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Journal of Neurology, Neurosurgery, and... Nov 1987A double-blind crossover study of sodium valproate and placebo was conducted in five patients with Meige syndrome. CSF neurotransmitter studies were performed at the end... (Clinical Trial)
Clinical Trial
A double-blind crossover study of sodium valproate and placebo was conducted in five patients with Meige syndrome. CSF neurotransmitter studies were performed at the end of each treatment period. GABA levels were not influenced by the administration of sodium valproate. An increase in HVA levels was observed in every patient, which may reflect an increase in central dopaminergic activity. This finding may explain the trend towards clinical deterioration which was observed during treatment with sodium valproate. Sodium valproate appears to be ineffective in Meige syndrome.
Topics: Basal Ganglia Diseases; Double-Blind Method; Female; Humans; Male; Meige Syndrome; Middle Aged; Valproic Acid; gamma-Aminobutyric Acid
PubMed: 3121795
DOI: 10.1136/jnnp.50.11.1522 -
Arquivos Brasileiros de Oftalmologia 2023Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the...
PURPOSE
Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the clinical features of patients with these conditions, followed up in two reference centers in Brazil.
METHODS
The study included patients with essential blepharospasm and hemifacial spasm, followed up at the Departments of Ophthalmology at Universidade Federal de São Paulo and Universidade de São Paulo. Apart from demographic and clinical features, past stressful events related to the first symptoms (triggering event), aggravating factors, sensory tricks, and other ameliorating factors for the eyelid spasms were assessed.
RESULTS
A total of 102 patients were included in this study. Most patients were female (67.7%). Essential blepharospasm was the most frequent movement disorder [51/102 patients (50%)], followed by hemifacial spasm (45%) and Meige's syndrome (5%). In 63.5% of the patients, the onset of the disorder was associated with a past stressful event. Ameliorating factors were reported by 76.5% of patients; 47% of patients reported sensory tricks. In addition, 87% of the patients reported the presence of an aggravating factor for the spasms; stress (51%) was the most frequent.
CONCLUSION
Our study provides information regarding the clinical features of patients treated in the two largest ophthalmology reference centers in Brazil.
Topics: Humans; Female; Male; Hemifacial Spasm; Brazil; Ophthalmology; Blepharospasm; Spasm; Facial Muscles
PubMed: 37851740
DOI: 10.5935/0004-2749.2022-0160 -
Journal of Thoracic Oncology : Official... Nov 2019
Topics: Adenocarcinoma of Lung; Aged; Fatal Outcome; Female; Gene Rearrangement; Humans; Lung Neoplasms; Meige Syndrome; Proto-Oncogene Proteins c-ret
PubMed: 31668323
DOI: 10.1016/j.jtho.2019.06.008 -
Psychiatry and Clinical Neurosciences Oct 2001We demonstrated the effect of clonazepam (2 mg/day) on Meige syndrome in two schizophrenic patients under continuous treatment with antipsychotic drugs, and changes in...
We demonstrated the effect of clonazepam (2 mg/day) on Meige syndrome in two schizophrenic patients under continuous treatment with antipsychotic drugs, and changes in the plasma levels of gamma-aminobutyric acid (GABA), homovanillic acid (HVA), and 3-methoxy-4-hydroxyphenylglycol (MHPG) in these cases. The plasma levels of HVA and MHPG during treatment with clonazepam were decreased in the responder, while not changed in the non-responder to clonazepam. A difference between the responder and the non-responder was not found in the plasma GABA levels. These results suggest that hyperactivities of the central dopaminergic and noradrenergic neurones are involved in the pathophysiology of Meige syndrome.
Topics: Adult; Antipsychotic Agents; Clonazepam; Female; Homovanillic Acid; Humans; Meige Syndrome; Methoxyhydroxyphenylglycol; Schizophrenia, Disorganized; Schizophrenia, Paranoid; gamma-Aminobutyric Acid
PubMed: 11555353
DOI: 10.1046/j.1440-1819.2001.00903.x -
Revista de Neurologia Jul 2020Dyskinesia of the ADCY5 mutation is a rare movement-onset disorder in childhood. It is characterized by isolated chorea movements or associated with myoclonus and... (Review)
Review
INTRODUCTION
Dyskinesia of the ADCY5 mutation is a rare movement-onset disorder in childhood. It is characterized by isolated chorea movements or associated with myoclonus and dystonia affecting the limbs, neck and face. The low number of patients and families still does not allow an adequate genotype-phenotype relationship.
AIMS
The case of a child with movement disorders of early onset is presented in a family with three generations of affected members. An updated review of the casuistry and management of this rare disease is made.
CASE REPORT
A 6-year-old boy referred for language delay and hyperactivity. After six months of follow-up he begins to show chorea movements of predominantly facial and limb roots, especially when waking up. At one year of follow-up, generalized chorea at rest with orofacial involvement and awkward gait begins to show. His family history includes his mother, grandfather, maternal uncle and cousin, who were diagnosed with Meige's syndrome (oromandibular dystonia and periorbital muscles) with choreiform-like movement disorders without affiliation since childhood. The brain study by MRI showed no alterations. A clinical exome targeting movement disorders was performed that discovered the pathogenic mutation in the ADCY5 gene causing autosomal familial dyskinesia.
CONCLUSION
The c.1126G>A p.A376T mutation shows a natural history with a non-progressive clinical phenotype in three generations of affected members, with childhood debut and response to guanfacine treatment.
Topics: Adenylyl Cyclases; Amino Acid Substitution; Attention Deficit Disorder with Hyperactivity; Child; Developmental Disabilities; Drug Resistance; Female; Guanfacine; Humans; Language Development Disorders; Levetiracetam; Male; Meige Syndrome; Movement Disorders; Mutation, Missense; Pedigree; Point Mutation
PubMed: 32627162
DOI: 10.33588/rn.7102.2020154 -
Journal of Movement Disorders Oct 2014Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We...
Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects.
PubMed: 25360233
DOI: 10.14802/jmd.14013 -
Psychiatry and Clinical Neurosciences Dec 2007
Topics: Adult; Antipsychotic Agents; Benzodiazepines; Fluvoxamine; Humans; Male; Meige Syndrome; Olanzapine; Risperidone; Schizophrenia; Selective Serotonin Reuptake Inhibitors
PubMed: 18081639
DOI: 10.1111/j.1440-1819.2007.01739.x -
Annals of Indian Academy of Neurology Apr 2011Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. It has been reported as a complication of typical antipsychotics. To the best of our...
Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. It has been reported as a complication of typical antipsychotics. To the best of our knowledge, case of olanzapine-induced tardive Meige's syndrome has not been reported in the literature. We we are reporting report a case of Meige's syndrome developing after long term therapy with olanzapine.
PubMed: 21808480
DOI: 10.4103/0972-2327.82808 -
Medicina Oral, Patologia Oral Y Cirugia... Sep 2021This retrospective study aimed in the evaluation of oral health and oral health-related quality of life (OHRQoL) of patients with oral dystonia (OD).
BACKGROUND
This retrospective study aimed in the evaluation of oral health and oral health-related quality of life (OHRQoL) of patients with oral dystonia (OD).
MATERIAL AND METHODS
Seventeen patients with OD (Meige Syndrome: n=11, Oromandibular Dystonia: n=6) were included, of which seven were examined again at three months after botulinum toxin injection. OHRQoL was assessed by the German short form of oral health impact profile (OHIP G14). Within oral examination, dental parameters, remaining teeth and periodontitis severity were assessed. A matched healthy control (HC) was composed for comparison.
RESULTS
The OD patients had significantly more carious teeth (0.94 ± 1.75 vs. 0; p<0.01), less remaining teeth (15.65 ± 9.89 vs. 22.22 ± 5.91; p=0.01) and higher dental treatment need than the HC (42.9% vs. 0%; p<0.01). The OHIP G14 sum score of 9.47± 9.82 vs. 1.58 ± 2.79 (p<0.01) as well its dimensions psychosocial impact (4.47 ± 6.45 vs. 0.53 ± 1.16; p=0.03) and oral function (4.35 ± 2.98 vs. 0.47 ± 1.34; p<0.01) were clinically relevant and statistically significant higher in OD compared to HC group. No significant differences could be detected at three months after botulinum toxin injection.
CONCLUSIONS
Patients with OD suffer from more dental diseases and have a worse OHRQoL than HC. Dental special care appears recommendable and should be fostered by everyone, who is involved in the treatment of patients with OD.
Topics: Cross-Sectional Studies; Diagnosis, Oral; Dystonia; Humans; Oral Health; Quality of Life; Retrospective Studies; Surveys and Questionnaires
PubMed: 34023846
DOI: 10.4317/medoral.24479 -
Journal of Neurology, Neurosurgery, and... Nov 1986
Topics: Aged; Basal Ganglia Diseases; Brain Stem; Cerebral Infarction; Female; Humans; Meige Syndrome; Muscles; Myoclonus; Palatal Muscles
PubMed: 3794743
DOI: 10.1136/jnnp.49.11.1324