What is CD?
Cervical Dystonia, also known as Spasmodic Torticollis, is a neurological movement disorder, characterised by neck muscles contracting involuntarily, which leads to abnormal movements and posture of the head and neck.
This term is used generally to describe spasms in any direction: forward (anterocollis), backwards (retrocollis), and sideways (torticollis). The movements may be sustained or jerky. More women than men are affected, with spasms in the muscles or pinching nerves in the neck resulting in considerable pain and discomfort. CD can be a stressful condition for some to cope with, causing embarrassment and loss of confidence. When one’s head is unnaturally turned to one side, one’s body language is affected, and others may misinterpret our interaction with them. Though Cervical Dystonia may progress, it is likely to plateau in 2 to 5 years with a chance of remission. It is not life threatening and does not affect other brain functions.
Cause
The basal ganglia, a cluster of brain cells (neurons) deep within the brain, are involved with the initiation and regulation of movement. Most cases of dystonia are thought to reflect dysfunction within the basal ganglia or related brain networks. Some evidence suggests that abnormal sensory input from different regions of the body may also be a contributing factor or trigger for dystonia. Ongoing research is being undertaken and progress made towards a better understanding of this condition.
Diagnosis
Diagnosis of primary CD is based on clinical findings, or information from the effected individual. A history of head or neck injury may be obtained, but the relationship between trauma and dystonia is still unclear. There are no specific tests to confirm the diagnosis. However, various investigations may be used to exclude other causes of abnormal position.
Cases of inherited Cervical Dystonia have been reported, usually in conjunction with early-onset generalized dystonia, which is associated with the DYT1 gene.
Cervical Dystonia should not be confused with other conditions which cause a twisted neck such as local orthopedic, congenital problems of the neck, ophthalmologic conditions where the head tilts to compensate for double vision. It is sometimes misdiagnosed as stiff neck, arthritis, or wry neck.
Treatment
The goal of any treatment is to achieve the greatest benefits while incurring the fewest risks. It is to allow the sufferer to lead a fuller, more productive life by reducing the effects of Dystonia. Establishing a satisfactory regimen requires patience on the part of both the affected individual and the physician.
There is a three-tiered approach to treating Dystonia: botulinum toxin (botox) injections, several types of medication, and surgery. These may be used alone or in combination. Medications and botox can both help block the communication between the nerve and the muscle and may lessen abnormal movements and postures.
Medications
A number of drugs have been studied to determine the benefits for people, but none appear to be uniformly effective.
Botulinum Toxin Injections
Botulinum Toxin injections, which weaken the muscles affected by spasm, are the most effective treatment. Injections need to be repeated every three months or so. In cases where little improvement results from the injections, it may be because they have not been accurately targeted, or the dose needs adjusting, or a different type of Botulinum Toxin is required.
For this reason it is important that the physician administering the injections be experienced with Botulinum Toxin injections and be very knowledgeable about the anatomy of the neck and surrounding areas. The muscular structure of the neck is very complicated and physicians must also be aware of anatomical variation.
Surgery
Surgery is considered when other treatments have proven ineffective. The goal of surgery is to interrupt the pathways responsible for the abnormal movements at various levels of the nervous system. Some operations purposely damage small regions of the thalamus (thalamotomy), globus pallidus (pallidotomy), or other deep centres in the brain. Deep brain stimulation (DBS) has been tried with some success. Other surgeries include:- cutting nerves leading to the nerve roots deep in the neck close to the spinal cord (anterior cervical rhizotomy), or removing the nerves at the point they enter the contracting muscles (selective peripheral denervation).
The benefits of surgery should always be weighed carefully against its risks. Although some Dystonia patients report significant symptom reduction after surgery, there is no guarantee that surgery will help every individual.
Complementary Therapy
Complementary care, such as physical therapy and speech therapy, may also have a role in the treatment management. For many people, supportive therapy provides an important adjunct to medical treatment. Sometimes, the condition may be partially relieved by touching the chin, other parts of the face, or the back of the head. Different sensory tricks work for different people, and if a person finds a sensory trick that works, it usually continues to work. And the use and/or need for a soft cervical collar is sometimes helpful, especially if it is molded to provide the perfect fit.
Because CD is not common, many doctors may see only a few cases in their working life, and it is advised that sufferers are referred to a specialist neurologist with an interest in movement disorders.
Dystonia Foundation USA
http://www.dystonia-foundation.org
Dystonia Society – UK
http://www.dystonia.org.uk
