Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1603  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (411 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this Article

 Article Access Statistics
    PDF Downloaded68    
    Comments [Add]    

Recommend this journal


Table of Contents    
Year : 2018  |  Volume : 66  |  Issue : 1  |  Page : 36-37

Current status of dystonias including Meige's syndrome

Department of Neurology, Fortis Hospital, Vasant Kunj, New Delhi, India

Date of Web Publication11-Jan-2018

Correspondence Address:
Dr. Madhuri Behari
Department of Neurology, Fortis Hospital, Vasant Kunj, New Delhi
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.222827

Rights and Permissions

How to cite this article:
Behari M. Current status of dystonias including Meige's syndrome. Neurol India 2018;66:36-7

How to cite this URL:
Behari M. Current status of dystonias including Meige's syndrome. Neurol India [serial online] 2018 [cited 2020 May 26];66:36-7. Available from:

“Meige's syndrome” is a type of segmental dystonia characterized by the presence of blepharospasm along with a oromandibulo-facio-cervical (cranio-cervical) dystonia. It was described by Henry Meige, a French neurologist in 1910.[1] It is very disfiguring as well as debilitating, causing functional blindness along with difficulty in handling food in the mouth and associated pain.

Treatment for all dystonias including Meige's syndrome has been very disappointing. Though a large number of oral medications have been tried, the result has been, at best, only modest. Among the oral medications that have been used to treat Meige's syndrome, anti-cholinergics, benzodiazepines, tetra-benazine and other atypical antipsychotics such as pimozide and baclofen (GABAB receptor agonist) have been tried. All of them are associated with significant side effects.[2],[3],[4],[5],[6],[7] Pharmacotherapy provides better control of symptoms in blepharospasm as compared to Meige's syndrome. Of all the mentioned drugs, benzodiazepines are better tolerated, and have shown a good response in cranio-cervical dystonia.[4],[5],[6],[7] More recently, leveteracetam and zolpidem have been found to be effective in some cases of focal dystonia.[8],[9],[10] However, sodium valproate was not found to be useful.[11],[12]

In the mid 1980's, injection botulinum toxin revolutionized the treatment of dystonias.[13],[14] It was first approved for use in strabismus.[14] Since then it has become a regular mode of therapy for focal and segmental dystonias.[15],[16],[17] Most of earlier authors have combined the results of treatment of blepharospasm and Meige's syndrome while reporting the results of injection botulinum toxin. The article by Pandey et al.,[18] appears to be timely in this regard and differs from most of the other reported series in that it includes only cases of Meige's syndrome treated with botulinum toxin. The number of case included is small. Response to botulinum toxin is, however, dramatic with the improvement starting in 24-48 hours. The improvement is upto the tune of 70-80% on the Burke-Fahn-Marsden dystonia scale (BFMDS). Similar to the results of pharmacotherapy, the clinical response in cases of blepharospasm is about 70-80%, whereas only 30-40% of patients suffering from Meige's syndrome respond to injection botulinum toxin.[19],[20],[21],[22] Complication rates in most experienced hands are rather low.

Deep brain stimulation (DBS) of globus pallidus interna (GPi) is another option of treatment especially in patients responding poorly to botulinum toxin injections.[23],[24],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40],[41] The number of patients studied has been small. Very good results have been seen in patients with primary generalized dystonia with DYT1 (torsion dystonia mutation 1).[33] In a small follow-up series of 5 patients suffering from Meige's syndrome with GPi DBS, a significant response lasting for about 4 years has been shown.[34] It is postulated that long-term GPi DBS may correct neural network abnormalities responsible for the appearance of dystonia so that these patients do not require continued stimulation.[42]

On the other hand, some patients have required bilateral sub-thalamic (STN) DBS after GPi DBS due to an inadequate, response with the latter procedure. Stimulation induced parkinsonian features have been observed after GPi stimulation in Huntington's disease and Tourrettes' syndrome, which were not related to the underlying pathology.[43],[44],[45] Hence, most neurologists prefer the STN DBS to the GPi DBS. It is also observed that patients with phasic contractions fare better with GPi DBS than those with tonic contractions.

  References Top

Meige H. Les convulsions de la face: Une forme clinique de convulsion faciale, bilaterale et mediane, Rev Neurol (Paris) 1910;10:437-43.  Back to cited text no. 1
LeDoux MS. Meige's syndrome: What's in a name? Parkinsonism Relat Disord 2009;15:483-9.  Back to cited text no. 2
Marsden CD. Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia. J Neurol Neurosurg Psychiatry 1976;39:1204-9.  Back to cited text no. 3
Tanner CM, Glantz RH, Klawans HL. Meige disease: Acute and chronic cholinergic effects. Neurology 1982;32:783-5.  Back to cited text no. 4
Duvoisin RC. Meige syndrome: Relief on high-dose anticholinergic therapy. Clin Neuropharmacol 1983;6:63-6.  Back to cited text no. 5
Nutt JG, Hammerstad JP, Carter JH, deGarmo P. Meige syndrome: Treatment with trihexyphenidyl. Adv Neurol 1983;37:201-5.  Back to cited text no. 6
Klawans HL, Tanner CM. Cholinergic pharmacology of blepharospasm with oromandibular dystonia (Meige's syndrome). Adv Neurol 1988;49:443-50.  Back to cited text no. 7
Zesiewicz TA, Louis ED, Sullivan KL, Menkin M, Dunne PB, Hauser RA. Substantial improvement in a Meige's syndrome patient with levetiracetam treatment. Mov Disord 2004;19:1518-21.  Back to cited text no. 8
Yardimci N, Karatas M, Kilinc M, Benli S. Levetiracetam in Meige's syndrome. Acta Neurol Scand 2006;114:63-6.  Back to cited text no. 9
An JY, Kim JS, Kim YI, Lee KS. Successful treatment of the Meige syndrome with oral zolpidem monotherapy. Mov Disord 2008;23:1619-21.  Back to cited text no. 10
Hipola D, D. Mateo D, Gimenez-Roldan S. Meige's syndrome: Acute and chronic responses to clonazepan and anticholinergics. Eur Neurol 1984;23:474-8.  Back to cited text no. 11
Snoek JW, van Weerden TW, Teelken AW, van den Burg W, Lakke JP. Meige syndrome: Double-blind crossover study of sodium valproate. J Neurol Neurosurg Psychiatry 1987;50:1522-5.  Back to cited text no. 12
Scott AB. Botulinum toxin injection of eye muscles to correct strabismus. Trans Am Ophthalmol Soc 1981;79:734-70.  Back to cited text no. 13
Mauriello Jr. JA. Blepharospasm, Meige syndrome, and hemifacial spasm: Treatment with botulinum toxin. Neurology 1985;35:1499-1500.  Back to cited text no. 14
Elston JS. Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injections. Br J Ophthalmol 1987; 71;664-8.  Back to cited text no. 15
Rai V, Goyal V, Shukla G, Rath G, Behari M. Adductor laryngeal breathing dystonia in NBIA treated with botulinum toxin-A. Ann Indian Acad Neurol. 2013;16:409-10.  Back to cited text no. 16
Bhaumik S, Behari M. Botulinum toxin-A injection for cervical dystonia. J Assoc Physicians India 1999;47:267-70.  Back to cited text no. 17
Pandey S, Sharma S. Botulinum toxin in patients with Meige's syndrome: A video-based case series. Neurol India 2018:66:71-6.  Back to cited text no. 18
Poungvarin N, Devahastin V, Chaisevikul R, Prayoonwiwat N, Viriyavejakul A. Botulinum A toxin treatment for blepharospasm and Meige syndrome: Report of 100 patients. J Med Assoc Thail 1977;80:1-8.  Back to cited text no. 19
Møller E, Werdelin LM, Bakke M, Dalager T, Prytz S, Regeur L. Treatment of perioral dystonia with botulinum toxin in 4 cases of Meige syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;96;544-9.  Back to cited text no. 20
Grandas F, Elston J, Quinn N, Marsden CD. Blepharospasm: A review of 264 patients. J Neurol Neurosurg Psychiatry 1988;51:767-72.  Back to cited text no. 21
Van Den Bergh P, Francart J, Mourin S, Kollmann P, Laterre EC. Five-year experience in the treatment of focal movement disorders with low-dose dysport botulinum toxin. Muscle Nerve 1995;18:720-9.  Back to cited text no. 22
Muta D, Goto S, Nishikawa S, Hamasaki T, Ushio Y, Inoue N, et al. Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy. Mov Disord 2001;16:774-77.  Back to cited text no. 23
Houser M, Waltz T. Meige syndrome and pallidal deep brain stimulation. Mov Disord 2005;20:1203-5.  Back to cited text no. 24
Ostrem JL, Marks Jr. WJ, Volz MM, Heath SL, Starr PA. Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome). Mov Disord 2007;22:1885-91.  Back to cited text no. 25
Blomstedt P, Tisch S, Hariz MI. Pallidal deep brain stimulation in the treatment of Meige syndrome. Acta Neurol. Scand 2008; 118:198-202.  Back to cited text no. 26
Inoue N, Nagahiro S, Kaji R, S. Goto S. Long-term suppression of Meige syndrome after pallidal stimulation: A 10-year follow-up study. Mov Disord 2010;25:1756-8.  Back to cited text no. 27
Ghang JY, Lee MK, Jun SM, Ghang CG. Outcome of pallidal deep brain stimulation in Meige syndrome. J Korean Neurosurg Soc 2010;48:134-8.  Back to cited text no. 28
Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VG. Long-term follow- up of deep brain stimulation for Meige syndrome. Neurosurg Focus 2010;29:E5.  Back to cited text no. 29
Markaki E, Kefalopoulou Z, Georgiopoulos M, Paschali A, Constantoyannis C. Meige syndrome: A cranial dystonia treated with bilateral pallidal deep brain stimulation. Clin Neurol Neurosurg 2010;112:344-6.  Back to cited text no. 30
Romito LM, Elia AE, Franzini A, Bugiani O, Albanese A. Low-voltage bilateral pallidal stimulation for severe Meige syndrome in a patient with primary segmental dystonia: Case report. Neurosurgery 2010;67:(suppl 3):E 308.  Back to cited text no. 31
Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y, et al. Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord 2011;17:123-5.  Back to cited text no. 32
Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, et al. Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 2011;26:691-8.  Back to cited text no. 33
Limotai N, Go C, Oyama G, Hwynn N, Zesiewicz T, Foote K, et al. Mixed results for GPi-DBS in the treatment of cranio-facial and cranio-cervical dystonia symptoms. J Neurol 2011;258:2069-74.  Back to cited text no. 34
Tai CH, Wu RM, Liu HM, Tsai CW, Tseng SH. Meige syndrome relieved by bilateral pallidal stimulation with cycling mode: Case report. Neurosurgery 2011;69:E1333-E1337.  Back to cited text no. 35
Sobstyl M, Zaąbek M, Mossakowski Z, Zaczynński A. Pallidal deep brain stimulation in the treatment of Meige syndrome. Neurol Neurochir Pol 2014;48:196-9.  Back to cited text no. 36
Bae DW, Son BC, Kim JS. Globus pallidus internal deep brain stimulation in a patient with medically intractable Meige syndrome. J Mov Disord 2014;7:92-4.  Back to cited text no. 37
Wang X, Zhang C, Wang Y, Liu C, Zhao B, Zhang JG, et al. Deep brain stimulation for craniocervical dystonia (Meige syndrome): A report of four patients and a literature-based analysis of its treatment effects. Neuromodulation 2016;19:818-23.  Back to cited text no. 38
Foote KD, Sanchez JC, Okun MS. Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: Case report and physiology. Neurosurgery 2005;56:E415.  Back to cited text no. 39
Alterman RL, Tagliati M. Deep brain stimulation for torsion dystonia in children. Childs Nerv Syst 2007;23:1033-40.  Back to cited text no. 40
Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y, et al. Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord 2011;17:123-5.  Back to cited text no. 41
Hebb MO, Chiasson Lang PAE, Brownstone ERM, Mendez I. Sustained relief of dystonia following cessation of deep brain stimulation. Mov Disord 2007; 22:1958 -62.  Back to cited text no. 42
Baizabal-Carvallo JF, Jankovic J. Movement disorders induced by deep brain stimulation. Parkinsonism Relat Disord 2016;25:1-9.  Back to cited text no. 43
Moro E, Lang AE, Strafella AP, Poon YY, Arango PM, Dagher A, et al. Bilateral globus pallidus stimulation for Huntington's disease. Ann Neurol 2004;56:290-4.  Back to cited text no. 44
Diederich NJ, Kalteis K, Stamenkovic M, Pieri V, Alesch F. Efficient internal pallidal stimulation in Gilles de la Tourette syndrome: A case report. Mov Disord 2005; 20:1496-9.  Back to cited text no. 45


Print this article  Email this article
Online since 20th March '04
Published by Wolters Kluwer - Medknow