Motor Neuron Disease, An Issue of Neurologic Clinics

Richard J. Barohn

Description

Dr. Richard Barohn and Dr. Mazen Dimachkie lead this publication on Motor Neuron Disease. Focus is on ALS, with inclusion of primary lateral sclerosis, primary muscular atrophy, leg amyotrophic diplegia, brachial amyotropic diplegia, and isolate bulbar ALS. Among the topics presented are. Patterns of weakness, classification of motor neuron disease  & clinical diagnosis of sporadic ALS;  Potential environmental factors in ALS;  Neuropathology;  Spinal muscular atrophy; Complementary and alternative therapies in ALS frontotemporal dysfunction and dementia in ALS;  Symptoms management and end of life care;  Research approaches to slowing progression of ALS; Familial ALS; Kennedy disease and more. Information in this issue presents: Description of the problem (Incidence, Prevalence, Severity, Natural history); 2. Review of pertinent data;  Controversial areas discussing aspects such as areas of practice for which there are disagreements and why? What are the arguments and counter arguments and what data support them?;  Conclusions using levels of clinical evidence that support or refute an intervention. Procedural steps are provided for diagnostic and treatment discussions along with clinical cases.

Sujets

• Médecine
• Médecine Générale
• Medical
• Neurology

Informations

• Publié par: Elsevier
• Date de parution: 07 janvier 2016
• Nombre de lectures: 1
• EAN13: 9780323413459
• Langue: English
• Poids de l'ouvrage: 2 Mo

Extrait

Motor Neuron Disease
Neurologic Clinics
Editors
Mazen M. Dimachkie, MD
Department of Neurology, Neuromuscular Section, University of Kansas Medical Center, Kansas City, KS, USA
Richard J. Barohn, MD
Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA
Consulting Editor
Randolph W. Evans

Clinics Review Articles

www.neurologic.theclinics.com

November 2015 Volume 33 Number 4
Table of Contents
Cover image
Title page
Copyright
Contributors
Consulting Editor
Editors
Authors
Forthcoming Issues
Forthcoming Issues
Recent Issues
Preface. Motor Neuron Disease
References
Amyotrophic Lateral Sclerosis: A Historical Perspective
Key points
References
Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis
Key points
Patterns of weakness
Classification of motor neuron diseases
Amyotrophic lateral sclerosis
Summary and future directions
Disclosure
References
Primary Lateral Sclerosis
Key points
Introduction
Clinical findings
Case history
Diagnosis
Pathophysiology
Prognosis
Therapeutic strategies
Summary and future directions
Disclosure
References
Progressive Muscular Atrophy
Key points
Introduction
Epidemiology
Clinical presentation
Pathophysiology
Diagnosis
Subclinical upper motor neuron involvement in progressive muscular atrophy
Differential diagnosis
Management
Summary
Acknowledgments
References
Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis)
Key points
Introduction
Brachial amyotrophic diplegia
Leg amyotrophic diplegia
Isolated bulbar amyotrophic lateral sclerosis
Summary and future directions
Disclosure statement
References
Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis
Key points
Introduction
Illustrative case
Cognitive deficits in amyotrophic lateral sclerosis
Mild behavioral impairment in amyotrophic lateral sclerosis
Psychiatric symptoms in amyotrophic lateral sclerosis
Detection of amyotrophic lateral sclerosis-frontotemporal dementia, amyotrophic lateral sclerosis with cognitive impairment, and amyotrophic lateral sclerosis with behavioral impairment
Neuroimaging
Molecular, clinical, and neuropathologic correlates of frontotemporal dysfunction in amyotrophic lateral sclerosis
Current controversies
References
Familial Amyotrophic Lateral Sclerosis
Key points
Background
Recent technological developments and amyotrophic lateral sclerosis gene discovery
Genetic susceptibility to amyotrophic lateral sclerosis
Familial inheritance patterns in amyotrophic lateral sclerosis
Gene variants linked to amyotrophic lateral sclerosis pathogenesis
Epigenetics of amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis gene testing in clinical practice
Summary
References
Spinal Muscular Atrophy
Key points
Incidence
Prevalence
Severity
Natural history
Introduction
Clinical features
Molecular genetics
Clinical management
Therapeutic development
Summary
References
Spinal and Bulbar Muscular Atrophy
Key points
Introduction
Mechanism
Disease course
Diagnosis
Clinical case
Management
A disease of nerve or muscle?
Summary
References
Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants
Key points
Introduction
Classic amyotrophic lateral sclerosis neuropathology
Molecular neuropathology: inclusions and proteinopathies
Sequential changes and neuropathologic staging
Familial amyotrophic lateral sclerosis: genetics and associated disease
Amyotrophic lateral sclerosis variants
Future directions and final remarks
Acknowledgments
References
Potential Environmental Factors in Amyotrophic Lateral Sclerosis
Key points
Introduction
Gene-environment interaction
Proposed environmental factors
Combined oxidative stress theory
Summary
References
Symptom Management and End-of-Life Care in Amyotrophic Lateral Sclerosis
Key points
Sialorrhea
Pseudobulbar affect
Sleep disruption
Respiratory insufficiency
Fatigue
Nutrition
Pain
Spasticity
Summary
Disclosure
References
Complementary and Alternative Therapies in Amyotrophic Lateral Sclerosis
Key points
Introduction
Types of complementary and alternative medicine that people with amyotrophic lateral sclerosis commonly ask about
Options for reviewing complementary and alternative medicine with people with amyotrophic lateral sclerosis
Summary
References
The Dilemma of the Clinical Trialist in Amyotrophic Lateral Sclerosis: The Hurdles to Finding a Cure
Key points
Report
Case examples of trial challenges
References
Index
Copyright
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NEUROLOGIC CLINICS Volume 33, Number 4
November 2015 ISSN 0733-8619, ISBN-13: 978-0-323-41344-2
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Contributors
Consulting Editor
RANDOLPH W. EVANS, MD , Clinical Professor, Department of Neurology, Baylor College of Medicine, Houston, Texas
Editors
MAZEN M. DIMACHKIE, MD , Vice-Chair for Research; Director, Clinical Neurophysiology Division; Director, Neuromuscular Section; Professor of Neurology, Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas
RICHARD J. BAROH