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Meige Syndrome

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A syndrome characterized by orofacial DYSTONIA; including BLEPHAROSPASM; forceful jaw opening; lip retraction; platysma muscle spasm; and tongue protrusion. It primarily affects older adults, with an incidence peak in the seventh decade of life. (From Adams et al., Principles of Neurology, 6th ed, p108)

Also Known As:

Blepharospasm-Oromandibular Dyskinesia; Blepharospasm-Oromandibular Dystonia Syndrome; Blepharospasm-Oromandibular Dystonia Syndrome, Idiopathic; Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome; Syndrome, Blepharospasm-Oromandibular Dystonia; Blepharospasm Oromandibular Dyskinesia; Blepharospasm Oromandibular Dystonia; Blepharospasm Oromandibular Dystonia Syndrome; Blepharospasm Oromandibular Dystonia Syndrome, Idiopathic; Blepharospasm-Oromandibular Dyskinesias; Blepharospasm-Oromandibular Dystonia Syndromes; Blepharospasm-Oromandibular Dystonias; Dyskinesia, Blepharospasm-Oromandibular; Dyskinesia, Idiopathic Orofacial; Dyskinesias, Blepharospasm-Oromandibular; Dyskinesias, Idiopathic Orofacial; Dystonia Syndrome, Blepharospasm-Oromandibular; Dystonia Syndromes, Blepharospasm-Oromandibular; Dystonia, Blepharospasm-Oromandibular; Dystonias, Blepharospasm-Oromandibular; Idiopathic Blepharospasm Oromandibular Dystonia Syndrome; Idiopathic Orofacial Dyskinesias; Orofacial Dyskinesia, Idiopathic; Orofacial Dyskinesias, Idiopathic; Blepharospasm-Oromandibular Dystonia; Brueghel Syndrome; Idiopathic Orofacial Dyskinesia

Networked: 60 relevant articles (7 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 6158
- Central Nervous System Diseases: 1649
  - Movement Disorders: 4614
    - Dystonic Disorders: 575
      - Meige Syndrome: 60
  - Brain Diseases: 2114
    - Basal Ganglia Diseases: 227
      - Meige Syndrome: 60

Related Diseases

1.	Blepharospasm
2.	Dystonia (Limb Dystonia)
3.	Hemifacial Spasm
4.	Dyskinesias (Dyskinesia)
5.	Torticollis (Wryneck)

Experts

1.	Kim, Joong-Seok: 2 articles (10/2014 - 08/2008)
2.	Sobstyl, Michał: 2 articles (01/2014 - 11/2011)
3.	Ząbek, Mirosław: 2 articles (01/2014 - 11/2011)
4.	Sako, Wataru: 2 articles (01/2012 - 02/2011)
5.	Kaji, Ryuji: 2 articles (01/2012 - 02/2011)
6.	Saito, Tsukasa: 1 article (02/2015)
7.	Kano, Kohei: 1 article (02/2015)
8.	Hasebe, Naoyuki: 1 article (02/2015)
9.	Katayama, Takayuki: 1 article (02/2015)
10.	Sato, Nobuyuki: 1 article (02/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Meige Syndrome:

1.	Botulinum Toxins (Botulinum Toxin)IBA 10/01/2014 - "Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. " 10/01/2008 - "A case of a 15-year-old boy with Meige syndrome who was managed with injection of botulinum toxin is presented." 10/01/2004 - "Six men and four women with a diagnosis of Meige syndrome undergoing botulinum toxin injection were recruited. " 10/01/2008 - "Meige syndrome: a rare form of cranial dystonia that was treated successfully with botulinum toxin." 05/01/2010 - "Initial data suggest that this procedure appears safe, well tolerated, and useful in the treatment of patients with essential blepharospasm and Meige syndrome responding poorly to botulinum toxin therapy."
2.	Benign essential blepharospasmIBA 06/01/1998 - "A retrospective review was performed on a population of 238 patients with presumed benign essential blepharospasm and Meige syndrome. " 12/01/1996 - "A retrospective review of 239 patients with benign essential blepharospasm and Meige syndrome was performed in order to determine patients' long term treatment preferences. " 07/01/2013 - "Seven patients had hemifacial spasm, 4 Meige syndrome, and 26 benign essential blepharospasm. " 06/01/1999 - "Charts of 358 patients with a diagnosis of benign essential blepharospasm, Meige syndrome (with eyelid involvement), and hemifacial spasm were reviewed. " 05/01/2010 - "Orbicularis oculi myo-osseous fixation: a new treatment for benign essential blepharospasm and blepharospasm associated with diffuse facial dystonia (meige syndrome)."
3.	Antipsychotic Agents (Antipsychotics)IBA 08/01/1982 - "Neuroleptics worsened symptoms in four patients with generalized dystonia and improved symptoms in one patient with Meige syndrome. " 10/01/2001 - "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. "
4.	pantogab (hopantenic acid)IBA 01/01/2010 - "Efficacy of pantogam active in the complex treatment of facial and cervical dystonic hyperkineses was studied in 37 patients (25 women, 12 men) with dystonic torticollis, blepharospasm, oromandibular dystonia with illness duration more than 6 months. "
5.	Vigabatrin FDA Link 08/01/1985 - "We conducted a single-blind trial of gamma-vinyl-GABA (GVG) in nine patients: seven with tardive dyskinesia, one with Meige syndrome, and one with Tourette syndrome. "
6.	Type A Botulinum Toxins (Botulinum A Toxin)FDA Link 11/01/1988 - "Four patients with severe Meige's disease (blepharospasm-oromandibular dystonia) have been treated, after having given an informed consent, by local injections of purified botulinum toxin type "A". " 01/01/1997 - "Botulinum A toxin treatment for blepharospasm and Meige syndrome: report of 100 patients." 01/01/1988 - "13 patients with hemifacial spasm and 19 patients with blepharospasm (essential blepharospasm or Meige syndrome) were treated with injection of botulinum A toxin in the eyelids. "
7.	Trihexyphenidyl (Artane)FDA LinkGeneric 01/01/1983 - "Meige syndrome: treatment with trihexyphenidyl." 02/01/2015 - "Combination therapy for segmental craniocervical dystonia (Meige syndrome) with aripiprazole, trihexyphenidyl, and botulinum toxin: three cases reports."
8.	LisurideIBA 06/01/1988 - "The patient described here had blepharospasm-oromandibular dystonia, which responded transiently to oral lisuride. " 06/01/1985 - "[Improvement of Meige syndrome with lisuride]."
9.	Clonazepam (Rivotril)FDA LinkGeneric 01/01/2011 - "Spasmodic dysphonia in Meige syndrome responding to clonazepam." 10/01/2001 - "Effect of clonazepam treatment on antipsychotic drug-induced Meige syndrome and changes in plasma levels of GABA, HVA, and MHPG during treatment." 10/01/2001 - "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. "
10.	Congenital hereditary lymphedemaIBA 01/01/1990 - "[Hereditary lymphedema (Nonne-Milroy-Meige syndrome) associated with chylothorax. " 01/01/2000 - "To describe a patient with Meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. "

Therapies and Procedures

1.	Injections 05/01/1993 - "Dose requirements for this indication were compared with doses used in 697 injections in 112 patients with essential blepharospasm and Meige syndrome. " 11/01/1988 - "Four patients with severe Meige's disease (blepharospasm-oromandibular dystonia) have been treated, after having given an informed consent, by local injections of purified botulinum toxin type "A". " 07/01/2004 - "The study was carried out in patients with essential blepharospasm or Meige syndrome undergoing routine treatment with botulinum toxin A injections. " 11/01/2011 - "These observations have led to the use of GPi stimulation in patients with severe torticollis and Meige syndrome refractory to pharmacological agents as well to botulinum toxin injections. " 05/01/2010 - "This article describes a novel surgical procedure, myo-osseous fixation, as an adjunct therapy for patients with reduced or poor response to repeated botulinum toxin injections for essential blepharospasm and Meige syndrome. "
2.	Drug Therapy (Chemotherapy) 07/01/2001 - "We report that all dystonic symptoms that had been refractory to both pharmacotherapy and bilateral thalamotomy were markedly alleviated by bilateral pallidal stimulation in a patient with segmental axial dystonia advanced from Meige syndrome." 12/01/1996 - "While botulinum toxin is the most highly effective treatment for benign essential blepharospasm and Meige syndrome over a long period of time, adjunctive oral drug therapy, including minor tranquillisers as well as eyelid surgery, may augment its effectiveness."
3.	Deep Brain Stimulation 10/01/2014 - "We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). " 10/01/2014 - "Globus pallidus interna deep brain stimulation in a patient with medically intractable meige syndrome." 01/01/2014 - "Pallidal deep brain stimulation in the treatment of Meige syndrome." 03/01/2011 - "Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation." 02/01/2011 - "Bilateral pallidal deep brain stimulation in primary Meige syndrome."
4.	Pallidotomy 01/01/2011 - "We report the case of a 64-year-old woman with bilateral manifestation of Meige syndrome (MS) successfully treated with left-side unilateral ventroposterolateral pallidotomy. " 01/01/2011 - "Effectiveness of unilateral pallidotomy for meige syndrome confirmed by motion analysis." 05/01/1997 - "Ventrolateral (VL)-thalamotomy and posteroventral pallidotomy were carried out in 37 patients with idiopathic dystonia, including 23 with dystonia musculorum deformans, 12 with Meige syndrome and 2 with focal dystonia. "
5.	Intravenous Infusions 02/01/1985 - "An intravenous infusion of a 250-ml, 10% ethanol solution decreased dystonic scores in five of seven patients with spasmodic torticollis, but had no effect in patients with Meige syndrome, tardive dystonia, or generalized torsion dystonia. "