DBS of Internal Globus Pallidus Improves Dystonia, Quality of Life in X-Linked Dystonia Parkinsonism

A study showed that deep brain stimulation (DBS) of the internal globus pallidus (GPi) is associated with improved quality of life and is possibly effective for reducing symptoms of dystonia in X-linked dystonia parkinsonism (XDP). Findings from this study were published in JAMA Neurology.

A total of 16 Filipino patients with XDP (mean [SD] age, 40.9 [7.3]; disease duration, 1 to 6 years) who were treated in a single German center with bilateral GPi-DBS were enrolled. In follow-up of up to 46 months, patients were assessed using the motor parts of the Burke-Fahn-Marsden scale (BFMDRS-M) and the Unified Parkinson’s Disease Rating Scale (UPDRS-III). These assessments were used to examine postoperative improvements in dystonia and parkinsonism. In addition, the researchers performed T1-based basal ganglia volumetry and correlated findings with postoperative outcomes.

Significant improvements in dystonia were observed in masked video ratings at 1 week (−55%; range, −94% to 59%; P <.01) and 6 months (−59%; range, −100% to 22%; P <.001) following surgery. The researchers also observed noticeable improvements in the UDPRS-III score after GPi-DBS (−19%; range, −54% to 95%; and −27%; range, −70% to 124%; respectively). At up to 46-month unmasked follow-up, GPi-DBS continued to prove effective for improving dystonia, with significant reduction of the BFMDRS-M (F_5,45 = 27.3; P <.001) and an association with the UPDRS-III score (F_5,30 = 7.23; P =.015) observed during this time. In addition, the investigators observed improvements in activities of daily living, pain severity, weight, and quality of life. A predictor of a less beneficial outcome included caudate atrophy (r = 0.817; P =.004).

Limitations of the study were the inclusion of only male patients and the small number of participants in the final cohort.

“Because advanced neurodegeneration was associated with a poorer response,” the researchers wrote, “DBS should be considered during the early stages of the disease.”

Reference

Brüggemann N, Domingo A, Rasche D, et al. Association of pallidal neurostimulation and outcome predictors with X-linked dystonia parkinsonism [published online December 3, 2018]. JAMA Neurol. doi:10.1001/jamaneurol.2018.3777