title: Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation.
abstract: Deep brain stimulation of the globus pallidus internus (GPi DBS) is effective in the treatment of primary segmental and generalized dystonia. Although limb, neck, or truncal dystonia are markedly improved, orofacial dystonia is ameliorated to a lesser extent. Nevertheless, several case reports and small cohort studies have described favorable short-term results of GPi DBS in patients with severe Meige syndrome. Here, we extend this preliminary experience by reporting long-term outcome in a multicenter case series, following 12 patients (6 women, 6 men) with Meige syndrome for up to 78 months after bilateral GPi DBS. We retrospectively assessed dystonia severity based on preoperative and postoperative video documentation. Mean age of patients at surgery was 64.5 ± 4.4 years, and mean disease duration 8.3 ± 4.4 years. Dystonia severity as assessed by the Burke-Fahn-Marsden Dystonia Rating Scale showed a mean improvement of 45% at short-term follow-up (4.4 ± 1.5 months; P < 0.001) and of 53% at long-term follow-up (38.8 ± 21.7 months; P < 0.001). Subscores for eyes were improved by 38% (P = 0.004) and 47% (P < 0.001), for mouth by 50% (P < 0.001) and 56% (P < 0.001), and for speech/swallowing by 44% (P = 0.058) and 64% (P = 0.004). Mean improvements were 25% (P = 0.006) and 38% (P < 0.001) on the Blepharospasm Movement Scale and 44% (P < 0.001) and 49% (P < 0.001) on the Abnormal Involuntary Movement Scale. This series, which is the first to demonstrate a long-term follow-up in a large number of patients, shows that GPi DBS is a safe and highly effective therapy for Meige syndrome. The benefit is preserved for up to 6 years.
authors.affiliation: Department of Neurosurgery, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625 Hannover, Germany.
authors.forename: René
authors.initials: R
authors.lastname: Reese
authors.forename: Doreen
authors.initials: D
authors.lastname: Gruber
authors.forename: Thomas
authors.initials: T
authors.lastname: Schoenecker
authors.forename: Hansjörg
authors.initials: H
authors.lastname: Bäzner
authors.forename: Christian
authors.initials: C
authors.lastname: Blahak
authors.forename: H Holger
authors.initials: HH
authors.lastname: Capelle
authors.forename: Daniela
authors.initials: D
authors.lastname: Falk
authors.forename: Jan
authors.initials: J
authors.lastname: Herzog
authors.forename: Marcus O
authors.initials: MO
authors.lastname: Pinsker
authors.forename: Gerd H
authors.initials: GH
authors.lastname: Schneider
authors.forename: Christoph
authors.initials: C
authors.lastname: Schrader
authors.forename: Günther
authors.initials: G
authors.lastname: Deuschl
authors.forename: Hubertus M
authors.initials: HM
authors.lastname: Mehdorn
authors.forename: Andreas
authors.initials: A
authors.lastname: Kupsch
authors.forename: Jens
authors.initials: J
authors.lastname: Volkmann
authors.forename: Joachim K
authors.initials: JK
authors.lastname: Krauss
mesh_terms: D000368:Aged; D000704:Analysis of Variance; D046690:Deep Brain Stimulation / Q000379:methods*; D004566:Electrodes; D005260:Female; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008538:Meige Syndrome / Q000503:physiopathology / Q000628:therapy*; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology; D012720:Severity of Illness Index; D016896:Treatment Outcome