Chronic high‐frequency globus pallidus internus stimulation in different types of dystonia: A clinical, video, and MRI report of six patients presenting with segmental, cervical, and generalized dystonia

Abstract

The results of deep brain stimulation (DBS) of the globus pallidus internus (Gpi) in six patients with generalized, focal, and segmental dystonia are presented. Pre- and postoperative assessments are given for one patient with generalized inherited dystonia and for five patients with idiopathic segmental or cervical dystonia. Clinical symptoms were evaluated before and 3-12 months after surgery using the Burke-Fahn-Marsden (BFM) dystonia rating scale for primary torsion dystonia and the Tsui scale for cervical dystonia. The Short-Form Health Survey (SF-36) was completed by each patient to document preoperative and postoperative health status. Also, neurological status was documented by video before and during chronic stimulation. Magnetic resonance imaging studies were performed to show the anatomical localization of the electrode leads. Five patients showed a progressive improvement within 7 days. One patient with cervical dystonia and Meige's syndrome showed no improvement for 3 months, but beneficial effects were observed after 12 months. On average, the BFM movement scale scores decreased by 72.5% and Tsui scale scores by 63%. SF-36 showed an improvement in health status by an average of 36% according to eight different health categories. We conclude that chronic high-frequency Gpi stimulation in different types of dystonia is a very effective and safe treatment.

Long-term follow-up of deep brain stimulation for Meige syndrome

“…Since Vercueil and coworkers 3 first reported their results of bilateral GPi DBS in a patient with Meige syndrome, several studies have shown that segmental dystonia responds to GPi DBS. 2,3,6,9,11,13,[18][19][20][21][22][23][24][25][29][30][31] The reports on Meige syndrome and its response to DBS are primarily single cases, aside from the excellent report by Ostrem and colleagues 25 ( Table 3). The follow-up time frame has been somewhat limited in most reports, except the cases of Loher et al 21 at 7 years, Hebb et al 11 at 5 years, and Capelle and associates 6 at 2 years.…”

“…15 Meige syndrome has become the most common eponym current used in the literature. 2,3,6,9,11,13,21,23,24,27,30 Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The underlying cause of Meige syndrome is unknown, but most investigators consider it a variant of idiopathic torsion dystonia.…”

“…Botulinum toxin has also been shown to be effective for blepharospasm, as well as for facial, cervical, and oromandibular dystonia; however, some patients may have diminished response with time and some may develop antibodies that make them resistant to continued therapy. 2,9,16,17,25,27 The advent of DBS for neuromodulatory treatment of a wide variety of movement and other disorders has prompted increased interest in applications to severe forms of medically refractory dystonias. Deep brain stimulation for dystonia, targeting the GPi, has been shown to be an effective treatment for primary generalized dystonia.…”

“…18,19,25,29,31 There have been several recent reports on the efficacy of GPi DBS in selected patients with Meige syndrome. 2,3,6,9,11,12,[21][22][23][24][25]30 We report on our experience in using DBS in 3 cases of Meige syndrome and 1 case of Parkinson disease and associated craniofacial dystonia, with long-term follow-up, and we review the current literature. Object.…”

Early Globus Pallidus Internus Stimulation in Pediatric Patients With Generalized Primary Dystonia: Long-Term Efficacy and Safety

“…Over the past years, the globus pallidus internus has been identified as the most promising target in different types of dystonia, and several study groups have presented convincing retrospective (long-term) data using the globus pallidus internus as a target for deep brain stimulation. [7][8][9][10][11][12][13][14] The efficacy of this approach was further underscored by the data from the prospective controlled studies by Vidailhet et al 15,16 along with the results of the prospective randomized controlled 2-armed study by Kupsch et al 17 The evidence of the effectiveness of globus pallidus internus deep brain stimulation in dystonia was thus taken to a very high level. However, these studies did not focus on early treatment in children and the associated special implications and possible restrictions: there are only sparse data available on (mainly) short-term outcome derived from some series or case reports focusing on pediatric patients 8,18,19 or children being part of a predominantly adult cohort.…”