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CASE REPORT
Year : 2016  |  Volume : 25  |  Issue : 2  |  Page : 232-233  Table of Contents     

Meige's syndrome


Department of Psychiatry, University College of Medical Sciences and GTB Hospital, New Delhi, India

Date of Web Publication13-Jun-2017

Correspondence Address:
Manjeet Singh Bhatia
D-1, Naraina Vihar, New Delhi - 110 028
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-6748.207853

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   Abstract 


Meige's syndrome consists of idiopathic blepharospasm and oromandibular dystonia. The exact etiology is not known and various hypotheses have been proposed for its causation. The hypothesis suggesting dopaminergic and cholinergic hyperactivity is most widely accepted. There is no curative drug for Meige's syndrome although a variety of treatments have been proposed. We report a case which responded to tetrabenazine.

Keywords: Etiology, Meige's syndrome, oromandibular dystonia, tetrabenazine, treatment


How to cite this article:
Gautam P, Bhatia MS, Kaur J, Rathi A. Meige's syndrome. Ind Psychiatry J 2016;25:232-3

How to cite this URL:
Gautam P, Bhatia MS, Kaur J, Rathi A. Meige's syndrome. Ind Psychiatry J [serial online] 2016 [cited 2019 Sep 21];25:232-3. Available from: http://www.industrialpsychiatry.org/text.asp?2016/25/2/232/207853



Meige's syndrome consisting of idiopathic blepharospasm and oromandibular dystonia was first described by Meige in 1910.[1]

Meige's syndrome usually begins between ages 30 and 70 years and it is twice more common in women than in men. Symptoms usually comprise difficulty in opening mouth, grinding of teeth, deviation of jaw, and lip tightening. There may also be blepharospasm, increased rate of blinking, uncontrollable squinting, and photophobia. The muscles commonly involved are masseter, temporalis, and platysma. The symptoms of Meige's syndrome can be increased by talking, chewing, biting and relieved by chewing gum, pressure over chin, and during sleep.[2]

The exact etiology is not known and various hypotheses have been proposed for its causation. Besides idiopathic Meige's syndrome, hypothesis suggesting dopaminergic and cholinergic hyperactivity is most widely accepted. Defect in the region of basal ganglia has also been implicated in the etiology.[3]

Acute and tardive Meige's syndrome has been seen to occur with typical and newer atypical antipsychotics, showing prompt improvement with antipsychotic withdrawal.[4] The central dopaminergic preponderance, as possible biochemical basis of Meige's syndrome, has been suggested in various pharmacological studies and further supported by observation that it improves with dopamine depleting agent, tetrabenazine.[5]

There is no curative treatment for Meige's syndrome. Botulinum injections in the facial musculature are considered the most effective treatment till date.[6] Electroconvulsive therapy and deep brain stimulation of globus pallidus internus are other treatment modalities which have been tried in Meige's syndrome with success.[7],[8]

Most of the patients are treated with oral drugs only and there are varieties of drugs which are used frequently in the treatment of Meige's syndrome. Anticholinergics,[9] tetrabenazine (monoamine storage inhibitor),[5] benzodiazepines,[9] and baclofen [10] have been used successfully. The atypical antipsychotics represent a new class of drugs used for the treatment of Meige's syndrome.


   Case Report Top


A 55-year-old male presented to the department of psychiatry with complaints of inability to speak, slurring of speech, and irresistible desire to blink his eyes from the last 1 ½ years. These complaints started 2 months after an accidental pouring of some ayurvedic medication in eye, which was prescribed for knee pain. Initially, there was increased blinking of eyes. It further increased in frequency over time and became very stressful and embarrassing for the patient. He gradually developed difficulty in speaking and his speech became slurred. There was also difficulty in chewing food. The symptoms got precipitated on emotional distress and in public and decreased when he was engaged or sleeping. He had no significant past or family history of any major medical and psychiatric illness and premorbid personality was well adjusted.

On examination, the patient was well oriented in time, place, and person and no abnormality was detected on general physical, systemic, and neurological examination. Mental status examination showed anxious affect and preoccupation with the illness. Higher mental functions were within normal limits. His ophthalmological examination was also within normal limits.

The complete blood count, liver and kidney function tests, blood sugar, serum electrolytes, and thyroid function tests were normal. Magnetic resonance imaging of the brain did not show any abnormality.

The patient was started on trihexyphenidyl 6 mg in divided doses, but he did not show any improvement. After few months, he was put on tetrabenazine 25 mg which was gradually increased to 75 mg. There was marked improvement in his symptoms and he started talking normally and his blinking rate got reduced.


   Discussion Top


This is a case of Meige's syndrome as the patient presented with oromandibular dystonia and blepharospasm, which gradually worsened and produced difficulty in speaking and chewing food. There was also increased rate of blinking. These patients can present with varied picture and can be easily misdiagnosed as conversion disorder.

Exact cause of this disorder is not known. The central dopaminergic preponderance is widely accepted hypothesis [5] though the role of striatal glutamate underactivity has also been suggested in some studies.

The present case did not show any response to anticholinergics (trihexyphenidyl) but improved with tetrabenazine, an antidopaminergic drug. It further supports the dopaminergic hyperactivity as the biochemical basis of Meige's syndrome.

Meige's syndrome is a combination of blepharospasm and oromandibular dystonia. Diagnosis is mainly clinical and requires a high index of suspicion to avoid misdiagnosis. No exact cause is known and striatal dopaminergic preponderance is the most widely supported hypothesis. Unfortunately, there is no cure but relief occurs with injection botulinum and different classes of drugs such as anticholinergics, baclofen, benzodiazepines, and tetrabenazine.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest



 
   References Top

1.
Meige H. Les convulsion de la face, une forme clinique de convulsion faciale, bilateral et mediane. Rev Neurol (Paris) 1910;10:437-43.  Back to cited text no. 1
    
2.
Akiyama K. Algorithms for neuroleptic-associated tardive movement disorders. Psychiatry Clin Neurosci 1999;53Suppl:S23-9.  Back to cited text no. 2
    
3.
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 Thai 1997;80:1-8.  Back to cited text no. 3
[PUBMED]    
4.
Ananth J, Edelmuth E, Dargan B. Meige's syndrome associated with neuroleptic treatment. Am J Psychiatry 1988;145:513-5.  Back to cited text no. 4
[PUBMED]    
5.
Chen JJ, Ondo WG, Dashtipour K, Swope DM. Tetrabenazine for the treatment of hyperkinetic movement disorders: A review of the literature. Clin Ther 2012;34:1487-504.  Back to cited text no. 5
    
6.
Czyz CN, Burns JA, Petrie TP, Watkins JR, Cahill KV, Foster JA. Long-term botulinum toxin treatment of benign essential blepharospasm, hemifacial spasm, and Meige syndrome. Am J Ophthalmol 2013;156:173-77.e2.  Back to cited text no. 6
    
7.
Boshes RA, Afonso JA, Tanev K. Treatment of Meige's syndrome with ECT. J ECT 1999;15:154-7.  Back to cited text no. 7
    
8.
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. 8
    
9.
Hípola D, Mateo D, Giménez-Roldán S. Meige's syndrome: Acute and chronic responses to clonazepan and anticholinergics. Eur Neurol 1984;23:474-8.  Back to cited text no. 9
    
10.
Ikawa M, Yoneda M, Nakagawa H, Kuriyama M. Case of oromandibular dystonia presenting with severe impairment of mouth-opening, and a marked effect by administration of baclofen. Rinsho Shinkeigaku 2006;46:661-3.  Back to cited text no. 10
    




 

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