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Continuum Postreading Test 29(5) October 2023 (Peripheral Nerve and Motor Neuron Disorders)

Continuum Postreading Test 29(5) October 2023 (Peripheral Nerve and Motor Neuron Disorders)

Learning Objectives

Upon completion of this Continuum: Lifelong Learning in Neurology Peripheral Nerve and Motor Neuron Disorders issue, participants will be able to:
  • Initiate diagnostic evaluation in patients with suspected peripheral nerve disease by applying a framework for clinical diagnosis
  • Discuss the clinical features, diagnostic criteria, differential diagnosis, pathogenesis, prognosis, and insights into the current and future diagnostic and therapeutic interventions of Guillain-Barré syndrome
  • Identify the types of chronic demyelinating neuropathies and differentiate and treat immune-mediated demyelinating neuropathies
  • Identify the common causes of autoimmune axonal neuropathy and develop appropriate clinical evaluation and management strategies based on subtype
  • Diagnose and manage the spectrum of peripheral neuropathies associated with diabetes
  • Discuss the infectious causes of peripheral neuropathies and the types of neuropathies associated with them
  • Perform the clinical diagnosis, workup, and management of the most clinically relevant toxic neuropathies
  • Discuss the clinical presentation, evaluation, and management of peripheral neuropathies caused by nutritional deficiencies
  • Identify common clinical phenotypic, electrophysiologic, and hematologic features of each paraproteinemic neuropathy and tailor the appropriate diagnostic investigation and multidisciplinary treatment approach
  • Guide the clinical evaluation of patients with suspected hereditary neuropathy and discuss the major clinical phenotypes and common genotypes of hereditary neuropathies
  • Describe the clinical spectrum, diagnostic process, evolving genetic landscape, and approach to management for amyotrophic lateral sclerosis
  • Discuss the diagnostic assessment of individuals with spinal muscular atrophy, available treatment options, and key outcome measures
  • Identify the role of multidisciplinary care in the management of patients with neuromuscular disorders

Core Competencies

This Continuum: Lifelong Learning in Neurology Peripheral Nerve and Motor Neuron Disorders issue covers the following core competencies:
  • Patient Care and Procedural Skills
  • Medical Knowledge
  • Practice-Based Learning and Improvement
  • Interpersonal and Communication Skills
  • Professionalism
  • Systems-Based Practice

Contributorsa

Kelly Gwathmey, MD,
Guest Editor
Associate Professor of Neurology; Neuromuscular Medicine Division Chair, Virginia Commonwealth University, Richmond, Virginia

Relationship Disclosure: Dr Gwathmey has received personal compensation in the range of $5000 to $9999 for serving on a scientific advisory or data safety monitoring board for Alexion Pharmaceuticals, Inc, and in the range of $500 to $4999 for serving as a consultant for Alexion Pharmaceuticals, Inc, argenx, UCB S.A., and Xeris Biopharma and for serving on a speakers bureau for Alexion Pharmaceuticals, Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Gwathmey reports no disclosure.

Said R. Beydoun MD, FAAN
Professor of Clinical Neurology; Richard Angus Grant Sr. Chair in Neurology; Division Chief, Neuromuscular Medicine; Program Director, Clinical Neurophysiology, Keck School of Medicine of USC, Los Angeles, California

Relationship Disclosure: Dr Beydoun has received personal compensation in the range of $500 to $4999 for serving as a consultant for Amylyx Pharmaceuticals, Biogen, Mitsubishi Chemical Group Corporation, Octapharma USA, Inc, and UCB S.A. and on a speakers bureau for Grifols S.A.; in the range of $5000 to $9999 for serving as a consultant for Alexion Pharmaceuticals, Inc, Alnylam Pharmaceuticals, Inc, argenx, Janssen Global Services, LLC, and Takeda Pharmaceutical Company Ltd and on speakers bureaus for Amylyx Pharmaceuticals and CSL; and in the range of $10,000 to $49,999 for serving as a consultant for CSL and on speakers bureaus for Alexion Pharmaceuticals, Inc, Alnylam Pharmaceuticals, Inc, argenx, and Takeda Pharmaceutical Company Ltd. The institution of Dr Beydoun has received research support from Alexion Pharmaceuticals, Inc, Amylyx Pharmaceuticals, argenx, Genentech, Inc, Janssen Global Services, LLC, Regeneron Pharmaceuticals Inc, Sanofi , the Sean M. Healey & AMG Center for ALS, UCB S.A.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Beydoun discusses the unlabeled/investigational use of ibrutinib, IV immunoglobulin, and rituximab for the treatment of IgM–myelin-associated glycoprotein polyneuropathy.

Aimee Boegle, MD, PhD
Instructor, Harvard Medical School; Director, Neuromuscular Medicine Fellowship Associate Program Director, Neurology Residency, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts

Relationship Disclosure: Dr Boegle has received publishing royalties from Wolters Kluwer Health N.V.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Boegle discusses the unlabeled use of doxycycline, amoxicillin, and cefotaxime for the treatment of Lyme disease and the unlabeled use of gabapentin, lamotrigine, oxcarbazepine, pregabalin, selective norepinephrine reuptake inhibitors (eg, duloxetine, venlafaxine), topical anesthetics (eg, capsaicin, lidocaine), and tricyclic antidepressants (eg, amitriptyline, nortriptyline) for the treatment of neuropathic pain.

Brian Callaghan, MD, FAAN
Associate Professor of Neurology, Department of Neurology, University of Michigan, Ann Arbor, Michigan

Relationship Disclosure: Dr Callaghan has received personal compensation in the range of $500 to $4999 for serving as an editorial board member for the American Academy of Neurology, a consultant for Dynamed (EBSCO Industries, Inc), and a grant reviewer for the National Institutes of Health; in the range of $10,000 to $49,999 for serving as an expert witness for medicolegal work; and in the range of $50,000 to $99,999 for serving as an expert witness for the Vaccine Injury Compensation Program. The institution of Dr Callaghan has received research support from the American Academy of Neurology, JDRF (5COE-2019-861-S-B), the National Institute of Diabetes and Digestive Kidney Diseases (R01DK115687), and Veterans Affairs Clinical Science and Research Development.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Callaghan discusses the unlabeled use of amitriptyline, lamotrigine, oxcarbazepine, and venlafaxine for the treatment of painful distal symmetric polyneuropathy.

Leila Darki, MD
Assistant Professor of Clinical Neurology, Keck Medicine of USC, Los Angeles, California

Relationship Disclosure: Dr Darki has received personal compensation in the range of $0 to $499 for serving as a consultant for Guidepoint Global LLC and in the range of $500 to $4999 for serving as a consultant for Trinity Partners, LLC, and on scientific advisory or data safety monitoring boards for Amylyx Pharmaceuticals and Global Access Meetings.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Darki discusses the unlabeled/investigational use of ibrutinib, IV immunoglobulin, and rituximab for the treatment of IgM–myelin-associated glycoprotein polyneuropathy.

Melissa Elafros, MD, PhD
Assistant Professor, Department of Neurology, University of Michigan, Ann Arbor, Michigan

Relationship Disclosure: The institution of Dr Elafros has received research support from the National Center for Advancing Translational Sciences (UL1TR002240), the National Institute of Diabetes and Digestive and Kidney Diseases (P30-DK-02926 and P30-DK089503), and the National Institute of Neurologic Disorders and Stroke (5R25NS089450).

Unlabeled Use of Products/Investigational Use Disclosure: Dr Elafros discusses the unlabeled use of amitriptyline, lamotrigine, oxcarbazepine, and venlafaxine for the treatment of painful distal symmetric polyneuropathy.

Karissa Gable, MD
Associate Professor, Department of Neurology, Neuromuscular Division, Duke University Medical Center, Durham, North Carolina

Relationship Disclosure: Dr Gable has received personal compensation in the range of $500 to $4999 for serving as a consultant for Cowen Inc, MJH Life Sciences, WebMD, LLC, and on the scientific advisory or data safety monitoring boards for argenx, Immunovant, Inc, and Ultragenyx Pharmaceutical Inc and has received personal compensation in the range of $5000 to $9999 for serving on scientific advisory or data safety monitoring boards for Sanofi and Takeda Pharmaceutical Company Ltd and as a lecturer for WebMD, LLC.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Gable reports no disclosure.

Ali Habib, MD
Associate Professor; Director, Electrodiagnostic Laboratory, Department of Neurology, University of California, Irvine, California

Relationship Disclosure: Dr Habib has received personal compensation in the range of $500 to $4999 for serving on scientific advisory or data safety monitoring boards for the National Institutes of Health/National Institute of Neurological Disorders and Stroke and UCB S.A., and as a consultant for Pfizer Inc and in the range of $5000 to $9999 for serving as a consultant for Alexion Pharmaceuticals, Inc, argenx, Genentech, Inc, Immunovant, Inc, and UCB S.A., and for serving on speakers bureaus for Alexion Pharmaceuticals, Inc, and argenx. The institution of Dr Habib has received research support from Alexion Pharmaceuticals, Inc, argenx, Cabaletta Bio, Inc, Genentech, Inc, Immunovant, Inc, Regeneron Pharmaceuticals Inc, and UCB S.A.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Habib discusses the unlabeled/investigational use of IV immunoglobulin for the treatment of Guillain-Barré syndrome.

Leslie H. Hayes, MD
Instructor in Neurology, Harvard Medical School; Attending Physician, Boston Children’s Hospital, Boston, Massachusetts

Relationship Disclosure: Dr Hayes has received personal compensation in the range of $500 to $4999 for serving as a consultant for PTC Therapeutics.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Hayes reports no disclosures.

Terry D. Heiman-Patterson, MD
Professor of Neurology; Director MDA/ALS Center of Hope, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania

Relationship Disclosure: Dr Heiman-Patterson has received personal compensation in the range of $500 to $4999 for serving as a consultant for ITF Pharma, Mitsubishi Tanabe Pharma America, and Samus Therapeutics, Inc, and on scientific advisory or data safety monitoring boards for Amylyx Pharmaceuticals, Biogen, Biohaven Ltd, Cytokinetics, and Mitsubishi Tanabe Pharma America.
Unlabeled Use of Products/Investigational Use Disclosure: Dr Heiman-Patterson reports no disclosure.

Aaron E. Izenberg, MD
Assistant Professor Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada

Relationship Disclosure: Dr Izenberg has received personal compensation in the range of $500 to $4999 for serving on scientific advisory or data safety monitoring boards for Biogen, F. Hoffman-La Roche Ltd, and Sanofi.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Izenberg reports no disclosure.

Peter Jin, MD
Assistant Professor, Department of Neurology, University of Maryland School of Medicine, Baltimore, Maryland

Relationship Disclosure: Dr Jin has received personal compensation in the range of $0 to $4999 for serving as a consultant for Ionis Pharmaceuticals.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Jin reports no disclosure.

Noah Kolb, MD
Associate Professor of Neurological Sciences, University of Vermont, Burlington, Vermont

Relationship Disclosure: Dr Kolb has received personal compensation in the range of $0 to $499 for serving as a consultant for Eisana Corp; in the range of $500 to $4999 for serving as a consultant for Abalone Bio, Inc, Alexion Pharmaceuticals, Inc, Lilly, and UCB S.A.; in the range of $5000 to $9999 for serving as a consultant for the National Institute of Neurological Disorders and Stroke and for serving as an expert witness for Locks Law and for Ralston, Pope & Diehl; and in the range of $10,000 to $49,999 for serving as an expert witness for Walkup, Melodia, Kelly & Schoenberger. The institution of Dr Kolb has received research support from the National Cancer Institute.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Kolb discusses the unlabeled use of baclofen-amitriptylineketamine, gabapentin, lidocaine, mexiletine, nortriptyline or amitriptyline, and pregabalin for the treatment of neuropathic pain.

Neeraj Kumar, MD
Professor of Neurology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota

Relationship Disclosure: Dr Kumar reports no disclosure.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Kumar reports no disclosure.

Brendan McNeish, MD
Assistant Professor, Department of Physical Medicine Rehabilitation and Neurology, University of Pittsburgh, Pittsburgh, Pennsylvania

Relationship Disclosure: Dr McNeish has received research support in the range of $100,000-$499,999 from the National Institutes of Health (NIA P30AG02482).

Unlabeled Use of Products/Investigational Use Disclosure: Dr McNeish discusses the unlabeled use of baclofen-amitriptylineketamine, gabapentin, lidocaine, mexiletine, nortriptyline or amitriptyline, and pregabalin for the treatment of neuropathic pain.

Pushpa Narayanaswami, MD, FAAN
Associate Professor of Neurology, Harvard Medical School; Vice Chair, Clinical Operations, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts

Relationship Disclosure: Dr Narayanaswami has received personal compensation in the range of $500 to $4999 for serving on scientific advisory or data safety monitoring boards for Alexion Pharmaceuticals, Inc, argenx, and Sanofi and for serving as a consultant for Novartis Pharmaceuticals Corporation; in the range of $5000 to $9999 for serving on a scientific advisory or data safety monitoring board for Janssen Global Services, LLC, and for serving as an editor, associate editor, or editorial advisory board member for Muscle & Nerve; and in the range of $10,000 to $49,999 for serving as a consultant for UCB S.A. Dr Narayanaswami has stock in Doximity, Inc, Dr Reddy’s Laboratories Ltd, Moderna, Inc, Pfi zer Inc, and Viatris Inc. Dr Narayanaswami has noncompensated relationships as a member of the boards of directors with the American Association of Neuromuscular & Electrodiagnostic Medicine and the Myasthenia Gravis Foundation Of America, Inc, that are relevant to the American Academy of Neurology interests or activities. The institution of Dr Narayanaswami has received research support from Alexion Pharmaceuticals, Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Narayanaswami discusses the unlabeled use of doxycycline, amoxicillin, and cefotaxime for the treatment of Lyme disease and the unlabeled use of gabapentin, lamotrigine, oxcarbazepine, pregabalin, selective norepinephrine reuptake inhibitors (eg, duloxetine, venlafaxine), topical anesthetics (eg, capsaicin, lidocaine), and tricyclic antidepressants (eg, amitriptyline, nortriptyline) for the treatment of neuropathic pain.

Maryam Oskoui, MD, FAAN
Associate Professor, Department of Pediatrics, Department of Neurology and Neurosurgery, McGill University; Director, Division of Pediatric Neurology, McGill University Health Center, Montreal, Quebec, Canada

Relationship Disclosure: Dr Oskoui has a noncompensated relationship as a member of the medical and scientific advisory committee with Muscular Dystrophy Canada that is relevant to the American Academy of Neurology (AAN) interests or activities and has received personal compensation in the range of $500 to $4999 for serving as a member of the board of directors for the Association des Neurologues du Québec. The institution of Dr Oskoui has received research support from Biogen, the Canadian Institutes of Health Research, Genetech, Inc, Muscular Dystrophy Canada, and Santhera Pharmaceuticals.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Oskoui discusses the unlabeled/investigational use of celecoxib, E1v1.11, flunarizine, moxifloxacin, reldesemtiv rigosertib, salbutamol, and securinine for the treatment of spinal muscular atrophy.

Reza Sadjadi, MD
Assistant Professor in Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts

Relationship Disclosure: Dr Sadjadi has received personal compensation in the range of $500 to $4999 for serving on scientific advisory or data safety monitoring boards for Alnylam Pharmaceuticals, Inc, and the Dysimmune Diseases Foundation. The institution of Dr Sadjadi has received research support from the American Academy of Neurology.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Sadjadi reports no disclosures.

Laurent Servais, MD, PhD
Professor of Paediatric Neuromuscular Diseases, University of Oxford, Oxford, United Kingdom

Relationship Disclosure: Dr Servais has received personal compensation in the range of $500 to $4999 for serving on scientific advisory or data safety monitoring boards for Lupin Pharmaceuticals, Inc, and FibroGen, Inc, and as a consultant for Affinia Healthcare, Anagenesis Biotechnologies, Audentes Therapeutics, Catabasis Pharmaceuticals, Evox Therapeutics, Flamingo Therapeutics, RegenexBio Inc, Santhera Pharmaceuticals, and Sarepta Therapeutics, Inc; in the range of $5000 to $9999 for serving as a consultant for Biogen and Novartis AG; and in the range of $10,000 to $49,999 for serving as a consultant for F. Hoffman-La Roche Ltd and Pfizer Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Servais discusses the unlabeled/investigational use of celecoxib, E1v1.11, lunarizine, moxifloxacin, reldesemtiv rigosertib, salbutamol, and securinine for the treatment of spinal muscular atrophy.

Jennifer Tracy, MD
Assistant Professor of Neurology, Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester, Minnesota

Relationship Disclosure: Dr Tracy reports no disclosure.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Tracy reports no disclosure.

Dr Tracy died September 26, 2023.

Waqar Waheed, MD
Professor of Neurology, Vice-Chair Department of Neurological Sciences, The University of Vermont and The University of Vermont Medical Center, Burlington, Vermont

Relationship Disclosure: Dr Waheed has received personal compensation in the range of $500 to $4999 for serving on a scientific advisory or data safety monitoring board for UCB S.A.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Waheed discusses the unlabeled/investigational use of IV immunoglobulin for the treatment of Guillain-Barré syndrome.


Self-Assessment and CME Test Writers

Douglas J. Gelb, MD, PhD, FAAN
Professor of Neurology, University of Michigan, Ann Arbor, Michigan

Relationship Disclosure: Dr Gelb has received personal compensation in the range of $500 to $4900 for serving as a multiple-choice question writer for Continuum with the American Academy of Neurology. Dr Gelb has received publishing royalties from a publication relating to health care.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Gelb reports no disclosure.

Nuri Jacoby, MD, FAAN
Associate Professor of Clinical Neurology, SUNY Downstate Health Sciences University; Attending Neurologist, Maimonides Medical Center, Brooklyn, New York

Relationship Disclosure: Dr Jacoby has received personal compensation in the range of $500 to $4999 for serving as a multiple-choice question writer for Continuum with the American Academy of Neurology and on scientific advisory or data safety monitoring boards for Alexion Pharmaceuticals, Inc and argenx and in the range of $5000 to $9999 for serving as an expert witness. The institution of Dr Jacoby has received research support from the American Board of Psychiatry and Neurology.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Jacoby reports no disclosure.

AAll relevant financial relationships have been mitigated.

ACCREDITATION

Accreditation Statement: The American Academy of Neurology Institute is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide CME for physicians. For information on Continuum Audio CME, please visit continuum.audio-digest.org.

AMA Credit: The American Academy of Neurology Institute designates this enduring material for a maximum of 20 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

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 authors 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 continuing medical education (CME) and self-assessment offerings.
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. Audio interviews with the authors of Continuum articles are published alongside each article, and 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.