The research, published in the journal Dystonia, followed 19 patients, 13 of them women, who received bilateral GPi DBS at the Florida center. The median age at surgery was 66 years, and the median follow-up stretched to six years, with some patients followed for as long as 24 years. Craniocervical dystonia encompasses blepharospasm, in which the muscles around the eyes contract involuntarily and forcefully; oromandibular dystonia, affecting the jaw, tongue, and lower face; and cervical dystonia, which distorts head posture. When these regions are affected together, as in the complex phenotype known as Meige syndrome, the functional consequences can be severe, touching vision, speech, swallowing, and social interaction, and frequently co-occurring with anxiety, depression, and reduced quality of life.

Botulinum toxin injections remain the first-line treatment for these conditions, relaxing overactive muscles at the site of the spasms. But for many patients the relief is incomplete, and oral medications such as benzodiazepines, baclofen, and trihexyphenidyl often provide only marginal benefit with troublesome side effects. DBS offers a fundamentally different approach: instead of targeting muscles, it modulates the abnormal neural circuitry in the brain itself. The GPi, a relay station in the basal ganglia motor network, has long been the preferred target for generalized and segmental dystonia, and its somatotopic organization means that the ventral, lateral, and posterior portions of the nucleus correspond to orofacial and cervical representations, precisely the regions involved in craniocervical dystonia.

In the study, the surgical team targeted the lateral portion of the sensorimotor posteroventral GPi, balancing the ability to deliver higher stimulation amplitudes against the risk of side effects from current spreading to the adjacent internal capsule. Surgeons used awake surgery with microelectrode recording to refine lead placement. The researchers then tracked outcomes at prespecified intervals: before surgery, at three to six months, at one to two years, at three to five years, and at the most recent follow-up visit. Severity was measured with the Unified Dystonia Rating Scale, or UDRS, which rates both the intensity and the proportion of time that dystonic movements or postures are present across 14 body regions, yielding a maximum total score of 112.

The motor results were striking. Median UDRS scores improved by 34.5 percent at three to six months, 51.0 percent at one to two years, 33.3 percent at three to five years, and 43.4 percent at the most recent follow-up, calculated as the median of individual percentage improvements. Every one of these comparisons reached statistical significance after correction for multiple testing. The trajectory was not a simple straight line, but the overall message was clear: the benefits of pallidal stimulation were durable, and in many patients they continued to deepen over time rather than fading, as can happen with some implanted therapies.

Perhaps the most intriguing finding came from a statistical model examining the relationship between therapy duration and symptom severity. Each additional month of DBS was associated with a reduction of 0.10 points on the UDRS, an association that remained robust after adjustment for sex, disease duration, age at onset, and the number of body areas affected, and that survived sensitivity analyses. The researchers also found that a greater number of affected body regions and a longer duration of dystonia before surgery predicted higher, meaning worse, UDRS scores. That latter result echoes a growing consensus in the field: shorter disease duration before surgical referral is associated with superior long-term motor improvement, an argument for referring appropriate patients to DBS earlier rather than after years of refractory suffering.

Body-region analysis added further texture. The researchers defined meaningful improvement for each region using thresholds equivalent to at least a 25 percent reduction: a two-point drop for the eyes and upper face, a two-point drop for the neck, and a six-point drop for the lower face, jaw, tongue, and larynx. By these criteria, the eyes responded best. At the longest follow-up window, spanning six to nine years after surgery, 83.3 percent of the remaining patients achieved meaningful improvement in the eye region, 50 percent in the lower face, and 50 percent in the neck. Overall, the proportion of patients classified as DBS responders, defined by at least a 25 to 30 percent improvement in UDRS scores, rose from roughly 31 to 44 percent early after surgery to between 50 and 83 percent at the six-to-nine-year mark, depending on the region examined.

The non-motor and quality-of-life findings were more muted, but not without signal. Using the Short Form Health Survey-36, the researchers found no statistically significant changes in the overall physical or mental component summaries, likely a consequence of the modest sample size and the generic, multidomain nature of the instrument, which dilutes changes driven by a segmental movement disorder, particularly as patients age and accumulate other health problems. Yet social functioning showed a directional trend toward improvement, and in longitudinal regression models, longer DBS duration was associated with significant gains in the social functioning and role-physical domains even after correction for multiple comparisons. Scores on the Beck Depression Inventory, Beck Anxiety Inventory, and Apathy Scale remained broadly stable, suggesting that stimulation did not worsen psychiatric symptoms over the long term.

The study also documented a meaningful shift in the pharmacologic burden of care. Before surgery, nearly 79 percent of patients were taking oral medications for dystonia, including 58 percent on benzodiazepines; at the most recent follow-up, that figure had fallen to about 47 percent, and the proportion of patients requiring no oral medications at all roughly doubled, from 21 to 42 percent. Use of botulinum toxin also declined modestly, from about 74 percent of patients before surgery to 58 percent afterward. For a population in which long-term polypharmacy carries cumulative risks, this lightening of the medication load is itself a clinically relevant outcome, independent of the motor scores.

The findings align closely with a growing body of work from Asian centers, where Meige syndrome cohorts have reported 50 to 70 percent improvements in dystonia severity following pallidal stimulation, and with recent comparative trials suggesting that stimulation of the subthalamic nucleus, an alternative target, produces broadly comparable results. What this study adds is a long-term, real-world perspective from a United States center, with a phenotype that includes substantial neck involvement, unlike some comparative cohorts in which cervical symptoms were nearly absent. The authors are candid about the limitations: the retrospective design, the small and attrition-prone sample, the lack of disease-specific quality-of-life metrics, and the possibility that patients who do well are more likely to remain in follow-up. Even so, the picture that emerges is one of a therapy whose benefits are not merely sustained but potentially progressive, a pattern the researchers suggest may reflect underlying neuroplastic changes in the motor circuitry. For patients and clinicians weighing DBS for craniocervical dystonia, the message is one of realistic optimism: meaningful relief may take time to reach its full effect, but the evidence now suggests it is worth the wait.

Subject of Research: Long-term motor and non-motor outcomes of pallidal deep brain stimulation in craniocervical dystonia

Article Title: Beyond the neck: long-term motor and non-motor outcomes of pallidal DBS in craniocervical dystonia

Article References: Remz, M., Zeilman, P., Senthil, S., Kumar, R., Gerzenshtein, I., Kamo, H., & Wagle Shukla, A. (2026). Beyond the neck: long-term motor and non-motor outcomes of pallidal DBS in craniocervical dystonia. Dystonia, 5, Article 16756. https://doi.org/10.3389/dyst.2026.16756

Image Credits: AI Generated

DOI: 10.3389/dyst.2026.16756

Keywords: craniocervical dystonia, deep brain stimulation, globus pallidus internus, blepharospasm, Meige syndrome, oromandibular dystonia, Unified Dystonia Rating Scale, quality of life, botulinum toxin, neuroplasticity, movement disorders, long-term outcomes