Meige syndrome (MS) is an idiopathic adult-onset segmental dystonia characterized initially by blepharospasm, followed by dystonia of the mid-facial and lower facial muscles, mouth, jaw, tongue, or pharyngeal muscles. Patients… Click to show full abstract
Meige syndrome (MS) is an idiopathic adult-onset segmental dystonia characterized initially by blepharospasm, followed by dystonia of the mid-facial and lower facial muscles, mouth, jaw, tongue, or pharyngeal muscles. Patients with MS may have further spread of dystonia to the cervical muscles, leading to craniocervical dystonia (CD). Severe Meige or CD is often refractory to oral medications. Botulinum toxin (BTX) injections remain the cornerstone of the management of MS or CD, although many patients experience a diminished response over time attributed to either progression of the disease or development of neutralizing antibodies. We and other groups have reported the effectiveness of deep brain stimulation (DBS) of either the globus pallidus internus or subthalamic nucleus for medically refractory MS or CD. We report on a patient with medically refractory CD who underwent bilateral asleep DBS surgery of the ventralis intermedius nucleus (VIM) with dramatic response.
               
Click one of the above tabs to view related content.