Deep Brain Stimulation for Dystonia

Overview

What is Deep Brain Stimulation for Dystonia?

Patients with generalized dystonia often require deep brain stimulation (DBS) for improvement in quality of life when medications have been found to be ineffective or difficult to tolerate.

In cervical dystonia and other types of focal dystonia, botulinum toxin (Botox) injections are often beneficial, but in some patients the dystonia is too severe for adequate benefit from medical treatment. These patients can enjoy significant improvements in quality of life and recovery of function with DBS.

Eligibiity

Eligibiity of Deep Brain Stimulation for Dystonia

Patients with dystonia that are most likely to have a good outcome from DBS exhibit the following characteristics:

  • Disabling dystonia
  • Incomplete response to medications
  • Younger patients (<21 years old)
  • Shorter duration of symptoms (<15 years)
  • Absence of fixed deformities or contractures
  • DYT1, DYT6, or DYT11 positive
  • Primary dystonia (not caused by an underlying disease)
  • Absence of dementia or severe depression
  • Normal MRI
  • Good medical health
  • Realistic expectations

Please note that patients who do not meet all the above criteria may still have dramatic benefit, therefore consultation with our team is useful to make specific recommendations.

Benefits

Potential Benefits of Deep Brain Stimulation for Dystonia

Studies of DBS for dystonia show a broader range of benefit of DBS compared to DBS for PD or ET. On average, patients with primary / genetic dystonia have about 50% reduction in their symptoms. However, around 20% of patients implanted may have very little benefit, whereas some patients may experience a larger degree of improvement, up to 80-90% reduction in symptoms and signs of dystonia. Some patients are able to reduce medications with DBS but the majority continue to take some medication or continue to have Botox injections.

Risks

Potential Risks of Deep Brain Stimulation for Dystonia

Post-operative headache and pain are possible but typically resolves gradually a few weeks after the procedure. Neck pain is also possible in the short term. Infection of the leads, extension or battery is very low risk and our surgical team does their utmost to prevent this. Hemorrhage is extremely low risk but when it occurs may lead to stroke-like symptoms.

Most frequently, hemorrhage does not result in permanent complications. Lead fracture or migration, also very low risk, may require repeat surgery.

Problems with speech, language and mood; muscle tightness, slurred speech and vision symptoms can be related to the stimulation and thus can usually be reduced or eliminated by adjusting the stimulation.

Team

Deep Brain Stimulation for Dystonia Specialist

Resources

Deep Brain Stimulation Resources

The incisions are small, about 2 inches on the top of the head, 1 inch behind the ear, and 2.5 inches below the collarbone. Typically only a small area near the incision on the top of the head requires shaving.

3-4 years depending on the settings required. A rechargeable option is also available which will last 9 years, but requires the patient to regularly charge the battery.

Once the electrodes are placed, a neurological exam is done to ensure that therapeutic benefit has been achieved. During this examination, the patient is awake. During the procedure, the anesthesia is tailored to the patient’s needs to make sure that pain and anxiety are properly relieved.

Patients typically go home after 1 or 2 days. Most patients feel they are back to their baseline level of energy after 2 weeks. The sutures are removed one week after surgery and DBS programming can be done at this visit. We recommend avoiding strenuous activity for the first month post-operatively. It can take several weeks, up to a couple months, to find the optimal settings for DBS programming and adjust medications.

Patients are given a patient controller which allows them to turn the DBS on and off (typically it’s left on the entire time); and depending on the patient we may allow for adjustment of one parameter of programming to a certain degree. Initial programming and major readjustments are done by our expert team.

After the recovery period, it is fine to fly. We will provide paperwork so the TSA is aware that you have a medical device and it is preferable to avoid going through airport scanners. However, there is only a very low risk of scanners turning off the device. When flying, patients should take their patient controller device with them to be able to verify whether the DBS is on or off.

MRIs, of the head or the body, may be considered if medically necessary, but the type of MRI machine may be restricted to a lower magnetic field (1.5 T), and certain types of MRI may require special equipment. Always tell the radiology technician of any implanted devices. Typically, the DBS is turned off during the MRI and turned on afterwards. We work closely with you to coordinate pre- and post-MRI management.

Yes. Medicare and most private insurances cover DBS because it is standard therapy for PD, ET and dystonia. DBS is FDA approved for PD. Our office would obtain prior approval from your insurance company.

Five-year and ten-year follow-up studies show sustained improvement in symptoms of PD, ET and dystonia over time. However, DBS is not a cure of PD, which typically continues to progress.

In the first few weeks, weekly visits are typical to fine-tune the adjustment of the DBS. Following that, visits are scheduled on an as-needed basis. At minimum, DBS patients follow up every 6 months to verify the battery level and ensure stability of treatment.

The leads and battery are all internalized, but in a slender person, there may be a slight bump visible under the skin of the neck and upper chest below the collarbone. In a person whose hairline is receding, the scalp incision scar may be visible and there may be two small bumps, about the size of a quarter, which can be felt or sometimes seen. The battery looks and feels very similar to a pacemaker battery.

Be sure to tell all providers that you have a DBS system in place. Of course, your referring physician and primary care physician will receive notes from our clinic. Dental care can be undertaken but with various precautions, such as avoiding dental drills and ultrasonic probes over the implant site and using antibiotics prior to invasive procedures such as root canals and implants.

Let your surgeon and the DBS team know prior to surgery. The DBS system should be turned off for the procedure or for CT scans. Electrocautery, electrolysis and radiation therapy should not be used over the implant site. There is no problem with X-ray or ultrasound.

The decision regarding driving is complex and should be determined by your providers who are well familiar with your case. In some cases, patients can resume driving as soon as they are no longer using sedating pain medications. In other cases, drivers’ evaluation testing may be advised to ensure that the reaction timing is intact.

No. Deep-heat treatments, also known as diathermy, deliver energy to treat specific parts of the body, and can result in severe injury or death in patients who have DBS in place. This includes microwave diathermy, ultrasound therapeutic diathermy and shortwave diathermy.

No.

Yes, but tell your doctor so they are aware of the DBS to avoid interference with the system.

Limited exposure (10-15 minutes) to heat equal or less than 100 degrees Fahrenheit should not cause any trouble, but hot tubs and tanning beds often result in heat exposure above the acceptable range, and therefore are best avoided.

Most household appliances will not harm the DBS, including radios, microwaves, computers. Older generation DBS systems were susceptible to accidentally being turned off or on by nearby magnets including those on refrigerators, but does not to occur with newer generation DBS. Still, you should always carry your patient programmer so you can check your system if you are not sure if it is on or off.

You can be around industrial equipment and use power tools but always carry your patient programmer so you can check to make sure your system is working properly after use. You should not arc weld.

Ask your DBS team prior to scuba diving. The device should function normally down to 33 feet of seawater. Skydiving should be avoiding because of the dramatic forces put on the head and neck which can damage the leads and connections of the DBS system.

Next Steps

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Our movement disorders neurology and restorative neurosurgery team can be reached at 310-582-7433.

Written and reviewed by:
The Pacific Neuroscience medical and editorial team
We are a highly specialized team of medical professionals with extensive neurological and cranial disorder knowledge, expertise and writing experience.

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