DOCSLIB.ORG

Diagnosis and Treatment of Pediatric Acute Transverse Myelitis Ayesha Ahmad, MD; Luis Seguias, MD; and Kathryn Ban, MD

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Diagnosis and Treatment of Pediatric Acute Transverse Myelitis Ayesha Ahmad, MD; Luis Seguias, MD; and Kathryn Ban, MD FEATURE Diagnosis and Treatment of Pediatric Acute Transverse Myelitis Ayesha Ahmad, MD; Luis Seguias, MD; and Kathryn Ban, MD cute transverse myelitis is a diagnosis. In addition to the clinical ticosteroids, plasma exchange (PLEX), rare, focal immune-mediated evaluation, magnetic resonance imag- intravenous immunoglobulin (IVIG), Adisease that affects the sensory ing (MRI) and cerebrospinal fluid (CSF) cytotoxic drugs, and other immuno- and motor pathways of the spinal cord. testing are common diagnostic tools modulatory agents; however, the use of It is characterized by a sudden clinical used as a first step in diagnosis. steroids followed by PLEX is the most onset of neurological motor dysfunction Children with ATM undergo differ- widely accepted treatment regimen. associated with sensory loss and auto- ent therapeutic modalities such as cor- ATM is commonly a monophasic ill- nomic disturbances. The following tetrad of symptoms has been classically described in acute transverse myelitis (ATM): 1) weakness in the arms/legs; 2) sensory symptoms such as numbness or tingling; 3) pain and discomfort; and 4) bladder dysfunc- tion and/or bowel motility problems. The etiology of ATM is diverse and includes infectious, post-infectious, vas- cular, collagen/autoimmune, neoplastic, and paraneoplastic causes. Clinical in- terview and careful neurological exami- nation are essential in making an ATM Ayesha Ahmad, MD, is an Assistant Professor of Pediatrics, University of Texas Southwestern Medical Center. Luis Seguias, MD, is an Assistant Professor of Pediatrics, University of Texas South- western Medical Center. Kathryn Ban, MD, is an Assistant Professor of Pediatrics, University of Texas Southwestern Medical Center. Address correspondence to: Luis Seguias, MD, University of Texas Southwestern Medical Center, 5151 Harry Hines Boulevard, Dallas, TX 75390; email: [email protected]. Disclosure: The authors have no relevant fi- nancial relationships to disclose. doi: 10.3928/00904481-20121022-15 © Shutterstock PEDIATRIC ANNALS 41:11 | NOVEMBER 2012 Healio.com/Pediatrics | 477 FEATURE increased drooling and trouble swallow- considered an immunologically “privi- ing. leged” organ. This old concept was based Initial clinical evaluation revealed on four principles: (1) the presence of a child with normal mental status and the blood–brain barrier; (2) the lack of severe truncal hypotonia. Her lower ex- constitutive expression of major histo- tremity muscle strength was markedly compatibility complex (MHC); (3) the decreased bilaterally with no antigrav- absence of lymphatic drainage; and (4) ity movement (grade: 2/5) and her upper the lack of T cell immune surveillance. extremity strength was also decreased However, it is now evident that most but to a lesser degree (grade: 3/5). Oth- of these assumptions were not valid. Re- Images courtesy of Ayesha Ahmad, MD. Reprinted with permission. Images courtesy of Ayesha Figure 1. MRI of brain showing swelling. erwise, vital signs and physical exami- cent studies have shown that activated T nation were unremarkable. Pertinent lymphocytes can penetrate the blood– diagnostic work-up included lumbar brain and the blood–nerve barriers and puncture revealing normal CSF indices, release cytokines that will engineer an and brain and spine MRI that demon- immune-response.2 Also, it has been strated swelling and marked T2 signal demonstrated that the nervous system prolongation of the spinal cord from microglial cells have the potential to act approximately C2 to T2-T3 suggestive as antigen presenters and regulate T-cell of an active inflammatory process (see activity.3 Figures 1 and 2). No optic nerve or brain Current research intends to elucidate demyelinating lesions were observed. the role of immune cells and immuno- She was admitted with the diagnosis of mediators in the initiation and progres- ATM for further diagnostic testing and sion of these neurological disorders.2 treatment. A comprehensive infectious and autoimmune work-up was obtained, HISTORY OF ATM Figure 2. MRI of spine showing marked T2 pro- with the only pertinent finding being a In 1882, H.C. Bastain, MD, reported longation of the spinal cord from C2 to T2-T3. positive parainfluenza virus type 3 on pathologic findings of several autopsies nasopharyngeal polymerase chain reac- from patients who died of “acute my- ness. Nevertheless, a small fraction of tion (PCR) swab. elitis” and divided the cases into those patients may have recurrence. Pediatric The patient was started on high-dose that he thought were due to “thrombotic outcomes are better than in adults, with intravenous corticosteroids followed by events to blood vessels supplying the children often regaining complete func- PLEX therapy per neuroimmunology spinal cord” and those that were due tion. recommendations. After completing five to acute inflammation. The “inflamma- PLEX sessions, she had significant re- tory” cases were postulated to be due to CASE SCENARIO covery of muscle strength, and was able an infectious or an allergic mechanism.4 A 1-year-old, previously healthy His- to sit and stand unassisted. Upon follow- In the early 1920s, health care pro- panic female presented to the emergency up visit 1 month after discharge, she was viders in England and Holland reported department (ED) with acute generalized reported to have a normal neurological multiple cases of “inflammation of the weakness. The sudden neurological exam. spinal cord and brain” after smallpox clinical picture was preceded by a 3-day immunization. At the time, clinical find- history of fever (Tmax: 38.8°C) and a SPECTRUM OF ings were interpreted as an “allergic” 2-week history of rhinorrhea and cough. NEUROIMMUNOLOGICAL post-vaccine complication.5 After picking her up from daycare at DISORDERS Several years later in 1928, Dr. Ford midday, the mother noticed that she re- The term neuroimmunological disor- theorized that many cases of acute my- fused to pull up or walk. The child had der refers to a group of illnesses that are elitis were post-infectious rather than previously been walking independently the result of acquired dysregulation of infectious in nature.6 A new terminology for 6 weeks. The mother tried repeatedly both the immune system and the central was introduced in 1948 by A.I. Suchett- to get her to sit-up alone, but she was un- nervous system (CNS)1 (see Sidebar 1, Kaye, MD, who described a case with able to do so. Also, the patient’s mother page 479). a “band-like” horizontal area of altered expressed concern about her daughter’s Historically, the CNS has long been sensation of the neck and torso. He 478 | Healio.com/Pediatrics PEDIATRIC ANNALS 41:11 | NOVEMBER 2012 FEATURE named this entity “acute transverse my- ATM: 1) weakness in the arms/legs; 2) SIDEBAR 1. elitis.”7 sensory symptoms such as numbness or tingling; 3) pain and discomfort; and 4) Spectrum of Neuro- PATHOGENESIS/PATHOLOGY bladder dysfunction and/or bowel motil- immunologic Disorders ATM is a heterogeneous inflamma- ity problems. Central Nervous System tory disorder of the spinal cord. Using The distribution of these symptoms • Brain acute disseminated immunochemistry techniques, spinal may be either symmetric or asymmetric, encephalomyelitis (ADEM) cord histopathology of ATM patients has affecting either legs, arms, or both. • Multiple sclerosis (MS) demonstrated perivascular infiltration by There is often a clearly defined ros- • Acute transverse myelitis (ATM) T lymphocytes (+CD3 stain) and focal tral border of sensory dysfunction, and • Neuromyelitis optica (NMO) infiltration by macrophages (+HLA-Dr spinal MRI and lumbar puncture often • Optic neuritis (ON) 1,2 stain). These immunopathological show evidence of acute inflammation. • Pediatric autoimmune neuropsychiatric observations substantiate that ATM is Autonomic symptoms are variable, con- disorders associated with streptococcal an immune-mediated inflammatory dis- sisting of bowel or bladder incontinence, infection (PANDAS) ease. increased urinary urgency, difficulty or • Hashimoto’s encephalitis ATM is often preceded by an in- inability to void, incomplete evacuation, • Paraneoplastic encephalomyelitis fectious process, which has led to the or constipation.12 • Rasmussen’s encephalitis hypothesis of microbial superantigen- ATM has an incidence of one to four • Tropical spastic paraparesis mediated immune response. Molecular new cases per million people per year, • Stiff person syndrome “mimicry” by infectious agents has been affecting individuals of all ages with bi- • Vasculitis acknowledged as a pathogenic mecha- modal peaks between the ages of 10 and Peripheral Nervous System nism in neurological disease (eg, Guil- 19 years and 30 and 39 years.12 There • Peripheral nerve chronic inflammatory 9,10 lain-Barré syndrome). In ATM, this may be a third peak involving children demyelinating polyneuropathy idea of cross-reactive immune activation aged younger than 3 years, as evidenced • Acute inflammatory demyelinating poly- against self-tissue is also embraced.2 by a recent study of 47 pediatric cases of neuropathy (Guillain-Barré syndrome) In addition, the humoral reaction to ATM, with 38% of their patients being Neuromuscular Junction microbial molecular “mimicry” could younger than the age of 3.9 It is estimat- • Myasthenia gravis trigger the production of autoautibod- ed that 20% to 30% of ATM cases occur • Lambert-Eaton myasthenic syndrome ies. This autoimmune response against in children. The number of affected male Muscle neuronal surface proteins
Load more

Recommended publications
  • Dermatomyositis: Observations on the Use of Cairo-Glasgow Study Group
    Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Postgraduate Medical Journal (August 1978) 54, 516-527. Dermatomyositis: observations on the use of immunosuppressive therapy and review of literature Cairo-Glasgow Study Group AHMED EL- GHOBAREY GEZA BALINT M.R.C.P. M.D. (Budapest) KAREL DE CEULAER W. CARSON DICK M.D. (Leuven) M.D., M.R.C.P. W. W. BUCHANAN M.D., F.R.C.P. TAHSIN HADIDI* T. A. HASSAN* F.R.C.P. M.R.C.P. The Centre for Rheumatic Diseases, University Department ofMedicine, Royal Infirmary, Protected by copyright. Glasgow, Scotland, and *Maadi Armed Forces Hospital, and Azhar University, Cairo, Egypt Summary The response to therapy and the subsequent Seven young adults, six of whom were male, all clinical course are briefly described as follows. suffering from dermatomyositis unassociated with Patient N.D. was treated with corticosteroids. The malignancy are described. These patients were not course of therapy, clinical response and changes in adequately controlled with high doses of corti- serum muscle enzymes are summarized in Fig. 1. costeroids but all responded when immunosuppressive Intravenous dexamethasone was discontinued at the therapy was also given. nineteenth week and by the end of the twenty-first week the dose of prednisolone was reduced to 10 mg/ day. Introduction http://pmj.bmj.com/ Dermatomyositis (as the name suggests) is a The patient was discharged at the end of the syndrome consisting of polymyositis associ- twenty-fourth week. clinical One year later, the patient had a relapse, the ated with skin lesions (Pearson, 1966a; Currie and subsequent course and response to treatment are Walton, 1971).
    [Show full text]
  • Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD
    Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD Richard K. Vehe, MD,* Mona M. Riskalla, MD* *Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children’s Hospital, Minneapolis, MN Practice Gap Timely and accurate recognition of collagen vascular disorders (CVDs), and implementation of effective screening and referral processes for patients suspected of having a CVD, remain a challenge for many physicians. The result, too often, is unnecessary testing and referrals, and in some cases unnecessary anxiety for physicians, patients, and parents. Objectives After completing this article, readers should be able to: 1. Recognize the common clinical symptoms and signs of systemic lupus erythematosus, dermatomyositis, and scleroderma, and their distinction from common infectious mimics. 2. Recognize the testing that can clarify the likelihood of whether a child has a rheumatic disease, including the limited utility of early serologic testing for autoantibodies. 3. Recognize the prognosis and management objectives for these often- chronic disorders, and practical steps to ensure excellent outcomes. AUTHOR DISCLOSURE Drs Vehe and Riskalla have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/ INTRODUCTION investigative use of a commercial product/ device. Timely and accurate recognition of collagen vascular diseases (CVDs), and ABBREVIATIONS implementation of effective screening and referral processes for patients sus- ANA antinuclear antibody pected of having a CVD, remain challenging for many physicians. The result, too CTD connective tissue disease often, is unnecessary testing with questionable results, leading in some cases to CVD collagen vascular disease unnecessary referrals and anxiety for physicians, patients, and parents.
    [Show full text]
  • Beneath the Surface: Derm Clues to Underlying Disorders
    Christian R. Halvorson, MD; Richard Colgan, MD Department of Family and Beneath the surface: Derm clues Community Medicine, University of Maryland School of Medicine, Baltimore to underlying disorders [email protected] Dermatologic fi ndings are frequent indicators of The authors reported no potential confl ict of interest connective tissue disorders. Here’s what to look for. relevant to this article. any systemic conditions are accompanied by skin PRACTICE manifestations. Th is is especially true for connec- RECOMMENDATIONS Mtive tissue disorders, for which dermatologic fi nd- › When evaluating patients ings are often the key to diagnosis. with suspected cutaneous In this review, we describe the dermatologic fi ndings of lupus erythematosus, use some well-known connective tissue disorders. Th e text and multiple criteria—including photographs in the pages that follow will help you hone your histologic and immuno- diagnostic skills, leading to earlier treatment and, possibly, fl uorescent biopsy fi ndings better outcomes. and American College of Rheumatology criteria—to rule out systemic disease. C Lupus erythematosus: Cutaneous › Cancer screening with a and systemic disease often overlap careful history and physi- Lupus erythematosus (LE), a chronic, infl ammatory autoim- cal examination is recom- mended for all adult patients mune condition that primarily aff ects women in their 20s and whom you suspect of having 30s, may initially present as a systemic disease or in a purely dermatomyositis. C cutaneous form. However, most patients with systemic LE have some skin manifestations, and those with cutaneous › Suspect mixed connective LE often have—or subsequently develop—systemic involve- tissue disease in patients 1 with skin fi ndings charac- ment.
    [Show full text]
  • Dermatomyositis
    DERMATOMYOSITIS http://www.aocd.org Dermatomyositis (DM) is a rare inflammatory muscle disease that affects both the muscles as well as the skin. DM can affect people of all races, sex and age. Although it affects both males and females equally in childhood, it is more common in females in adults. The exact cause of DM is unknown but it is believed to result from an immune-mediated process triggered by outside factors (e.g. malignancy, drugs, and infectious agents) in genetically predisposed individuals. Dermatomyositis can occur with other connective tissue disorders such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma, Sjogren’s syndrome, and mixed connective tissue disease. Patients with dermatomyositis usually present with complaints of tiredness and loss of energy. The skin changes occur before the onset of muscle disease in most patients. The earliest signs of skin manifestations may begin with a red to bluish-purple rash most commonly in the sun exposed areas (face, neck, shoulders, upper chest and back). The eyelids may get the typical purple rash known as the heliotrope rash. Early clinicians thought that this violaceous rash around the eyes reminded them of the color of a heliotrope flower, and thus referred to this as the ‘heliotrope sign’. The knuckles may have purple spots known as ‘Gottron’s papules’. The most important diagnostic feature of skin eruption of DM is poikiloderma, which is the pale, thin skin with blood vessels and dark spots located in the sun exposed areas. Hardened deposits of yellow to white lumps under the skin can develop especially in children and adolescents known as calcinosis.
    [Show full text]
  • Diagnosis and Treatment of Dermatomyositis-Systemic Lupus
    Diagnosis and Treatment of Dermatomyositis-Systemic Lupus Erythematosus Overlap Syndrome Preston Williams1; Benjamin McKinney, MD2 1Texas A&M College of Medicine; 2Baylor University Medical Center Family Medicine Residency Introduction Case Description Discussion Dermatomyositis is an autoimmune condition classically A punch biopsy of her rash showed atrophic epithelium with This case of overlap syndrome between dermatomyositis and characterized by symmetric proximal muscle weakness, dyskeratotic keratinocytes, vacuolar interface changes, superficial systemic lupus erythematosus presents a rare but important inflammatory muscle changes, and dermatologic abnormalities.1 perivascular and lichenoid inflammation, and pigment challenge to the primary care physician. Our patient presented Several studies have shown that the inflammatory myopathies, incontinence consistent with systemic lupus erythematosus initially with arthralgias and fatigue, symptoms more such as dermatomyositis, commonly overlap with other (SLE). The patient was started on prednisone 40 mg daily for a 2- characteristic of systemic lupus erythematosus. However, these connective tissue disorders, significantly complicating the week taper to 10 mg and hydroxychloroquine 200 mg daily with symptoms were followed by a facial rash that involved the diagnosis.2 The reported incidence of overlap syndrome ranges marked improvement in symptoms. Further lab work-up was nasolabial folds and periorbital regions more in line with from 11% to 40% in patients diagnosed with significant
    [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]
  • PATIENT FACT SHEET Myopathies
    Inflammatory PATIENT FACT SHEET Myopathies Inflammatory myopathies are muscle diseases caused skin rashes also. Muscle pain is not a common symptom. by inflammation. They are autoimmune diseases where Some people can have breathing problems. the body’s immune system attacks its own muscles by People of all ages and races may get inflammatory mistake. The most common inflammatory myopathies are myopathies, but they’re rare. Children usually get them polymyositis and dermatomyositis. between ages 5 and 10. Adults usually get these diseases CONDITION Inflammatory myopathies cause muscle weakness, usually between 40 and 50. Women get inflammatory myopathies DESCRIPTION in the neck, shoulders and hips. Dermatomyositis causes twice as often as men. The most common sign of inflammatory myopathies is • Shortness of breath weakness in the large muscles of the shoulders, neck or • Cough hips. Inflammation damages tissue so you lose strength Dermatomyositis causes skin rashes that look like red or in these muscles. Inflammatory myopathies may cause purple spots on the eyelids, or scaly, red bumps on the problems like these: elbows, knuckles or knees. Children may also have white • Trouble climbing stairs, lifting objects over your head spots on their skin called calcinosis or vasculitis, a blood or getting out of a seat vessel inflammation that causes skin lesions. SIGNS/ • Choking while eating or intake of food into the lungs SYMPTOMS Diagnosing inflammatory myopathies starts with (Deltasone, Orasone), to reduce inflammation. Muscle a muscle strength exam. A rheumatologist may also enzymes usually return to normal at 4 to 6 weeks, and do blood tests to measure certain muscle enzymes or strength returns in 2 to 3 months.
    [Show full text]
  • Multiple Myeloma Associated With
    ISSN: 2469-5696 Islam and Kotoucek. Int J Blood Res Disord 2018, 5:029 DOI: 10.23937/2469-5696/1410029 International Journal of Volume 5 | Issue 1 Open Access Blood Research and Disorders RESEARCH ARTICLE Multiple Myeloma Associated with Dermatomyositis: A Short Report and Mini-Review Md Serajul Islam1,2* and Pavel Kotoucek2 Check for 1Department of Haematology, Guys’ & St Thomas’ Hospital, London, UK updates 2Department of Haematology, Broomfield Hospital, Chelmsford, UK *Corresponding author: Md Serajul Islam, Department of Haematology, Guys’ & St Thomas’ Hospital, London, UK; De- partment of Haematology, Broomfield Hospital, Chelmsford, UK, Tel: +44-7769580452, Fax: +44-1245-51-6669, E-mail: [email protected] amyloid deposits in various tissues [3]. The American Abstract Cancer Society has estimated that 30,770 new MM Multiple myeloma (MM) is characterized by the neoplastic proliferation of plasma cell clones that produce monoclo- cases will occur in the United States in 2018, with an nal immunoglobulin. Dermatomyositis (DM), and to a lesser estimated 12,770 deaths [4]. Idiopathic inflammatory extent polymyositis (PM), carry a higher risk of cancer than myopathies (IIM), a heterogeneous group of disorders that of the general population as demonstrated by several characterised by weakness and inflammation of skele- studies with the prevalence being 32% and 15% for DM and PM respectively. The mechanism underlying the association tal muscle, are often associated with malignancies [5]. between idiopathic inflammatory myopathies (IIM) and ma- Polymyositis (PM) and dermatomyositis (DM) are clas- lignancies remains unclear. Both conditions predominantly sified as IIM, with these conditions predominantly af- affect adults. About 15% of DM is refractory to conventional fecting adults [6].
    [Show full text]
  • First Case Report of Association of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Pneumatosis Intestinalis
    Open Access Austin Critical Care Case Reports Case Report First Case Report of Association of Anti-N-Methyl- D-Aspartate Receptor Encephalitis and Pneumatosis Intestinalis Abdul-Aziz R1*, Bout-Tabaku S1, O’Donovan JC2, Sivaraman V1 and Spencer CH1 Abstract 1Department of Pediatric Rheumatology, Ohio State Anti-N-Methyl-D-Aspartate Receptor (anti-NMDA-R) encephalitis is an University, USA immune-mediated syndrome that was first described in 2007. We describe two 2Department of Radiology, Ohio State University, USA patients with anti-NMDAR encephalitis who developed Pneumatosis Intestinalis *Corresponding author: Rabheh Abdul-Aziz, (PI) during the course of their disease. Pneumatosis intestinalis in the setting Department of Pediatric Rheumatology, Ohio State of anti-NMDA-R encephalitis has not previously been reported in the literature. University, 700 Children’s Drive, Columbus, Ohio, 43205, That led to speculation whether the association between anti-N-methyl-D- USA aspartate receptor encephalitis and pneumatosis intestinalis is secondary to immunosuppression, medication effect, or a direct manifestation of auto Received: November 02, 2016; Accepted: January 27, antibodies will require further study. Our patients highlight the occurrence of this 2017; Published: January 30, 2017 rare complication in critically ill patients with NMDA-R encephalitis who usually admit to pediatric intensive care unit. Early recognition and appropriate therapy are essential to recovery in patients who develop this complication. Keywords: Anti-n-methyl-d-aspartate receptor; Pneumatosis intestinalis; Encephalitis Abbreviations history of attention deficit hyperactivity disorder was admitted to the psychiatric unit due to sub-acute altered mental status, auditory and Anti-NMDA-R: Anti-N-Methyl-D-Aspartate Receptor; visual hallucinations, and violent behavior.
    [Show full text]
  • Liver Damage in Patients with Polymyositis and Dermatomyositis
    Original Contribution Kitasato Med J 2016; 46: 40-46 Liver damage in patients with polymyositis and dermatomyositis Tatsuhiko Wada, Gakurou Abe, Takeo Kudou, Eisuke Ogawa, Tatsuo Nagai, Sumiaki Tanaka, Shunsei Hirohata Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine Objective: To clarify the prevalence of liver damage associated with polymyositis (PM) and dermatomyositis (DM) Methods: Forty-two patients with PM/DM were exhaustively studied. Six patients showed liver diseases of known etiology, including drug-induced hepatitis, fatty liver, metastatic liver tumor, primary biliary cirrhosis and autoimmune hepatitis. As a control, 8 patients with miscellaneous diseases who showed elevation of the creatine kinase (CK) due to extreme exercise were studied. Serum levels of aspirate aminotransferase (AST), alanine aminotransferase (ALT) and CK, as well as the ratios of AST/CK, ALT/CK and AST/ALT were evaluated. Results: ALT levels were significantly elevated in PM/DM compared with control group. The ratios of AST/CK and ALT/CK were significantly elevated in PM/DM compared with the control group. AST/ALT ratios in PM/DM were significantly lower than those in control group (P < 0.0001). Twenty- six of 36 patients (72.2%) with PM/DM showed AST/ALT ratios below the minimum value of AST/ ALT ratios in the control patients. Additionally, 10 of 26 patients (38.5%) showed hepatocellular liver damage. There were no significant differences in the levels of AST, ALT or CK, or in the ratios of AST/CK, ALT/CK or AST/ALT between PM and DM. Conclusions: The results indicate that a disproportionate elevation of ALT takes places in patients with PM/DM.
    [Show full text]
  • Polymyositis / Dermatomyositis)
    Myositis (Polymyositis / Dermatomyositis) Myositis is a disease characterized by inflammation of the muscles and is often associated with severe muscle weakness. Myositis can also affect other organ systems including the skin, joints, lungs, heart, and gastrointestinal tract. It is a chronic disease, meaning it lasts a long time. The most common forms of myositis are polymyositis and dermatomyositis. Myositis is a systemic autoimmune disease. This means that the body’s natural immune system does not behave normally. Instead of serving to fight infections such as bacteria and viruses, the body’s own immune system attacks itself. In myositis, autoimmunity may cause the immune system to attack specific muscles resulting in muscle damage and destruction. The immune system may also attack other organs such as the lungs, skin, joints and gastrointestinal tract. What are Some of the Symptoms of Myositis? As an autoimmune disease that mostly targets muscles, myositis most obvious symptoms manifest themselves in muscle fatigue and pain. The disease may have many other symptoms. Common symptoms of myositis include: • Muscle weakness • Muscle pains • Rashes • Fatigue • Weight loss • Low-grade fevers • Arthritis • Color changes of hands and feet with cold exposure (known as Raynaud’s) • Difficulty swallowing • Heartburn • Cough • Shortness of breath Who Gets Myositis? Although myositis is a rare disease, people of all races and ethnic backgrounds get the disease. The peak age of onset is in the 50s, although it can occur at any age. Inclusion body myositis is more common in men, while dermatomyositis and polymyositis are more common in women. What Causes Myositis? The cause of myositis remains unknown.
    [Show full text]
  • A Case of Dermatomyositis with Secondary Sjögren's Syndrome
    162 A Case of Dermatomyositis with Secondary Sjögren’s Syndrome- Diagnosis with Follow-up Study of Technetium-99m Pyrophosphate Scintigraphy Ching-Tang Huang1, Ying-Chu Chen1, Chingtsai Lin2, Yu-Chun Hsiao3, Lai-Fa Sheu4, Min-Chien Tu1 Abstract Purpose: To report a case of dermatomyositis (DM) with secondary Sjögren’s syndrome (SS) and propose the clinical application of technetium-99m pyrophosphate (99mTc-PYP) scan. Case Report: A 50-year-old woman had progressive proximal muscle weakness of bilateral thighs, myalgia, tea-colored urine, and exercise intolerance for 6 months. Physical examination showed malar rash, V-sign, periungual erythema, and mechanic hands. Neurological assessment showed symmetric pelvic-girdle weakness, myopathic face, waddling gait, but preserved deep tendon reflex and sensory functions. DM was diagnosed on the basis of typical rashes and serum creatinine kinase elevation (7397 IU/L). Aside from myopathic symptoms, dry eye and mouth were reported. Thorough autoantibody searches showed positive anti-SSA/Ro antibody (198 U/ml). Both Schirmer's test and sialoscintigraphy were positive, leading secondary SS as diagnosis. Initial 99mTc-PYP scan revealed increased radiouptake in the muscles of bilateral thighs, compatible with clinical assessment. Follow- up scan three months later shows abnormal but attenuated radiouptake at bilateral thighs, in the presence of nearly-complete clinical recovery. Conclusion: DM with secondary SS in adult is a unique disease entity, with predominantly myopathic symptoms and satisfactory therapeutic response as its characteristics. Our serial muscle imaging studies suggest that 99mTc-PYP scan is at once anatomically-specific and persistently-sensitive to microstructural damages within inflammatory muscles, enabling clinician to monitor disease activity and therapeutic response.
    [Show full text]