Oromandibular Dystonia: Clinical Characteristics and Impact on Quality of Life Open Access
Scorr, Laura M. (2017)
Abstract
Objective: To determine the clinical characteristics of oromandibular dystonia (OMD) and estimate the association between quality of life (QOL) and location of cranial dystonia.
Background : OMD and blepharospasm (BSP) are sub-types of cranial dystonia that occur in isolation or combination. BSP is characterized by spasms of the upper facial muscles, known to result in poor QOL. OMD affects the lower facial muscles causing jaw dysfunction and pain. Patients anecdotally report OMD to be particularly disabling. OMD is not well characterized in the literature, neglected on cranial dystonia rating scales, and often untreated. We hypothesized location of dystonia is associated with QOL.
Methods : Subjects include 200 patients with OMD, BSP, and combined cranial dystonia enrolled in the Dystonia Coalition database from 26 international sites. Demographics and clinical characteristics were collected via standardized questionnaires. Anxiety was assessed with Liebowitz Social Anxiety Scale (LSA), and QOL with SF-36. Descriptive statistical analysis was performed for sample characteristics. The association between location of dystonia and QOL was estimated with linear regression.
Results : Among 165 cases of OMD, 65% were female and average age of onset was 55±12 (range: 9-74). The pattern of dystonia was segmental in 48% of cases. Average LSA scores for OMD (32±26) and combined cranial dystonia (36±29) indicated social anxiety. Mean SF-36 QOL score for healthy subjects is 50±10, with lower scores indicating worse QOL. Average mental QOL scores for OMD, BSP, and combined cranial dystonia were 48±11, 50±9, and 43±12. Average physical QOL scores were 48±10, 46±12, and 44±8. Combined cranial dystonia was associated lower mental QOL scores compared to BSP ( β =-7.89, p=0.03). OMD had a similar impact on QOL as BSP ( β =-0.42, p=0.90).
Conclusions : OMD typically presents in the fifth decade as part of a segmental pattern and is more common in women. On average, patients with OMD had social anxiety and poor quality of life. The presence of combined cranial dystonia had a more negative impact on QOL than OMD or BSP alone. To improve QOL in cranial dystonia patients it will be essential to revise our clinical paradigm to include assessment and treatment for both OMD and BSP.
Table of Contents
Section
Pages
Introduction
1-2
Background
3-5
Methods
6-10
Results
11-13
Discussion
14-19
References
20
Tables and Figures
21-27
· Table 1: Characteristics of oromandibular dystonia patients enrolled in the Dystonia Coalition database from 2011-2016
21
· Table 2: Characteristics of cranial dystonia patients enrolled in the Dystonia Coalition database from 2011-2016
22
· Figure 1: Distribution of SF-36 physical component quality of life scores by location of dystonia
23
· Figure 2: Distribution of SF-36 mental component quality of life scores by location of dystonia
24
· Figure 3: Distribution of SF-36 mental component quality of life in combined cranial dystonia compared to expected normal distribution
25
· Table 3: Univariate linear regression analysis of SF-36 quality of life scores of patients with cranial dystonia enrolled in the Dystonia Coalition database from 2011-2016
26
· Table 3: Multivariate linear regression analysis of SF-36 quality of life scores of patients with cranial dystonia enrolled in the Dystonia Coalition database from 2011-2016
27
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