The aim of this study was to provide an objective assessment of deep brain stimulation (DBS) for groups of patients with mixed secondary dystonia and primary torticollis syndromes by a blinded evaluation of 13 consecutive patients who underwent ineffective medical treatment and botulinum toxin injections.
Nine patients with secondary dystonia and 4 with cranial dystonia involving prominent spasmodic torticollis were selected for a DBS implant after they underwent unsuccessful medical treatment. Preoperative videos and neurological assessments were obtained and the DBS implant was inserted into the globus pallidus internus. Postoperatively, DBS parameters were adjusted to provide optimal benefit. Postoperative videotapes and quality of life scores were obtained. Blinded randomized evaluation of videotapes was performed by a neurologist specializing in movement disorders. Videos were scored using the Unified Dystonia Rating Scale, Toronto Western Spasmodic Torticollis Rating Scale, Burke-Fahn-Marsden Dystonia Rating Scale, or Abnormal Involuntary Movement Scale. Quality of life scoring was assessed using a standardized 7-point Global Rating Scale.
All 13 patients completed preoperative videotaping, medical assessment, and surgery. Optimal DBS programming was completed in 6.5 visits over 5.9 months. Seven patients reported marked improvement, 3 reported moderate improvement, 2 reported slight improvement or no change, and 1 was lost to follow-up. Examiner scores on the Global Rating Scale reflected patient self-reported scores.
Global subjective gains and notable objective improvement were observed in 11 of 13 patients. Although the benefits were variable and not fully predictable, they were of sufficient magnitude to justify offering the procedure when medications and botulinum toxin injections have failed.
Abbreviations used in this paper: AIMS = Abnormal Involuntary Movement Scale; BFMDRS = Burke-Fahn-Marsden Dystonia Rating Scale; DBS = deep brain stimulation; GPI = globus pallidus internus; GRS = Global Rating Scale; PD = Parkinson disease; QOL = quality of life; TWSTRS = Toronto Western Spasmodic Torticollis Rating Scale; UDRS = Unified Dystonia Rating Scale.
Address correspondence to: Theresa E. Pretto, R.N., Neurosurgery Nurse Associate, University of Chicago, Section of Neurosurgery, 5841 South Maryland, MC 3026, Chicago, Illinois 60637. email:
BereznaiBSteudeUSeelosKBotzelK: 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. Mov Disord17:138–1442002
EltahawyHASaint-CyrJGiladiNLangAELozanoAM: Primary dystonia is more responsive than secondary dystonia to pallidal interventions: outcome after pallidotomy or pallidal deep brain stimulation. Neurosurgery54:613–6212004
KraussJKLoherTJPohleTWeberSTaubEBärlocherCB: Pallidal deep brain stimulation in patients with cervical dystonia and severe cervical dyskinesias with cervical myelopathy. J Neurol Neurosurg Psychiatry72:249–2562002
KraussJKLoherTJWeigelRCapelleHWeberSBergunderJM: Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg98:785–7922003
StarrPATurnerRSRauFLindseyNHeathSVolzM: Microelectrode-guided implantation of deep brain stimulators into the globus pallidus for dystonia: techniques, electrode locations, and outcomes. J Neurosurg104:488– 5012006