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BMC Neurology Mar 2022To test, in a two-arm, single center, superiority, randomized controlled trial, the effectiveness of and costs associated with a patient-initiated treatment model for... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
To test, in a two-arm, single center, superiority, randomized controlled trial, the effectiveness of and costs associated with a patient-initiated treatment model for people with hemifacial spasm (HFS) and blepharospasm (BEB) in comparison to usual care.
METHODS
One hundred and thirty patients with HFS or BEB, aged 18 years or over, were recruited from a nurse-led botulinum toxin type A clinic at an eye hospital in the United Kingdom (UK), completed baseline measures and were randomized (1:1). The intervention group determined their own botulinum toxin type A (BoNT/A) treatment schedule during the trial period (9 months) and received an information leaflet with a "hotline" number to book an appointment. Usual care appointments were scheduled by treating clinicians. Data analysts were blind to study group. The primary outcomes were disease severity and functional disability, as measured by the Jankovic Rating Scale and Blepharospasm Disability Index, respectively. Secondary outcomes included quality of life, anxiety and depression, satisfaction with care, confidence in the service, economic costs and employment days lost.
RESULTS
Sixty-five patients were randomized to each group. The intervention demonstrated no statistically significant difference to usual care for any of primary outcomes. On secondary outcomes the levels of anxiety differed significantly (F = 1.65, p = 0.02), with the intervention arm exhibiting a decrease and the control arm an increase (Hedges' g = - 0.26 [99% CI -0.83, 0.32]). No other statistically significant differences were found for secondary outcomes. Overall healthcare costs and costs to the patient were on average £198.95 less (95% CI -£256.76, £654.67; p = 0.10) per participant for those in the intervention compared to usual care, although this finding was not significant.
CONCLUSIONS
We did not observe differences between the patient-initiated treatment model and usual care for people with BEB or HFS, on any primary outcome measure, quality of life, or depression. The patient-initiated treatment model may, however, have the potential to save healthcare costs and reduce anxiety. Patients using this new model were also equally as satisfied in the service and confident in their care as those receiving treatment as usual.
TRIAL REGISTRATION
Clinicaltrials.gov ID NCT02577224 , 16th October 2015.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Blepharospasm; Botulinum Toxins, Type A; Health Care Costs; Hemifacial Spasm; Humans; Middle Aged; Quality of Life; Young Adult
PubMed: 35300599
DOI: 10.1186/s12883-022-02603-7 -
Turkish Journal of Ophthalmology Dec 2016Botulinum neurotoxin (BoNT) is the first biological toxin used in the treatment of ophthalmic diseases and to decrease skin wrinkles as an aesthetic agent. When used... (Review)
Review
Botulinum neurotoxin (BoNT) is the first biological toxin used in the treatment of ophthalmic diseases and to decrease skin wrinkles as an aesthetic agent. When used appropriately, it weakens the force of muscular contraction and/or inhibits glandular secretion. The most common areas for botulinum toxin treatment are the upper face, including the glabella, forehead, brows, and lateral canthal lines, or crow's feet. By relaxing the muscles causing wrinkles, non-permanent results may be achieved with its use. BoNT has gained widespread use in a variety of ophthalmic diseases. The effect of BoNT is temporary, but the therapeutic benefit is usually maintained even after repeated injections. Treatment is usually well tolerated. Complications and side effects associated with the treatment are rare and temporary. Complications occur due to weakness (chemodenervation) of adjacent muscle groups, immunological mechanisms and injection technique. Current therapeutic indications, doses, complications and contraindications of BoNT use in the following disorders related to ophthalmology were investigated: aesthetic use, strabismus, blepharospasm, hemifacial spasm, eyelid retraction, entropion, lacrimal hypersecretion syndrome, and facial paralysis.
PubMed: 28050326
DOI: 10.4274/tjo.57701 -
Journal Francais D'ophtalmologie Sep 2020To study whether there is an association between benign essential blepharospasm and Sjögren's syndrome by analyzing the presence of antibodies to extractable nuclear...
PURPOSE
To study whether there is an association between benign essential blepharospasm and Sjögren's syndrome by analyzing the presence of antibodies to extractable nuclear antigens in this population.
METHODS
Seventy-two patients with benign essential blepharospasm (BEB) were included in this study. We excluded patients with hemifacial spasm or blepharospasm secondary to known corneal pathology. We recorded results of Schirmer I testing as well as levels of anti-SSA/Ro and anti-SSB/La antibodies.
RESULTS
Our study included 72 patients (144 eyes), of which 62 (86.1%) were women. The mean age was 74.3±10.73 years. The mean Schirmer I test result was 3.14±4.00mm. Five women (8% of this female population) were found to have positive anti-SSA/Ro and anti-SSB/La antibodies. Their mean age was 65.66±13.24 years, while the mean age of the antibody-negative patients was 75.42±9.27 years. There was no statistically significant difference between the Schirmer I tests of the antibody positive and negative patients.
CONCLUSION
This study demonstrates a possible association between Sjögren's syndrome and benign essential blepharospasm, justifying anti-SSA/Ro and anti-SSB/La testing in these patients.
Topics: Adult; Aged; Aged, 80 and over; Antibodies, Antinuclear; Antigens, Nuclear; Blepharospasm; Comorbidity; Dry Eye Syndromes; Female; Humans; Male; Middle Aged; Sjogren's Syndrome
PubMed: 32532571
DOI: 10.1016/j.jfo.2020.06.001 -
Frontiers in Neurology 2017Temporal discrimination is the ability to determine that two sequential sensory stimuli are separated in time. For any individual, the temporal discrimination threshold... (Review)
Review
Temporal discrimination is the ability to determine that two sequential sensory stimuli are separated in time. For any individual, the temporal discrimination threshold (TDT) is the minimum interval at which paired sequential stimuli are perceived as being asynchronous; this can be assessed, with high test-retest and inter-rater reliability, using a simple psychophysical test. Temporal discrimination is disordered in a number of basal ganglia diseases including adult-onset dystonia, of which the two most common phenotypes are cervical dystonia and blepharospasm. The causes of adult-onset focal dystonia are unknown; genetic, epigenetic, and environmental factors are relevant. Abnormal TDTs in adult-onset dystonia are associated with structural and neurophysiological changes considered to reflect defective inhibitory interneuronal processing within a network which includes the superior colliculus, basal ganglia, and primary somatosensory cortex. It is hypothesized that abnormal temporal discrimination is a mediational endophenotype and, when present in unaffected relatives of patients with adult-onset dystonia, indicates non-manifesting gene carriage. Using the mediational endophenotype concept, etiological factors in adult-onset dystonia may be examined including (i) the role of environmental exposures in disease penetrance and expression; (ii) sexual dimorphism in sex ratios at age of onset; (iii) the pathogenesis of non-motor symptoms of adult-onset dystonia; and (iv) subcortical mechanisms in disease pathogenesis.
PubMed: 29234300
DOI: 10.3389/fneur.2017.00625 -
International Journal of Molecular... Mar 2024A neurological condition called dystonia results in abnormal, uncontrollable postures or movements because of sporadic or continuous muscular spasms. Several varieties... (Review)
Review
A neurological condition called dystonia results in abnormal, uncontrollable postures or movements because of sporadic or continuous muscular spasms. Several varieties of dystonia can impact people of all ages, leading to severe impairment and a decreased standard of living. The discovery of genes causing variations of single or mixed dystonia has improved our understanding of the disease's etiology. Genetic dystonias are linked to several genes, including pathogenic variations of VPS16, TOR1A, THAP1, GNAL, and ANO3. Diagnosis of dystonia is primarily based on clinical symptoms, which can be challenging due to overlapping symptoms with other neurological conditions, such as Parkinson's disease. This review aims to summarize recent advances in the genetic origins and management of focal dystonia.
Topics: Humans; Dystonia; Dystonic Disorders; Movement; Parkinson Disease; Molecular Chaperones; DNA-Binding Proteins; Apoptosis Regulatory Proteins; Anoctamins
PubMed: 38612382
DOI: 10.3390/ijms25073571 -
Therapeutic Advances in Neurological... Mar 2015Blepharospasm is a focal (although usually bilateral) dystonia of the orbicularis oculi muscles, producing excessive eye closure. This produces significant disability... (Review)
Review
Blepharospasm is a focal (although usually bilateral) dystonia of the orbicularis oculi muscles, producing excessive eye closure. This produces significant disability through functional blindness. Botulinum neurotoxins (BoNT) have become the treatment of choice for blepharospasm; the impressive response rate and the tolerable safety profile have been proven through multiple clinical studies. There are currently four BoNT approved in the United States for different indications - we review the data on blepharospasm for each of these drugs. Currently, incobotulinumtoxinA and onabotulinumtoxinA have the most evidence of benefit for patients with blepharospasm. Current evidence, recent development and future directions are discussed.
PubMed: 25922620
DOI: 10.1177/1756285614557475 -
Medicine Jul 2023Strabismus, deviation of the ocular alignment, can adversely affect quality of life and activities of daily living. Surgery was the prior standard of care for...
Strabismus, deviation of the ocular alignment, can adversely affect quality of life and activities of daily living. Surgery was the prior standard of care for strabismus, but up to 40% of patients required additional surgeries. This need for more effective and less invasive treatment, along with the convergence of other events such as the development of electromyography, purification of botulinum toxin A, and the finding that injection of botulinum toxin type A could paralyze the hind limbs of chicks, led Dr. Alan Scott to investigate injection of his formulation for strabismus. The positive results of initial trials in monkeys segued to human trials with observations of alignment improvements and few adverse events. The success of botulinum toxin type A in the treatment of strabismus led to interest in its use to treat other skeletal muscles, particularly in blepharospasm, a type of focal dystonia involving eyelid spasms and involuntary eye closure that lacked an effective pharmacological treatment. Patient groups helped to increase awareness of this novel treatment, and results from clinical trials confirmed its effectiveness. Dr. Scott's formulation, then known as Oculinum, received its first Food and Drug Administration approvals in 1989 for strabismus and blepharospasm. Allergan acquired Oculinum in 1991, renaming it Botox. These initial uses led to its application in a myriad of other indications as outlined in other articles of this supplement.
Topics: Humans; Botulinum Toxins, Type A; Blepharospasm; Activities of Daily Living; Quality of Life; Strabismus
PubMed: 37499080
DOI: 10.1097/MD.0000000000032374 -
Journal of Neuro-ophthalmology : the... Mar 2012Photophobia is a common yet debilitating symptom seen in many ophthalmic and neurologic disorders. Despite its prevalence, it is poorly understood and difficult to... (Review)
Review
Photophobia is a common yet debilitating symptom seen in many ophthalmic and neurologic disorders. Despite its prevalence, it is poorly understood and difficult to treat. However, the past few years have seen significant advances in our understanding of this symptom. We review the clinical characteristics and disorders associated with photophobia, discuss the anatomy and physiology of this phenomenon, and conclude with a practical approach to diagnosis and treatment.
Topics: Blepharospasm; Brain Injuries; Eye Diseases; Humans; Migraine Disorders; Photophobia; Visual Pathways
PubMed: 22330853
DOI: 10.1097/WNO.0b013e3182474548 -
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 -
Scientific Reports Apr 2023Mental rotation (mR) bases on imagination of actual movements. It remains unclear whether there is a specific pattern of mR impairment in focal dystonia. We aimed to...
Mental rotation (mR) bases on imagination of actual movements. It remains unclear whether there is a specific pattern of mR impairment in focal dystonia. We aimed to investigate mR in patients with cervical dystonia (CD) and blepharospasm (BS) and to assess potential confounders. 23 CD patients and 23 healthy controls (HC) as well as 21 BS and 19 hemifacial spasm (HS) patients were matched for sex, age, and education level. Handedness, finger dexterity, general reaction time, and cognitive status were assessed. Disease severity was evaluated by clinical scales. During mR, photographs of body parts (head, hand, or foot) and a non-corporal object (car) were displayed at different angles rotated within their plane. Subjects were asked to judge laterality of the presented image by keystroke. Both speed and correctness were evaluated. Compared to HC, CD and HS patients performed worse in mR of hands, whereas BS group showed comparable performance. There was a significant association of prolonged mR reaction time (RT) with reduced MoCA scores and with increased RT in an unspecific reaction speed task. After exclusion of cognitively impaired patients, increased RT in the mR of hands was confined to CD group, but not HS. While the question of whether specific patterns of mR impairment reliably define a dystonic endophenotype remains elusive, our findings point to mR as a useful tool, when used carefully with control measures and tasks, which may be capable of identifying specific deficits that distinguish between subtypes of dystonia.
Topics: Humans; Blepharospasm; Torticollis; Fingers; Motor Skills; Dystonic Disorders; Hemifacial Spasm
PubMed: 37055560
DOI: 10.1038/s41598-023-33262-4