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Journal of the Neurological Sciences Aug 2022Blepharospasm is one of the most common subtypes of dystonia, and often spreads to other body regions. Despite published guidelines, the approach to diagnosis and...
BACKGROUND
Blepharospasm is one of the most common subtypes of dystonia, and often spreads to other body regions. Despite published guidelines, the approach to diagnosis and classification of affected body regions varies among clinicians.
OBJECTIVE
To delineate the clinical features used by movement disorder specialists in the diagnosis and classification of blepharospasm according to body regions affected, and to develop recommendations for a more consistent approach.
METHODS
Cross-sectional data for subjects diagnosed with all types of isolated dystonia were acquired from the Dystonia Coalition, an international, multicenter collaborative research network. Data were evaluated to determine how examinations recorded by movement disorder specialists were used to classify blepharospasm as focal, segmental, or multifocal.
RESULTS
Among all 3222 participants with isolated dystonia, 210 (6.5%) had a diagnosis of focal blepharospasm. Among these 210 participants, 34 (16.2%) had dystonia outside of upper face region. Factors such as dystonia severity across different body regions and number of body regions affected influenced the classification of blepharospasm as focal, segmental, or multifocal.
CONCLUSIONS
Although focal blepharospasm is the second most common type of dystonia, a high percentage of individuals given this diagnosis had dystonia outside of the eye/upper face region. These findings are not consistent with existing guidelines for the diagnosis and classification of focal blepharospasm, and point to the need for more specific guidelines for more consistent application of existing recommendations for diagnosis and classification.
Topics: Blepharospasm; Cross-Sectional Studies; Dystonia; Dystonic Disorders; Humans
PubMed: 35716653
DOI: 10.1016/j.jns.2022.120319 -
Neurological Sciences : Official... Nov 2021Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of...
Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of movement disorders and cognitive\behavioral changes. Its pathogenesis is debated and the symptoms are poorly responsive to medical therapy. Meige's syndrome is a form of cranial dystonia, considered an idiopathic form of adult onset dystonia. We report a 60-year-old man with HCV-related liver cirrhosis and hepatocarcinoma who developed Meige's syndrome associated with cognitive and behavioral manifestations, unrelated to acute metabolic derangement. CAHD was diagnosed. Liver transplantation reversed the clinical picture and MR abnormalities, reinforcing the idea that CAHD is a potentially reversible syndrome, which may be healed by liver transplantation and should not be considered a contraindication for this operation.
Topics: Adult; Behavioral Symptoms; Hepatolenticular Degeneration; Humans; Liver Transplantation; Male; Meige Syndrome; Middle Aged
PubMed: 34278516
DOI: 10.1007/s10072-021-05475-w -
Parkinsonism & Related Disorders Jul 2021Anterocollis (AC) is a rare form of cervical dystonia, which responds poorly to botulinum toxin treatment.
BACKGROUND
Anterocollis (AC) is a rare form of cervical dystonia, which responds poorly to botulinum toxin treatment.
OBJECTIVES
To recognise the different clinical phenotypes of AC and to detail the selection of muscles from the results of treating a cohort of 15 AC patients with Botulinum Toxin.
METHODS
The study was performed using prospectively collected data. We included 15 patients with cervical dystonia and AC posture, treated between 2016 and 2019 in our joint Neuro-ENT clinic. We excluded patients with posterior cervical muscle weakness and patients with Parkinsonism. We characterised the primary dystonic posture of every AC patient as posterior sagittal shift, head flexion or neck flexion, or a combination of the three.
RESULTS
All AC patients had a more widespread dystonic picture with a majority having Meige syndrome, but AC was the most problematic feature. Treatment with botulinum toxin required the injection not only of the deep cervical flexor (DCF), but also the sterno-cleido-mastoid (SCM) and moreover the supra-hyoid (SH) muscles. The choice between the longus capiti and the longus colli depended on the AC posture. Half of the patients had a dramatic improvement with 90% satisfaction or above.
CONCLUSION
AC posture is a complex but treatable type of CD. A joint Neuro-ENT clinic is an ideal setting in which to target all the dystonic muscles. This allows the injection of the longus capiti (under nasal endoscopic approach) as well as the supra-hyoid and SCM muscles in the same session.
Topics: Adult; Aged; Botulinum Toxins; Female; Humans; Male; Meige Syndrome; Middle Aged; Neck Muscles; Neuromuscular Agents; Prospective Studies; Torticollis
PubMed: 34102419
DOI: 10.1016/j.parkreldis.2021.05.024 -
Neurological Sciences : Official... May 2023The clinical efficacy of deep brain stimulation (DBS) relies on the optimal electrode placement in a large extent. Subthalamic nucleus (STN) DBS was recognized as...
OBJECTIVE
The clinical efficacy of deep brain stimulation (DBS) relies on the optimal electrode placement in a large extent. Subthalamic nucleus (STN) DBS was recognized as clinically effective for Meige syndrome. This study identified the correlations of volume of tissue activated (VTA) within the motor STN and the final efficacy of the surgical procedure.
METHODS
Clinical outcomes of the patients (n=25) were evaluated with the percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) scores at the last follow-up (LFU) visit. Pearson's correlation coefficients were calculated to identify the relationship of the final clinical outcomes with the VTA within the STN, VTA within the different STN territories, and other clinical variables.
RESULTS
On the whole, the patients showed an average of 59.21% improvement at the LFU visit relative to the baseline (5.72 ± 7.31 vs. 13.70 ± 7.36, P ˂ 0.001). Active electrode contacts mainly clustered in the STN motor territories. There were significant positive correlations between the BFMDRS-M percentage improvement and VTA within the STN (Pearson r = 0.434, P = 0.039) and the STN motor territories (r = 0.430, P = 0.041), but not associative or limbic STN. Other basic clinical characteristics including age, disease duration, and preoperative scores were not significantly correlated with the final outcomes.
CONCLUSIONS
Our study further validated the efficacy of STN-DBS in even the cases with intractable Meige syndrome. Furthermore, VTA within the motor STN could serve as a potential prognostic factor for the final clinical outcomes.
Topics: Humans; Subthalamic Nucleus; Meige Syndrome; Deep Brain Stimulation; Treatment Outcome; Dystonic Disorders; Dystonia
PubMed: 36622476
DOI: 10.1007/s10072-022-06594-8 -
Neurological Sciences : Official... May 2016Bilateral hemifacial spasm and Meige syndrome can be easily confused due to their similar clinical manifestation. Here, we aimed to investigate the application of...
Bilateral hemifacial spasm and Meige syndrome can be easily confused due to their similar clinical manifestation. Here, we aimed to investigate the application of electrophysiological methods and magnetic resonance tomographic angiography (MRTA) in the differentiation between hemifacial spasm and Meige syndrome. 10 patients with bilateral hemifacial spasm and 9 patients with Meige syndrome received electrophysiological monitoring of nerves. There were two males and eight females with bilateral hemifacial spasm, aged 16-58 years with a course of 5-54 months. For the patients with Meige syndrome, there were three males and six females, aged 51-68 years with a course of 12-36 months. All patients received conventional MRTA of the brain blood vessels before decompression. We found that all patients with Meige syndrome showed synchronous contraction of bilateral orbicularis oculi muscles and (or) burst discharge from orbicularis oris muscles in surface electromyography (sEMG). However, those with hemifacial spasm presented with bilaterally asynchronous burst discharge. Electromyography for patients with Meige syndrome did not record abnormal muscle response (AMR), but recorded AMR for those with bilateral hemifacial spasm. The offending vessels were compressed in patients with hemifacial spasm in MRTA, while MRTA results were generally negative for those with Meige syndrome. Combining sEMG and AMR detection in EMG and MRTA, bilateral hemifacial spasm can be differentiated from Meige syndrome with a reduction of misdiagnosis rate.
Topics: Adolescent; Adult; Aged; Electromyography; Evoked Potentials, Motor; Female; Hemifacial Spasm; Humans; Magnetic Resonance Angiography; Male; Meige Syndrome; Middle Aged; Retrospective Studies; Young Adult
PubMed: 26838523
DOI: 10.1007/s10072-016-2492-2 -
Noro Psikiyatri Arsivi Jun 2015Meige syndrome, which has been presented in tardive syndromes, is a form of blepharospasm accompanied by oromandibular dystonia with manifestations over the face, jaw,...
Meige syndrome, which has been presented in tardive syndromes, is a form of blepharospasm accompanied by oromandibular dystonia with manifestations over the face, jaw, and neck. A blepharospasm can be induced by antihistamines, dopaminomimetic or sympathomimetic drugs, or long-term exposure to dopamine antagonists. Atypical antipsychotics have less extrapyramidal side effects because of a weak dopamine D2 receptor binding affinity or a strong antagonistic effect to serotonin 5-HT2a receptor and have been known to cause less tardive dyskinesia than typical antipsychotics. Thus, in literature, atypical antipsychotics are recommended for the treatment of psychosis in cases of tardive dyskinesia. The potential risk factors associated with the development of tardive dyskinesia are extrapyramidal symptoms' history, diabetes mellitus, affective disorder, female gender, older age, and long-term therapy with neuroleptics at higher dosages. As reported below, a patient with an affective disorder who had quetiapine-induced oromandibular dystonia and olanzapine-induced Meige syndrome after antipsychotic augmentation in different stages of the disease process was presented.
PubMed: 28360708
DOI: 10.5152/npa.2015.7411 -
Frontiers in Neurology 2022Facial appearance and expressions influence social interaction. Hemifacial spasm (HFS), blepharospasm (BPS), and blepharospasm-oromandibular dystonia (BOD) are common...
BACKGROUND
Facial appearance and expressions influence social interaction. Hemifacial spasm (HFS), blepharospasm (BPS), and blepharospasm-oromandibular dystonia (BOD) are common forms of craniofacial movement disorders. Few studies have focused on the mental burden and quality of life (QoL) in patients with craniofacial movement disorders. Therefore, this study investigated mental health and QoL in these patients.
METHODS
This cross-sectional study included 90 patients with craniofacial movement disorders (HFS, BPS, and BOD; 30 patients per group) and 30 healthy individuals without craniofacial movement disorders (control group) recruited from October 2019 to November 2020. All participants underwent QoL and mental health evaluations for depression, anxiety, and stigma using the 36-item Short Form Health Survey (SF-36), Hamilton Anxiety Rating Scale (HAMA), Hamilton Rating Scale for Depression-24 (HAMD-24) and a questionnaire related to stigma.
RESULTS
Depression was diagnosed in 37 (41.11%) patients, whereas 30 patients (33.33%) had anxiety. HAMA scores were significantly higher in the BPS and BOD groups than in the control group. Nineteen patients (21.11%) experienced stigma and SF-36 scores were lower in various dimensions in the movement disorders groups compared to healthy controls. The role-physical and social function scores were significantly lower in the movement disorders groups than in the control group all < 0.05. The vitality scores of the BPS group and mental health scores of the BPS and BOD groups were significantly lower than those of the control group. Correlation analysis showed that the eight dimensions of SF-36 correlated with education level, disease duration, HAMD score, and HAMA score (all < 0.05). Regression analysis demonstrated that the HAMD score correlated with general health, vitality, social function, role-emotional, and mental health (all < 0.05). The HAMA score correlated with body pain after adjusting for education level and disease duration.
CONCLUSION
This study highlights the significant frequency of mental symptoms, including depression, anxiety, and stigma, which lower QoL in patients with craniofacial movement disorders.
PubMed: 36212649
DOI: 10.3389/fneur.2022.938632 -
Movement Disorders : Official Journal... Aug 2021Systematic perceptual distortions of tactile space have been documented in healthy adults. In isolated focal dystonia impaired spatial somatosensory processing is...
BACKGROUND
Systematic perceptual distortions of tactile space have been documented in healthy adults. In isolated focal dystonia impaired spatial somatosensory processing is suggested to be a central pathophysiological finding, but the structure of tactile space for different body parts has not been previously explored.
OBJECTIVES
The objective of this study was to assess tactile space organization with a novel behavioral paradigm of tactile distance perception in patients with isolated focal dystonia and controls.
METHODS
Three groups of isolated focal dystonia patients (cervical dystonia, blepharospasm/Meige syndrome, focal hand dystonia) and controls estimated perceived distances between 2 touches across 8 orientations on the back of both hands and the forehead.
RESULTS
Stimulus size judgments differed significantly across orientations in all groups replicating distortions of tactile space known for healthy individuals. There were no differences between groups in the behavioral parameters we assessed on the hands and forehead.
CONCLUSIONS
Tactile space organization is comparable between patients with isolated focal dystonia and healthy controls in dystonic and unaffected body parts. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
Topics: Adult; Dystonic Disorders; Hand; Humans; Space Perception; Touch; Touch Perception
PubMed: 33942381
DOI: 10.1002/mds.28607 -
Arquivos de Neuro-psiquiatria Apr 2024Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type...
BACKGROUND
Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type A (OBTA) injections are the standard treatment, evidence of their effectiveness and safety in this scenario is still lacking.
OBJECTIVE
Our research aimed to evaluate the improvement and occurrence of side effects following injections of onabotulinum toxin type A (OBTA) in patients with Meige's syndrome.
METHODS
Patients with Meige's syndrome undergoing botulinum toxin injections were enrolled in this study. We assessed dystonia intensity before and 14 days after OBTA injection using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) to measure the response of symptoms in the eyes (blepharospasm) and mouth (oromandibular dystonia). Other variables, such as dosage, side effects, and demographic data, were also recorded.
RESULTS
The study included 41 participants, with a mean age of 67.7 years and a female-to-male ratio of 3.5:1. The mean BFMDRS score before the injections was 8.89, and after 14 days, it was 2.88. The most reported side effect was ptosis, with a 7.3% incidence. OBTA significantly reduced dystonia severity ( < 0.0001). The clinical response for the blepharospasm component was superior to the oromandibular dystonia component.
CONCLUSION
Our results support that OBTA seems to be an effective and safe therapeutic option for treating Meige's syndrome. The effect of OBTA was more pronounced in the treatment of blepharospasm than in oromandibular dystonia.
Topics: Humans; Male; Female; Aged; Botulinum Toxins, Type A; Blepharospasm; Dystonia; Meige Syndrome; Dystonic Disorders
PubMed: 38641339
DOI: 10.1055/s-0044-1785691 -
Neurosurgical Review Feb 2022This study evaluated the long-term efficacy of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of craniofacial dystonia (Meige syndrome) and...
This study evaluated the long-term efficacy of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of craniofacial dystonia (Meige syndrome) and investigated the correlation between the volume of tissue activated (VTA) in the GPi and each subregion and movement score improvement. We retrospectively analyzed the clinical data of 13 patients with drug-refractory Meige syndrome who were treated with GPi DBS. The pre- and postoperative Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores were compared. The relationships between the preoperative baseline variables and improvement in the BFMDRS-Movement (BFMDRS-M) score were analyzed. LEAD-DBS software was used for the three-dimensional reconstruction of the GPi and implanted electrodes. The correlations between the GPi-VTA and score improvement were analyzed. The average follow-up period was 36.6 ± 11.0 months (18-55 months). At 3 months after the stimulation and the final follow-up visit, the improvements in the BFMDRS-M score were 58.2 and 54.6%, and the improvements in the BFMDRS-Disability (BFMDRS-D) score were 53.6 and 51.7%, respectively. At the final follow-up visit, the improvements in the BFMDRS-M scores of the eye, mouth, and speech/swallowing were significant (P < 0.001). Age was an independent predictor of improvement in the BFMDRS-M score after DBS (P = 0.005). A decrease in the BFMDRS-M score was significantly positively correlated with the GPi-VTA (r = 0.757, P = 0.003). GPi DBS is an effective method for treating drug-refractory Meige syndrome. LEAD-DBS software can be used as an effective aid for visualization programming after DBS.
Topics: Deep Brain Stimulation; Dystonia; Globus Pallidus; Humans; Retrospective Studies; Treatment Outcome
PubMed: 34185189
DOI: 10.1007/s10143-021-01584-4