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Dystonia (Lausanne, Switzerland) 2022Blepharospasm is a type of dystonia where the diagnosis is often delayed because its varied clinical manifestations are not well recognized. The purpose of this study...
OBJECTIVE
Blepharospasm is a type of dystonia where the diagnosis is often delayed because its varied clinical manifestations are not well recognized. The purpose of this study was to provide a comprehensive picture of its clinical features including presenting features, motor features, and non-motor features.
METHODS
This was a two-part study. The first part involved a systematic literature review that summarized clinical features for 10,324 cases taken from 41 prior reports. The second part involved a summary of clinical features for 884 cases enrolled in a large multicenter cohort collected by the Dystonia Coalition investigators, along with an analysis of the factors that contribute to the spread of dystonia beyond the periocular region.
RESULTS
For cases in the literature and the Dystonia Coalition, blepharospasm emerged in the 50s and was more frequent in women. Many presented with non-specific motor symptoms such as increased blinking (51.9%) or non-motor sensory features such as eye soreness or pain (38.7%), photophobia (35.5%), or dry eyes (10.7%). Non-motor psychiatric features were also common including anxiety disorders (34-40%) and depression (21-24%). Among cases presenting with blepharospasm in the Dystonia Coalition cohort, 61% experienced spread of dystonia to other regions, most commonly the oromandibular region and neck. Features associated with spread included severity of blepharospasm, family history of dystonia, depression, and anxiety.
CONCLUSIONS
This study provides a comprehensive summary of motor and non-motor features of blepharospasm, along with novel insights into factors that may be responsible for its poor diagnostic recognition and natural history.
PubMed: 36248010
DOI: 10.3389/dyst.2022.10359 -
Heliyon Mar 2024Globus pallidus internus (GPi) and subthalamic nucleus (STN) are two common deep brain stimulation (DBS) targets. This meta-analysis was to compared the efficacy and...
BACKGROUND
Globus pallidus internus (GPi) and subthalamic nucleus (STN) are two common deep brain stimulation (DBS) targets. This meta-analysis was to compared the efficacy and safety of these two DBS targets for the treatment of Meige syndrome (MS).
METHODS
A systematic search was performed using EMBASE, MEDLINE, the Cochrane Library, and ClinicalTrials.gov to identify DBS trials for MS. Review Manager 5.3 was used to perform meta-analysis and the mean difference (MD) was analyzed and calculated with a random effect model. Pearson's correlation coefficients and meta-regression analyses were utilized to identify relevant predictive markers.
RESULTS
Twenty trials involving 188 participants with GPi-DBS and 110 individuals with STN-DBS were eligible. Both groups showed improvement of the Burke-Fahn-Marsden Dystonia Rating Scale-Movement (BFMDRS-M) and Disability (BFMDRS-D) scores (BFMDRS-M: MD = 10.57 [7.74-13.41] for GPi-DBS, and MD = 8.59 [4.08-13.11] for STN-DBS; BFMDRS-D: MD = 5.96 [3.15-8.77] for GPi-DBS, and MD = 4.71 [1.38-8.04] for STN-DBS; all < 0.001) from baseline to the final follow-up, while no notable disparity in improvement rates was observed between them. Stimulation-related complications occurrence was also similar between two groups (38.54 ± 24.07% vs. 43.17 ± 29.12%, P = 0.7594). Simultaneously, preoperative BFMDRS-M score and disease duration were positively connected with the relative changes in BFMDRS-M score at the final visit.
CONCLUSION
Both GPi-DBS and STN-DBS are effective MS therapies, with no differences in efficacy or the frequency of stimulation-related problems. Higher preoperative scores and longer disease duration probably predict greater improvement.
PubMed: 38510025
DOI: 10.1016/j.heliyon.2024.e27945 -
Parkinsonism & Related Disorders Sep 2019In isolated, sporadic dystonia, it has been occasionally reported that some patients might undergo symptom remission. However, the exact clinical characteristics of... (Meta-Analysis)
Meta-Analysis
In isolated, sporadic dystonia, it has been occasionally reported that some patients might undergo symptom remission. However, the exact clinical characteristics of patients with remission remain understudied. Given the important prognostic and pathophysiological implications of dystonic remission, we here provide a systematic review of the literature and a meta-analysis to assess demographic and clinical features associated with this phenomenon. We also provide a list of operational criteria to better define dystonic remission. Using PubMed and Embase, we conducted a systematic literature search in March 2018. 626 records were screened, 31 studies comprising data of 2551 cases with reports predominantly from patients with cervical dystonia (n = 1319) or blepharospasm/Meige syndrome (n = 704) were included in qualitative analysis. Five studies reporting remission in cervical dystonia were eligible for meta-analysis. Complete remission was reported in 11.8% and partial remission for 4.4% of cases. Remission rates were higher in cervical dystonia than in blepharospasm/Meige (e.g. complete remission 15.4% vs. 5.8% respectively). Remission occurred on average 4.5 years after onset of dystonic symptoms. However, the majority of patients (63.8%) relapsed. Meta-analysis for cervical dystonia showed that patients with remission were significantly younger at symptom onset than patients without remission (mean difference -7.13 years [95% CI: 10.58, -3.68], p < 0.0001). Based on our findings, we propose that the degree, the conditions associated with the onset, and the duration of remission are key factors to be considered in a unifying definition of dystonic remission.
Topics: Dystonic Disorders; Humans; Recurrence; Remission, Spontaneous
PubMed: 30898428
DOI: 10.1016/j.parkreldis.2019.02.020 -
Frontiers in Neurology 2020The effect of deep brain stimulation (DBS) on swallowing function in movement disorders is unclear. Here, we systematically reviewed this topic by searching keywords...
The effect of deep brain stimulation (DBS) on swallowing function in movement disorders is unclear. Here, we systematically reviewed this topic by searching keywords following PICOS strategy of problem (swallowing or swallow or dysphagia or aspiration) and intervention (deep brain stimulation, or DBS) in the PubMed and Web of Science in English in April 2020, with comparators [subthalamic nucleus (STN), globus pallidus interna (GPi), ventralis intermedius, (ViM), post-subthalamic area, or caudal zona incerta (PSA/cZi); ON/OFF DBS state/settings, ON/OFF medication state, Parkinson's disease (PD), dystonia, tremor], outcomes (swallowing function measures, subjective/objective) and study types (good quality original studies) in mind. We found that STN DBS at usual high-frequency stimulation could have beneficial effect (more so on subjective measures and/or OFF medication), no effect, or detrimental effect (more so on objective measures and/or ON medication) on swallowing function in patients with PD, while low-frequency stimulation (LFS) could have beneficial effect on swallowing function in patients with freezing of gait. GPi DBS could have a beneficial effect (regardless of medication state and outcome measures) or no effect, but no detrimental effect, on swallowing function in PD. GPi DBS also has beneficial effects on swallowing function in majority of the studies on Meige syndrome but not in other diseases with dystonia. PSA/cZi DBS rarely has detrimental effect on swallowing functions in patients with PD or tremor. There is limited information on ViM to assess. Information on swallowing function by DBS remains limited. Well-designed studies and direct comparison of targets are further needed.
PubMed: 32765388
DOI: 10.3389/fneur.2020.00547