Health Issues Playing the Marimba - Repetitive Motion, Posture, and More -- Music and Medicine on MondayClick Here

Meige's Syndrome

last modified on: Tue, 12/12/2023 - 20:26

return to: Botulinum Toxin Protocols

"De Gaper' by Pieter Brueghel, The Elder (c. 1525-1569) from wiki commons (public domain) accessed 5 26 2019 https://commons.wikimedia.org/wiki/File:DeGaper.png

Meige's syndrome is a syndrome of oral mandibular dystonia with blepharospasm.

  • Oromandibular dystonia (OMD) consists of prolonged spasms caused by contraction of the muscles of the mouth and mandible and involves the muscles of mastication, facial expression, tongue, and eye lids. This results in difficulty opening and closing the mandible, often affecting mastication and speech. It can also create myofacial pain symptoms.
  • Symptoms often begin between ages 40-70 years with blepharospasm usually the earliest clinical manifestation which spreads over time to involve other cranial and extra-cranial muscles with anatomic variants leading to different eponyms (Pandy 2017);
    • ​Meige's syndrome - Dr. Henir Meige, a French neurologist, in 1910 described 10 patients with 'facial convulsions' accompanied by spasms in pharynx, jaw, floor of mouth and tongue through to be provoked by irritative lesion in bulbopontine region
    • Segmanetal cranio-cervical dystonia
    • Segmental cranial dystonia
    • Blepharospasm plus
    • Wood syndrome - Dr. Horatio Wood, a Philadelphia neurologist, described blepharospasm accompanied by other cranial dystonias in 1887
    • Brueghel syndrome - named after Pieter Brueghel, the Elder, with consideration that jaw-opening dystonia without blepharospasm occurred but was less common than Meige's syndrome
  • Women are more affected than men
  • Symptoms: Forceful opening of the jaw or jaw clamping shut, retraction of lips or lip pursing, spasm of platysma, protrusion of tongue, lateral jaw deviation, difficulty speaking or swallowing, blepharospasm
  • There is no definitive diagnostic medical test for OMD. The diagnosis is based on information from the individual along with a neurological examination
  • Treatment is tailored to the individual patient. Oral medications have been used to treat OMD. Approximately one-third of a patient's symptoms improve with clonazepam, trihexyphenidyl, diazepam, and other oral medications.
  • Approximately 70% of patients with OMD may experience some reduction of spasm and improvement in mastication and speech after injection of botulinum toxin (Botox; Allergan, Inc, Irvine, Calif) into muscles of mastication (masseter, temporalis, and lateral pterygoid). These injections are most effective in mandibular closing dystonia and less effective in mandibular opening dystonia. Use of botulinum toxin has been reported to be occasionally effective in the treatment of lingual dystonia; however, side effects such as swallowing difficulties, speech problems, and excessive muscle weakness where the injection was placed have been reported.
  • Clinical Report:Schneider R, Hoffman HT. Oromandibular dystonia: a clinical report. J Prosthet Dent. 2011 Dec; 106(6):355-8. - with longer term f/u identifying use of a dental appliance offered remarkable improvement for 3 months - but with benefit that did not continue past that time - leading to abandoning the prosthesis in favor or repeated (every 3 months) EMG guided botox injection to the anterior bellies of digastric muscles.
  • 'Sensory tricks' have found some benefit in decreasing symptoms of blepharospasm (humming or singing, pulling on the upper eyelid, using a toothpick, blowing cheeks)

Secondary Meige's Syndrome

  • Considered a manifestation of tardive dystonia - that may result from underlying neurological disorder
    • Rare causes associated have been listed as head trauma, kernicterus, stroke, brainstem demyelination, normal pressure hydrocephalus, bilateral thalamotomy, mass lesions, post-encephalitis, and cerebral hypoxia (Pandey 2017)
  • May arise from use of neuroleptic medications - developing tardive dystonia involving cranio-cervical musculature
    • Thought to be due to blockade of dopamine receptor leading to denervation supersensititivty.
    • Genetic factors potentially involved in predisposition to neuroleptic induced movement disords. 
    • Drugs listed as potentially contributing (Pandy 2017): metoclopramide, levodopa, bromocriptine and rarely olanzapine
      • antidepressants and selective serotonin reuptake inhibitors (SSRIs) potentially associated with development of facial dyskinesa (Mauriello 1998)
      • Antihistamines implicated in drug induced blepharospasm

Pathogenesis

  • Not well understood
  • Hallet (2002) suggested - environmental trigger and genetic predisposition cause plastic changes and reduced cortical inhibition

Botulinum toxin injection

  • Multiple publications identify the potential value for use of botulinum toxin injection to selected affected muscles as an alternative (or supplement) to medical therapy - with general repords identifying blepharospasm is more likely to respond to botulinum toxin than oromandibular dystonia - and that jaw closing dystonia is more likely to respond than either jaw opening or mixed dystonia (Tan 1999 and Jankovic 1987)

References

Schneider R, Hoffman HT. Oromandibular dystonia: a clinical report. J Prosthet Dent. 2011 Dec; 106(6):355-8.

Gonzalez-Alegre P, Schneider RL, Hoffman H: Clinical, Etiological, and Therapeutic Features of Jaw-opening and Jaw-closing Oromandibular Dystonias: A Decade of Experience at a Single Treatment Center.Tremor Other Hyperkinet Mov (N Y). 2014 Apr 30;4:231.

Pandey S, Sharma S: Meige's syndrome: History, epidemiology, clinical features, pathogenesis and treatment.J Neurol Sci. 2017 Jan 15;372:162-170. doi: 10.1016/j.jns.2016.11.053. Epub 2016 Nov 23

Mauriello JA, Carbonarao P, Dhillon S, Leone T, and Franklin M: Drugassociated facial dyskinesias - a study of 238 patients. J. Neuroophthalmol. 18(2) 1998 June 153-157

Hallett M: Blepharospasm: recent advances Neurology, 59 (2002), pp. 1306-1312

Tan EK and Jankovic J: Botulinum toxin a in patients with oromandibular dystonia: long-term follow-up Neurology, 53 (9) (Dec 10, 1999), pp. 2102-2107

Jankovic J and Orman J: Botulinum a toxin for cranial-cervical dystonia: a double-blind, placebo-controlled study Neurology, 37 (4) (Apr 1987), pp. 616-623

Marsden CD: Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia J. Neurol. Neurosurg. Psychiatry, 39 (1976), pp. 1204-1209