Elan D Louis1. 1. Department of Neurology, Yale School of Medicine, Yale University, New Haven, CT, USA; Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, CT, USA; Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, CT, USA. Electronic address: elan.louis@yale.edu.
Abstract
BACKGROUND: Although essential tremor (ET) is commonly encountered in clinical practice, historically, there has been considerable disagreement as how to best define it, and now with a growing sense of its clinical complexity, how to best encapsulate it. Here, I draw attention to five issues of current uncertainty. METHODS: A PubMed search conducted on June 19, 2017 crossed "essential tremor" with 9 second search terms (e.g., definition, diagnosis). RESULTS: There are several major issues of clinical and diagnostic uncertainty. Underlying each issue is a larger question about the nature of the underlying pathophysiology of ET. Does age of onset of ET matter? How much dystonia is acceptable in ET? How much in the way of "cerebellar signs" are acceptable? Are non-motor features due to the underlying disease or merely secondary to the clinical features? Is ET a single disease entity or something else? CONCLUSIONS: We are learning more about ET and, as a by-product of these efforts, are struggling with its definition. Further understanding the nature of the underlying disease pathogenesis as well as the role the cerebellum and cerebellar relays play in this process will likely provide important clues to enable us to bring order to areas of uncertainty.
BACKGROUND: Although essential tremor (ET) is commonly encountered in clinical practice, historically, there has been considerable disagreement as how to best define it, and now with a growing sense of its clinical complexity, how to best encapsulate it. Here, I draw attention to five issues of current uncertainty. METHODS: A PubMed search conducted on June 19, 2017 crossed "essential tremor" with 9 second search terms (e.g., definition, diagnosis). RESULTS: There are several major issues of clinical and diagnostic uncertainty. Underlying each issue is a larger question about the nature of the underlying pathophysiology of ET. Does age of onset of ET matter? How much dystonia is acceptable in ET? How much in the way of "cerebellar signs" are acceptable? Are non-motor features due to the underlying disease or merely secondary to the clinical features? Is ET a single disease entity or something else? CONCLUSIONS: We are learning more about ET and, as a by-product of these efforts, are struggling with its definition. Further understanding the nature of the underlying disease pathogenesis as well as the role the cerebellum and cerebellar relays play in this process will likely provide important clues to enable us to bring order to areas of uncertainty.
Authors: Elan D Louis; Phyllis L Faust; Jean-Paul G Vonsattel; Lawrence S Honig; Claire Henchcliffe; Rajesh Pahwa; Kelly E Lyons; Eileen Rios; Cordelia Erickson-Davis; Carol B Moskowitz; Arlene Lawton Journal: Mov Disord Date: 2009-08-15 Impact factor: 10.338
Authors: Takashi Tsuboi; Zakia Jabarkheel; Pamela R Zeilman; Matthew J Barabas; Kelly D Foote; Michael S Okun; Aparna Wagle Shukla Journal: Neurology Date: 2020-02-11 Impact factor: 9.910
Authors: Daniella Iglesias-Hernandez; Silvia Chapman; Keith Radler; Hollie Dowd; Edward D Huey; Stephanie Cosentino; Elan D Louis Journal: Tremor Other Hyperkinet Mov (N Y) Date: 2021-05-11
Authors: Myung Ji Kim; Kyung Won Chang; So Hee Park; Won Seok Chang; Hyun Ho Jung; Jin Woo Chang Journal: Front Neurol Date: 2021-06-09 Impact factor: 4.003