DOCSLIB.ORG

ALS, MS and MD | Fact Sheet ALS, MS, and MD: How Do They Differ? Often, the Public Confuses Multiple Sclerosis, Muscular Dystrophy, and Amyotrophic Lateral Sclerosis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
ALS, MS and MD | Fact Sheet ALS, MS, and MD: How Do They Differ? Often, the Public Confuses Multiple Sclerosis, Muscular Dystrophy, and Amyotrophic Lateral Sclerosis AMYOTROPHIC LATERAL SCLEROSIS SOCIETY OF CANADA www.als.ca ALS, MS AND MD | Fact Sheet ALS, MS, and MD: How do they differ? Often, the public confuses Multiple Sclerosis, Muscular Dystrophy, and Amyotrophic Lateral Sclerosis. AMYOTROPHIC LATERAL The disease attacks the protective myelin wastes away, it is replaced by fatty and SCLEROSIS (ALS) covering of the central nervous system, connective tissue. MDs are not diseases ALS is a form of motor neuron disease. causing inflammation and often destroy- of the central or peripheral nervous sys- It involves the loss of the actual motor ing the myelin in patches. The severity of tem, but exclusively of the muscle. nerve cells. The nerves affected are in MS, rate of progression, and specific the spinal cord and those that travel to symptoms cannot be predicted at the Two-thirds of MD strikes children, the voluntary muscles, so there are time of diagnosis. although dystrophies such as opthalmo- symptoms of both central and peripher- plegic (affects muscles of the eyes and al involvement, with weakness and wast- While symptoms may be similar to those pharynx) and late-onset MD strike in ing in arms, legs, and mouth/throat of ALS (weakness and wasting of the adulthood. (bulbar). arms and legs) sensation is often affected in MS because of demyelination in parts MD is inherited or caused through The loss of nerve cells results in second- of the nervous system where sensation is genetic mutation. ary atrophy – or wasting (amyotrophy) – transmitted. of the muscles served by those cells. The Although there is no cure for muscular hardening in ALS involves the lateral The actual word ‘sclerosis’ comes from a dystrophy as yet, physiotherapy and columns of the spine as a secondary Greek word meaning ‘scar’. Scarring in occupational therapy can help people phenomenon, providing the ‘lateral scle- MS distorts or blocks nerve signals. with muscular dystrophy achieve their rosis’ part of the name. maximum level of independence in daily An estimated 50,000 Canadians have living. In some cases, certain surgical Although people with ALS usually have multiple sclerosis. Canada is a high-risk procedures can also improve the quality initial symptoms in one side more than area for MS, which occurs more often in of life for many individuals with muscu- countries which are further from the the other, both sides are involved and the lar dystrophy. effects usually become more symmetrical equator. as the disorder progresses. Sensation is ALS vs MS MS is not a fatal disease. The vast not affected. How to explain the majority of people can be expected to differences Approximately 3,000 Canadians live with live a normal or near-normal lifespan, ALS. Two to three Canadians a day die thanks to improvements in the treatment of symptoms and in other therapies. • ALS is a rapidly progressive and fatal of ALS. Less than 10 per cent of all neuromuscular disease. cases of ALS are genetic. The cause of MS is unknown but researchers are closer to finding the • MS is a scarring and hardening of the Treatments for ALS are being developed answer. sheath around the nerves in the brain, spinal cord, and optic nerve. and while the cause of ALS is unknown, research provides hope for a cure. MUSCULAR DYSTROPHY (MD) • MD is a muscular disorder with Muscular Dystrophy (MD) is the name specific kinds of MD involving different muscles in the body. MULTIPLE SCLEROSIS (MS) of a group of about 40 different muscle Multiple sclerosis (MS) is an unpre- disorders that are characterized by pro- • MD is almost exclusively hereditary. dictable – at times disabling – disease of gressive weakness and wasting of the MS is sporadic with some familial tendency. ALS is sporadic in all but the central nervous system: which con- voluntary muscles that control body 10 per cent of cases. sists of the brain and the spinal cord. movement. As muscle tissue weakens and ALS. Three letters that change people’s lives. FOREVER..
Load more

Recommended publications
  • Clinically Undetected Motor Neuron Disease in Pathologically Proven Frontotemporal Lobar Degeneration with Motor Neuron Disease
    ORIGINAL CONTRIBUTION Clinically Undetected Motor Neuron Disease in Pathologically Proven Frontotemporal Lobar Degeneration With Motor Neuron Disease Keith A. Josephs, MST, MD; Joseph E. Parisi, MD; David S. Knopman, MD; Bradley F. Boeve, MD; Ronald C. Petersen, MD, PhD; Dennis W. Dickson, MD Background: Frontotemporal lobar degeneration with evidence of motor neuron disease. Semiquantitative motor neuron disease (FTLD-MND) is a pathological analysis of motor and extramotor pathological findings entity characterized by motor neuron degeneration and revealed a spectrum of pathological changes underlying frontotemporal lobar degeneration. The ability to detect FTLD-MND. Hippocampal sclerosis, predominantly of the clinical signs of dementia and motor neuron disease the subiculum, was a significantly more frequent occur- in pathologically confirmed FTLD-MND has not been rence in the cases without clinical evidence of motor assessed. neuron disease (PϽ.01). In addition, neuronal loss, gliosis, and corticospinal tract degeneration were less Objectives: To determine if all cases of pathologically severe in the other 3 cases without clinical evidence of confirmed FTLD-MND have clinical evidence of fronto- motor neuron disease. temporal dementia and motor neuron disease, and to de- termine the possible reasons for misdiagnosis. Conclusions: Clinical diagnostic sensitivity for the el- ements of FTLD-MND is modest and may be affected by Method: Review of historical records and semiquantita- the fact that FTLD-MND represents a spectrum of patho- tive analysis of the motor and extramotor pathological find- logical findings, rather than a single homogeneous en- ings of all cases of pathologically confirmed FTLD-MND. tity. Detection of signs of clinical motor neuron disease is also difficult when motor neuron degeneration is mild Results: From a total of 17 cases of pathologically con- and in patients with hippocampal sclerosis.
    [Show full text]
  • Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
    brain sciences Review Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia Timothy Fullam and Jeffrey Statland * Department of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USA; [email protected] * Correspondence: [email protected] Abstract: Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics. Keywords: primary lateral sclerosis; amyotrophic lateral sclerosis; hereditary spastic paraplegia Citation: Fullam, T.; Statland, J. Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper 1. Introduction Motor Neuron Dominant Jean-Martin Charcot (1825–1893) and Wilhelm Erb (1840–1921) are credited with first Amyotrophic Lateral Sclerosis, and describing a distinct clinical syndrome of upper motor neuron (UMN) tract degeneration in Hereditary Spastic Paraplegia. Brain isolation with symptoms including spasticity, hyperreflexia, and mild weakness [1,2]. Many Sci. 2021, 11, 611. https:// of the earliest described cases included cases of hereditary spastic paraplegia, amyotrophic doi.org/10.3390/brainsci11050611 lateral sclerosis, and underrecognized structural, infectious, or inflammatory etiologies for upper motor neuron dysfunction which have since become routinely diagnosed with the Academic Editors: P.
    [Show full text]
  • A Schematic Approach to Hypotonia in Infancy
    Leyenaar.qxd 8/26/2005 4:03 PM Page 397 NEUROLOGY SUBSPECIALTY ARTICLE A schematic approach to hypotonia in infancy JoAnna Leyenaar MD MPH, Peter Camfield MD FRCPC, Carol Camfield MD FRCPC J Leyenaar, P Camfield, C Camfield. A schematic approach Une démarche schématique envers l’hypotonie to hypotonia in infancy. Paediatr Child Health 2005; pendant la première enfance 10(7):397-400. L’hypotonie peut être le signe révélateur de nombreuses maladies Hypotonia may be the presenting sign for many systemic diseases and systémiques ou du système nerveux. Le présent article traite d’une diseases of the nervous system. The present paper discusses a rational, démarche diagnostique rationnelle, simple et précise envers l’hypotonie simple and accurate diagnostic approach to hypotonia in infancy, pendant la première enfance, illustrée par le cas d’une fillette de cinq mois illustrated by the case of a five-month-old infant girl recently referred récemment aiguillée vers le IWK Health Centre de Halifax, en Nouvelle- to the IWK Health Centre in Halifax, Nova Scotia. Key points in the Écosse. Les principaux points de l’anamnèse et de l’examen physique sont history and physical examination are outlined to allow a tailored exposés afin de permettre une exploration personnalisée de la patiente et investigation both for the patient and for other hypotonic infants. A des autres nourrissons hypotoniques. Un exposé sur une importante discussion of an important neuromuscular disease, diagnosed in the maladie neuromusculaire, diagnostiquée chez la patiente, conclut l’article. present patient, concludes the paper. Key Words: Hypotonia; Infant; Spinal muscular atrophy nfants with hypotonia pose challenges for clinicians respiratory syncytial virus-positive bronchiolitis.
    [Show full text]
  • ALS and Other Motor Neuron Diseases Can Represent Diagnostic Challenges
    Review Article Address correspondence to Dr Ezgi Tiryaki, Hennepin ALS and Other Motor County Medical Center, Department of Neurology, 701 Park Avenue P5-200, Neuron Diseases Minneapolis, MN 55415, [email protected]. Ezgi Tiryaki, MD; Holli A. Horak, MD, FAAN Relationship Disclosure: Dr Tiryaki’s institution receives support from The ALS Association. Dr Horak’s ABSTRACT institution receives a grant from the Centers for Disease Purpose of Review: This review describes the most common motor neuron disease, Control and Prevention. ALS. It discusses the diagnosis and evaluation of ALS and the current understanding of its Unlabeled Use of pathophysiology, including new genetic underpinnings of the disease. This article also Products/Investigational covers other motor neuron diseases, reviews how to distinguish them from ALS, and Use Disclosure: Drs Tiryaki and Horak discuss discusses their pathophysiology. the unlabeled use of various Recent Findings: In this article, the spectrum of cognitive involvement in ALS, new concepts drugs for the symptomatic about protein synthesis pathology in the etiology of ALS, and new genetic associations will be management of ALS. * 2014, American Academy covered. This concept has changed over the past 3 to 4 years with the discovery of new of Neurology. genes and genetic processes that may trigger the disease. As of 2014, two-thirds of familial ALS and 10% of sporadic ALS can be explained by genetics. TAR DNA binding protein 43 kDa (TDP-43), for instance, has been shown to cause frontotemporal dementia as well as some cases of familial ALS, and is associated with frontotemporal dysfunction in ALS. Summary: The anterior horn cells control all voluntary movement: motor activity, res- piratory, speech, and swallowing functions are dependent upon signals from the anterior horn cells.
    [Show full text]
  • Motor Neuron Disease and the Elderly
    Neurology 61 Motor neuron disease and the elderly Motor neuron disease is a devastating condition characterised by degeneration of motor nerves. Many of the presenting symptoms, such as fatigue, muscle weakness and difficulty in swallowing have a broad differential diagnoses in the elderly population. Dr Sheba Azam and Professor PN Leigh explain how ensuring quality of life for patients requires preventing unnecessary delay in diagnosis and early referral to an appropriate multidisciplinary team. myotrophic lateral sclerosis (ALS) also cognitive changes. Thus, misdiagnosis as well as known as motor neuron disease (MND) under-investigation has been suggested as possible (the terms are used interchangeably), was causes of an apparent decrease in the incidence of A 4 fi rst described in 1869 by the French neurologist MND in later life . Jean-Martin Charcot1. It is a progressive, fatal neurological disease characterised by degeneration of motor nerve cells in the motor cortex, Prognostic factors corticospinal tract and the spinal cord anterior horn Although the average survival in MND is around cells. The degeneration of motor nerve cells results 36 months, some patients live for 10 years or more. in progressive muscle wasting leading to signifi cant Certain phenotypic variants appear to determine disability and ultimately death. Death usually survival rates. Using information held in a tertiary results from respiratory failure due to weakness of referral MND database, a group of researchers the respiratory muscles. analysed data on onset of disease, site of onset and duration of survival5. The authors concluded that typical MND with bulbar onset, onset later in life Incidence and prevalence or in the defi nite category of El Escorial (where The worldwide incidence of MND is approximately the World Federation of Neurologist meet to a professor of clinical two per 100,000 and the prevalence is four to seven decide diagnostic criteria) at presentation, per 100,000.
    [Show full text]
  • Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis
    1340 • The Journal of Neuroscience, February 11, 2004 • 24(6):1340–1349 Neurobiology of Disease Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis Minh Dang Nguyen,1 Thierry D’Aigle,2 Genevie`ve Gowing,1,2 Jean-Pierre Julien,1,2 and Serge Rivest2 1McGill University Health Center, Centre for Research in Neurosciences, McGill University, The Montreal General Hospital Research Institute, Montre´al, Que´bec H3G 1A4, Canada, and 2Laboratory of Molecular Endocrinology, Laval University Medical Center Research Center and Department of Anatomy and Physiology, Laval University, Sainte-Foy, Que´bec G1V 4G2, Canada Innate immunity is a specific and organized immunological program engaged by peripheral organs and the CNS to maintain homeostasis after stress and injury. In neurodegenerative disorders, its putative deregulation, featured by inflammation and activation of glial cells resulting from inherited mutations or viral/bacterial infections, likely contributes to neuronal death. However, it remains unclear to what extent environmental factors and innate immunity cooperate to modulate the interactions between the neuronal and non-neuronal elements in the perturbed CNS. In the present study, we addressed the effects of acute and chronic administration of lipopolysaccharide (LPS), a Gram-negative bacterial wall component, in a genetic model of neurodegeneration. Transgenic mice expressing a mutant form of the superoxide dismutase 1 (SOD1 G37R) linked to familial amyotrophic lateral sclerosis were challenged intraperitoneally with a single nontoxic or repeated injections of LPS (1 mg/kg). At different ages, SOD1 G37R mice responded normally to acute endotoxemia. Remark- ably, only a chronic challenge with LPS in presymptomatic 6-month-old SOD1 G37R mice exacerbated disease progression by 3 weeks and motor axon degeneration.
    [Show full text]
  • Myasthenia and Related Disorders of the Neuromuscular Junction Jennifer Spillane, David J Beeson, Dimitri M Kullmann
    Myasthenia and related disorders of the neuromuscular junction Jennifer Spillane, David J Beeson, Dimitri M Kullmann To cite this version: Jennifer Spillane, David J Beeson, Dimitri M Kullmann. Myasthenia and related disorders of the neuromuscular junction. Journal of Neurology, Neurosurgery and Psychiatry, BMJ Publishing Group, 2010, 81 (8), pp.850. 10.1136/jnnp.2008.169367. hal-00557404 HAL Id: hal-00557404 https://hal.archives-ouvertes.fr/hal-00557404 Submitted on 19 Jan 2011 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. Myasthenia and related disorders of the neuromuscular junction Jennifer Spillane1, David J Beeson2 and Dimitri M Kullmann1 1UCL Institute of Neurology 2Weatherall Institute for Molecular Medicine, Oxford University Abtract Our understanding of transmission at the neuromuscular junction has increased greatly in recent years. We now recognise a wide variety of autoimmune and genetic diseases that affect this specialised synapse, causing muscle weakness and fatigue. These disorders greatly affect quality of life and rarely can be fatal. Myasthenia Gravis is the most common disorder and is most commonly caused by auto‐antibodies targeting postsynaptic acetylcholine receptors (AChRs). Antibodies to muscle‐specific kinase (MuSK) are detected in a variable proportion of the remainder.
    [Show full text]
  • Combined Web 759..782
    Movement Disorders Vol. 24, No. 5, 2009, pp. 759–782 Ó 2009 Movement Disorder Society Brief Reports Clinical Characteristics of Psychogenic movement disorders (PMDs) are not uncommon in movement disorder clinics.1 PMDs may 49 Patients with Psychogenic phenomenologically mimic almost all movement disor- Movement Disorders in a Tertiary ders. The most common movement disorder is tremor, followed by dystonia and others.2–5 Clinic in Turkey Diagnostic criteria for PMDs was first identified by Fahn and Williams, based on atypical and common Sibel Ertan, MD,1 Derya Uluduz, MD,1 clinical clues.6 Later, other authors described additional 1* 1 Sibel O¨ zekmekc¸i, MD, Gu¨nes Kiziltan, MD, features to distinguish PMD patients from those with 2 1 1 Turan Ertan, MD, Cengiz Yalc¸inkaya, MD, , and neurogenic movement disorders.7–9 ¨ 1 and C¸ igdem Ozkara, MD Because there is no study written in English on any 1Department of Neurology, Cerrahpasa Faculty of Medicine, hospital-based data of PMDs in Turkey, we aimed to Istanbul University, Istanbul, Turkey; 2Department of identify the frequency and phenomenological features Psychiatry, Cerrahpasa Faculty of Medicine, Istanbul of PMDs in our patient population with movement dis- University, Istanbul, Turkey orders. Abstract: Patients admitted to movement disorders outpa- tient unit at a university hospital between January 2002 and June 2007 were screened for psychogenic movement PATIENTS AND METHODS disorders (PMDs). Out of 1,743 patients, 49 patients Patients admitted to our Movement Disorders Unit (2.8%), including four children, were diagnosed to have between January 2002 and June 2007, were screened PMDs. Women to men ratio was 34/15.
    [Show full text]
  • Diagnosis and Treatment of Facioscapulohumeral Muscular Dystrophy: 2015 Guidelines Steven Karceski Neurology 2015;85;E41-E43 DOI 10.1212/WNL.0000000000001865
    PATIENT PAGE Section Editors Diagnosis and treatment of DavidC.Spencer,MD Steven Karceski, MD facioscapulohumeral muscular dystrophy 2015 guidelines Steven Karceski, MD WHAT DID THE AUTHORS STUDY? Dr. Tawil led a in people with FSHD. However, a person with committee of doctors who specialize in diagnosing FSHD could develop heart problems unrelated to and treating facioscapulohumeral muscular dystrophy FSHD. If a person with FSHD developed heart prob- (FSHD). Together, they reviewed published articles lems, he or she would need to see a doctor for an eval- and research in FSHD and similar muscular dystro- uation and treatment. phies. They assembled detailed recommendations Although rare, patients with a low number of about the diagnosis and treatment of people with copies of D4Z4 may develop problems with their FSHD.1 vision. They develop Coats disease, which can be de- tected by an ophthalmologist using special equip- HOW IS FSHD DIAGNOSED? The initial step to the ment called indirect ophthalmoscopy. In short, a diagnosis of FSHD is taking a careful medical history. person who has a low number of copies should be This starts in the doctor’s office. The doctor will ask screened and evaluated for this possibility by a many questions about the person’s weakness: how it trained eye specialist. started, where it is most noticeable, how quickly it is Pain is common in people with FSHD. The pain worsening, and whether there is a family history of occurs in the muscles and bones. It often responds to the same kind of problem. If there is a family history several medications and physical therapy.
    [Show full text]
  • Tremor in X-Linked Recessive Spinal and Bulbar Muscular Atrophy (Kennedy’S Disease)
    CLINICS 2011;66(6):955-957 DOI:10.1590/S1807-59322011000600006 CLINICAL SCIENCE Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy’s disease) Francisco A. Dias,I Renato P. Munhoz,I Salmo Raskin,II Lineu Ce´sar Werneck,I He´lio A. G. TeiveI I Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clı´nicas, Federal University of Parana´ , Curitiba, PR, Brazil. II Genetika Laboratory, Curitiba, PR, Brazil. OBJECTIVE: To study tremor in patients with X-linked recessive spinobulbar muscular atrophy or Kennedy’s disease. METHODS: Ten patients (from 7 families) with a genetic diagnosis of Kennedy’s disease were screened for the presence of tremor using a standardized clinical protocol and followed up at a neurology outpatient clinic. All index patients were genotyped and showed an expanded allele in the androgen receptor gene. RESULTS: Mean patient age was 37.6 years and mean number of CAG repeats 47 (44-53). Tremor was present in 8 (80%) patients and was predominantly postural hand tremor. Alcohol responsiveness was detected in 7 (88%) patients with tremor, who all responded well to treatment with a b-blocker (propranolol). CONCLUSION: Tremor is a common feature in patients with Kennedy’s disease and has characteristics similar to those of essential tremor. KEYWORDS: Kennedy’s disease; X-linked recessive bulbospinal neuronopathy; Spinal and bulbar muscular atrophy; Motor neuron disease; Tremor. Dias FA, Munhoz RP, Raskin S, Werneck LC, Teive HAG. Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy’s disease). Clinics. 2011;66(6):955-957. Received for publication on December 24, 2010; First review completed on January 18, 2011; Accepted for publication on February 25, 2011 E-mail: [email protected] Tel.: 55 41 3019-5060 INTRODUCTION compatible with a long life.
    [Show full text]
  • Motor Neuron Disease Motor Neuron Disease
    Motor Neuron Disease Motor Neuron Disease • Incidence: 2-4 per 100 000 • Onset: usually 50-70 years • Pathology: – Degenerative condition – anterior horn cells and upper motor neurons in spinal cord, resulting in mixed upper and lower motor neuron signs • Cause unknown – 10% familial (SOD-1 mutation) – ? Related to athleticism Presentation • Several variations in onset, but progress to the same endpoint • Motor nerves only affected • May be just UMN or just LMN at onset, but other features will appear over time • Main patterns: – Amyotrophic lateral sclerosis – Bulbar presentaion – Primary lateral sclerosis (UMN onset) – Progressive muscular atrophy (LMN onset) Questions Wasting Classification • Amyotrophic Lateral Sclerosis • Progressive Bulbar Palsy • Progressive Muscular Atrophy • Primary Lateral Sclerosis • Multifocal Motor Neuropathy • Spinal Muscular Atrophy • Kennedy’s Disease • Monomelic Amyotrophy • Brachial Amyotrophic Diplegia El Escorial Criteria for Diagnosis Tongue fasiculations Amyotrophic lateral sclerosis • ‘Typical’ presentation (60%+) • Usually one limb initially – Foot drop – Clumsy weak hand – May complain of cramps • Gradual progression over months • May be some wasting at presentation • Usually fasiculations (often more widespread) • Brisk reflexes, extensor plantars • No sensory signs; MAY occasionally be mild symptoms • Relentless progression, noticable over weeks/ months Bulbar MND • Approximately 30% of cases • Onset with dysarthria, dysphagia • Bulbar and pseudobulbar symptoms • On examination – Dysarthria –
    [Show full text]
  • Consensus-Based Care Recommendations for Adults with Myotonic Dystrophy Type 1
    Consensus-based Care Recommendations for Adults with Myotonic Dystrophy Type 1 I Consensus-based Care Recommendations for Adults with Myotonic Dystrophy Type 1 Due to the multisystemic nature of this disease, the studies and rigorous evidence needed to drive the creation of an evidence-based guideline for the clinical care of adult myotonic dystrophy type 1 (DM1) patients are not currently available for all affected body systems and symptoms. In order to improve and standardize care for this disorder now, more than 65 leading myotonic dystrophy (DM) clinicians in Western Europe, the UK, Canada and the US joined in a process started in Spring 2015 and concluded in Spring 2017 to create the Consensus-based Care Recommendations for Adults with Myotonic Dystrophy Type 1. The project was organized and supported by the Myotonic Dystrophy Foundation (MDF). A complete list of authors and an overview of the process is available in Addendum 1. A complete reading list for each of the study area sections is available in Addendum 2. An Update Policy has been adopted for this document and will direct a systematic review of literature and appropriate follow up every three years. Myotonic Dystrophy Foundation staff will provide logistical and staff support for the update process. A Quick Reference Guide extrapolated from the Consensus-based Care Recommendations is available here http://www.myotonic.org/clinical-resources For more information, visit myotonic.org. Myotonic Dystrophy Foundation 1 www.myotonic.org Table of Contents Life-threatening symptoms
    [Show full text]