Peripheral Nervous System Disorders

October 2014, Volume 20, Issue 5
BROWSE ISSUES

Peripheral Nervous System Disorders

October 2014, Vol.20, No.5

Guest Editor:

Michelle L. Mauermann, MD; Ted M. Burns, MD

ISSN: 1080-2371

Online ISSN: 1538-6899

REVIEW ARTICLES
ALS and Other Motor Neuron Diseases
Tiryaki, Ezgi; Horak, Holli A.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1185-1207
doi: 10.1212/01.CON.0000455886.14298.a4
Charcot-Marie-Tooth Disease and Other Inherited Neuropathies
Saporta, Mario A.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1208-1225
doi: 10.1212/01.CON.0000455885.37169.4c
Diabetic Neuropathies
Russell, James W.; Zilliox, Lindsay A.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1226-1240
doi: 10.1212/01.CON.0000455884.29545.d2
Acquired Immune Demyelinating Neuropathies
Dimachkie, Mazen M.; Saperstein, David S.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1241-1260
doi: 10.1212/01.CON.0000455883.91426.12
Acquired Immune Axonal Neuropathies
Bril, Vera; Katzberg, Hans D.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1261-1273
doi: 10.1212/01.CON.0000455882.83803.72
Infectious Neuropathies
Hehir, Michael K. II; Logigian, Eric L.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1274-1292
doi: 10.1212/01.CON.0000455881.83803.a9
Peripheral Neuropathy Due to Vitamin Deficiency, Toxins, and Medications
Staff, Nathan P.; Windebank, Anthony J.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1293-1306
doi: 10.1212/01.CON.0000455880.06675.5a
Paraproteinemic Neuropathies
Mauermann, Michelle L.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1307-1322
doi: 10.1212/01.CON.0000455879.99050.26
Brachial Plexopathies
Ferrante, Mark A.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1323-1342
doi: 10.1212/01.CON.0000455878.60932.37
Lumbosacral Plexopathy
Dyck, P. James B.; Thaisetthawatkul, Pariwat
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1343-1358
doi: 10.1212/01.CON.0000455877.60932.d3
Paraneoplastic Neuropathies
Muppidi, Srikanth; Vernino, Steven
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1359-1372
doi: 10.1212/01.CON.0000455876.53309.ec
Autonomic Neuropathies
Iodice, Valeria; Sandroni, Paola
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1373-1397
doi: 10.1212/01.CON.0000455875.76179.b1
Small Fiber Neuropathies
Gibbons, Christopher H.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1398-1412
doi: 10.1212/01.CON.0000455874.68556.02
Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome
Sanders, Donald B.; Guptill, Jeffrey T.
CONTINUUM: Lifelong Learning in Neurology
October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - p 1413-1425
doi: 10.1212/01.CON.0000455873.30438.9b
Editor's Preface
Articles
Key Points
Abbreviations
Appendix
Issue Overview

Issue Overview

CONTINUUM: Lifelong Learning in Neurology October 2014 - Volume 20 - Issue 5, Peripheral Nervous System Disorders - [no page #] doi: 10.1212/01.CON.0000455895.42114.02

Continuum: Lifelong Learning in NeurologyPeripheral Nervous System Disorders, Volume 20, Issue 5, October 2014

Issue Overview

Peripheral Nervous System Disorders, October 2014;20(5)

Continuum: Lifelong Learning in Neurology® is designed to help practicing neurologists stay abreast of advances in the field while simultaneously developing lifelong self-directed learning skills.

Learning Objectives

Upon completion of this Continuum: Lifelong Learning in Neurology Peripheral Nervous System Disorders issue, participants will be able to:

▸ Understand the pathophysiology of the various genetic forms of ALS and the associated clinical presentations

▸ Formulate an algorithm for genetic testing in Charcot-Marie-Tooth disease based on nerve conduction studies

▸ Distinguish the clinical symptoms and signs of various forms of diabetic peripheral neuropathy

▸ Define, diagnose, and manage acute inflammatory demyelinating polyradiculoneuropathy, including both supportive and immune treatment

▸ Appropriately recognize, evaluate, and manage the clinical presentation of vasculitic neuropathies

▸ Identify and manage the various bacterial, viral, and spirochetal infections that cause peripheral neuropathy

▸ List and effectively treat the various vitamin deficiencies that lead to peripheral neuropathy

▸ Differentiate the paraproteinemic neuropathies based on monoclonal protein subtype

▸ Assess the potential differential diagnoses of a brachial plexopathy based on anatomic location

▸ Diagnose and manage diabetic and nondiabetic lumbosacral plexopathies

▸ List the various forms of paraneoplastic neuropathies and their associated autoantibodies and malignancies

▸ Classify acute autonomic neuropathies and choose the appropriate laboratory evaluation

▸ Recognize the clinical symptoms, signs, and risk factors for the development of treatment-induced neuropathy

▸ Identify the clinical signs, symptoms, and laboratory and electrophysiological findings of neuromuscular junction disorders and initiate appropriate management

▸ List the important factors in deciding when to order an EMG for neuropathy evaluation in order to more appropriately make EMG referrals and integrate the results into clinical practice

Core Competencies

The Continuum Sports Neurology issue covers the following core competencies:

▸ Patient Care

▸ Medical Knowledge

▸ Practice-Based Learning and Improvement

▸ Interpersonal and Communication Skills

▸ Professionalism

▸ Systems-Based Practice

Disclosures

CONTRIBUTORS

Ted M. Burns, MD, Co-Guest Editor

Professor of Neurology, University of Virginia, Charlottesville, Virginia

a,bDr Burns reports no disclosures.

Michelle L. Mauermann, MD, Co-Guest Editor

Assistant Professor of Neurology, Mayo Clinic, Rochester, Minnesota

aDr Mauermann reports no disclosure.

bDr Mauermann discusses the unlabeled use of IV immunoglobulin G, plasma exchange, and rituximab for the treatment of immunoglobulin M neuropathies and rituximab for the treatment of Waldenstrom macroglobulinemia.

Vera Bril, MD, BSc, FRCPC

Professor, University of Toronto; Chief of Neurology, University Health Network and Mount Sinai Hospital, Toronto, Canada

aDr Bril serves as a consultant for and her institution receives grants from CSL Behring and Grifols, Canada, Ltd.

bDr Bril reports no disclosure.

Mazen M. Dimachkie, MD, FAAN, FANA

Professor of Neurology, Department of Neurology; Director, Neurophysiology Division; Director, Neuromuscular Section, University of Kansas Medical Center, Kansas City, Kansas

aDr Dimachkie has received grant support from CSL Behring and has served as a consultant, speaker, advisory board member, or steering committee member for Baxter; BioMarin Pharmaceutical, Inc; Catalyst Pharmaceutical Partners, Inc; CSL Behring; Depomed, Inc; Merck & Co, Inc; NuFACTOR; and Pfizer Inc.

bDr Dimachkie discusses the unlabeled use of IV immunoglobulin G, plasma exchange, and gabapentin for the treatment of acquired immune demyelinating neuropathies.

P. James B. Dyck, MD, FAAN

Professor of Neurology, Head of Peripheral Nerve Section and Peripheral Nerve Laboratory, Mayo Clinic, Rochester, Minnesota

aDr Dyck reports no disclosure.

bDr Dyck discusses the unlabeled use of methylprednisolone for the treatment of inflammatory lumbosacral plexopathy.

Mark A. Ferrante, MD

Professor, Co-director Neurophysiology Fellowship, University of Tennessee Health Science Center; Director, EMG Laboratory, VAMC, Memphis, Tennessee

aDr Ferrante receives royalties from the American Association of Neuromuscular & Electrodiagnostic Medicine.

bDr Ferrante reports no disclosure.

Christopher H. Gibbons, MD, FAAN

Assistant Professor of Neurology, Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, Massachusetts

aDr Gibbons serves on the advisory panel of Pfizer Inc.

bDr Gibbons reports no disclosure.

Kenneth C. Gorson, MD

Professor of Neurology, Tufts University School of Medicine, St. Elizabeth’s Medical Center, Boston, Massachusetts

aDr Gorson serves as a consultant for Baxter and CSL Behring.

bDr Gorson discusses the unlabeled use of IV immunoglobulin G and plasma exchange for the treatment of Guillain-Barre syndrome.

Jeffrey T. Guptill, MD, MA, MHS

Assistant Professor, Department of Neurology, Duke University Medical Center, Durham, North Carolina

aDr Guptill serves as a consultant for Grifols and UCB, Inc, and receives research support from the Myasthenia Gravis Foundation of America. Dr Guptill’s institution receives grants from Grifols and UCB, Inc.

bDr Guptill discusses the unlabeled use of amifampridine and rituximab for the treatment of Lambert-Eaton myasthenic syndrome and the unlabeled use of azathioprine, cyclosporine A, mycophenolate mofetil, and rituximab for the treatment of myasthenia gravis.

Michael K. Hehir II, MD

Assistant Professor, University of Vermont, Department of Neurosciences, Burlington, Vermont

a,bDr Hehir reports no disclosures.

Holli A. Horak, MD, FAAN

Associate Professor of Neurology, Co-director of the Muscular Dystrophy Association ALS Clinic, University of Arizona College of Medicine, Tucson, Arizona

aDr Horak’s institution receives a grant from the Centers for Disease Control and Prevention.

bDr Horak discusses the unlabeled use of various drugs for the symptomatic management of ALS.

Valeria Iodice, MD, PhD

Consultant in Neurology, University College London, London, United Kingdom

aDr Iodice reports no disclosures.

bDr Iodice discusses the unlabeled use of ephedrine, octreotide, acarbose, voglibose, erythropoietin, desmopressin, yohimbine, pyridostigmine, and dihydroergotamine for the treatment of orthostatic hypotension as well as the use of plasma exchange, IV immunoglobulin, azathioprine, prednisone, cyclophosphamide, and mycophenolate mofetil for autoimmune autonomic ganglionopathies.

Lyell K. Jones Jr, MD

Assistant Professor of Neurology, Residency Program Director, Associate Chair for Hospital Practice, Mayo Clinic, Rochester, Minnesota

a,bDr Jones reports no disclosures.

Hans D. Katzberg, MD, MSC, FRCPC

Assistant Professor, University of Toronto, University Health Network, Toronto, Ontario, Canada

aDr Katzberg has received travel support from Genzyme Canada and serves as a speaker, and his institution receives grants from CSL Behring; Grifols, Canada, Ltd; and the University of Toronto.

bDr Katzberg reports no disclosure.

Daniel G. Larriviere, MD, JD, FAAN

Director, Neuromuscular Division, Department of Neurology, Ochsner Neuroscience Institute, New Orleans, Louisiana

aDr Larriviere is a compensated faculty member of the American Academy for the Advancement of Science and serves as an associate editor for Continuum Audio.

bDr Larriviere discusses the unlabeled use of cannabis for the treatment of HIV-associated sensory neuropathy.

Eric L. Logigian, MD, FAAN

Professor of Neurology, Unit Chief, Neuromuscular Division, University of Rochester Medical Center, University of Rochester School of Medicine and Dentistry, Rochester, New York

aDr Logigian serves on the editorial board of Muscle and Nerve, as a consultant for Genzyme Corporation, and has given expert testimony for Matis Baum O’Connor and Harvey Pennington LTD.

bDr Logigian reports no disclosure.

Srikanth Muppidi, MD

Assistant Professor, Department of Neurology, Stanford School of Medicine, Stanford, California

aDr Muppidi reports no disclosure.

bDr Muppidi discusses the unlabeled use of rituximab in the treatment of paraneoplastic neuropathies.

James W. Russell, MD, MS, FRCP, FACP, FAAN

Professor, Departments of Neurology and Neurobiology; Director, Peripheral Nerve Center, University of Maryland; Associate Chief of Neurology, Baltimore VA Medical Center, Maryland Health Care System, Baltimore, Maryland

aDr Russell’s institution receives grants from the Department of Veterans Affairs and the NIH.

bDr Russell discusses the unlabeled use of α-lipoic acid for the treatment of diabetic neuropathies.

Donald B. Sanders, MD, FAAN

Professor of Neurology, Duke University Medical Center, Durham, North Carolina

aDr Sanders has served as a speaker for Athena Diagnostics, Inc; as a consultant for Accordant Health Services, Jacobus Pharmaceutical Company, Inc, GlaxoSmithKline, and UCB, Inc; and has participated in review activities with Alexion and Cytokinetics, Inc.

bDr Sanders discusses the unlabeled use of amifampridine and rituximab for the treatment of Lambert-Eaton myasthenic syndrome and the unlabeled use of azathioprine, cyclosporine A, mycophenolate mofetil, and rituximab for the treatment of myasthenia gravis.

Paola Sandroni, MD, PhD, FAAN

Professor of Neurology, Mayo Clinic, Rochester, Minnesota

aDr Sandroni reports no disclosures.

bDr Sandroni discusses the unlabeled use of ephedrine, octreotide, acarbose, voglibose, erythropoietin, desmopressin, yohimbine, pyridostigmine, and dihydroergotamine for the treatment of orthostatic hypotension as well as the use of plasma exchange, IV immunoglobulin, azathioprine, prednisone, cyclophosphamide, and mycophenolate mofetil for autoimmune autonomic ganglionopathies.

David S. Saperstein, MD

Clinical Associate Professor of Neurology, University of Arizona, Phoenix, Arizona; Phoenix Neurological Associates, Phoenix, Arizona; Adjunct Professor of Neurology, University of Kansas Medical Center, Kansas City, Kansas

aDr Saperstein has served as a consultant, speaker, and advisory board member for Baxter, CLS Behring, Grifols, and NuFACTOR.

bDr Saperstein discusses the unlabeled use of IV immunoglobulin G, plasma exchange, and gabapentin for the treatment of acquired immune demyelinating neuropathies.

Mario A. Saporta, MD, PhD

Adjunct Professor of Clinical Neurophysiology, Fluminense Federal University, Rio de Janeiro, Brazil

a,bDr Saporta reports no disclosures.

A. Gordon Smith, MD, FAAN

Professor, Vice Chair of Neurology, Chief, Division of Neuromuscular Medicine, University of Utah, Salt Lake City, Utah

a,bDr Smith reports no disclosures.

Nathan P. Staff, MD, PhD

Assistant Professor of Neurology, Mayo Clinic, Rochester, Minnesota

aDr Staff receives grants from the National Cancer Institute and National Center for Advancing Translational Sciences and research funding from BrainStorm Cell Therapeutics.

bDr Staff reports no disclosure.

Pariwat Thaisetthawatkul, MD

Associate Professor, University of Nebraska Medical Center, Omaha, Nebraska

aDr Thaisetthawatkul reports no disclosure.

bDr Thaisetthawatkul discusses the unlabeled use of methylprednisolone for the treatment of inflammatory lumbosacral plexopathy.

Ezgi Tiryaki, MD

Associate Professor of Neurology, University of Minnesota; Medical Director of the ALS Association Certified ALS Center of Excellence at Hennepin County Medical Center, Minneapolis, Minnesota

aDr Tiryaki’s institution receives support from the ALS Association.

bDr Tiryaki discusses the unlabeled use of various drugs for the symptomatic management of ALS.

Steven Vernino, MD, PhD, FAAN

Professor, Academic Vice Chair, Dr Bob & Jean Smith Foundation Distinguished Chair in Neuromuscular Disease Research, Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas

aDr Vernino served on the advisory board of Chelsea Therapeutics and has received honorarium for speaking engagements from the American Academy of Neurology and the Texas Neurological Society. Dr Vernino’s institution receives laboratory support from Athena Diagnostics, Inc.

bDr Vernino discusses the unlabeled use of rituximab in the treatment of paraneoplastic neuropathies.

Anthony J. Windebank, MD, FAAN

Professor of Neurology, Department of Neuroscience Research, Mayo Clinic, Rochester, Minnesota

aDr Windebank receives grants from the National Institute of Aging, National Center for Advancing Translational Sciences, Armed Forces Institute of Regenerative Medicine, Morton Cure Paralysis Fund, Craig H. Neilsen Foundation, and research funding from BrainStorm Cell Therapeutics.

bDr Windebank reports no disclosure.

Lindsay A. Zilliox, MD

Assistant Professor of Neurology, University of Maryland, School of Medicine and Baltimore VA-Medical Center, Baltimore, Maryland

aDr Zilliox receives salary support from the Department of Veterans Affairs.

bDr Zilliox discusses the unlabeled use of alpha lipoic acid for the treatment of diabetic neuropathies.

MULTIPLE-CHOICE QUESTION WRITERS

Adam G. Kelly, MD

Assistant Professor of Neurology, University of Rochester Medical Center; Chief of Neurology, Highland Hospital, Rochester, New York

aDr Kelly has received research support from the Donald W. Reynolds Foundation and compensation from the American Academy of Neurology as a question writer for Continuum.

bDr Kelly reports no disclosure.

Joseph E. Safdieh, MD, FAAN

Vice Chairman for Education; Associate Professor of Neurology, Weill Cornell Medical College and New York-Presbyterian Hospital, New York, New York

aDr Safdieh receives research support from Accera, Inc, and serves on the speakers bureau for Supernus Pharmaceuticals, Inc.

bDr Safdieh reports no disclosure.

aRelationship Disclosure

bUnlabeled Use of Products/Investigational Use Disclosure

Methods of Participation and Instructions for Use

Continuum: Lifelong Learning in Neurology® is designed to help practicing neurologists stay abreast of advances in the field while simultaneously developing lifelong self-directed learning skills. In Continuum, the process of absorbing, integrating, and applying the material presented is as important as, if not more important than, the material itself.

The goals of Continuum include disseminating up-to-date information to the practicing neurologist in a lively, interactive format; fostering self-assessment and lifelong study skills; encouraging critical thinking; and, in the final analysis, strengthening and improving patient care.

Each Continuum issue is prepared by distinguished faculty who are acknowledged leaders in their respective fields. Six issues are published annually and are composed of review articles, case-based discussions on ethical and practice issues related to the issue topic, coding information, and comprehensive CME and self-assessment offerings, including a self-assessment pretest, multiple-choice questions with preferred responses, and a patient management problem. For detailed instructions regarding Continuum CME and self-assessment activities, visit aan.com/continuum/cme.

The review articles emphasize clinical issues emerging in the field in recent years. Case reports and vignettes are used liberally, as are tables and illustrations. Video material relating to the issue topic accompanies issues when applicable.

The text can be reviewed and digested most effectively by establishing a regular schedule of study in the office or at home, either alone or in an interactive group. If subscribers use such regular and perhaps new study habits, Continuum’s goal of establishing lifelong learning patterns can be met.

© 2014 American Academy of Neurology