Objective: To report on deep brain stimulation (DBS) outcomes in dystonia and related movement disorders at Mass General Brigham (MGB) hospitals.

Background: Dystonia is a movement disorder involving repetitive, twisting, involuntary muscle contractions and abnormal postures that can affect any part of the body. Although medication and botulinum toxin are first-line therapies, DBS is an important treatment option for dystonia that fails to respond or is too widespread for chemodenervation. Positive predictors of response to DBS include younger age at surgery and shorter disease duration [1, 2]. Additionally, previous studies have characterized the effectiveness of DBS through the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), showing relief up to one year post surgery [3, 4]. Here we report changes of the BFMDRS for 61 patients from pre to post DBS at a single non-profit hospital network.

Method: Subjects were included if they were diagnosed with dystonia and received DBS surgery at MGB. Movement disorder physicians reviewed medical records and imaging both pre and post surgery. Independent variables included sex, genotype, age of onset, disease duration, age at surgery, DBS lead placement and volume of tissue activated. Outcomes measured included medication use, device complications, length of follow-up, and symptom relief as assessed by the BFMDRS. By reporting from a single healthcare network with a small number of DBS programmers, variability in DBS programming and scoring was reduced. Statistical analyses performed include descriptive statistics and a generalized linear regression model.

Results: The cohort consisted of 61 subjects (26 female, 35 male), and included 11 DYT1, 7 KMT2B, several other genotypes, and 33 idiopathic or secondary dystonias. The mean follow-up period was 5.2 years (range 0.22 – 13.93). At one-year, there was an improvement of 62% in BFMDRS across the entire cohort (p < 10e-8). At last follow-up the improvement was 49% relative to the pre-DBS baseline (p < 10e-8).  Out of 61 cases, 10 device complications occurred.

Conclusion: Deep brain stimulation is an effective treatment for idiopathic and genetic generalized dystonia, with sustained benefit beyond 5 years.

References: 1. Valldeoriola, F., Regidor, I., Mínguez-Castellanos, A., Lezcano, E., García-Ruiz, P., Rojo, A., Salvador, A., Castro, A., Grandas, F., Kulisevsky, J., Martí, M. J., Martínez-Martín, P., Relova, L., Rumià, J., Cámara, A., Burguera, J. A., Linazasoro, G., de Val, J. L., Obeso, J., Rodríguez-Oroz, M. C., … Grupo ESpañol para el EStudio de la EStimulación PALidal en la DIStonía (2010). Efficacy and safety of pallidal stimulation in primary dystonia: results of the Spanish multicentric study. Journal of neurology, neurosurgery, and psychiatry, 81(1), 65–69. https://doi.org/10.1136/jnnp.2009.174342 2. Isaias, I. U., Volkmann, J., Kupsch, A., Burgunder, J. M., Ostrem, J. L., Alterman, R. L., Mehdorn, H. M., Schönecker, T., Krauss, J. K., Starr, P., Reese, R., Kühn, A. A., Schüpbach, W. M., & Tagliati, M. (2011). Factors predicting protracted improvement after pallidal DBS for primary dystonia: the role of age and disease duration. Journal of neurology, 258(8), 1469–1476. https://doi.org/10.1007/s00415-011-5961-9 3. Vidailhet, M., Vercueil, L., Houeto, J. L., Krystkowiak, P., Benabid, A. L., Cornu, P., Lagrange, C., Tézenas du Montcel, S., Dormont, D., Grand, S., Blond, S., Detante, O., Pillon, B., Ardouin, C., Agid, Y., Destée, A., Pollak, P., & French Stimulation du Pallidum Interne dans la Dystonie (SPIDY) Study Group (2005). Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. The New England journal of medicine, 352(5), 459–467. https://doi.org/10.1056/NEJMoa042187 4. Kupsch, A., Benecke, R., Müller, J., Trottenberg, T., Schneider, G. H., Poewe, W., Eisner, W., Wolters, A., Müller, J. U., Deuschl, G., Pinsker, M. O., Skogseid, I. M., Roeste, G. K., Vollmer-Haase, J., Brentrup, A., Krause, M., Tronnier, V., Schnitzler, A., Voges, J., Nikkhah, G., … Deep-Brain Stimulation for Dystonia Study Group (2006). Pallidal deep-brain stimulation in primary generalized or segmental dystonia. The New England journal of medicine, 355(19), 1978–1990. https://doi.org/10.1056/NEJMoa063618

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MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-for-dystonia-a-single-center-experience/