ABSTRACT
OBJECT: Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The medical treatment of this condition is largely unsuccessful over time and is a major source of decreased quality of life in those patients suffering from this disease. Recent advances in the application of deep brain stimulation (DBS) surgery techniques for many disorders have prompted several recent reports of DBS for medically refractory cases of Meige syndrome. While the etiology for this disorder is unknown, it is considered by many investigators to be a form of idiopathic torsion dystonia. Pallidal stimulation is widely considered to be effective for dystonia. METHODS: The authors report the long-term results of bilateral globus pallidus internus (GPi) or subthalamic nucleus (STN) stimulation in 3 patients with Meige syndrome and 1 patient with Parkinson disease and associated craniofacial dystonia treated at their center. RESULTS: Initial 12-month and long-term follow-up Burke-Fahn-Marsden scores were substantially improved in all 4 patients compared with preoperative scores. CONCLUSIONS: Bilateral GPi DBS may be an effective and safe treatment for medically refractory Meige syndrome. The results are comparable with those reported in the literature. Sustained and long-term improvement in symptoms does appear to be reproducible across reports. The authors' patient with Parkinson disease and associated craniofacial dystonia syndrome undergoing bilateral STN DBS noted immediate and sustained improvement in his symptoms. Further study is required, but these results, along with the other reports, suggest that bilateral GPi DBS is an effective treatment for medically refractory Meige syndrome.
Subject(s)
Deep Brain Stimulation/methods , Aged , Female , Follow-Up Studies , Functional Laterality/physiology , Globus Pallidus/physiology , Humans , Longitudinal Studies , Male , Meige Syndrome/therapy , Middle Aged , Parkinson Disease/therapy , Subthalamic Nucleus/physiology , Treatment OutcomeABSTRACT
"Lubag" or X-linked dystonia-parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45-year-old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw-opening dystonia. At 1-year follow-up, his Burke-Fahn-Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms.
Subject(s)
Electric Stimulation Therapy , Genetic Diseases, X-Linked/therapy , Globus Pallidus/physiopathology , Parkinson Disease/genetics , Parkinson Disease/therapy , Genetic Diseases, X-Linked/pathology , Globus Pallidus/radiation effects , Humans , Male , Middle Aged , Parkinson Disease/pathologyABSTRACT
We report on 6 advanced Parkinson's disease (PD) patients who underwent bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) surgery whose restless legs syndrome (RLS) improved postoperatively. Despite a mean 56% decrease in their levodopa equivalents postoperatively, their RLS scores dropped by a mean of 84% (100% in three). Our findings suggest that bilateral STN DBS surgery can improve RLS in patients with advanced PD.