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NI FEATURE: THE EDITORIAL DEBATE I-- PROS AND CONS
Year : 2018  |  Volume : 66  |  Issue : 1  |  Page : 38-39

Botulinum toxin in patients with Meige's syndrome


Department of Medicine and Neurology, Melbourne Medical School, The University of Melbourne and Western Health, Sunshine Hospital, St Albans, Victoria, Australia

Date of Web Publication11-Jan-2018

Correspondence Address:
Dr. Tissa Wijeratne
Department of Medicine and Neurology, Melbourne Medical School, The University of Melbourne and Western Health, Sunshine Hospital, 176 Furlong Rd, St Albans VIC 3021
Australia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.222831

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How to cite this article:
Vijiaratnam N, Wijeratne T. Botulinum toxin in patients with Meige's syndrome. Neurol India 2018;66:38-9

How to cite this URL:
Vijiaratnam N, Wijeratne T. Botulinum toxin in patients with Meige's syndrome. Neurol India [serial online] 2018 [cited 2019 Oct 18];66:38-9. Available from: http://www.neurologyindia.com/text.asp?2018/66/1/38/222831




We read with interest Pandey and Sharma's contribution in the current edition of this journal on the merits of botulinum toxin therapy in a group of patients with Meige's syndrome with supplementary video evidence.[1] This condition is of some fascination and has certainly evolved a great deal since Henri Meige's initial description of “spasm facial median”.[2] Simplistically put, we have come to define this disorder in current day practice as a segmental cranial dystonia with a descriptive emphasis on the body distribution.[3] The inference, however, is that of spread in the muscles involved, with variability in the onset region, manifesting either as blepharospasm or oromandibular dystonia, and rarely with involvement of other regions (spasmodic, lingual, pharyngeal, laryngeal and cervical dystonia).[4] While there are postulations for this evolution in patients presenting with isolated dystonia, no cohesive, all-encompassing explanation has been offered to date.

The evidence for the use of botulinum toxin for this group of disorders, either in isolation or as a combination, has been available for a number of decades and is not dissimilar to the efficacy of the toxin for any form of dystonia.[5] It is worthy to note though that although some previous studies of large patient groups report this efficacy, they attribute conclusions to patients suffering from Meige's syndrome by extrapolating responses obtained in cases of coexistent essential blepharospasm and oromandibular dystonia that have initially been treated before progression, rather than fulfillment of our current day definition of Meige's syndrome prior to treatment initiation. Nevertheless, in these large initial studies, responses for blepharospasm were highly reassuring with over three quarters of patients studied appreciating a significant clinical benefit. This response rate is, however, somewhat attenuated in dystonias debilitating the lower aspect of the face, and in particular cases of oromandibular dystonia, with only a third reporting a sizeable positive response.[6],[7] A smaller individual study examining the effect of the toxin on lower facial spasm, however, found a far more robust response.[8] Pandey and Sharma's current contribution of eight prospective cases of Meige's syndrome who had never been treated with botulinum toxin (BTX) adds significant clarity to this discussion in the sense that it examines patients who are given the diagnosis of Meige's syndrome prior to toxin administration, with their documentation using high quality video recordings and the use of the Burke-Fahn-Marsden dystonia rating (BFMDRS) scale. Their findings suggest that all the cranial dystonias described equally respond to toxin administration.[1] This aspect of response certainly warrants further consideration. It is likely that with current electromyographic (EMG) and imaging guidance techniques, lower face dystonia are better managed with more suitable doses of toxin administered. Although evidence for this view is lacking in the dystonia literature, some evidence from the cosmetics sphere of toxin use suggests that EMG findings are different in lower facial muscles and certainly does direct a need for higher doses of toxin to achieve the same degree of muscle impact as the upper face.[9] The beneficial response to toxin has certainly stood the test of time with follow up data over a four- year period in these groups of patients showing no waning of response.[10] Although, patients typically proceed to toxin upon failure of oral medication, there is some suggestion that a combination of these approaches may be of synergistic value in these cases.[11]

On balance, we feel there is sufficient evidence to recommend the use of botulinum toxin in patients presenting with the full constellation of features suggesting Meige's syndrome. While treatment principles should be tailored to treating the isolated dystonias observed, guided techniques to administration should probably be employed in particular during administration of toxin to lower facial aspects. This approach is likely to allay historic concerns of the lack of response rates in these regions. As our understanding as to why dystonia spreads to involve different cranial regions matures, we may come to appreciate why only a subset of patients develop these features. This understanding may enlighten a differential approach on how we institute treatment to patients with Meige's syndrome.

It would have been valuable to know the exact injection paradigm of this case series to add further clarity to the discussion. This case series helps us to reflect on the issue of whether patients with Meige's syndrome indeed constitute a different clinical phenotype when compared with patients with oromandibular dystonia alone or those with blepharospasm.



 
  References Top

1.
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. 1
    
2.
Meigem H. Les convulsions de la face: Une forme Clinique de convulsion faciale, bilateral et mediane. Rev. Neurol. (Paris) 1910;10:437-43.  Back to cited text no. 2
    
3.
Albanese A, Bhatia K, Bressman SB, Delong MR, Fahn S, Fung VS, et al. Phenomenology and classification of dystonia: A consensus update. Mov Disord 2013;28:863-73.  Back to cited text no. 3
    
4.
Casala B, Deuschl G. Craniocervical dystonia. Pragmatic general concept or nosological entity? Nervenarzt 1994;65:75-94.  Back to cited text no. 4
    
5.
Pandey S, Sharma S. Meige's syndrome: History, epidemiology, clinical features, pathogenesis and treatment. J Neurol Sciences 2017;372:162-70.  Back to cited text no. 5
    
6.
Maurri S, Brogelli S, Alfieri G, Barontini F. Use of botulinum toxin in Meige disease. Riv Neurol 1988; 58: 245-8.  Back to cited text no. 6
    
7.
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. 7
    
8.
Mauriello Jr JA., Coniaris H, Haupt EJ. Use of botulinum toxin in the treatment of one hundred patients with facial dyskinesias. Opthalmology 1987;94:976-9.  Back to cited text no. 8
    
9.
Winn BJ, Sires BS. Electromyographic differences between normal upper and lower facial muscles and the influence of onabotulinum toxin A. JAMA Facial Plast Surg 2013;15:211-7.  Back to cited text no. 9
    
10.
Tan EK, Jankovic J. Botulinum toxin in patients with oromandibular dystonia: Long-term follow-up. Neurology 1999;53:2102-7.  Back to cited text no. 10
    
11.
Mauriello Jr JA, Dhillon S, Leone T, Pakeman B, Mostafavi R, Yepez MC. Treatment selections of 239 patients with blepharospasm and Meige syndrome over 11 years. Br J Ophthalmol 1996; 80:1073-6.  Back to cited text no. 11
    




 

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