Deep brain stimulation for people with involuntary posturing, or dystonia

The review question

We reviewed the evidence about the effect of deep brain stimulation (DBS) for adults with dystonia. We assessed the efficacy, safety, and tolerability of this procedure.


Dystonia is a disease that causes undesired, uncontrollable, often painful, abnormal movement of an affected limb or body region. It is a relatively uncommon condition, which can be very disabling and negatively affect a person's quality of life. In most cases, the cause is unknown; no cure exists. Dystonia is normally a long-term disease that requires long-term treatment.

Deep brain stimulation (DBS) involves a surgical procedure to place electrical stimulators in the brain. Afterwards, the stimulators are connected to a battery, and deliver electrical impulses to the brain over time. For people with dystonia, DBS is usually considered to be a therapeutic option for severe cases only, once other treatments have failed.

Study characteristics

We conducted a literature search on 29 May 2018 for studies that compared DBS with sham stimulation (same surgical procedure, but no electrical impulses are delivered through the electrodes placed in the brain), best medical therapy, and placebo (a pretend medicine). We found two studies that compared DBS with sham stimulation, and included a total of 102 participants. One study included participants with dystonia of the limbs and trunk, and the other with dystonia of the neck. Participants received active DBS for a total of six months. The average age of people in the studies was 50 years; the average duration of the disease was 16 years. Both studies were funded by a DBS device manufacturer with possible interests in the results of the studies.

Key results

For limb and trunk dystonia, DBS may improve symptoms, self-assessed clinical status, and functioning. The results showed that for neck dystonia, DBS may improve symptoms, clinical status, functioning, and mood. For either type of dystonia, we are uncertain about the impact that DBS has on harmful or undesired events, or treatment tolerability.

Quality of the evidence

The overall quality of the evidence for neck, limb, and trunk dystonia was low to very low. Further research is needed to draw conclusions about the clinical efficacy, safety, and tolerability of DBS in people with dystonia, especially beyond the three- to six-month duration of the included studies.

Authors' conclusions: 

DBS of the internal globus pallidus nucleus may reduce symptom severity and improve functional capacity in adults with cervical, segmental or generalised moderate to severe dystonia (low-quality evidence), and may improve quality of life in adults with generalised or segmental dystonia (low-quality evidence). We are uncertain whether the procedure improves quality of life in cervical dystonia (very low-quality evidence). We are also uncertain about the safety and tolerability of the procedure in adults with either cervical and generalised, or segmental dystonia (very-low quality evidence).

We could draw no conclusions for other populations with dystonia (i.e. children and adolescents, and adults with other types of dystonia), or for other DBS protocols (i.e. other target nuclei or stimulation paradigms). Further research is needed to establish the long-term efficacy and safety of DBS of the internal globus pallidus nucleus.

Read the full abstract...

Dystonia is a painful and disabling disorder, characterised by painful, involuntary posturing of the affected body region(s). Deep brain stimulation is an intervention typically reserved for severe and drug-refractory cases, although uncertainty exists regarding its efficacy, safety, and tolerability.


To compare the efficacy, safety, and tolerability of deep brain stimulation (DBS) versus placebo, sham intervention, or best medical care, including botulinum toxin and resective or lesional surgery, in adults with dystonia.

Search strategy: 

We identified studies by searching the CENTRAL, MEDLINE, Embase, three other databases, four clinical trial registries, four grey literature databases, and reference lists of included articles. We ran the last search of all elements of the search strategy, with no language restrictions, on 29 May 2018.

Selection criteria: 

Double-blind, parallel, randomised, controlled trials (RCTs) comparing DBS with sham stimulation, best medical care, or placebo in adults with dystonia.

Data collection and analysis: 

Two independent review authors assessed records, selected included studies, extracted data onto a standardised (or prespecified) data extraction form, and evaluated the risk of bias. We resolved disagreements by consensus or by consulting a third review author. We conducted meta-analyses using a random-effects model, to estimate pooled effects and corresponding 95% confidence intervals (95% CI). We assessed the quality of the evidence with GRADE methods. The primary efficacy outcome was symptom improvement on any validated symptomatic rating scale, and the primary safety outcome was adverse events.

Main results: 

We included two RCTs, enrolling a total of 102 participants. Both trials evaluated the effect of DBS on the internal globus pallidus nucleus, and assessed outcomes after three and six months of stimulation. One of the studies included participants with generalised and segmental dystonia; the other included participants with focal (cervical) dystonia. We assessed both studies at high risk for performance and for-profit bias. One study was retrospectively registered with a clinical trial register, we judged the second at high risk of detection bias.

Low-quality evidence suggests that DBS of the internal globus pallidus nucleus may improve overall cervical dystonia-related symptoms (mean difference (MD) 9.8 units, 95% CI 3.52 to 16.08 units; 1 RCT, 59 participants), cervical dystonia-related functional capacity (MD 3.8 units, 95% CI 1.41 to 6.19; 1 RCT, 61 participants), and mood at three months (MD 3.1 units, 95% CI 0.73 to 5.47; 1 RCT, 61 participants).

Low-quality evidence suggests that In people with cervical dystonia, DBS may slightly improve the overall clinical status (MD 2.3 units, 95% CI 1.15 to 3.45; 1 RCT, 61 participants). We are uncertain whether DBS improves quality of life in cervical dystonia (MD 3 units, 95% CI -7.71 to 13.71; 1 RCT, 57 participants; very low-quality evidence), or emotional state (MD 2.4 units, 95% CI -6.2 to 11.00; 1 RCT, 56 participants; very low-quality evidence).

Low-quality evidence suggests that DBS of the internal globus pallidus nucleus may improve generalised or segmental dystonia-related symptoms (MD 14.4 units, 95% CI 8.0 to 20.8; 1 RCT, 40 participants), overall clinical status (MD 3.5 units, 95% CI 2.33 to 4.67; 1 RCT, 37 participants), physical functioning-related quality of life (MD 6.3 units, 95% CI 1.06 to 11.54; 1 RCT, 33 participants), and overall dystonia-related functional capacity at three months (MD 3.1 units, 95% CI 1.71 to 4.48; 1 RCT, 39 participants). We are uncertain whether DBS improves physical functioning-related quality of life (MD 5.0 units, 95% CI -2.14 to 12.14, 1 RCT, 33 participants; very low-quality evidence), or mental health-related quality of life (MD -4.6 units, 95% CI -11.26 to 2.06; 1 RCT, 30 participants; very low-quality evidence) in generalised or segmental dystonia.

We pooled outcomes related to safety and tolerability, since both trials used the same intervention and comparison. We found very low-quality evidence of inconclusive results for risk of adverse events (relative risk (RR) 1.58, 95% 0.98 to 2.54; 2 RCTs, 102 participants), and tolerability (RR 1.86, 95% CI 0.16 to 21.57; 2 RCTs,102 participants).