Parkinson’s Disease and Nonmotor Dysfunction C C N U R R E N T L I N I C A L E U R O L O G Y Daniel Tarsy, , S E MD ERIES DITOR Parkinson’s Disease and Nonmotor Dysfunction, edited by Ronald F. Pfeiffer and Ivan Bodis-Wollner, 2005 Thrombolytic Therapy for Acute Stroke, Second Edition, edited by Patrick D. Lyden, 2005 Movement Disorder Emergencies: Diagnosis and Treatment,edited by Steven J. Frucht and Stanley Fahn, 2005 Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management,edited by Alireza Minagar and J. Steven Alexander, 2005 Neurological and Psychiatric Disorders: From Bench to Bedside,edited by Frank I. Tarazi and John A. Schetz, 2005 Multiple Sclerosis: Etiology, Diagnosis, and New Treatment Strategies,edited byMichael J. Olek, 2005 Seizures in Critical Care: A Guide to Diagnosis and Therapeutics,edited byPanayiotis N. Varelas, 2005 Vascular Dementia: Cerebrovascular Mechanisms and Clinical Management, edited by Robert H. 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Kaminski, 2003 Seizures:Medical Causes and Management, edited by Norman Delanty, 2002 Clinical Evaluation and Management of Spasticity, edited by David A. Gelber and Douglas R. Jeffery, 2002 Early Diagnosis of Alzheimer's Disease, edited by Leonard F. M. Scinto and Kirk R. Daffner, 2000 Sexual and Reproductive Neurorehabilitation, edited by Mindy Aisen, 1997 Parkinson’s Disease and Nonmotor Dysfunction Edited by Ronald F. Pfeiffer, MD Department of Neurology, University of Tennessee Health Science Center, Memphis, TN and Ivan Bodis-Wollner, , MD DSc Departments of Neurology and Ophthalmology, State University of New York, Downstate Medical Center, Brooklyn, NY © 2005 Humana Press Inc. 999 Riverview Drive, Suite 208 Totowa, New Jersey 07512 humanapress.com All rights reserved. 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For those organizations that have been granted a photocopy license from the CCC, a separate system of payment has been arranged and is acceptable to Humana Press Inc. The fee code for users of the Transactional Reporting Service is: [1-58829-316-5/05 $30.00 ]. e-ISBN 1-59259-859-5 Printed in the United States of America. 10 9 8 7 6 5 4 3 2 1 Library of Congress Cataloging-in-Publication Data Parkinson's disease and nonmotor dysfunction / edited by Ronald F. Pfeiffer and Ivan Bodis-Wollner. p. ; cm. -- (Current clinical neurology) Includes bibliographical references and index. ISBN 1-58829-316-5 (alk. paper) 1. Parkinson's disease. [DNLM: 1. Parkinson Disease--complications. 2. Autonomic Nervous System Diseases--etiology. 3. Behavioral Symptoms--etiology. 4. Sensation Disorders--etiology. 5. Sleep Disorders--etiology. WL 359 P24654 2005] I. Pfeiffer, Ronald. II. Bodis-Wollner, Ivan, 1937- III. Title. IV. Series. RC382.P2577 2005 616.8'33--dc22 2004025976 Series Editor’s Introduction Parkinson’s Disease and Nonmotor Dysfunction fills a major gap in the current rapidly growing body of knowledge concerning Parkinson’s disease. Drs. Pfeiffer and Bodis-Wollner have correctly perceived that many nonmotor features of Parkinson’s disease are given insufficient attention in the medical literature. Unfortunately, they are often also given insufficient attention by the practicing neurologists who see these patients. As recently pointed out, there is clearly much more to Parkinson’s disease than depletion of the nigrostriatal dopamine system (1). Parkinson’s disease (not just mul- tiple system atrophy) is a multisystem disorder, both pathologically and in its clinical manifestations. This is clearly true for the various motor system abnormalities, which are not fully corrected by dopamine replacement therapy strategies, but also for the nonmotor system abnormalities that are the subject of this volume. Although recently there has been increased awareness of the cognitive, psychiatric, and sleep disorders commonly associated with Parkinson’s disease, many of their manifestations remain under-recognized and their importance in managing patients is underestimated. Even less attention is paid to the myriad of other nonmotor disturbances that plague these patients. For example, among the autonomic disorders, although orthostatic hypotension is well recognized, it is usually attributed to dopaminergic medications rather than to effects of the underlying disease. Urologic disorders are also very familiar in these patients, but may not be properly understood or well managed. Beyond this, there is much less awareness of the less common and less obvious autonomic disorders which are herein reviewed chapter by chapter. Their link to Parkinson’s disease is usually unappreciated and there is frequently an unfortunate tendency to attribute the symptoms they produce to advancing age. Finally, unusual sensory and painful phenomena, dysphagia, fatigue, and visual deficits are other less common features of this remarkably varied disease that readers of this book may become acquainted with as signs of Parkinson’s disease for the first time. This book highlights the fact that management of Parkinson’s disease requires a multidisciplinary approach. This may not always require referral to a consultant but at least requires awareness of the spectrum of nonmotor symptoms. Some of these symptoms may be more subtle than others. Under- standably, many will not be forthcoming in the typical initial encounter in which more troublesome motor symptoms usually dominate the visit. Some need to be elicited by careful inquiry on the part of the medical caregiver. Symptom questionnaires may be especially useful in gathering symptoms that can be returned to in subsequent visits. Knowledge and understanding of Parkinson’s disease is grow- ing exponentially. This volume establishes the baseline of current knowledge and undoubtedly will stimulate further fruitful inquiry in the field. 1. Lang AE, Obeso JA. Challenges in Parkinson’s disease: restoration of the nigrostriatal dopam- ine system is not enough. Lancet 2004; 3:309–316. Daniel Tarsy, MD Parkinson’s Disease & Movement Disorders Center Beth Israel Deaconess Medical Center Harvard Medical School Boston, MA v Preface The idea that Parkinson’s disease (PD) is characterized only by such motor features as tremor, rigidity, bradykinesia, and postural instability is deeply embedded not only in the minds of patients and their family members, but also in the training and practice of many physicians. However, even a quick perusal of the amazingly perceptive clinical description that James Parkinson put to paper in 1817 reveals that from the beginning, various features not reflective of motor dysfunction were recognized and described as being part of PD. It has only been relatively recently that attention has been refocused on these nonmotor features, and the realization has grown that nonmotor features are frequently present in PD and can be the source of considerable discomfort and disability for affected individuals. In fact, it is not at all infrequent that these nonmotor features play a dominant role in the clinical picture. The growing recognition that nonmotor features are an important component of PD has, in turn, led to the realization that these aspects of the condition have often received insufficient attention in the current medical and lay literature. This volume on Parkinson’s Disease and Nonmotor Dysfunc- tion is an attempt to at least partially correct that deficiency and provide a source of detailed informa- tion describing and explaining these nonmotor features that can be readily accessed by the practicing physician. To this end, a truly outstanding group of experienced researchers and clinicians has been assembled to provide this in-depth review of nonmotor dysfunction in PD, which has been subdi- vided into five diverse domains. Behavioral abnormalities are problems encountered in the management of PD, particularly in individuals with more advanced disease, which are both distressingly frequent and frequently dis- tressing. They may be intrinsic components of the disease process itself (depression and dementia), treatment-induced complications (psychosis and postsurgical behavioral changes), or a combination of both (anxiety, obsessionality). Whatever their derivation, behavioral abnormalities can seriously impact and impair quality of life for both patients and family members. Autonomic dysfunction is often mistakenly considered to be a feature of multiple system atrophy and not PD. In reality, individuals with PD can, and frequently do, display various features indicative of autonomic dysfunction. Gastrointestinal, urogenital, cardiorespiratory, thermoregulatory, and other aspects of autonomic function may become impaired in PD, not simply as consequences of medica- tion-induced derangements, but as part of the disease process itself. These autonomic features often develop in the later stages of the illness but may also appear early, occasionally even before the classic motor components become evident. Sleep-related dysfunction can be a source of considerable consternation, not only to patients but also to their family members, who often suffer the indirect, and sometimes the direct (at least in the setting of rapid eye movement sleep behavior disorder), consequences of the patient’s sleep distur- bance. As with behavioral and autonomic dysfunction, sleep-related disturbances can be either dis- ease-related or medication-induced and may occur both early and later in the course of PD. Sensory dysfunction is perhaps the least well-known or recognized and also the most purely nonmotor facet of nonmotor dysfunction in PD. Abnormalities of primary sensory function (vision and olfaction) occur, as do more complex sensory phenomena, as exemplified by the visuo-cognitive deficits and the various pain syndromes and disorders of sensation that may plague the patient with PD. Finally, a section of this volume is devoted to several problems (oculomotor dysfunction and fatigue) that tread on, or perhaps cross over, the line between motor and nonmotor dysfunction in PD. However, they are included here because they often are not covered extensively in the more tradi- tional discussions of the motor features of PD. vii viii Preface It is our hope that this collection of contributions by a truly tremendous contingent of authors will serve to increase awareness of the contributions that nonmotor features may make to the collective clinical picture experienced by the patient with PD. Early recognition of these features will lead, we hope, to more prompt and effective treatment of them, a goal that can be firmly shared and appreci- ated by both patient and physician alike. Ronald F. Pfeiffer, MD Ivan Bodis-Wollner, MD,DSc Contents Series Editor’s Introduction.............................................................................................................v Preface................................................................................................................................................vii Contributors.......................................................................................................................................xi Part I. Behavioral Dysfunction in Parkinson’s Disease 1 Depression.................................................................................................................................3 William J. Burke, Steven P. Wengel, and Daryl Bohac 2 Anxiety.....................................................................................................................................13 Hubert H. Fernandez and Tanya Simuni 3 Obsessionality.........................................................................................................................25 Marie-Andrée Bruneau 4 Dementia..................................................................................................................................35 Patricia Kavanagh and Karen Marder 5 Psychosis..................................................................................................................................49 Eric S. Molho and Stewart A. Factor 6 Postsurgical Behavioral Changes........................................................................................75 Laurie M. Rilling, John A. Lucas, and Ryan J. Uitti Part II. Autonomic Dysfunction in Parkinson’s Disease 7 Dysphagia................................................................................................................................95 Norman A. Leopold 8 Gastric Dysfunction.............................................................................................................105 Tanya Gurevich, Amos D. Korczyn, and Nir Giladi 9 Intestinal Dysfunction.........................................................................................................115 Ronald F. Pfeiffer 10 Impaired Sexual Function...................................................................................................127 Cheryl Waters and Janice Smolowitz 11 Urological Dysfunction.......................................................................................................139 Carlos Singer 12 Cardiovascular Autonomic Dysfunction.........................................................................149 David S. Goldstein 13 Thermoregulatory Dysfunction.........................................................................................159 Mark S. LeDoux 14 Respiratory Dysfunction.....................................................................................................173 Holly A. Shill Part III. Sleep-Related Dysfunction in Parkinson’s Disease 15 Insomnia.................................................................................................................................181 Maria L. Moro-de-Casillas and David E. Riley 16 Rapid Eye Movement Sleep Behavior Disorder.............................................................191 Suzanne Stevens and Cynthia Comella ix x Contents 17 Excessive Daytime Sleepiness............................................................................................199 David Hardesty, Daryl Victor, and Steven J. Frucht 18 Sleep Apnea...........................................................................................................................209 Cheryl M. Carlucci and Robert A. Hauser Part IV. Sensory Dysfunction in Parkinson’s Disease 19 Visual Dysfunction ..............................................................................................................223 Robert L. Rodnitzky 20 Primary Visual and Visuocognitive Deficits...................................................................233 Ivan Bodis-Wollner and Andrea Antal 21 Olfactory Dysfunction.........................................................................................................245 Sarah Furtado and Zbigniew K. Wszolek 22 Pain Syndromes and Disorders of Sensation..................................................................255 Blair Ford and Ronald F. Pfeiffer Part V. Sensorimotor Dysfunction in Parkinson’s Disease 23 Oculomotor Dysfunction....................................................................................................271 Parashkev Nachev and Christopher Kennard 24 Fatigue....................................................................................................................................281 Carol Ewing Garber and Joseph H. Friedman Index.................................................................................................................................................295