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NI FEATURE: THE EDITORIAL DEBATE I-- PROS AND CONS
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
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.222827

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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 2019 Jun 18];66:36-7. Available from: http://www.neurologyindia.com/text.asp?2018/66/1/36/222827




“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.



 
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