Cervical dystonia is characterized by long-lasting (chronic sustained) involuntary contractions or periodic, intermittent spasms of the neck muscles, causing the neck to turn in different ways.
The cause of cervical dystonia is usually unknown.
Doctors base the diagnosis on symptoms and results of the physical examination.
Injections with botulinum toxin are used first, but if they are ineffective, medications, taken by mouth, can be used.
Physical therapy may help relieve some symptoms.
(See also Overview of Movement Disorders.)
Dystonia can also occur in muscles other than those in the neck.
In cervical dystonia, contraction of the neck muscles causes the neck to turn from its usual position. Cervical dystonia is the most common dystonia.
The neck may move in one or more of the following ways:
Rotate (called torticollis)
Tilt (called laterocollis)
Bend forward (called anterocollis)
Bend backward (called retrocollis)
One form (called adult-onset cervical dystonia) begins in adulthood. Because it causes the neck to rotate, it is sometimes called spasmodic torticollis (in Latin, "torti" refers to twisted and "collis" refers to neck). It is the most common form of cervical dystonia. Usually, the cause is unknown, but in some people, spasmodic torticollis is caused by a genetic mutation. Stress and emotional problems may make spasmodic torticollis worse.
Cervical dystonia can
Be present at birth
Occur later, caused by various neurologic disorders
Result from the use of medications that block dopamineantipsychotic medications)
Rarely, an emotional problem is thought to contribute.
Symptoms of Cervical Dystonia
Symptoms of cervical dystonia may begin at any age but usually begin between the ages of 20 and 60, most often between ages 30 and 50.
Symptoms usually begin gradually. Rarely, they begin suddenly and progress rapidly.
Sometimes symptoms begin with shaking the head from side to side, as if people are shaking their head to say no. Some neck muscles may contract and stay contracted, or the contractions may come and go (be intermittent), twisting the neck. The contractions may be painful. The head may be turned to one side or pulled forward or backward. Sometimes one of the shoulders is raised.
During sleep, muscle spasms disappear.
Symptoms range from mild to severe. Usually, the spasms worsen slowly for 1 to 5 years, then plateau. In about 10 to 15% of people, they disappear or lessen substantially on their own, within 5 years from when symptoms begin. Spasms are more likely to disappear when they are mild and begin at a young age. However, in these people, spasms may come back. Spasms may persist for life, restricting movement and permanently twisting the head, neck, and shoulders in a distorted position.
Diagnosis of Cervical Dystonia
A doctor's evaluation
The diagnosis of cervical dystonia is based on symptoms and a neurologic examination.
Treatment of Cervical Dystonia
Physical techniques
Injections of botulinum toxin
Sometimes medications taken by mouth
Some physical techniques (such as physical therapy) can sometimes temporarily relieve spasms. Physical therapy may help by improving flexibility. Therapists may also help people identify which movements worsen spasms and which ones relieve them. Biofeedback (using relaxation techniques to control unconscious body processes, such as heart rate and muscle tension) or massage may help some people.
People may find certain tricks that may briefly relieve spasms. They include lightly touching the chin, cheek, upper face, or back of the head. These tricks are usually more effective if done on the side opposite the spasm.
When an emotional problem contributes, treatment is best managed by a team of doctors, including a psychiatrist, a psychologist, and a neurologist.
Medications
In about 70% of people with cervical dystonia, injections of botulinum toxin into the affected muscles can reduce painful spasms for 1 to 4 months and allow the head to return to a more normal position. However, for continuous relief, injections must be repeated every 3 to 4 months because the benefit of botulinum toxin wears off over time. In a few people who are repeatedly given botulinum injections, the body produces antibodies that inactivate the toxin. If the affected muscles are tiny or deep in the body, electromyography (stimulating muscles and recording their electrical activity) may be done to identify the muscles to be injected.
Certain medications, given by mouth, may help, but they control the spasms in only about 25 to 33% of people. These medications include
Anticholinergic medications can lessen spasms. However, these medications can also have side effects (such as confusion, drowsiness, dry mouth, blurred vision, dizziness, constipation, difficulty urinating, and loss of bladder control) These effects are troublesome, especially in older adults. Thus. their use may be limited.
All of these medications are started in low doses. Doses should be increased until symptoms are controlled or side effects cannot be tolerated. Side effects from these medications are particularly likely in older adults.
Surgery
In people with cervical dystonia, the role of surgery involving areas outside the brain is controversial. For example, in one such procedure, the nerves to the affected muscles are cut. As a result, the nerves can no longer stimulate the muscle to contract. However, after this procedure, the muscles are permanently weakened or may be paralyzed. When the procedure is done by skilled surgeons, benefits outweigh potential complications.
If symptoms are severe and all of the usual treatments have been ineffective, deep brain stimulation can be done. For this procedure, tiny electrodes are surgically implanted in part of the basal ganglia (collections of nerve cells that help initiate and smooth out voluntary muscle movements). The electrodes send small amounts of electricity to the specific area of the basal ganglia that causes cervical dystonia and thus help lessen symptoms.
