Movement Disorders

October 2022, Volume 28, Issue 5
BROWSE ISSUES

Movement Disorders

October 2022, Vol.28, No.5

Guest Editor:

Kathleen L. Poston, MD, MS, FAAN

Editor-in-Chief:

STEVEN L. LEWIS, MD, FAAN

ISSN: 1080-2371

Online ISSN: 1538-6899

REVIEW ARTICLES
Prodromal α-Synucleinopathies
Chahine, Lana M.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1268-1280
doi: 10.1212/CON.0000000000001153
Diagnosis and Medical Management of Parkinson Disease
Thaler, Avner; Alcalay, Roy N.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1281-1300
doi: 10.1212/CON.0000000000001152
Surgical Therapies for Parkinson Disease
Rawls, Ashley E.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1301-1313
doi: 10.1212/CON.0000000000001160
Diagnosis and Treatment of Cognitive and Neuropsychiatric Symptoms in Parkinson Disease and Dementia With Lewy Bodies
Weintraub, Daniel; Irwin, David
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1314-1332
doi: 10.1212/CON.0000000000001151
Diagnosis and Treatment of Essential Tremor
Wagle Shukla, Aparna
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1333-1349
doi: 10.1212/CON.0000000000001181
Multiple System Atrophy
Claassen, Daniel O.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1350-1363
doi: 10.1212/CON.0000000000001154
Progressive Supranuclear Palsy and Corticobasal Syndrome
Pantelyat, Alexander
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1364-1378
doi: 10.1212/CON.0000000000001158
Chorea
Stimming, Erin Furr; Bega, Danny
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1379-1408
doi: 10.1212/CON.0000000000001169
Neurodegenerative Cerebellar Ataxia
Rosenthal, Liana S.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1409-1434
doi: 10.1212/CON.0000000000001180
The Dystonias
Stephen, Christopher D.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1435-1475
doi: 10.1212/CON.0000000000001159
Diagnosing Common Movement Disorders in Children
O’Malley, Jennifer A.
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1476-1519
doi: 10.1212/CON.0000000000001187
Palliative Care and Movement Disorders
Katz, Maya
CONTINUUM: Lifelong Learning in Neurology
October 2022 - Volume 28 - Issue 5, Movement Disorders - p 1520-1529
doi: 10.1212/CON.0000000000001162
Editor's Preface
Articles
Key Points
Abbreviations
Appendix
Issue Overview

Issue Overview

CONTINUUM: Lifelong Learning in Neurology October 2022 - Volume 28 - Issue 5, Movement Disorders -10.1212/01.CON.0000892540.33029.c0 doi: 10.1212/01.CON.0000892540.33029.c0

Issue Overview

Movement Disorders, Volume 28, Number 5, October 2022

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 Movement Disorders issue, participants will be able to:

  • Assess and manage individuals who are prodromal or at risk for α-synucleinopathies
  • Describe the key symptoms, prodromal stage, genetic basis, and current treatments of Parkinson disease
  • Discuss advanced surgical therapies for Parkinson disease, appropriate patient selection, and outcomes of each therapy
  • Assess and manage the wide range of psychiatric and cognitive complications occurring in Parkinson disease and dementia with Lewy bodies
  • Diagnose and manage patients with essential tremor and distinguish essential tremor from other tremor disorders
  • Develop a comprehensive approach to the diagnosis and treatment of multiple system atrophy
  • Diagnose and treat patients with progressive supranuclear palsy and corticobasal syndrome
  • Discuss the phenomenology of chorea and the differential diagnosis and management strategies for hereditary and acquired causes of chorea
  • Describe the differential diagnosis, evaluation, and management of neurodegenerative cerebellar ataxia
  • Diagnose and classify genetic and idiopathic dystonia and discuss the multidisciplinary treatment of dystonia, including pharmacologic, surgical, and rehabilitation therapies, and the emergency treatment of status dystonicus/dystonic storm
  • Identify the most common pediatric movement disorders and recognize benign versus pathologic movements in infancy and childhood with a particular focus on treatable conditions and those that should not be missed
  • Discuss the role of palliative care in the treatment of life-limiting neurodegenerative movement disorders, including the specialized approach of palliative care to symptom management, clinical communication, and advance care planning

Core Competencies

This Continuum: Lifelong Learning in Neurology Movement 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

Contributors

Kathleen L. Poston, MD, MS, FAAN, Guest Editor

Edward F. and Irene Thiele Pimley Professor in Neurology and Neurological Sciences; Chief, Movement Disorders, Stanford University, Stanford, California

Relationship Disclosure: Dr Poston has received personal compensation in the range of $0 to $499 for serving on a clinical advisory board for Amprion Inc and a data safety monitoring board for the National Institutes of Health, in the range of $500 to $4999 for serving as an associate editor for the Movement Disorders Journal and as an assistant editor for Annals of Neurology, and in the range of $10,000 to $49,999 for serving on a scientific advisory board for CuraSen Therapeutics, Inc. Dr Poston has stock in Amprion Inc and CuraSen Therapeutics, Inc, and has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr Poston has received research support from the Alzheimer's Drug Discovery Foundation, the Lewy Body Dementia Association, Inc, The Michael J. Fox Foundation, and the National Institutes of Health.

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

Roy N. Alcalay, MD, MS

Chief, Movement Disorders Division, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Associate Professor of Clinical Neurology, Department of Neurology, Columbia University Irving Medical Center, New York, New York

Relationship Disclosure: Dr Alcalay has received personal compensation in the range of $500 to $4999 for serving as a consultant for AVROBIO, Inc; Caraway Therapeutics, Inc; GlaxoSmithKline plc; Janssen Global Services, LLC; Merck & Co, Inc; Ono Pharmaceutical Co, Ltd; and Takeda Pharmaceutical Company. Dr Alcalay has received personal compensation in the range of $10,000 to $49,999 for serving as a consultant for Sanofi. The institution of Dr Alcalay has received research support from Biogen, the Department of Defense, The Michael J. Fox Foundation, the National Institutes of Health, and the Parkinson’s Foundation.

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

Danny Bega, MD

Associate Professor of Neurology and Director, Neurology Residency, Director, Huntington’s Disease Society of America Center of Excellence, Northwestern University Feinberg School of Medicine, Chicago, Illinois

Relationship Disclosure: Dr Bega has received personal compensation in the range of $500 to $4999 for serving on a speakers bureau for Acorda Therapeutics; Adamas Pharmaceuticals, Inc; Kyowa Kirin Co, Ltd; Neurocrine Biosciences, Inc; and Teva Pharmaceutical Industries Ltd and as an editor, associate editor, or editorial advisory board member for the Annals of Clinical and Translational Neurology/American Neurological Association. The institution of Dr Bega has received research support from the Huntington’s Disease Society of America.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Bega discusses the unlabeled/investigational use of amantadine, benzodiazepines, botulinum toxin injections, cannabinoids, carbamazepine, clozapine, deep brain stimulation, olanzapine, quetiapine, risperidone, and valbenazine for the treatment of Huntington disease and other causes of chorea.

Lana Chahine, MD, FAAN

Associate Professor, Department of Neurology, University of Pittsburgh, Pittsburgh, Pennsylvania

Relationship Disclosure: Dr Chahine has received personal compensation in the range of $500 to $4999 for serving as a consultant for Gray Matters Technology Services. Dr Chahine has received publishing royalties from a publication relating to health care. The institution of Dr Chahine has received research support from the Biogen/Parkinson Study Group, The Michael J. Fox Foundation, and University of Pittsburgh Medical Center.

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

Daniel O. Claassen, MD, MS, FAAN

Division Chief, Behavioral and Cognitive Neurology; Professor, Neurology, Vanderbilt University Medical Center, Nashville, Tennessee

Relationship Disclosure: Dr Claassen has received personal compensation in the range of $500 to $4999 for serving as a consultant for Spark Therapeutics, Inc and as an editor, associate editor, or editorial advisory board member for HD Insights. Dr Claassen has received personal compensation in the range of $5000 to $9999 for serving as a consultant and on a scientific advisory or data safety monitoring board for Teva Neuroscience, Inc. Dr Claassen has received personal compensation in the range of $50,000 to $99,999 for serving as a consultant for Alterity Therapeutics. The institution of Dr Claassen has received research support from AbbVie Inc; the CHDI Foundation; Genentech, Inc/R. Hoffman-La Roche Ltd; the Griffin Family Foundation; the Huntington’s Disease Society of America; the National Institutes of Health, Neurocrine Biosciences, Inc/Huntington Study Group; Prilenia Therapeutics; the US Department of Defense; and Vaccinex Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Claassen discusses the unlabeled/investigational use of acarbose (α-glucosidase inhibitor) for postprandial hypotension, baclofen and gabapentin for pain/dystonia, levetiracetam for cerebellar outflow tremor, serotonin norepinephrine reuptake inhibitors (eg, duloxetine) for pain, and short-acting antihypertensives (eg, nifedipine, transdermal topical nitroglycerin) for supine hypertension.

Erin Furr Stimming, MD, FAAN

Professor of Neurology and Director, Neurology Clerkship, Director, Huntington’s Disease Society of America Center of Excellence, The University of Texas Health Science Center at Houston, McGovern Medical School, Houston, Texas

Relationship Disclosure: Relationship Disclosure: Dr Furr-Stimming has received personal compensation in the range of $500 to $4999 for serving as a consultant for Teva Pharmaceutical Industries Ltd and Wave Life Sciences, on a scientific advisory board for Amneal Pharmaceuticals LLC, and on a speakers bureau for Sunovion Pharmaceuticals Inc and Teva Pharmaceutical Industries Ltd. Dr Furr Stimming has served as an editor and author for McGraw Hill and Oxford University Press. The institution of Dr Furr Stimming has received research support from the CHDI Foundation; Cures Within Reach; Huntington Study Group/Neurocrine Biosciences, Inc; the Huntington's Disease Society of America; F. Hoffman-La Roche/Genetech, Inc; the National Institutes of Health/University of Iowa; uniQure NV; and Vaccinex Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Furr Stimming discusses the unlabeled/investigational use of amantadine, benzodiazepines, botulinum toxin injections, cannabinoids, carbamazepine, clozapine, deep brain stimulation, olanzapine, quetiapine, risperidone, and valbenazine for the treatment of Huntington disease and other causes of chorea.

David Irwin, MD

Assistant Professor of Neurology, University of Pennsylvania, Perelman School of Medicine Philadelphia, Pennsylvania

Relationship Disclosure: Dr Irwin has received personal compensation in the range of $500 to $4999 for serving on a scientific advisory or data safety monitoring board for Denali Therapeutics. The institution of Dr Irwin has received research support from the National Institutes of Health (U19-AG062418).

Unlabeled Use of Products/Investigational Use Disclosure: Dr Irwin discusses the use of several medications and treatments for the neuropsychiatric symptoms of Parkinson disease and dementia with Lewy bodies, none of which are approved by the US Food and Drug Administration, except for rivastigmine for the treatment of Parkinson dementia and pimavanserin for the treatment of psychosis in Parkinson disease.

Maya Katz, MD

Clinical Associate Professor of Neurology, Stanford University, Stanford, California

Relationship Disclosure: Dr Katz has received research support from the National Institutes of Health (1R01NR016037-01A1).

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

Jennifer A. O’Malley, MD, PhD

Clinical Assistant Professor, Neurology and Pediatrics, Stanford University School of Medicine, Palo Alto, California

Relationship Disclosure: Dr O’Malley has received personal compensation in the range of $5000 to $9999 for serving on a speakers bureau for PTC Therapeutics and has stock in Doximity. The institution of Dr O’Malley has received research support from Grace Science, LLC.

Unlabeled Use of Products/Investigational Use Disclosure: Dr O’Malley discusses the unlabeled/investigational use of deep brain stimulation for movement disorders in children and gene therapy for aromatic l-amino acid decarboxylase deficiency.

Alexander Pantelyat, MD, FAAN

Director, Atypical Parkinsonism Center; Assistant Professor of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Relationship Disclosure: Dr Pantelyat has received personal compensation in the range of $5000 to $9999 for serving as a scientific advisory board member for MedRhythms, Inc, and in the range of $10,000 to $49,999 for serving as an expert witness for Kelly & Ignoffo Law Group. The institution of Dr Pantelyat has received research support from the National Institutes of Health/National Institute on Aging.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Pantelyat discusses the unlabeled/investigational use of dextromethorphan/quinidine or selective serotonin reuptake inhibitors (SSRIs) for the treatment of pseudobulbar affect in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS); methylphenidate or modafinil for the treatment of apathy in PSP and CBS; miglustat for Niemann-Pick disease type C; onabotulinumtoxinA for the treatment of sialorrhea, cervical and limb dystonia/spasticity, muscle pain, and eyelid opening apraxia/blepharospasm in PSP and CBS; and SSRIs for the treatment of depression and anxiety in PSP and CBS.

Ashley E. Rawls, MD, MS

Assistant Professor of Neurology, University of Florida, Norman Fixel Institute for Neurological Diseases, Gainesville, Florida

Relationship Disclosure: Dr Rawls has received personal compensation in the range of $500 to $4999 for serving as an editor, associate editor, or editorial advisory board member for JAMA Neurology and as a physician expert panelist with Mediflix, Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Rawls discusses the unlabeled/investigational use of closed-loop deep brain stimulation for the treatment of Parkinson disease.

Liana S. Rosenthal, MD, PhD

Director, Johns Hopkins Ataxia Center; Co-Director, Johns Hopkins Lewy Body Disease Association Research Center of Excellence; Associate Professor of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Relationship Disclosure: Dr Rosenthal has received personal compensation in the range of $500 to $4999 for serving as a consultant for Biohaven Pharmaceuticals and on a scientific advisory or data safety monitoring board for Reata Pharmaceuticals, Inc; has received research support from Biohaven Pharmaceuticals, The Daniel B. and Florence E. Green Foundation, the Gordon and Marilyn Macklin Foundation, The Michael J. Fox Foundation, the National Ataxia Foundation, the National Institutes of Health, and Pfizer Inc; and has a noncompensated relationship as a Medical Director, ex-officio Member of the Board with National Ataxia Foundation that is relevant to American Academy of Neurology interests or activities.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Rosenthal discusses the unlabeled/investigational use of medications for the treatment of neurodegenerative spinocerebellar ataxias, none of which are approved by the US Food and Drug Administration.

Christopher D. Stephen, MB ChB, FRCP, SM

Instructor in Neurology, Harvard Medical School; Attending Neurologist, Movement Disorders Unit, Ataxia Center, Dystonia Clinic, Department of Neurology, Massachusetts General Hospital, Brigham and Women's Hospital, Boston, Massachusetts

Relationship Disclosure: Dr Stephen has received personal compensation in the range of $500 to $4999 for serving on a scientific advisory or data safety monitoring board for SwanBio Therapeutics, Inc, has received research support from the National Institutes of Health/National Institute of Neurological Disorders and Stroke (1K23NS118045-01A1), and has received honoraria in the range of $500 to $4999 from the International Parkinson and Movement Disorder Society. The institution of Dr Stephen has received research support from Sanofi.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Stephen discusses the unlabeled/investigational use of medications and therapies, none of which are approved by the US Food and Drug Administration (FDA) except for botulinum toxin injections; medications and therapies for the treatment of paroxysmal dystonia, none of which are approved by the FDA for this indication; medications and therapies for the treatment of status dystonicus/dystonic storm, none of which are approved by the FDA for this indication; dopamine agonists for the treatment of dystonia parkinsonism; ethylenediaminetetraacetic acid chelation therapy for the treatment of dystonia/parkinsonism with manganese accumulation; dopamine agonists, monoamine oxidase inhibitors, and pyridoxine for the treatment of aromatic l-amino acid decarboxylase deficiency; levodopa and 5-hydroxytryptophan for the treatment of complex dopa-responsive dystonia; L-carnitine for the treatment of glutaric aciduria type 1, methylmalonic aciduria, and propionic acidemia; vitamin B6 for the treatment of homocystinuria; biotin and thiamine for the treatment of biotin-thiamine–responsive basal ganglia disease and biotinidase deficiency; and coenzyme Q10 for the treatment of coenzyme Q10 deficiency.

Avner Thaler, MD, PhD

Assistant Professor of Neurology, Sackler Faculty of Medicine, Sagol School of Neuroscience, Tel Aviv University; Tel Aviv Sourasky Medical Center, Tel Aviv, Israel

Relationship Disclosure: Dr Thaler has received personal compensation in the range of $500 to $4999 for serving as a consultant for AbbVie Inc. The institution of Dr Thaler has received research support from Biogen and The Michael J. Fox Foundation.

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

Aparna Wagle Shukla, MD

Professor, Department of Neurology, Norman Fixel Institute for Neurological Diseases, University of Florida Health, Gainesville, Florida

Relationship Disclosure: Dr Wagle Shukla has received personal compensation in the range of $500 to $4999 for serving as a consultant for Jazz Pharmaceuticals, Inc, as a reviewer with the National Institutes of Health, and as the Vice President of the board of directors for the Tremor Research Group and in the range of $5000 to $9999 for serving on a scientific advisory board for Acadia Pharmaceuticals Inc. The institution of Dr Wagle Shukla has received research support from the National Institutes of Health.

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

Daniel Weintraub, MD

Professor of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

Relationship Disclosure: Dr Weintraub has received personal compensation in the range of $10,000 to $49,999 for serving as a consultant for Clintrex and on a scientific advisory or data safety monitoring board for Acadia Pharmaceuticals Inc. Dr Weintraub has received personal compensation in the range of $500 to $4999 for serving as a consultant for Eisai Co, Ltd; Jansen Global Services, LLC; Sage Therapeutics, Inc; Scion Pharmaceuticals, Signant Health, and Sunovian Pharmaceuticals Inc, and for serving as an editor, associate editor, or editorial advisory board member for the Movement Disorder Society. Dr Weintraub has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr Weintraub has received research support from The Michael J. Fox Foundation, the International Parkinson and Movement Disorder Society, and the National Institutes of Health.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Weintraub discusses the use of several medications and treatments for the neuropsychiatric symptoms of Parkinson disease and dementia with Lewy bodies, none of which are approved by the US Food and Drug Administration, except for rivastigmine for the treatment of Parkinson dementia and pimavanserin for the treatment of psychosis in Parkinson disease.

Self-Assessment and CME Test Writers

Adam G. Kelly, MD, FAAN

Associate Professor of Neurology; Director of Teleneurology and Regional Neurology, University of Rochester, Rochester, New York

Relationship Disclosure: Dr Kelly has received personal compensation in the range of $500 to $4999 for serving as a consultant for Included Health, Inc, and a CME question writer for the American Academy of Neurology and in the range of $5000 to $9999 for serving as a CME editor for the American Academy of Neurology.

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

D. Joanne Lynn, MD, FAAN

Clinical Professor Emerita, Department of Neurology, The Ohio State University College of Medicine, Columbus, Ohio

Relationship Disclosure: Dr Lynn has received personal compensation in the range of $500 to $4999 for serving as a Continuum Self-Assessment and CME question writer for the American Academy of Neurology; has received publishing royalties from Wolters Kluwer Health, Inc; and has stock in Abbott Laboratories, AbbVie Inc, Amgen Inc, Biogen, Bristol-Myers Squibb Company, Merck & Co Inc, Pfizer Inc, Regeneron Pharmaceuticals Inc, and Zimmer Biomet.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Lynn reports no 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 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. For detailed instructions regarding Continuum CME and self-assessment activities, visit continpub.com/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. 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.

© 2022 American Academy of Neurology.