Meige's syndrome: A cranial dystonia treated with bilateral pallidal deep brain stimulation

Meige's syndrome is a rare form of segmental dystonia characterized by blepharospasm and oromandibular dystonia. Medical treatment including botulinum toxin injections usually present disappointing results. The experience on Deep Brain Stimulation (DBS) in the treatment of Meige's syndrome and other segmental dystonias is still limited. At the moment, only a few cases of pallidal DBS have been reported to improve this rare form of dystonia.

We report on a case of a woman with a 7-year history of Meige's syndrome, which rendered her functionally blind. The treatment with botulinum toxin injections failed to improve her symptoms, whereas stereotactic bilateral DBS of the pallidum led to a dramatic clinical improvement. Clinical assessment using the Burke-Fahn-Mardsen Dystonia Rating Scale (BFMDRS) in a double-blind manner, showed an improvement of 70% in the Movement score and 93.33% in the Disability score (84% reduction of the total score) on the 3 and 6 month follow-up.

Stereotactic pallidal DBS might be considered as a potential treatment in the management of Meige's syndrome.