The Autonomic Nervous System

This is the first Seminars in Neurology devoted to the autonomic nervous system. The topics covered include autonomic nervous system diseases, the investigations of autonomic function that are used in clinical practice and research, and the treatment of common autonomic problems. The material should be of interest to both the general neurologist and the academician. This issue is loosely based on a series of courses on the autonomic nervous system held at the American Academy of Neurology annual meeting over the past 5 years, and many of the authors have participated in one or more of these courses. The authors who have contributed to this issue of Seminars in Neurology represent, in no small measure, the diversity of the autonomic nervous system.

Horacio Kaufmann (Professor of Neurology, Mount Sinai Medical School) and Italo Biaggioni (Professor of Medicine, Vanderbilt University) wrote the article “Autonomic Failure in Neurodegenerative Disorders.” Dr. Kaufmann's many interests include the role played by the vestibular apparatus in maintaining blood pressure during movements against gravity, therapeutics, and syncope. Of relevance to this article is his recent work unraveling the relationships among the synucleinopathies that are associated with autonomic dysfunction. These disorders are characterized by the accumulation of α-synuclein within cytoplasmic inclusions in the central and peripheral neurons. Dr. Biaggioni's work exemplifies the use of pharmacological probes to dissect autonomic control mechanisms. He has used this approach to examine disorders of orthostatic intolerance and hypertension. He has coauthored a book on autonomic disorders. Additional research areas of interest include the role played by adenosine in physiological and pathophysiological processes and the therapy of autonomic disorders.

Arthur Vinik (Professor of Medicine, Eastern Virginia Medical School), Tomris Erbas (Professor of Medicine, Hacettepe Medical School), and I coauthored the article on diabetic autonomic neuropathy. Dr. Vinik has diverse research interests and is a leading authority on many aspects of diabetes and other endocrine disorders. His areas of expertise include hormone secreting tumors, lipid disorders, and the complications of diabetes, particularly diabetic neuropathy. Of special relevance is his current work on the molecular biology of the islet cell and the role played by genetics in islet cell regeneration. Dr. Erbas is an endocrinologist. She did a research fellowship with Dr. Vinik at the East Virginia Medical School and has returned to Turkey. Her research interests are in diabetic peripheral neuropathy and neuroendocrinology.

Cory Toth (Clinical Fellow) and Doug Zochodne (Professor in the Department of Clinical Neurosciences, University of Calgary) wrote the article on “other” autonomic neuropathies. Dr. Zochodne has made major contributions to our understanding of peripheral nerve injury, repair, and regeneration. An important focus of his work has been the investigation into the pathogenesis of diabetic polyneuropathy. During his fellowship in the Neuromuscular Unit at the University of Calgary, Dr. Toth has focused on clinical neuromuscular disease, experimental diabetes, and nerve regeneration.

Felicia B. Axelrod and Max Hilz contributed the article on inherited autonomic neuropathies. Dr. Axelrod is the Professor of Dysautonomia Treatment and Research and Professor of Neurology, New York University School of Medicine. Her research has concentrated on the inherited disorders of the autonomic and sensory nervous systems. Her extensive studies of the clinical features, physiology, and therapeutic interventions for these disorders laid the groundwork for the recent discovery of the genetic mutations responsible for familial dysautonomia. Max Hilz is Professor of Neurology and Medicine, New York University School of Medicine, and Professor of Neurology, University of Erlangen. In addition to his collaborative studies with Dr. Axelrod on the neurophysiology of inherited autonomic neuropathies, Dr. Hilz has studied and published extensively on autonomic topics that include cerebral autoregulation, autonomic control of urogenital function, and autonomic aspects of epilepsy.

Indu Taneja (Research Associate) and David Robertson (Professor of Medicine, Pharmacology, and Neurology, Vanderbilt University) contributed the article on the genetic basis of autonomic dysfunction. Dr. Robertson's unique contributions to autonomic research include the elucidation of the disorders of orthostatic tolerance and the investigation of autonomic dysfunction associated with manned space flight. He has coauthored a text on autonomic disorders and a primer on the autonomic nervous system, which is now in its second edition. Of relevance to this article is the pioneering role played by Dr. Robertson in linking the molecular biology of the autonomic nervous system with clinical autonomic disorders. This is exemplified in his series of papers on the genotype and phenotype of dopamine β-hydroxylase deficiency. Dr. Taneja's research aims at better understanding of the genetic basis of autonomic disorders.

William Cheshire (Associate Professor of Neurology, Mayo Clinic School of Medicine) and I contributed the article on disorders of sweating. Dr. Cheshire has a long-standing history in sweating disorders and has published on the treatment of hyperhidrosis. His other interests include trigeminal neuralgia, an area in which he has published extensively, and medical ethics.

Phillip A. Low (Professor of Neurology, Mayo Clinic School of Medicine) authored the article on testing the autonomic nervous system. Dr. Low's contributions to autonomic neuroscience are extensive. He has introduced tests of the autonomic nervous system that are now used in clinical practice. In his early work he elucidated the autonomic neuropathies associated with diabetes and alcohol ingestion. More recently, he has clarified the pathophysiology of that common disorder of orthostatic tolerance, the postural tachycardia syndrome. His book, Clinical Autonomic Disorders, which is now in its second edition, is widely regarded as the autonomic reference text of first choice. His basic research has focused on diverse autonomic topics and, in addition, the elucidation of the pathophysiological mechanisms underlying the peripheral neuropathy of diabetes.

David S. Goldstein (Chief of the Clinical Neurocardiology Section, NIH National Institute of Neurological Disorders and Stroke) wrote the article on imaging the autonomic nervous system. Dr. Goldstein is a widely recognized authority on catecholamines and the autonomic response to stress. Among several single author books that he has written, the text Stress, Catecholamines and Cardiovascular Disease merits particular mention. His work on imaging the autonomic nervous system has helped explain the complex relationship between the central and peripheral autonomic neurodegenerative disorders.

Apostolos N. Apostolidis (Clinical Research Fellow, Institute of Neurology, University College of London) and Clare Fowler (Professor of Neurology, University College of London, and Consultant Uro-Neurologist at the National Hospital for Neurology and Neurosurgery, Queens Square) contributed the article on urogenital disorders. Dr. Apostolidis' research focuses on the immunohistochemistry of the primary afferent mechanisms in human detrusor overactivity and bladder hypersensitivity. Dr. Fowler, the preeminent neuro-urologist in the United Kingdom, has published extensively on neuro-urological disorders. She has harnessed contemporary neurophysiological and neuroimaging techniques to enhance our understanding of the urogenital neurophysiology and the diseases of the urogenital system. She is the only member of the faculty to have her own syndrome (Fowler's syndrome), a rare accomplishment in the days of vanishing eponyms.

Thomas Chelimsky (Associate Professor of Neurology, Case Western Reserve University) and Gisela Chelimsky (Assistant Professor of Pediatrics, Case Western Reserve University) coauthored the article on gastrointestinal autonomic disorders. Dr. Thomas Chelimsky directs the Autonomic Disorders Center at the University Hospitals of Cleveland and, in addition to his interest in gastrointestinal disease, his research endeavors to clarify autonomic aspects of pain. Dr. Gisela Chelimsky directs the Motility Laboratory at the Rainbow Babies and Children's Hospital. This team is uniquely positioned to enhance our understanding of the autonomic aspects of gastrointestinal disorders.