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Oromandibular Dystonia: A Clinical Examination of 2,020 Cases
- Source :
- Frontiers in Neurology, Vol 12 (2021), Frontiers in Neurology, Frontiers in Neurology, Frontiers, 2021, 12, ⟨10.3389/fneur.2021.700714⟩
- Publication Year :
- 2021
- Publisher :
- Frontiers Media SA, 2021.
-
Abstract
- Objective: The goal of this study is to better characterize the phenotypic heterogeneity of oromandibular dystonia (OMD) for the purpose of facilitating early diagnosis.Methods: First, we provide a comprehensive summary of the literature encompassing 1,121 cases. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter cohort. Finally, we summarize clinical features and treatment outcomes from cross-sectional analysis of 172 OMD subjects from two expert centers.Results: In all cohorts, typical age at onset was in the 50s and 70% of cases were female. The Dystonia Coalition cohort revealed perioral musculature was involved most commonly (85%), followed by jaw (61%) and tongue (17%). OMD more commonly appeared as part of a segmental dystonia (43%), and less commonly focal (39%) or generalized (10%). OMD was found to be associated with impaired quality of life, independent of disease severity. On average, social anxiety (LSA score: 33 ± 28) was more common than depression (BDI II score: 9.7 ± 7.8). In the expert center cohorts, botulinum toxin injections improved symptom severity by more than 50% in ~80% of subjects, regardless of etiology.Conclusions: This comprehensive description of OMD cases has revealed novel insights into the most common OMD phenotypes, pattern of dystonia distribution, associated psychiatric disturbances, and effect on QoL. We hope these findings will improve clinical recognition to aid in timely diagnosis and inform treatment strategies.
- Subjects :
- Pediatrics
medicine.medical_specialty
Physical examination
03 medical and health sciences
jaw
0302 clinical medicine
Quality of life
tongue
030225 pediatrics
medicine
RC346-429
Depression (differential diagnoses)
Original Research
Dystonia
treatment
medicine.diagnostic_test
business.industry
medicine.disease
Oromandibular dystonia
botulinum (neuro)toxin
Botulinum toxin
3. Good health
Neurology
Cohort
Etiology
dystonia
Neurology. Diseases of the nervous system
Neurology (clinical)
business
[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
030217 neurology & neurosurgery
medicine.drug
Subjects
Details
- ISSN :
- 16642295
- Volume :
- 12
- Database :
- OpenAIRE
- Journal :
- Frontiers in Neurology
- Accession number :
- edsair.doi.dedup.....8ba5cd301fdb4cfd20741bd0bbbff7fb
- Full Text :
- https://doi.org/10.3389/fneur.2021.700714