenital muscular dystrophy orthopaedic abnormalities, frequency of orthopaedic CMP Congenital myopathy abnormalities, and possible therapeutic interven - DHS Dropped head syndrome tions. DMD Duchenne muscular dystrophy Orthopaedic abnormalities in myopathies can be EDMD Emery-Dreifuss muscular dystrophy most simply classified according to the anatomical FHl1 Four-and-a-half Lin11, isl-1, Mec-3-domain 1 location into those of : the spine, including dropped gene head, camptocormia, scoliosis, hyperlordosis, hyper - FSH Facioscapulohumeral muscular dystrophy kyphosis, or rigid spine ; the thorax, including pectus lGMD limb girdle muscular dystrophy excavatum (cobbler’s chest), anterior/posterior flat - lMNA lamin A/C tening, or pectus carinatum (pigeon’s chest) ; the MD1 Myotonic dystrophy 1 limb girdles, including scapular winging and pelvic MD Muscular dystrophy deformities ; and the extremities, including con - MP Myopathy tractures, hyperlaxity of joints, and hand or foot MYH Myosin heavy chain deformities. these orthopaedic abnormalities can be NMDs Neuromuscular disorders most frequently found in arthrogryposis, muscular PVCr Posterior vertebral column resection dystrophies, congenital myopathies, myofibrillar myopathies, and myotonic dystrophies. Occasionally, they also occur in metabolic myopathies or other I Josef Finsterer, MD, PhD, Neurologist. types of myopathy. Most of the orthopaedic abnor - Krankenanstalt Rudolfstiftung, Vienna and Danube Univer - malities are sufficiently accessible to conservative or sity Krems, Vienna, Austria. surgical orthopaedic treatment. I Walter Strobl, MD, Orthopaedic Surgeon. Orthopaedic abnormalities have major implications Orthopaedic Hospital Speising, Vienna, Austria. in the management and outcome of myopathy Correspondence : Josef Finsterer, Postfach 20, 1180 Vienna, patients ; they should be closely monitored and treat - Austria. E-mail : [email protected] ed on time. © 2011, Acta Orthopædica Belgica. No benefits or funds were received in support of this study Acta Orthopædica Belgica, Vol. 77 - 5 - 2011 finsterer-_Opmaak 1 5/10/11 12:33 Pagina 564 564 J. FiNStErEr , W. StrOBl rYr1 ryanodine receptor 1 table i. — Orthopaedic problems in myopathies SEPN1 Selenoprotein 1 tPM tropomyosin Spine USS Universal spine system – Dropped head syndrome VC Vital capacity – Bent-spine syndrome (camptocormia) VCP Valosin-containing protein – Scoliosis – Hyperlordosis – Hyperkyphosis (gibbus) intrODUCtiOn – rigid spine Shoulder girdle though myopathies are per definition disorders – Scapula alata exclusively affecting the skeletal muscles, they fre - thorax (chest) quently cause secondary problems in other organs – Cobbler’s chest (pectus excavatum) or systems as well. this is particularly the case for – Anterior/posterior flattening of chest cavity – Pectus carinatum (pigeon’s chest) the cerebrum and the heart. in addition to these Pelvic deformities organs patients with myopathy frequently develop – Anterior/posterior tilt orthopaedic abnormalities and in single cases, such – Pelvic obliquity as arthrogryposis multiplex congenita (AMC, – Windblown (windswept) hip Extremities multiple joint contractures and talipes present at – Contractures of distal / proximal joints, small / large birth) or Escobar syndrome (multiple pterygium joints, spine (rigid spine) syndrome), the orthopaedic abnormality dominates – Joint hypermotility / hyperlaxity the phenotype (table i) (84,116 ). Since orthopaedic – luxation of joints, hips problems can substantially influence daily living, – Hand / foot deformities they are a major issue in the management of these – tip-toe, pes equinovarus (clubfoot, talipes), pes patients. the following review aims to summarize planovalgus the current knowledge and recent findings about orthopaedic abnormalities in patients with myopathies, their frequency in these patients and problems, frequency of orthopaedic problems, and possi - frequently utilized therapeutic interventions. ble therapeutic options in these patients. MetHODs resULts A MEDliNE search for the terms “spinal deformity”, “dropped head”, “camptocormia”, “bent spine”, “hyper - reviewing the papers selected according to the lordosis”, “hyperkyphosis”, “rigid spine”, “thoracic criteria mentioned above, it turned out that a num - deformity”, “pectus excavatum” (“cobbler’s chest”), ber of different orthopaedic abnormalities occur in “pectus carinatum” (“pigeon’s chest”), “scapula alata”, patients with myopathies. Generally, a mobility “scapular winging”, “winging scapula”, “scapulopexy” unit, such as a joint or the intervertebral discs, may (fixation of the scapula to the thorax), “scapulothoracic undergo pathological alterations, which lead to arthrodesis” (fixation of the shoulder blade to the complete immobility of the unit, decreased motility, thoracic wall), “pelvic obliquity”, “pelvic tilt”, “wind - or hypermotility. these changes most frequently blown hip”, “windswept hip”, “contracture”, “contrac - include irregular positioning or dislocation of the tures”, “genu recurvatum”, “genu valgus”, “genu varus”, combined elements. rarely, there may be deformi - “tip-toe walking”, “joint laxity”, “pes varus”, “pes val - ties, which are not accompanied by impaired motil - gus”, “pes cavus”, “pes equinovarus”, “talipes”, “pes planovalgus”, “Achilles tendon contracture”, and ity of the affected joints. Accordingly, orthopaedic “arthrogryposis” in combination with “myopathy”, abnormalities in myopathies include : spinal abnor - “myopathies”, “neuromuscular disorder”, and “skeletal malities, such as dropped head syndrome (DHS), muscle” was carried out and appropriate articles camptocormia, scoliosis, hyperlordosis, hyper - reviewed for the type of myopathy, types of orthopaedic kyphosis, or rigid spine ; thoracic deformities or Acta Orthopædica Belgica, Vol. 77 - 5 - 2011 finsterer-_Opmaak 1 5/10/11 12:33 Pagina 565 OrtHOPAEDiC ABNOrMAlitiES iN PriMArY MYOPAtHiES 565 impaired mobility of the thorax, such as pectus table ii. — Myopathies associated with excavatum, pectus carinatum ; shoulder girdle or dropped head syndrome pelvic girdle abnormalities, such as scapular wing - Muscular dystrophies ing (scapula alata), anterior / posterior pelvic tilt, – FSH* pelvic obliquity or windblown (windswept) hip ; – laminopathy limb abnormalities, such as contractures of small or – CMD (lMNA) large joints with or without deformities of the Congenital myopathies limbs, hypermobility or hyperlaxity of joints with – Nemaline myopathy – Congenital myopathy or without habitual subluxation or luxation of Myotonic myopathies joints, or hand or foot deformities (table i). Most – Myotonic dystrophy type 1# deformities of the extremities result from a combi - – Myotonic dystrophy type 2# nation of muscle weakness and imbalance, and sur - Metabolic myopathies gical procedures aim at correcting these deformities – Mitochondrial myopathy* – Acid maltase deficiency (Pompe) and rebalancing existing musculature by release or Unclassified myopathies transfer (4). – isolated cervical myopathy * : variable childhood or adult onset, # : predominantly adult spine onset. Dropped head syndrome DHS, also known as cervical kyphosis or floppy has been described so far are listed in table ii. head syndrome, is characterized by the inability of therapeutic approaches for DHS in primary the patient to voluntarily lift his head from the chin- myopathies include physiotherapy, neck extension on-chest position into the upright position due to orthoses, analgetics, or surgical stabilisation of weakness of the head extensor muscles. Weakness the cervical spine in selected cases. the latter may of these muscles may be complete or incomplete, be carried out by instrumented, posterior, cervico- allowing at least a limited head lift. Dropping of the thoracic spinal fusion (37 ). head may either go along with neck pain or may be painless. DHS was initially described in myasthenia Bent spine syndrome (camptocormia) gravis and motor neuron disease. Before the head Camptocormia, also known as bent spine drop can be attributed to a primary muscle disease, syndrome or progressive lumbar kyphosis, is an a number of differentials, including secondary acquired postural abnormality with forward-flexed muscle disorders and central nervous system (CNS) posture, leading to prominent lumbar kyphosis. disease have to be excluded. Secondary muscle Camptocormia usually increases when the patient disorders, which need to be considered in paediatric gets up or walks (5,21 ). Occasionally,
Load more

Recommended publications
  • Statin Myopathy: a Common Dilemma Not Reflected in Clinical Trials
    REVIEW CME EDUCATIONAL OBJECTIVE: Readers will assess possible statin-induced myopathy in their patients on statins CREDIT GENARO FERNANDEZ, MD ERICA S. SPATZ, MD CHARLES JABLECKI, MD PAUL S. PHILLIPS, MD Internal Medicine Residency Program, Robert Wood Johnson Clinical Scholars Department of Neurosciences, University Director, Interventional Cardiology, The University of Utah, Salt Lake City Program, Cardiovascular Disease Fellow, of California San Diego, La Jolla Department of Cardiology, Scripps Mercy Yale University School of Medicine, New Hospital, San Diego, CA Haven, CT Statin myopathy: A common dilemma not reflected in clinical trials ■■ ABSTRACT hen a patient taking a statin complains Wof muscle aches, is he or she experiencing Although statins are remarkably effective, they are still statin-induced myopathy or some other prob- underprescribed because of concerns about muscle toxic- lem? Should statin therapy be discontinued? Statins have proven efficacy in preventing ity. We review the aspects of statin myopathy that are 1 important to the primary care physician and provide a heart attacks and death, and they are the most guide for evaluating patients on statins who present with widely prescribed drugs worldwide. Neverthe- less, they remain underused, with only 50% of muscle complaints. We outline the differential diagnosis, those who would benefit from being on a statin the risks and benefits of statin therapy in patients with receiving one.2,3 In addition, at least 25% of possible toxicity, and the subsequent treatment options. adults who start taking statins stop taking them 4 ■■ by 6 months, and up to 60% stop by 2 years. KEY POINTS Patient and physician fears about myopathy There is little consensus on the definition of statin-in- remain a key reason for stopping.
    [Show full text]
  • A Acanthosis Nigricans, 139 Acquired Ichthyosis, 53, 126, 127, 159 Acute
    Index A Anti-EJ, 213, 214, 216 Acanthosis nigricans, 139 Anti-Ferc, 217 Acquired ichthyosis, 53, 126, 127, 159 Antigliadin antibodies, 336 Acute interstitial pneumonia (AIP), 79, 81 Antihistamines, 324 Adenocarcinoma, 115, 116, 151, 173 Anti-histidyl-tRNA-synthetase antibody Adenosine triphosphate (ATP), 229 (Anti-Jo-1), 6, 14, 140, 166, 183, Adhesion molecules, 225–226 213–216 Adrenal gland carcinoma, 115 Anti-histone antibodies (AHA), 174, 217 Age, 30–32, 157–159 Anti-Jo-1 antibody syndrome, 34, 129 Alanine aminotransferase (ALT, ALAT), 16, Anti-Ki-67 antibody, 247 128, 205, 207, 255 Anti-KJ antibodies, 216–217 Alanyl-tRNA synthetase, 216 Anti-KS, 82 Aldolase, 14, 16, 128, 129, 205, 207, 255, 257 Anti-Ku antibodies, 163, 165, 217 Aledronate, 325 Anti-Mas, 217 Algorithm, 256, 259 Anti-Mi-2 Allergic contact dermatitis, 261 antibody syndrome, 11, 129, 215 Alopecia, 62, 199, 290 antibodies, 6, 15, 129, 142, 212 Aluminum hydroxide, 325, 326 Anti-Myo 22/25 antibodies, 217 Alzheimer’s disease-related proteins, 190 Anti-Myosin scintigraphy, 230 Aminoacyl-tRNA synthetases, 151, 166, 182, Antineoplastic agents, 172 212, 215 Antineoplastic medicines, 169 Aminoquinolone antimalarials, 309–310, 323 Antinuclear antibody (ANA), 1, 141, 152, 171, Amyloid, 188–190 172, 174, 213, 217 Amyopathic DM, 6, 9, 29–30, 32–33, 36, 104, Anti-OJ, 213–214, 216 116, 117, 147–153 Anti-p155, 214–215 Amyotrophic lateral sclerosis, 263 Antiphospholipid syndrome (APS), 127, Antisynthetase syndrome, 11, 33–34, 81 130, 219 Anaphylaxi, 316 Anti-PL-7 antibody, 82, 214 Anasarca,
    [Show full text]
  • Muscle Biopsy Features of Idiopathic Inflammatory Myopathies And
    Autoimmun Highlights (2014) 5:77–85 DOI 10.1007/s13317-014-0062-2 REVIEW ARTICLE Muscle biopsy features of idiopathic inflammatory myopathies and differential diagnosis Gaetano Vattemi • Massimiliano Mirabella • Valeria Guglielmi • Matteo Lucchini • Giuliano Tomelleri • Anna Ghirardello • Andrea Doria Received: 1 August 2014 / Accepted: 22 August 2014 / Published online: 10 September 2014 Ó Springer International Publishing Switzerland 2014 Abstract The gold standard to characterize idiopathic Keywords Inflammatory myopathy Á Autoimmune inflammatory myopathies is the morphological, immuno- myositis Á Histopathology Á Differential diagnosis histochemical and immunopathological analysis of muscle biopsy. Mononuclear cell infiltrates and muscle fiber necrosis are commonly shared histopathological features. Introduction Inflammatory cells that surround, invade and destroy healthy muscle fibers expressing MHC class I antigen are Idiopathic inflammatory myopathies (IIM) are a heteroge- the typical pathological finding of polymyositis. Perifas- neous group of acquired muscle diseases, which have dis- cicular atrophy and microangiopathy strongly support a tinct clinical, pathological and histological features [1, 2]. diagnosis of dermatomyositis. Randomly distributed The most common IIM seen in clinical practice can be necrotic muscle fibers without mononuclear cell infiltrates separated into four categories including polymyositis (PM), represent the histopathological hallmark of immune-med- dermatomyositis (DM), immune-mediated necrotizing iated necrotizing myopathy; meanwhile, endomysial myopathy (NM) and sporadic inclusion body myositis inflammation and muscle fiber degeneration are the two (sIBM) [1, 3]. main pathological features in sporadic inclusion body In the diagnostic workup of an inflammatory myopathy, myositis. A correct differential diagnosis requires immu- muscle biopsy is an indispensable and sensitive tool for nopathological analysis of the muscle biopsy and has establishing the diagnosis.
    [Show full text]
  • SUPPLEMENTARY MATERIAL Supplementary 1. International
    SUPPLEMENTARY MATERIAL Supplementary 1. International Myositis Classification Criteria Project Steering Committee Supplementary 2. Pilot study Supplementary 3. International Myositis Classification Criteria Project questionnaire Supplementary 4. Glossary and definitions for the International Myositis Classification Criteria Project questionnaire Supplementary 5. Adult comparator cases in the International Myositis Classification Criteria Project dataset Supplementary 6. Juvenile comparator cases in the International Myositis Classification Criteria Project dataset Supplementary 7. Validation cohort from the Euromyositis register Supplementary 8. Validation cohort from the Juvenile dermatomyositis cohort biomarker study and repository (UK and Ireland) 1 Supplementary 1. International Myositis Classification Criteria Project Steering Committee Name Affiliation Lars Alfredsson Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden Anthony A Amato Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, USA Richard J Barohn Department of Neurology, University of Kansas Medical Center, Kansas City, USA Matteo Bottai Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden Matthew H Liang Division of Rheumatology, Immunology and Allergy, Brigham and Women´s Hospital, Boston, USA Ingrid E Lundberg (Project Director) Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden Frederick W Miller Environmental
    [Show full text]
  • Eosinophilic Fasciitis: Typical Abnormalities
    Diagnostic and Interventional Imaging (2015) 96, 341—348 REVIEW /Muskuloskeletal imaging Eosinophilic fasciitis: Typical abnormalities, variants and differential diagnosis of fasciae abnormalities using MR imaging a,∗ b,c a T. Kirchgesner , B. Dallaudière , P. Omoumi , a a a J. Malghem , B. Vande Berg , F. Lecouvet , d e a F. Houssiau , C. Galant , A. Larbi a Service de radiologie, Département d’imagerie musculo-squelettique, Cliniques Universitaires Saint-Luc, avenue Hippocrate 10-1200, Brussels, Belgium b Département d’imagerie, centre hospitalier universitaire Pellegrin, place Amélie-Léon-Rabat, 33000 Bordeaux, France c Clinique du sport de Bordeaux-Mérignac, 2, rue Négrevergne, 33700 Mérignac, France d Service de Rhumatologie, Cliniques Universitaires Saint-Luc, avenue Hippocrate 10-1200 Brussels, Belgium e Service d’anatomo-pathologie, Cliniques Universitaires Saint-Luc, avenue Hippocrate 10-1200, Brussels, Belgium KEYWORDS Abstract Eosinophilic fasciitis is a rare condition. It is generally limited to the distal parts of Fascia; the arms and legs. MRI is the ideal imaging modality for diagnosing and monitoring this condi- Fasciitis; tion. MRI findings typically evidence only fascial involvement but on a less regular basis signal Eosinophilic; abnormalities may be observed in neighboring muscle tissue and hypodermic fat. Differential Shulman; diagnosis of eosinophilic fasciitis by MRI requires the exclusion of several other superficial and MRI deep soft tissue disorders. © 2015 Éditions franc¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved. Eosinophilic fasciitis is a rare condition that was first described by Shulman in 1974 [1]. Magnetic resonance imaging (MRI) is the ideal imaging modality both for diagnosing and monitoring this condition. MRI examination typically evidences only fascial involvement but on a less regular basis signal abnormalities may be observed in neighboring muscle tissue and hypodermic fat.
    [Show full text]
  • Inclusion Body Myositis: a Case with Associated Collagen Vascular Disease Responding to Treatment
    J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.3.270 on 1 March 1985. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry 1985;48:270-273 Short report Inclusion body myositis: a case with associated collagen vascular disease responding to treatment RJM LANE, JJ FULTHORPE, P HUDGSON UK From the Regional Neurological Centre, Newcastle General Hospital, Newcastle-upon-Tyne, elec- SUMMARY Patients with inclusion body myositis demonstrate characteristic histological and muscle and are generally considered refractory to treatment. tronmicroscopical abnormalities in autoimmune A patient with inclusion body myositis is described with evidence of associated disease, who responded to steroids. muscles. He felt that his legs were quite normal. He denied guest. Protected by copyright. The diagnosis of inclusion body myositis depends symptoms. There was no relevant family or of the characteristic any sensory ultimately on the demonstration drug history. dis- intracytoplasmic and intranuclear filamentous inclu- On examination, he had a prominent bluish/purple sions, and cytoplasmic vacuoles originally described colouration of the knuckles, thickening of the skin on the by Chou in 1968.' However, reviews of reported dorsum of the hands and a slight heliotrope facial rash. The features which facial muscles were slightly wasted and he had marked cases have also emphasised clinical sternomastoids, deltoids, appear to distinguish inclusion body myositis from weakness and wasting of the Prominent among spinatti, biceps and triceps, with relative preservation of other forms of polymyositis.2-7 distal muscles. All upper limb reflexes were grossly these are the lack of associated skin changes or other bulk, power and to diminished or absent.
    [Show full text]
  • NEUROLOGY NEUROSURGERY & PSYCHIATRY Editorial
    Journal ofNeurology, Neurosurgery, and Psychiatry 1991;54:285-287 285 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.4.285 on 1 April 1991. Downloaded from Joural of NEUROLOGY NEUROSURGERY & PSYCHIATRY Editorial The idiopathic inflammatory myopathies and their treatment The inflammatory myopathies are the largest group of As new knowledge has accumulated over the course of acquired myopathies of adult life and may also occur in the last 10 years, it has become increasingly clear that there infancy and childhood. They have in common the presence are distinct pathological and immunological differences of inflammatory infiltrates within skeletal muscle, usually between polymyositis on the one hand and dermato- in association with muscle fibre destruction. They can be myositis on the other, though in some cases there is clearly subdivided into those which are due to known viral, an overlap between the two conditions. In polymyositis bacterial, protozoal or other microbial agents and those in there is usually scattered necrosis of single muscle fibres which no such agent can be identified and in which which appear hyalinised in the early stages and are immunological mechanisms have been implicated.' The subsequently invaded by mononuclear phagocytic cells. latter group includes polymyositis, dermatomyositis and Regenerating fibres are usually seen singly or in small inclusion body myositis. The evidence for an autoimmune groups distributed focally and randomly throughout the aetiology consists of: 1) an association with other auto- muscle. The inflammatory cell infiltrate is predominantly immune diseases; 2) serological tests which reflect an intrafascicular (endomysial) surrounding muscle fibres altered immune state; and 3) the responsiveness of rather than in the interfascicular septa, though perivascular polymyositis and dermatomyositis, if not of the inclusion infiltrates may also be found; the cellular infiltrate consists body variety, to immunotherapy.2 Polymyositis may rarely mainly of lymphocytes, plasma cells and macrophages.
    [Show full text]
  • EM Guidemap - Myopathy and Myoglobulinuria
    myopathy EM guidemap - Myopathy and myoglobulinuria Click on any of the headings or subheadings to rapidly navigate to the relevant section of the guidemap Introduction General principles ● endocrine myopathy ● toxic myopathy ● periodic paralyses ● myoglobinuria Introduction - this short guidemap supplements the neuromuscular weakness guidemap and offers the reader supplementary information on myopathies, and a short section on myoglobulinuria - this guidemap only consists of a few brief checklists of "causes of the different types of myopathy" that an emergency physician may encounter in clinical practice when dealing with a patient with acute/subacute muscular weakness General principles - a myopathy is suggested when generalized muscle weakness involves large proximal muscle groups, especially around the shoulder and proximal girdle, and when the diffuse muscle weakness is associated with normal tendon reflexes and no sensory findings - a simple classification of myopathy:- Hereditary ● muscular dystrophies ● congenital myopathies http://www.homestead.com/emguidemaps/files/myopathy.html (1 of 13)8/20/2004 5:14:27 PM myopathy ● myotonias ● channelopathies (periodic paralysis syndromes) ● metabolic myopathies ● mitochondrial myopathies Acquired ● inflammatory myopathy ● endocrine myopathies ● drug-induced/toxic myopathies ● myopathy associated with systemic illness - a myopathy can present with fixed weakness (muscular dystrophy, inflammatory myopathy) or episodic weakness (periodic paralysis due to a channelopathy, metabolic myopathy
    [Show full text]
  • Full Application Instructions and Review Procedure NOTE: Full Application Is by Invitation Only After Review of Pre-Application
    ORPHAN DISEASE CENTER MILLION DOLLAR BIKE RIDE PILOT GRANT PROGRAM The ODC MDBR Pilot Grant Program provides a one‐year grant to support research related to a rare disease represented in the 2020 Million Dollar Bike Ride. Number of awards and dollar amounts vary per disease based on fundraising totals by each disease team. Eligibility All individuals holding a faculty‐level appointment at an academic institution or a senior scientific position at a non-profit institution or foundation are eligible to respond to this RFA. Letter of Interest Instructions: Please visit our website to submit your Letter of Interest (LOI), which can also be found here. This one-page LOI is due no later than Friday, September 18, 2020 by 8pm (EST). Full Application Instructions and Review Procedure NOTE: Full Application is by invitation only after review of Pre-Application Proposal Due Date: Monday, October 26, 2020 no later than 8pm (EST) Full application documents are to be uploaded on our website, by invitation only. FORMAT for documents: Font and Page Margins: Use Arial typeface, a black font color, and a font size of 11 points. A symbol font may be used to insert Greek letters or special characters. Use 0.5 inch margins (top, bottom, left, and right) for all pages, including continuation pages. Print must be clear and legible; all text should be single-spaced. Header: There should be a header at the top right on all pages of the PDF indicating the full name of the PI (e.g., PI: Smith, John D.). For your convenience, a continuation page template is included at the end of the application document.
    [Show full text]
  • Myositis 101
    MYOSITIS 101 Your guide to understanding myositis Patients who are informed, who seek out other patients, and who develop helpful ways of communicating with their doctors have better outcomes. Because the disease is so rare, TMA seeks to provide as much information as possible to myositis patients so they can understand the challenges of their disease as well as the options for treating it. The opinions expressed in this publication are not necessarily those of The Myositis Association. We do not endorse any product or treatment we report. We ask that you always check any treatment with your physician. Copyright 2012 by TMA, Inc. TABLE OF CONTENTS contents Myositis basics ...........................................................1 Diagnosis ....................................................................5 Blood tests .............................................................. 11 Common questions ................................................. 15 Treatment ................................................................ 19 Disease management.............................................. 25 Be an informed patient ............................................ 29 Glossary of terms .................................................... 33 1 MYOSITIS BASICS “Myositis” means general inflammation or swelling of the muscle. There are many causes: infection, muscle injury from medications, inherited diseases, disorders of electrolyte levels, and thyroid disease. Exercise can cause temporary muscle inflammation that improves after rest. myositis
    [Show full text]
  • Connective Tissue 5.2.04
    Other Connective Tissue Diseases Chester V. Oddis, MD Thomas A. Medsger, Jr, MD Arthur Weinstein, MD Contents 1. Undifferentiated Connective Tissue Disease 2. Idiopathic Inflammatory Myopathy 3. Scleroderma 4. Sjögren’s Syndrome 5. References OTHER CONNECTIVE TISSUE DISEASES 1 1. Undifferentiated Connective Tissue Disease Table 1 The American College of Rheumatology (ACR) has published criteria for several different diseases Clinical Features and Autoantibody Findings commonly referred to as connective tissue disease Possibly Specific for a Defined CTD (CTD). The primary aim of such classification crite - ria is to ensure the comparability among CTD stud - ies in the scientific community. These diseases Clinical Feature include rheumatoid arthritis (RA), systemic sclero - sis (SSc), systemic lupus erythematosus (SLE), Malar rash polymyositis (PM), dermatomyositis (DM), and Sjögren’s syndrome (SS). These are systemic Subacute cutaneous lupus rheumatic diseases which reflects their inflamma - tory nature and protean clinical manifestations with Sclerodermatous skin changes resultant tissue injury. Although there are unifying immunologic features that pathogenetically tie Heliotrope rash these separate CTDs to each other, the individual disorders often remain clinically and even serologi - Gottron’s papules cally distinct. Immunogenetic data and autoanti - body findings in the different CTDs lend further Erosive arthritis support for their distinctive identity and often serves to subset the individual CTD even further, as seen with the myositis syndromes, SLE and SSc. In other cases, it remains difficult to classify individuals Autoantibody with a combination of signs, symptoms, and labora - tory test results. It is this group of patients that have Anti-dsDNA an “undifferentiated” connective tissue disease (UCTD), or perhaps more accurately, an undifferen - Anti-Sm tiated systemic rheumatic disease.
    [Show full text]
  • Occupational Diseases
    OCCUPATIONAL DISEASES OCCUPATIONAL DISEASES ОДЕСЬКИЙ ДЕРЖАВНИЙ МЕДИЧНИЙ УНІВЕРСИТЕТ THE ODESSA STATE MEDICAL UNIVERSITY Áiáëiîòåêà ñòóäåíòà-ìåäèêà Medical Student’s Library Започатковано 1999 р. на честь 100-річчя Одеського державного медичного університету (1900–2000 рр.) Initiated in 1999 to mark the Centenary of the Odessa State Medical University (1900–2000) 1 OCCUPATIONAL DISEASES Recommended by the Central Methodical Committee for Higher Medical Education of the Ministry of Health of Ukraine as a manual for students of higher medical educational establishments of the IV level of accreditation using English Odessa The Odessa State Medical University 2009 BBC 54.1,7я73 UDC 616-057(075.8) Authors: O. M. Ignatyev, N. A. Matsegora, T. O. Yermolenko, T. P. Oparina, K. A. Yarmula, Yu. M. Vorokhta Reviewers: Professor G. A. Bondarenko, the head of the Department of Occupational Diseases and Radiation Medicine of the Donetzk Medical University named after M. Gorky, MD Professor I. F. Kostyuk, the head of the Department of Internal and Occupational Diseases of the Kharkiv State Medical University, MD This manual contains information about etiology, epidemiology, patho- genesis of occupational diseases, classifications, new methods of exami- nation, clinical forms and presentation, differential diagnosis, complica- tions and treatment. It includes the questions of prophylaxis, modern trends in treatment according to WHO adopted instructions, working capacity expert exam. The represented material is composed according to occupational dis- eases study programme and it is recommended for the students of higher medical educational establishments of the IV accreditation standard and doctors of various specialities. Рекомендовано Центральним методичним кабінетом з вищої медичної освіти МОЗ України як навчальний посібник для студентів вищих медичних навчальних закладів IV рівня акредитації, які опановують навчальну дисципліну англiйською мовою (Протокол № 4 від 24.12.2007 р.
    [Show full text]