Giovanni Defazio1, Tommaso Ercoli1, Roberto Erro2, Roberta Pellicciari3, Marcello Mario Mascia1, Giovanni Fabbrini4,5, Alberto Albanese6, Stefania Lalli6, Roberto Eleopra7, Paolo Barone2, Roberta Marchese8, Roberto Ceravolo9, Cesa Scaglione10, Rocco Liguori10, Marcello Esposito11, Anna Rita Bentivoglio12,13, Laura Bertolasi14, Maria Concetta Altavista15, Francesco Bono16, Antonio Pisani17, Paolo Girlanda18, Alfredo Berardelli4,5. 1. Department of Medical Science and Public Health, Institute of Neurology, University of Cagliari, Cagliari, Italy. 2. Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana," University of Salerno, Baronissi (SA), Italy. 3. Department of Basic Science, Neuroscience and Sense Organs, Aldo Moro University of Bari, Bari, Italy. 4. Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy. 5. IRCSS Neuromed, Pozzili, Italy. 6. Department of Neurology, IRCCS Istituto Clinico Humanitas, Rozzano, Milan, Italy. 7. Neurology 1, Carlo Besta Neurological Institute, Milan, Italy. 8. San Martino Hospital - IRCCS, Genoa, Italy. 9. Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. 10. IRCCS - Institute of Neurological Sciences, Bologna, Italy. 11. Clinical Neurophysiology Unit, Cardarelli Hospital, Naples, Italy. 12. Gemelli University Hospital - IRCCS, Rome, Italy. 13. Institute of Neurology, Università Cattolica del Sacro Cuore, Rome, Italy. 14. Neurologic Unit, University Hospital, Verona, Italy. 15. Neurology Unit, San Filippo Neri Hospital, Rome, Italy. 16. Center for Botulinum Toxin Therapy, Neurologic Unit, Mater Domini University Hospital, Catanzaro, Italy. 17. Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy. 18. Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
Abstract
OBJECTIVE: The objective of this study was to describe the clinical and demographic features of idiopathic non-task-specific upper limb dystonia compared with the task-specific form. METHODS: In this retrospective study, adult patients with idiopathic upper limb dystonia, either focal or as part of a segmental/multifocal dystonia, from the Italian Dystonia Registry were enrolled. In patients with focal upper limb dystonia, dystonia spread was estimated by survival analysis. RESULTS: Of the 1522 patients with idiopathic adult-onset dystonia included in the Italian Dystonia Registry, we identified 182 patients with upper limb dystonia. Non-task-specific dystonia was present in 61.5% of enrolled cases. Women predominated among non-task-specific patients, whereas men predominated in the task-specific group. Peak age of upper limb dystonia onset was in the sixth decade in the non-task-specific group and in the fourth decade in the task-specific group. In both groups, upper limb dystonia started as focal dystonia or as part of a segmental dystonia. Segmental onset was more frequent among non-task-specific patients, whereas focal onset predominated among task-specific patients. Dystonic action tremor was more frequent among non-task-specific patients. No significant differences between groups emerged in terms of sensory trick frequency, rest tremor, or family history of dystonia. In patients with focal upper limb dystonia, dystonia spread was greater in the non-task-specific group. CONCLUSION: Novel information on upper limb dystonia patients suggests that non-task-specific and task-specific upper limb dystonia have different demographic and clinical features. However, it remains to be determined whether these differences also reflect pathophysiological differences.
OBJECTIVE: The objective of this study was to describe the clinical and demographic features of idiopathic non-task-specific upper limb dystonia compared with the task-specific form. METHODS: In this retrospective study, adult patients with idiopathic upper limb dystonia, either focal or as part of a segmental/multifocal dystonia, from the Italian Dystonia Registry were enrolled. In patients with focal upper limb dystonia, dystonia spread was estimated by survival analysis. RESULTS: Of the 1522 patients with idiopathic adult-onset dystonia included in the Italian Dystonia Registry, we identified 182 patients with upper limb dystonia. Non-task-specific dystonia was present in 61.5% of enrolled cases. Women predominated among non-task-specific patients, whereas men predominated in the task-specific group. Peak age of upper limb dystonia onset was in the sixth decade in the non-task-specific group and in the fourth decade in the task-specific group. In both groups, upper limb dystonia started as focal dystonia or as part of a segmental dystonia. Segmental onset was more frequent among non-task-specific patients, whereas focal onset predominated among task-specific patients. Dystonic action tremor was more frequent among non-task-specific patients. No significant differences between groups emerged in terms of sensory trick frequency, rest tremor, or family history of dystonia. In patients with focal upper limb dystonia, dystonia spread was greater in the non-task-specific group. CONCLUSION: Novel information on upper limb dystoniapatients suggests that non-task-specific and task-specific upper limb dystonia have different demographic and clinical features. However, it remains to be determined whether these differences also reflect pathophysiological differences.
Authors: Devdutta S Warhadpande; Jiayan Huo; William A Libling; Carol Stuehm; Bijan Najafi; Scott Sherman; Hong Lei; Janet Meiling Roveda; Phillip H Kuo Journal: Sensors (Basel) Date: 2022-07-05 Impact factor: 3.847
Authors: Giovanni Defazio; Hyder A Jinnah; Alfredo Berardelli; Joel S Perlmutter; Gamze Kilic Berkmen; Brian D Berman; Joseph Jankovic; Tobias Bäumer; Cynthia Comella; Adam C Cotton; Tommaso Ercoli; Gina Ferrazzano; Susan Fox; Han-Joon Kim; Emile Sami Moukheiber; Sarah Pirio Richardson; Anne Weissbach; Laura J Wrigth; Mark Hallett Journal: Parkinsonism Relat Disord Date: 2021-09-08 Impact factor: 4.402
Authors: Tommaso Ercoli; Giovanni Defazio; Christian Geroin; Enrico Marcuzzo; Giovanni Fabbrini; Francesco Bono; Alessandro Mechelli; Roberto Ceravolo; Luigi Michele Romito; Alberto Albanese; Antonio Pisani; Maurizio Zibetti; Maria Concetta Altavista; Luca Maderna; Martina Petracca; Paolo Girlanda; Marcello Mario Mascia; Alfredo Berardelli; Michele Tinazzi Journal: Mov Disord Clin Pract Date: 2021-09-10