Current status of dystonias including Meige's syndrome

摘要

“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, causingfunctional 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. Allof them are associated with significant side effects.[2],[3],[4],[5],[6],[7] Pharmacotherapy provides better control of symptoms in blepharospasmas compared to Meige's syndrome. Of all the mentioned drugs, benzodiazepines are better tolerated, and have shown a good response in craniocervicaldystonia.[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.