Bilateral Pallidal Deep Brain Stimulation Controls Dystonia in X-Linked Dystonia-Parkinsonism

Dystonia diagnosis
Dystonia diagnosis
Investigators examine the long-term outcomes of pallidal deep brain stimulation in x-linked dystonia-parkinsonism.

Bilateral pallidal deep brain stimulation (DBS) can effectively control dystonia and may control parkinsonism for patients with X-linked dystonia-parkinsonism, according to results published in Parkinsonism and Related Disorders.

The study included participants with X-linked dystonia-parkinsonism who underwent bilateral pallidal DBS from October 2009 through September 2018. The researchers reviewed the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Unified Parkinson’s Disease Rating Scale (UPDRS)-III from baseline to the longest follow-up, along with demographic and clinical data.

The 11 participants had a mean age of 39±9.2 years with a mean disease duration of 3 years (range, 1-9 years). Mean baseline BFMDRS scores were 36.3±12.1, and mean UPDRS-III was 24.04±8.74.

At 1 month after DBS, all participants showed an immediate response for dystonia, with a mean BFMDRS score of 23.3±12.12 and a mean UPDRS-III score of 20±10.39.

At 12 months (n=10), the mean BFMDRS score was 13.7±10.63, and the mean UPDRS-III score was 19±13.19.

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At their last follow-up, participants showed improvement in the clinical and functional stages, with the majority of participants in stage 1 or 2 (n=3 and 5, respectively).

“The long-term benefit beyond 12 months suggested benefit for controlling dystonia as seen in cases with longest follow-ups but parkinsonism seemed to progress despite this treatment,” the researchers wrote.


Abejero JEE, Jamora RDG, Vesagas TS, et al. Long-term outcomes of pallidal deep brain stimulation in X-linked dystonia parkinsonism (XDP): up to 84 months follow-up and review of literature [published online September 21, 2018]. Parkinsonism Relat Disord. doi: 10.1016/j.parkreldis.2018.09.022