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Spasmodic torticollis, also known as cervical dystonia, is a focal dystonia in which neck muscles contract involuntarily, giving rise to abnormal movements and posture of the head and neck. This term is generally to describe spasms in any direction: forward (anterocollis), backwards (retrocollis), and sideways (torticollis). The movements may be sustained or jerky. Spasms in the muscles or pinching nerves in the neck can give rise to devastating pain.
Spasmodic Torticollis should not be confused with other conditions giving rise to a twisted neck: local orthopedic or congenital problems of the neck, ophthalmologic conditions where the head tilts to compensate for double vision. Torticollis is due to abnormal functioning of the basal ganglia. In spasmodic torticollis, the motor program for neck movement is at fault, and the neck muscles contract involuntary in various combinations. Sustained contractions give rise to abnormal posture of the head and neck, while periodic spasms produce jerky head movements. The severity may vary from mild to severe.
What goes wrong in the basal ganglia is still unknown.
Spontaneous recovery has been reported in about 10 percent of patients, but this is highly unpredictable. Usually the torticollis reaches a plateau and remains stable within five years of onset. This form of focal dystonia is unlikely to spread to become generalized dystonia, though patients with generalized dystonia may also have torticollis. Occasionally, there may be associated focal dystonia of the eyelids, face, and hand.
Patients usually have no neurological deficit other than torticollis. About 20 percent, however, may have a fine tremor of the hand, head, and occasionally the voice. This is called essential tremor.
Since the underlying cause of spasmodic torticollis remains unknown, there is as present no cure for the condition. Treatment is directed towards symptomatic relief, the patients usually requiring treatment when they feel the condition is giving rise to disabling pain or when the abnormal neck posture and movement is causing them social embarrassment or is threatening to the job. It is often partially relieved by a gentle touch on the chin or other parts of the face.
Various medications are used for treating spasmodic torticollis. They have totally different mechanisms of action and generally produce unpredictable and short-lasting benefits. One drug may work for some patients and not for others. When the effects of one drug wears off, sometimes the replacement with another drug helps. There is, therefore, no fixed or best regimen. Establishing a satisfactory treatment scheme requires patience on the part of both the physician and the patient.
Some of the medicines your doctor might consider include: Artane (trihexyphenidyl), Cogentin (benztropine), Valium (diazepam), Klonopin (clonazepam), Lioresal (baclofen), Tegretol (carbamzepine), Sinemet or Madopar (levodopa), Parlodel (bromocriptine), Symmetrel (amantadine). Nitoman (tetrabenazine) is often effective, but it is not easily available. Thorazine (chlorpromazine) and Haldol (haloperidol) and other medicines of the phenothiazine or butyrophenone groups may help but may produce a side effect called tardive dyskinesia and should be used with great caution.
The list is by no means complete, and there are many more new drugs being developed. The use of these medications requires close supervision from a neurologist, and it is important that the patient not change the dosage or stop the medications without the neurologist's approval.
Surgery is undertaken to interrupt, at various levels of the nervous system, the pathways maintaining the abnormal neck movements. Some operations intentionally damage small regions of the thalamus (thalamotomy), globus pallidus (pallidotomy), or other deep centers in the brain. Other surgical approaches include severing one or more of the contracting neck muscles (muscle resection), cutting nerves going to the nerve roots deep in the neck close to the spinal cord (anterior cervical rhizonotomy), and removing the nerves at the point they enter the contracting muscles (selective peripheral denervation).
We do not recommend most of these procedures. However, selective peripheral denervation or thalamotomy may be considered in very severe cases when other treatment modalities, including botulinum toxin, have failed and when done be the few neurosurgeons who have significant experience in these specific operations.
Botulinum toxin paralyzes muscles by blocking the impulse transmitted from the nerve endings to the muscles. When diluted and given intramuscularly in extremely small quantities, it can give rise to selective paralysis of the injected muscle and is safe. In spasmodic torticollis, the abnormally overactive neck muscles can be identified by clinical examination and palpation. These muscles can be injected with the toxin. Occasionally, an elctromyogram (EMG) can confirm or aid in the identification of overactive neck muscles. Studies of this treatment have been shown to produce significant relief of pain in over 85% of patients and improvement in the torticollis in about 70%.
The improvement following treatment lasts for about three months when patients need to be re-injected. The injections cause very little discomfort, are well tolerated, and produce no significant side effects. Difficulty swallowing occasionally occurs. This, however, has been mild, transient, and infrequent. Resistance to the toxin after repeated treatment is rare, though circulating antibodies have been detected in about 10% of patients. The significance of this remains to be studied.
Stress makes all movement disorders, including torticollis, worse. Some patients may benefit from a trained professional in learning stress management.
A physiotherapist may be able to help patients with torticollis through an acute episode of pain and/or spasm through the use of local moist heat, ice, or ultrasound. A trained physiotherapist can suggest exercise and fitness programs suitable for the disability and can advise on the maintenance of good posture and strength in the back and shoulder muscles which are often secondarily affected by torticollis. Treatment involving manipulation of the neck is NOT RECOMMENDED for spasmodic torticollis.
For someone with torticollis, an occupational therapist may be able to help functional disability with the use of a soft collar. Sometimes a custom-fitted soft collar is necessary. A patient may use a collar in public to prevent unwanted questioning from strangers. The occupational therapist can suggest adaptations in the home or workplace which will reduce fatigue, promote safety, and improve the mobility for the patient with spasmodic torticollis. Support from family and friends is important. Thousand of person are experiencing the same symptoms. The Dystonia Medical Research Foundation has numerous support groups where sharing the experience will reassure a patient and his/her family.
For more information, contact:
National Spasmodic Torticollis Association (NSTA)
9920 Talbert Ave., Suite 233
Fountain Valley, Ca
USA 92708
Tel: (800) HURTFUL [487-8385]
Tel: (714) 378-7838
Fax: (714) 378-7830
E-mail: NSTAmail@aol.com
NSTA Web pages
