A prospective blinded evaluation of deep brain stimulation for the treatment of secondary dystonia and primary torticollis syndromes

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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.

Article Information

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: tpretto@surgery.bsd.uchicago.edu.

© AANS, except where prohibited by US copyright law.



  • View in gallery

    Graphs showing the preoperative and postoperative blinded video rating scores of the TWSTRS, UDRS, and BFMDRS (BFM). pt. = patient.

  • View in gallery

    Bar graph of the GRS scores as rated by the patients and the examiner.


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