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Tremor and Other Hyperkinetic Movements... 2015The aim was to elucidate clinical trial efficacy, safety, and dosing practices of abobotulinumtoxinA (ABO) treatment in adult patients with blepharospasm and hemifacial... (Review)
Review
BACKGROUND
The aim was to elucidate clinical trial efficacy, safety, and dosing practices of abobotulinumtoxinA (ABO) treatment in adult patients with blepharospasm and hemifacial spasm. To date, most literature reviews for blepharospasm and hemifacial spasm have examined the effectiveness of all botulinum neurotoxin type A products as a class. However, differences in dosing units and recommended schemes provide a clear rationale for reviewing each product separately.
METHODS
A systematic literature review was performed to identify randomized controlled trials and other comparative clinical studies of ABO in the treatment of blepharospasm and hemifacial spasm published in English between January 1991 and March 2015. Medical literature databases (PubMed, Cochrane library, EMBASE) were searched. A total of five primary publications that evaluated ABO for the management of blepharospasm and hemifacial spasm were identified and summarized.
RESULTS
Data included 374 subjects with blepharospasm and 172 subjects with hemifacial spasm treated with ABO. Total ABO doses ranged between 80 and 340 U for blepharospasm and 25 and 85 U for hemifacial spasm, depending on the severity of the clinical condition. All studies showed statistically significant benefits for the treatment of blepharospasm and hemifacial spasm. ABO was generally well tolerated across the individual studies. Adverse events considered to be associated with ABO treatment included: ptosis, tearing, blurred vision, double vision, dry eyes, and facial weakness.
DISCUSSION
These data from 5 randomized clinical studies represents the available evidence base of ABO in blepharospasm and hemifacial spasm. Future studies in this area will add to this evidence base.
PubMed: 26566457
DOI: 10.7916/D8CJ8CVR -
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 -
Movement Disorders : Official Journal... Jun 2013Many rating scales have been applied to the evaluation of dystonia, but only few have been assessed for clinimetric properties. The Movement Disorders Society... (Review)
Review
Many rating scales have been applied to the evaluation of dystonia, but only few have been assessed for clinimetric properties. The Movement Disorders Society commissioned this task force to critique existing dystonia rating scales and place them in the clinical and clinimetric context. A systematic literature review was conducted to identify rating scales that have either been validated or used in dystonia. Thirty-six potential scales were identified. Eight were excluded because they did not meet review criteria, leaving 28 scales that were critiqued and rated by the task force. Seven scales were found to meet criteria to be "recommended": the Blepharospasm Disability Index is recommended for rating blepharospasm; the Cervical Dystonia Impact Scale and the Toronto Western Spasmodic Torticollis Rating Scale for rating cervical dystonia; the Craniocervical Dystonia Questionnaire for blepharospasm and cervical dystonia; the Voice Handicap Index (VHI) and the Vocal Performance Questionnaire (VPQ) for laryngeal dystonia; and the Fahn-Marsden Dystonia Rating Scale for rating generalized dystonia. Two "recommended" scales (VHI and VPQ) are generic scales validated on few patients with laryngeal dystonia, whereas the others are disease-specific scales. Twelve scales met criteria for "suggested" and 7 scales met criteria for "listed." All the scales are individually reviewed in the online information. The task force recommends 5 specific dystonia scales and suggests to further validate 2 recommended generic voice-disorder scales in dystonia. Existing scales for oromandibular, arm, and task-specific dystonia should be refined and fully assessed. Scales should be developed for body regions for which no scales are available, such as lower limbs and trunk.
Topics: Databases, Factual; Dystonia; Health Planning Guidelines; Humans; Psychometrics; Severity of Illness Index; Surveys and Questionnaires
PubMed: 23893443
DOI: 10.1002/mds.25579 -
Movement Disorders Clinical Practice Oct 2022A systematic review of epidemiological studies of primary dystonia from 1985 and 2010 found an overall prevalence of 16.43 per 100,000 (95% CI = 12.09-22.32). (Review)
Review
BACKGROUND
A systematic review of epidemiological studies of primary dystonia from 1985 and 2010 found an overall prevalence of 16.43 per 100,000 (95% CI = 12.09-22.32).
METHODS
We performed a systematic review of studies from 2010 and 2022 to determine if there are important differences in epidemiology between these time periods.
RESULTS
Nineteen studies were included. Incidence of cervical dystonia, blepharospasm, and oromandibular dystonia were each reported in one study; one study reported incidence for all adult onset idiopathic focal dystonias combined. Using data from 11 studies, we performed random effects meta-analyses of the prevalence of cervical dystonia (9.95 per 100,000; 95% CI = 3.51-28.17), blepharospasm (2.82 per 100,000; 95% CI = 1.12-7.12), laryngeal dystonia (0.40 per 100,000; 95% CI = 0.09-1.83), upper limb dystonia (1.27 per 100,000; 95% CI = 0.36-4.52), oromandibular dystonia (0.57 per 100,000; 95% CI = 0.15-2.15), and idiopathic or inherited isolated dystonia all subtypes combined (30.85 per 100,000; 95% CI = 5.06-187.74). All studies reported more cases of dystonia in females. There was no significant difference in prevalence by subgroup analysis based on time of study publication (1985-2010 vs. 2010-2022). Subgroup analysis of differences in prevalence by dystonia subtype by continent using all studies published (1985-2022) revealed significant regional differences in the prevalence of cervical and laryngeal dystonia.
CONCLUSION
The incidence and prevalence of idiopathic or inherited isolated dystonia in the last decade was not significantly different from earlier reports. Population-based studies across multiple geographic areas are needed to obtain a clearer understanding of the epidemiology of this condition.
PubMed: 36247920
DOI: 10.1002/mdc3.13524 -
Therapeutic Advances in Infectious... 2023Fascioliasis is a parasitic zoonosis that can infect humans and be a source of significant morbidity. The World Health Organization lists human fascioliasis as a...
BACKGROUND
Fascioliasis is a parasitic zoonosis that can infect humans and be a source of significant morbidity. The World Health Organization lists human fascioliasis as a neglected tropical disease, but the worldwide prevalence of fascioliasis data is unknown.
OBJECTIVE
We aimed to estimate the global prevalence of human fascioliasis.
DATA SOURCES AND METHODS
We performed a systematic review and prevalence meta-analysis. We used the following inclusion criteria: articles published in the English, Portuguese, or Spanish languages from December 1985 to October 2022 and studies assessing the prevalence of in the general population with an appropriate diagnostic methodology, including longitudinal studies, prospective and retrospective cohorts, case series, and randomized clinical trials (RCTs). We excluded animal studies. Two reviewers independently reviewed the selected studies for methodological quality, performing critical standard measures from JBI SUMARI. A random-effects model was conducted of the summary extracted data on the prevalence proportions. We reported the estimates according to the GATHER statement.
RESULTS
In all, 5617 studies were screened for eligibility. Fifty-five studies from 15 countries were selected, including 154,697 patients and 3987 cases. The meta-analysis revealed a pooled prevalence of 4.5% [95% confidence interval (CI): 3.1-6.1; = 99.4%; = 0.07]. The prevalence in South America, Africa, and Asia was 9.0%, 4.8%, and 2.0%, respectively. The highest prevalence was found in Bolivia (21%), Peru (11%), and Egypt (6%). Subgroup analysis showed higher prevalence estimates in children, in studies from South America, and when Fas2-enzyme-linked immunosorbent assay (ELISA) was used as a diagnostic method. A larger study sample size ( = 0.027) and an increase in female percentage ( = 0.043) correlated with a decrease in prevalence. Multiple meta-regression showed a higher prevalence for hyperendemic than hypoendemic ( = 0.002) or mesoendemic ( = 0.013) regions.
CONCLUSION
The estimated prevalence and projected disease burden of human fascioliasis are high. Study findings support that fascioliasis continues to be a globally neglected tropical disease. Strengthening epidemiological surveillance and implementing measures to control and treat fascioliasis is imperative in the most affected areas.
PubMed: 37434654
DOI: 10.1177/20499361231185413 -
Journal of the Neurological Sciences Aug 2013Blepharospasm is a form of focal dystonia that manifests as repetitive involuntary closure of the eyes. The pathogenesis of blepharospasm and the neuroanatomic... (Review)
Review
BACKGROUND
Blepharospasm is a form of focal dystonia that manifests as repetitive involuntary closure of the eyes. The pathogenesis of blepharospasm and the neuroanatomic substrates involved are not fully understood. Dysfunction of the basal ganglia traditionally is presumed to be the main cause of most forms of dystonia, but a growing body of evidence suggests that a network of additional cortical and subcortical structures may be involved.
METHODS
The medical records of 1114 patients with blepharospasm seen over past 10 years at Emory University were reviewed to identify potentially contributing brain lesions. A systematic review of the published literature was also conducted to identify potentially contributing brain lesions.
RESULTS
Among patients with blepharospasm at Emory University, 18 had focal lesions on imaging studies available for review. The literature review revealed 25 articles describing 30 additional cases of blepharospasm associated with focal lesions. Among all 48 cases, lesions were found in multiple regions including the thalamus (n=12), lower brainstem (n=11), basal ganglia (n=9), cerebellum (n=9), midbrain (n=7), and cortex (n=1).
CONCLUSIONS
These data in combination with functional imaging studies of primary blepharospasm support a model in which a network of different regions plays a role in the pathogenesis of blepharospasm.
Topics: Adult; Aged; Aged, 80 and over; Blepharospasm; Brain; Brain Injuries; Female; Humans; Longitudinal Studies; Male; Middle Aged; Neural Pathways; PubMed
PubMed: 23747003
DOI: 10.1016/j.jns.2013.05.022 -
Frontiers in Neurology 2022Neuroimaging studies have shown gray matter structural and functional alterations in patients with idiopathic blepharospasm (iBSP) but with variations. Here we aimed to...
BACKGROUND
Neuroimaging studies have shown gray matter structural and functional alterations in patients with idiopathic blepharospasm (iBSP) but with variations. Here we aimed to investigate the specific and common neurostructural/functional abnormalities in patients with iBSP.
METHODS
A systematic literature search from PubMed, Web of Science and Embase was conducted to identify relevant publications. We conducted separate meta-analysis for whole-brain voxel-based morphometry (VBM) studies and for functional imaging studies, and a multimodal meta-analysis across VBM and functional studies in iBSP, using anisotropic effect size-based signed differential mapping.
RESULTS
The structural database comprised 129 patients with iBSP and 144 healthy controls whilst the functional database included 183 patients with iBSP and 253 healthy controls. The meta-analysis of VBM studies showed increased gray matter in bilateral precentral and postcentral gyri, right supplementary motor area and bilateral paracentral lobules, while decreased gray matter in right superior and inferior parietal gyri, left inferior parietal gyrus, left inferior temporal gyrus, left fusiform gyrus and parahippocampal gyrus. The meta-analysis of functional studies revealed hyperactivity in right dorsolateral superior frontal gyrus, left thalamus and right fusiform gyrus, while hypoactivity in left temporal pole, left insula, left precentral gyrus, bilateral precuneus and paracentral lobules, right supplementary motor area and middle frontal gyrus. The multimodal meta-analysis identified conjoint anatomic and functional changes in left precentral gyrus, bilateral supplementary motor areas and paracentral lobules, right inferior occipital gyrus and fusiform gyrus.
CONCLUSIONS
The patterns of conjoint and dissociated gray matter alterations identified in the meta-analysis may enhance our understanding of the pathophysiological mechanisms underlying iBSP.
PubMed: 35734475
DOI: 10.3389/fneur.2022.889714 -
The Cochrane Database of Systematic... Nov 2020This is an update of a Cochrane Review first published in 2005. Blepharospasm is the second most common form of focal dystonia. It is a disabling disorder, characterised... (Meta-Analysis)
Meta-Analysis
BACKGROUND
This is an update of a Cochrane Review first published in 2005. Blepharospasm is the second most common form of focal dystonia. It is a disabling disorder, characterised by chronic, intermittent or persistent, involuntary eyelid closure, due to spasmodic contractions of the orbicularis oculi muscles. Currently, botulinum toxin type A (BtA) is considered the first line of therapy for this condition.
OBJECTIVES
To compare the efficacy, safety, and tolerability of BtA versus placebo in people with blepharospasm.
SEARCH METHODS
We searched Cochrane Movement Disorders' Trials Register, CENTRAL, MEDLINE, Embase, reference lists of included articles, and conference proceedings. We ran all elements of the search, with no language restrictions, in July 2020.
SELECTION CRITERIA
Double-blind, parallel, randomised, placebo-controlled trials (RCTs) of BtA versus placebo in adults with blepharospasm.
DATA COLLECTION AND ANALYSIS
Two review authors independently assessed records, selected included studies, extracted data using a paper pro forma, and evaluated the risk of bias. We resolved disagreements by consensus, or by consulting a third review author. We performed meta-analyses using a random-effects model, for the comparison of BtA versus placebo, to estimate pooled effects and corresponding 95% confidence intervals (95% CI). We did not carry out any prespecified subgroup analyses. The primary efficacy outcome was improvement on any validated symptomatic rating scale. The primary safety outcome was the proportion of participants with any adverse event.
MAIN RESULTS
We included three RCTs, assessed at low to moderate overall risk of bias, which randomised 313 participants with blepharospasm. Two studies excluded participants with poorer prior responses to BtA treatment, therefore, they included an enriched population with a higher probability of benefiting from this therapy. All trials were industry-funded. All RCTs evaluated the effect of a single BtA treatment session. BtA resulted in a moderate to large improvement in blepharospasm-specific severity, with a reduction of 0.93 points on the Jankovic Rating Scale (JRS) severity subscale at four to six weeks after injection (95% confidence interval (CI) 0.61 to 1.25; I² = 9%) compared to placebo. BtA was also resulted in a moderate to large improvement in blepharospasm-specific disability and blepharospasm-specific involuntary movements at four to six weeks after injection (disability: 0.69 JRS disability subscale points, 95% CI 0.18 to 1.19; I² = 74%; blepharospasm-specific involuntary movements: standardised mean difference (SMD) 0.79, 0.31 to 1.27; I² = 58%) compared to placebo. BtA did not show a risk of adverse events (risk ratio (RR) 1.18, 95% CI 0.87 to 1.60; I² = 0%). However, BtA increased the risk of vision complaints and eyelid ptosis (vision complaints: RR 5.73, 95% CI 1.79 to 18.36; I² = 51%; eyelid ptosis: RR 4.02, 95% CI 1.61 to 10.00; I² = 39%). There was no distinction between BtA and placebo in the number of participants who dropped out of the trial. A single trial estimated the duration of effects to be 10.6 weeks (range 6.1 to 19.1). We found no evidence supporting the existence of a clear dose-response relationship with BtA. We found no data reporting the impact of BtA on health-related quality of life, or the development of secondary non-responsiveness.
AUTHORS' CONCLUSIONS
We are moderately certain that a single BtA treatment resulted in a clinically relevant reduction of blepharospasm-specific severity and disability, and have low certainty that it is well tolerated, when compared with placebo. There is low-certainty evidence that people treated with BtA are not at an increased risk of developing adverse events, though BtA treatment likely increases the risk of visual complaints and eyelid ptosis. There are no data from RCTs evaluating the effectiveness and safety of repeated BtA injection cycles. There is no evidence from RCTs to allow us to draw definitive conclusions on the optimal treatment intervals and doses, or the impact on quality of life.
Topics: Bias; Blepharospasm; Botulinum Toxins, Type A; Dose-Response Relationship, Drug; Female; Humans; Male; Neuromuscular Agents; Placebos; Randomized Controlled Trials as Topic
PubMed: 33211907
DOI: 10.1002/14651858.CD004900.pub3 -
Movement Disorders Clinical Practice 2016This systematic review was performed to elucidate dosing practices, dosing conversions, and related outcomes from randomized controlled trials that directly compared...
OBJECTIVE
This systematic review was performed to elucidate dosing practices, dosing conversions, and related outcomes from randomized controlled trials that directly compared onabotulinumtoxinA (ONA) and abobotulinumtoxinA (ABO) at various dose conversion ratios for therapeutic use in movement disorders.
METHODS
A systematic review of 3 medical literature databases (PubMed, the Cochrane Library, and EMBASE) was performed to identify relevant comparative clinical studies, systematic reviews, and meta-analyses published in the English language between January 1991 and January 2015. Studies that met predefined inclusion criteria were selected for formal data extraction and quality assessment.
RESULTS
A total of 182 manuscripts were identified, of which 4 were included for analysis. Targeted clinical applications included neurological disorders. The studies compared ONA to ABO dose conversion ratios of 1:2.5 (n=1), 1:3 (n=2), and 1:4 (n=2). One study compared both 1:3 and 1:4 ratios. An ONA:ABO conversion factor of 1:2.5 was associated with similar efficacy and side effects. An ONA:ABO ratio of 1:3 provided similar or higher efficacy but an increased rate of adverse effects, and an ONA:ABO ratio of 1:4 was associated with higher efficacy but with an excessive rate of intolerable side effects.
CONCLUSION
A dose conversion ratio of ONA to ABO between 1:2.5 and 1:3.0 provides comparable safety and efficacy for therapeutic movement disorders chemodenervation procedures.
PubMed: 27110585
DOI: 10.1002/mdc3.12235 -
BMC Neurology Mar 2016Primary dystonia is a chronic neurological movement disorder that causes abnormal muscle movements. Pain and emotional distress may accompany these physical symptoms.... (Review)
Review
BACKGROUND
Primary dystonia is a chronic neurological movement disorder that causes abnormal muscle movements. Pain and emotional distress may accompany these physical symptoms. Behavioural interventions are used to help people with long term conditions improve their quality of life. Little is known about behavioural interventions applied to Dystonia. We report a systematic review of studies reporting current evidence of behavioural interventions for people with primary dystonia.
METHODS
We did systematic searches of Medline, PsycINFO, AHMED and CINAHL. We assessed the methodological quality of included studies using a risk of bias tool. Any disagreements were resolved by liaising with an independent rater. Physiological outcomes such as dystonia severity and psychological outcomes such as sleep and depression were selected on the basis that primary dystonia causes motor and non-motor symptoms. No time limit was placed on the searches. A narrative synthesis of the results is presented.
RESULTS
Of 1798 titles and abstracts screened, 14 full articles were retrieved and inclusion and exclusion criteria applied. Of these a final nine were eligible for the review (N = 73). Only two were Randomised Controlled Trials (RCTs). Using the Movement Disorders Society (MDS) dystonia classification, that was published after this work started, all of the included studies were of idiopathic adult onset focal dystonia without associated features. These included: blepharospasm (eye dystonia) (N = 1), cervical dystonia (neck dystonia) (N = 2), writer's cramp (hand dystonia) (N = 3) and the yips (N = 3). No studies reported on dystonia that affects two or more body regions. Studies reported good adherence and response rates to treatment. Physiological and psychological improvements were noted in all studies at weekly, monthly and yearly follow-ups. Caution should be taken when interpreting the results because of the scarcity of RCTs identified, use of small sample sizes, and inappropriate statistical methods.
CONCLUSION
We identified few studies; mainly of poor methodological quality that all studied a focal dystonia. It is not possible to draw firm conclusions. Nevertheless, the data suggests that a combined behavioural therapy approach including relaxation practice for people with idiopathic adult onset focal dystonia merits further investigation.
Topics: Adult; Behavior Therapy; Depression; Dystonic Disorders; Humans; Quality of Life
PubMed: 27000094
DOI: 10.1186/s12883-016-0562-y