Surgery may be a treatment option for individuals whose dystonia symptoms do not respond to other therapies.
Selective Peripheral Denervation Surgery – Cervical Dystonia
Selective peripheral denervation surgery for cervical dystonia was developed to treat cervical dystonia before the availability of botulinum neurotoxin injections. The term selective refers to the care taken to identify the muscles of the neck affected by dystonia, and the term denervation refers to cutting the nerves that supply those muscles. The purpose of the procedure is to reduce abnormal contractions in the affected muscles by severing the nerves to these muscles.
Only a percentage of cervical dystonia patients are candidates for this surgery, and few clinical centers in the United States offer the procedure. Studies have demonstrated that selective peripheral denervation can significantly improve the posture of the neck with a better range of motion. Physical therapy following the procedure is an important part of the process. Patients may still require botulinum neurotoxin injections following the procedure, and repeat procedures may be needed.
Selective laryngeal adduction denervation – Spasmodic Dysphonia
Selective laryngeal adduction denervation and reinnervation (SLAD/R) is a surgical procedure to treat adductor spasmodic dysphonia/laryngeal dystonia by cutting (denervating) selected end branches of the nerves of the vocal cords.
SLAD/R may be an option for persons for specific individuals with adductor spasmodic dysphonia/laryngeal dystonia who are not satisfied with botulinum neurotoxin treatments. Only a very select number of surgical teams in the United States offer this highly specialized procedure. During the initial recovery period, all patients experience temporary voice breathiness and some experience swallowing difficulty. Patient satisfaction with the procedure in published reports tends to be high and results are long-term.
Thyroplasty surgeries include a group of surgical techniques to treat spasmodic dysphonia/laryngeal dystonia by modifying the cartilage surrounding the larynx. These adjustable and reversible procedures involve manipulating the cartilage by implanting wedges or shims to hold the tissue in place. A number of variations of this procedure are currently used and are effective for restoration of the voice after paralysis or in changing the pitch of the voice.
Myectomy procedure is the surgical removal of eyelid and brow-squeezing muscles to treat blepharospasm. Myectomy prevents the muscles surrounding the eyes from being stimulated by removing the muscle.
Before the availability of botulinum neurotoxin injections, myectomy was essentially the only treatment option for blepharospasm. The introduction of botulinum neurotoxin injections benefited many persons with blepharospasm and changed the population of individuals eligible for myectomy. Candidates for myectomy became those for whom botulinum neurotoxin neurotoxin is not sufficient. Only a very select number of surgical teams in the United States offer this highly specialized procedure.
Baclofen (Lioresol®) is a medication approved by the US Food & Drug Administration for the treatment of spasticity, a condition in which damage to the brain or spinal cord cause muses to tighten and stiffen. Baclofen is also used off-label to treat dystonia. Baclofen in the spinal fluid around the brain and spinal cord supplements the body's supply of a chemical neurotransmitter called GABA, which relaxes muscle movement. The drug may be given orally, but very high doses must often be used to ensure that the drug saturates the blood stream and reaches the spinal fluid. High doses of oral baclofen may cause intolerable side effects such as muscle weakness and fatigue. A surgically implanted baclofen pump delivers baclofen directly to the spinal fluid, and only very small doses are needed.
Intrathecal baclofen therapy has been used for children and adults with a combination of dystonia and spasticity, for example as the result of cerebral palsy. It is primarily used to treat dystonia and spasticity affecting the limbs.
In order to determine if an individual is eligible for intrathecal baclofen, they must undergo a screening test to observe the body's response to baclofen.
Starting intrathecal baclofen therapy involves surgically implanting a pacemaker-like device into the abdomen, beneath the skin of the abdomen. The pump is connected to a thin tube that is tunneled around the body and delivers baclofen to the spinal canal. The pump is adjusted by a remote control to deliver the amount of medication appropriate for the individual. Hospital stay may range from four to seven days. Modest improvement of symptoms may be noticeable before the individual is discharged from the hospital, and it make take six months or more to achieve the full extent of benefit.
Regular maintenance is a key component of intrathecal baclofen therapy. Regular exams and physical therapy may be a component of postoperative care. Pumps must be refilled regularly in the physician's office as an outpatient procedure. The frequency of refilling the pump depends on the dose required. The pump battery needs to be replaced about every five to seven years.
Studies have shown that intrathecal baclofen can dramatically improve symptoms and quality of life. Some centers have reported significant improvement in as much as 85% of patients. However, like any surgery, the procedure is not without risks. Pump complications can arise including infection and device breakage and disconnection. In a small percentage of cases, patients may lose effect within the first year of therapy or experience a worsening of symptoms. Common side effects are constipation, decreased muscle control, and drowsiness.
Pallidotomy & Thalamotomy
Before the availability of deep brain stimulation, dystonia was treated surgically by creating permanent lesions in the brain.
Pallidotomy is a procedure that involves creating a therapeutic lesion in the globus pallidus. Thalamotomy creates a lesion in the thalamus. The lesion is made by heating the tip of an electrode and coagulating the intended tissue, or similar methods.
Although lesioning procedures are performed much less frequently than deep brain stimulation, these procedures may provide a reasonable alternative to deep brain stimulation for some patients, when offered by a qualified neurosurgical team.
Suggested Questions for Your Surgeon
If you are considering surgery or if surgery has been recommended to you by a movement disorder specialist, consider discussing the following questions with your doctors:
- What is the name of the procedure and what does the name mean?
- Is the procedure a single surgery, or are there multiple steps?
- Why is this specific surgery appropriate for my case?
- What are the advantages of having surgery?
- What benefits might I expect?
- What are the risks?
- What happens if I don't have the surgery? Are their alternative treatments?
- What is the experience of the medical center and surgeon with this procedure?
- Does the medical team publish the results of surgical case studies?
- Where will the surgery be done?
- What kind of anesthetic will be used (general or local)?
- How long is the recovery and what rehabilitation/physical therapy is necessary?
- Will the procedure reduce the need for oral medications or botulinum neurotoxin injections?
- Is there a chance the surgery will need to be repeated?
- How much will the surgery cost and who will pay for it?
- Where can I get a second opinion?
- Is there anything about this procedure we have not discussed that I should know about?
Page added 2020