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Indian Journal of Psychological Medicine Jul 2013Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old...
Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.
PubMed: 24249940
DOI: 10.4103/0253-7176.119493 -
The British Journal of Oral &... Dec 2016Oromandibular dystonia is a disorder of movement that is often misdiagnosed. We present our experience of six patients treated with botulinum A toxin, for whom we...
Oromandibular dystonia is a disorder of movement that is often misdiagnosed. We present our experience of six patients treated with botulinum A toxin, for whom we recorded the symptoms, the group of muscles targeted, and the quantity of botulinum toxin used. We used the Glasgow Benefit Inventory (GBI) questionnaire to record and analyse outcomes after treatment. Five of the six patients had a good outcome as recorded by the GBI, the mean (SD) score of which was +40 (28), which reflected that the patients were well satisfied. The overall improvement in the categories of general health, social health, and physical health were 82%, 37%, and 31%, respectively. Botulinum type A toxin is an effective treatment of oromandibular dystonia. It is important for clinicians to recognise the condition, as treatment is well-tolerated and can improve quality of life.
Topics: Botulinum Toxins, Type A; Dystonia; Humans; Neuromuscular Agents; Quality of Life; Surveys and Questionnaires; Treatment Outcome
PubMed: 27776922
DOI: 10.1016/j.bjoms.2016.06.028 -
Journal of Korean Neurosurgical Society Aug 2010Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain...
OBJECTIVE
Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome.
METHODS
Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was 23.1 ± 6.4 months. The mean age at time of surgery was 58.0 ± 7.8 years. The mean duration of symptoms was 8.7 ± 7.6 years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After 2.4 ± 1.3 days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS).
RESULTS
BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant.
CONCLUSION
The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.
PubMed: 20856662
DOI: 10.3340/jkns.2010.48.2.134 -
Frontiers in Neurology 2012Although there are some newly developed options to treat dystonia, its medical treatment is not always satisfactory. Zolpidem, an imidazopyridine agonist with a high...
Although there are some newly developed options to treat dystonia, its medical treatment is not always satisfactory. Zolpidem, an imidazopyridine agonist with a high affinity on benzodiazepine subtype receptor BZ1 (ω1), was found to improve clinical symptoms of dystonia in a limited number of case reports. To investigate what subtype of dystonia is responsive to the therapy, we conducted an open label study to assess the efficacy of zolpidem (5-20 mg) in 34 patients suffering from miscellaneous types of dystonia using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Patients were entered into the study if they had been refractory to other medications as evaluated by BFMDRS (no change in the previous two successive visits). After zolpidem therapy, the scores in the patients as a whole were decreased from 7.2 ± 7.9 to 5.5 ± 5.0 (P = 0.042). Patients with generalized dystonia, Meige syndrome/blepharospasm, and hand dystonia improved in the scale by 27.8, 17.8, and 31.0%, respectively, whereas no improvement was found in cervical dystonia patients. Overall response rate among patients were comparable to that of trihexyphenidyl. Zolpidem may be a therapeutic option for generalized dystonia, Meige syndrome, and hand dystonia including musician's. Drowsiness was the dose-limiting factor.
PubMed: 22529836
DOI: 10.3389/fneur.2012.00058 -
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 -
Journal of Neurology, Neurosurgery, and... Nov 1998Oromandibular dystonia (OMD) is a focal dystonia manifested by involuntary muscle contractions producing repetitive, patterned mouth, jaw, and tongue movements. Dystonia... (Comparative Study)
Comparative Study
OBJECTIVES
Oromandibular dystonia (OMD) is a focal dystonia manifested by involuntary muscle contractions producing repetitive, patterned mouth, jaw, and tongue movements. Dystonia is usually idiopathic (primary), but in some cases it follows peripheral injury. Peripherally induced cervical and limb dystonia is well recognised, and the aim of this study was to characterise peripherally induced OMD.
METHODS
The following inclusion criteria were used for peripherally induced OMD: (1) the onset of the dystonia was within a few days or months (up to 1 year) after the injury; (2) the trauma was well documented by the patient's history or a review of their medical and dental records; and (3) the onset of dystonia was anatomically related to the site of injury (facial and oral).
RESULTS
Twenty seven patients were identified in the database with OMD, temporally and anatomically related to prior injury or surgery. No additional precipitant other than trauma could be detected. None of the patients had any litigation pending. The mean age at onset was 50.11 (SD 14.15) (range 23-74) years and there was a 2:1 female preponderance. Mean latency between the initial trauma and the onset of OMD was 65 days (range 1 day-1 year). Ten (37%) patients had some evidence of predisposing factors such as family history of movement disorders, prior exposure to neuroleptic drugs, and associated dystonia affecting other regions or essential tremor. When compared with 21 patients with primary OMD, there was no difference for age at onset, female preponderance, and phenomenology. The frequency of dystonic writer's cramp, spasmodic dysphonia, bruxism, essential tremor, and family history of movement disorder, however, was lower in the post-traumatic group (p<0.05). In both groups the response to botulinum toxin treatment was superior to medical therapy (p<0.005). Surgical intervention for temporomandibular disorders was more frequent in the post-traumatic group and was associated with worsening of dystonia.
CONCLUSION
The study indicates that oromandibular-facial trauma, including dental procedures, may precipitate the onset of OMD, especially in predisposed people. Prompt recognition and treatment may prevent further complications.
Topics: Adolescent; Adult; Aged; Anti-Dyskinesia Agents; Botulinum Toxins; Facial Injuries; Female; Humans; Meige Syndrome; Middle Aged
PubMed: 9810945
DOI: 10.1136/jnnp.65.5.722 -
BMJ Case Reports Mar 2016Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an established procedure to improve generalised, segmental craniofacial dystonia. However, no...
Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an established procedure to improve generalised, segmental craniofacial dystonia. However, no studies have addressed the applicability of DBS for blepharospasm as a focal craniofacial dystonia. A 52-year-old man developed medically intractable involuntary eye closure. Because the abnormal movement was observed exclusively in the eyelids, he was diagnosed not with Meige's syndrome but with blepharospasm as a focal craniofacial dystonia. He underwent stereotactic surgery under general anaesthesia for bilateral GPi-DBS. Continuous GPi stimulation almost completely abolished the blepharospasm. 15 months after the operation, his preoperative scores on the Burke-Fahn-Marsden Dystonia Rating Scale (=8 points) decreased to 1 (87.5% improvement). The present study demonstrates the applicability of GPi-DBS for treating blepharospasm presenting as focal dystonia. Further studies with accumulated case series are needed to confirm the effect of DBS on blepharospasm and other focal craniofacial dystonias.
Topics: Blepharospasm; Deep Brain Stimulation; Diagnosis, Differential; Humans; Male; Middle Aged; Stereotaxic Techniques; Treatment Outcome
PubMed: 27033410
DOI: 10.1136/bcr-2015-214241 -
BMC Ophthalmology Dec 2020We report a case of Meige's syndrome induced by an atypical antipsychotic (blonanserin) that presented with refractory dry eye disease.
BACKGROUND
We report a case of Meige's syndrome induced by an atypical antipsychotic (blonanserin) that presented with refractory dry eye disease.
CASE PRESENTATION
A 37-year-old woman with a 6-month history of foreign body sensation in the eyes and difficulty in opening her eyes was treated at a local clinic for dry eye disease. Despite this treatment, her symptoms did not improve and she was transferred to our attention. Our assessment revealed involuntary movements of her eyelids accompanied by repetitive pursing of her lips. She had been undergoing treatment with blonanserin for 5 years for schizophrenia. She was diagnosed with drug-induced Meige's syndrome after a psychiatric and neurological consultation. After a 2-month gradual dose reduction and discontinuing blonanserin, involuntary movements of the eyelids with oromandibular dystonia were resolved. Three months after discontinuing blonanserin, there was no recurrence of symptoms, and she had no exacerbation of psychotic symptoms.
CONCLUSIONS
In patients with refractory dry eye disease, especially those with involuntary movements of the eyelids with oromandibular dystonia, it is important to ask about their psychotropic medications and to consider the possibility of drug-induced Meige's syndrome and discontinuation of medications, if possible.
Topics: Adult; Antipsychotic Agents; Dry Eye Syndromes; Female; Humans; Meige Syndrome
PubMed: 33267850
DOI: 10.1186/s12886-020-01738-w -
Asian Journal of Surgery May 2024
PubMed: 38824021
DOI: 10.1016/j.asjsur.2024.05.156 -
Innovations in Clinical Neuroscience Jul 2012Meige syndrome is a rare form of segmental dystonia characterized by blepharospasm and oromandibular dystonia. A few case reports of Meige syndrome have been associated...
Meige syndrome is a rare form of segmental dystonia characterized by blepharospasm and oromandibular dystonia. A few case reports of Meige syndrome have been associated with Lewy body pathologies, and the syndrome was also proposed for inclusion in the spectrum of Lewy body disease. We report a case of an elderly gentleman with a history of Meige syndrome for more than 10 years who developed dementia with Lewy bodies. Updated clinical and pathological evidence of linkages between these two conditions is also presented.
PubMed: 22984651
DOI: No ID Found