Pallidal deep brain stimulation in patients with cranial–cervical dystonia (Meige syndrome)

Abstract

Idiopathic cranial-cervical dystonia (ICCD) is an adult-onset dystonia syndrome affecting orbicularis oculi, facial, oromandibular, and cervical musculature. ICCD is frequently difficult to treat medically. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a highly effective treatment for idiopathic generalized dystonia, however less is known about the effect of GPi DBS on ICCD. In this article, we present the results from a pilot study assessing the effect of GPi DBS in a series of patients with ICCD. Six patients underwent bilateral stereotactic implantation of DBS leads into the sensorimotor GPi. Patients were evaluated with the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) and Toronto western spamodic torticollis rating scale (TWSTRS) before surgery and 6 months postoperatively. At 6 months, patients showed a 72% mean improvement in the BFMDRS total movement score (P < 0.028, Wilcoxin signed rank test). The mean BFMDRS disability score showed a trend toward improvement (P < 0.06). The total TWSTRS score improved 54% (P < 0.043). Despite improvement in dystonia, mild worsening of motor function was reported in previously nondystonic body regions with stimulation in 4 patients. Although GPi DBS was effective in these patients, the influence of GPi DBS on nondystonic body regions deserves further investigation.

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.…”

“…14 However, bilateral stimulation is generally considered necessary for optimal treatment of disorders manifesting with midline symptoms, 9,25 and that unilateral stimulation may fail to alleviate the axial symptoms in patients with Meige syndrome. …”

“…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.…”

“…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.…”

Dystonia and DBS: The Jury Arrives

“…However, it has been suggested that lead migration and lead fracture is more common in dystonia than in parkinsonian patients (Yianni et al, 2003). Stimulation-related adverse effects specific for GPi DBS in dystonia include the development of reversible bradykinesia and parkinsonian gait problems in previously nondystonic body regions (Berman et al, 2009;Ostrem et al, 2007;Zauber et al, 2009). …”