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Blepharospasm-oromandibular dystonia syndrome

MedGen UID:
44341
•Concept ID:
C0025183
•
Disease or Syndrome
Synonyms: Blepharospasm Oromandibular Dyskinesia; Blepharospasm Oromandibular Dystonia; Blepharospasm Oromandibular Dystonia Syndrome; Blepharospasm Oromandibular Dystonia Syndrome, Idiopathic; Blepharospasm-Oromandibular Dyskinesia; Blepharospasm-Oromandibular Dyskinesias; Blepharospasm-Oromandibular Dystonia; Blepharospasm-Oromandibular Dystonia Syndrome; Blepharospasm-Oromandibular Dystonia Syndrome, Idiopathic; Blepharospasm-Oromandibular Dystonia Syndromes; Blepharospasm-Oromandibular Dystonias; Brueghel Syndrome; 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 Blepharospasm-Oromandibular Dystonia Syndrome; Idiopathic Orofacial Dyskinesia; Idiopathic Orofacial Dyskinesias; Meige Syndrome; Orofacial Dyskinesia, Idiopathic; Orofacial Dyskinesias, Idiopathic; Syndrome, Blepharospasm-Oromandibular Dystonia
SNOMED CT: Meige syndrome (230325003); Blepharospasm - oromandibular dystonia (230325003); Brueghel syndrome (230325003)
 
Monarch Initiative: MONDO:0019772
Orphanet: ORPHA93964

Definition

A focal dystonia involving symmetrical benign essential blepharospasm (BEB) and oromandibular dystonia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBlepharospasm-oromandibular dystonia syndrome

Professional guidelines

PubMed

Treatment of Blepharospasm and Oromandibular Dystonia with Botulinum Toxins.
Hassell TJW, Charles D
Toxins (Basel) 2020 Apr 22;12(4) doi: 10.3390/toxins12040269. PMID: 32331272Free PMC Article
Meige's syndrome: History, epidemiology, clinical features, pathogenesis and treatment.
Pandey S, Sharma S
J Neurol Sci 2017 Jan 15;372:162-170. Epub 2016 Nov 23 doi: 10.1016/j.jns.2016.11.053. PMID: 28017205
Diagnosis and treatment of dystonia.
Jinnah HA, Factor SA
Neurol Clin 2015 Feb;33(1):77-100. doi: 10.1016/j.ncl.2014.09.002. PMID: 25432724Free PMC Article

Recent clinical studies

Diagnosis

Idiopathic blepharospasm-oromandibular dystonia syndrome (Meige's syndrome) presenting as chronic temporomandibular joint dislocation.
Gray AR, Barker GR
Br J Oral Maxillofac Surg 1991 Apr;29(2):97-9. doi: 10.1016/0266-4356(91)90090-r. PMID: 2049364

Therapy

Generalized and focal dystonic syndromes: possible therapy with salmon calcitonin.
Patti F, Marano P, Nicoletti F, Giammona G, Nicoletti F
Eur Neurol 1985;24(6):386-91. doi: 10.1159/000115831. PMID: 4065158
Pharmacology of blepharospasm-oromandibular dystonia syndrome.
Casey DE
Neurology 1980 Jul;30(7 Pt 1):690-5. doi: 10.1212/wnl.30.7.690. PMID: 7190236

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