Session Information
Date: Thursday, June 23, 2016
Session Title: Dystonia and Pediatric Movement Disorders
Session Time: 12:00pm-1:30pm
Objective: The overall goal of this study is to better define the clinical characteristics and natural history of oromandibular dystonia (OMD).
Background: OMD is a rare form of dystonia that affects the masticatory, lower facial, and lingual muscles. Diagnosis and treatment are usually delayed primarily because this disease is under-recognized and treatment is poorly described. Meanwhile, these patients endure significant disability because eating and speaking are impaired, which has a significant negative impact on quality of life (QOL). The Dystonia Coalition is an NIH-funded multi-center collaboration aimed at advancing clinical research for isolated dystonia syndromes. Review of clinical data and videos collected by Dystonia Coalition investigators and additional Emory cases offers a unique opportunity to review an unusually large sample of cases to better describe the clinical characteristics and natural history of the disorder.
Methods: The subjects include OMD patients enrolled in the Dystonia Coalition Biorepository and Natural History studies from 26 sites and additional cases followed at Emory. As part of enrollment, information collected includes demographics, clinical characteristics, severity, mood, and QOL. Additional data comes from a retrospective chart review of all Emory cases for demographics, clinical characteristics, and response to botulinum toxin. Descriptive statistics for sample characteristics will be reported. Correlational analyses will be used to examine relationships among study variables.
Results: A total of 155 cases of OMD were identified. Average age of onset was 54 years and 65% were female. Approximately 45% were described as having focal dystonia, 36% segmental dystonia and 19% generalized dystonia. Within the last year, 43 cases of OMD have been evaluated and treated with botulinum toxin at Emory.
Conclusions: This large cohort from the Dystonia Coalition along with the Emory Movement Disorder Center provides a significant opportunity to better understand OMD. The results of further analyses addressing type (jaw opening, jaw closing, jaw deviation, tongue), severity, impact on quality of life, and treatment response will be presented.
To cite this abstract in AMA style:
L. Scorr, S. Factor, H. Jinnah. Clinical characteristics and natural history of oromandibular dystonia [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/clinical-characteristics-and-natural-history-of-oromandibular-dystonia/. Accessed November 22, 2024.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-characteristics-and-natural-history-of-oromandibular-dystonia/