About Cervical Dystonia
Cervical dystonia is a form of abnormal movement characterized by abnormal head and neck position caused by involuntary contraction of neck muscles. This may result in sustained abnormal position or jerky repetitive movements mimicking tremor.
Cervical dystonia is a form of abnormal movement characterized by abnormal head and neck position caused by involuntary contraction of neck muscles. This may result in sustained abnormal position or jerky repetitive movements mimicking tremor.
The abnormal contractions of the neck may also involve the shoulder resulting in shoulder elevation or forward deviation. Jerky tremulous-‐like movements often occur and the majority of patients have associated neck and shoulder pain. Pain in some patients may be even more disabling than the abnormal movements. Symptoms of cervical dystonia gradually come on over one to two years and then tend to stabilize. Remission is seen in only a small percentage of patients, and even in those in whom remission occurs, the symptoms typically come back. Cervical dystonia can occur by itself and is then considered a focal cervical dystonia or can be part of more widespread dystonia. When cervical dystonia is combined with dystonia involving the face, it is referred to as craniocervical segmental dystonia. Cervical dystonia may also occur as part of widespread dystonia involving several limbs and the trunk, in which case it is part of generalized dystonia.
Treating Cervical Dystonia
Treatment is aimed at reducing symptoms and, as a result, improving quality of life. The first goal is usually to improve the abnormal head position and jerky movements of the head and neck. Since pain occurs in the majority of patients with cervical dystonia, reduction of these abnormal movements and reduction of muscle spasm can easily and substantially improve pain. Secondary complications may occur as a result of the abnormal head posturing and jerking movements. Contractures may occur, which means that there can be limitation in range of motion of the neck due to permanent shortening of the muscles in the neck. Accelerated arthritis may occur, resulting in compression of the nerve roots which exit the neck resulting in pain and weakness in the neck and arms. This is referred to as a cervical radiculopathy. Accelerated arthritis may also result in compression of the spinal cord, resulting in weakness and numbness in the arms and the legs, as well as difficulties controlling the bladder and bowels. This is referred to as cervical myelopathy. Therefore, another important goal of treatment is to reduce the risk of developing secondary complications.
When the botulinum toxin is injected into the overactive muscle, it is taken up by the nerve ending which supplies the signal to the muscle to contract. Once inside the nerve ending, the toxin breaks down a protein which is necessary for the release of acetylcholine by the nerve ending. Acetylcholine acts as a signal from the nerve ending to the muscle to contract. The end result is that the toxin prevents the communication from the nerve ending to the overactive muscle resulting in temporary weakening of the overactive muscle reducing involuntary movement and muscle spasm. The effect is not permanent and the nerve ending eventually regenerates the protein which has been broken down by the botulinum toxin in the nerve ending that prevents release of the acetylcholine signal from the nerve ending to the muscle. When this important signaling protein is regenerated, the nerve ending can communicate with the muscle and the improvement of symptoms goes away.
There are two major forms of botulinum toxin, Type A and Type B. Type A botulinum toxins are used more commonly and the name brands are Botox, Dysport, and Xeomin. The name brand of Type B botulinum injection is Myobloc. Type A and Type B toxins work similarly, except that the type A toxins break down a different protein in nerve endings than does Type B toxin. The different forms of botulinum toxin have similar benefits and adverse effects. The effect of botulinum toxin injection is temporary and as a result patients need periodic injections. A very small percentage of patients, perhaps 1 or 2%, may develop antibodies to one type of botulinum toxin so that injections are no longer effective. If this occurs, patients may be switched from one type of toxin to another type of toxin.
Injections are distributed amongst the various muscles, customized and based on the patient’s pattern of abnormal movements. EMG guidance is often used to help localize the overactive muscles. EMG stands for electromyography and is a neurophysiologic method by which the electrical activity of muscle activity can be recorded.
Treating Cervical Dystonia with Surgery and Oral Medications
Surgery is reserved for patients with severe dystonia with significant impairment in quality of life. Usually, this means that patients have an inadequate response to aggressive treatment with botulinum toxin injections and oral medications. Surgery may also be used in patients who initially responded well, but who have subsequently developed resistance to both botulinum toxin Type A and Type B. Most commonly, surgery is especially helpful in patients who have marked anterocollis (neck flexion) or retrocollis (neck extension) since it may be difficult to adequately improve patients with severe anterocollis without causing substantial trouble swallowing. It may be difficult to adequately improve patients with severe retrocollis since this may result in excessive weakness of the neck extensor muscles. Cervical dystonia may also occur as part of more widespread segmental and generalized dystonia, in which case botulinum toxin injections may not be able to be applied throughout the entire affected areas.
Typical complications include tingling and burning in the areas of the skin where the nerves have been cut, trouble swallowing, or excessive weakness of the neck. Many of these adverse effects gradually resolve aYer surgery over a period of weeks or months.
Dystonia Overview
Dystonia is a movement disorder that causes involuntary muscle spasms and contractions. Dystonia may effect a single area of the body or throughout a group of muscles. The American Dystonia Society estimates that as many as 300,000 people in North America are diagnosed with the disease. Many dystonia patients have pain, muscle spasms, and cramping associated with the movement disorder.
Dystonia can affect patients at any age. At early onset age, it is more likely that dystonia will become more severe and spread to other parts of the body. If a patient has a late onset of dystonia, it is more likely that disorder will stay focal. In addition to staying focal, if the dystonia spreads, it will likely only spread to adjacent segments.
Treating Dystonia
Botulinum toxin injections are commonly used for the treatment of dystonia. Botulinum toxin is injected into the affected muscle(s) causing them to relax . This treatment typically provides approximately three months of relief. Botulinum toxin injections may also reduce the paid associated with dystonia. These injections will need to be repeated over time as the effects wear off. A rare portion of patients build an immunity to Botulinum toxin injections.
In the event oral medications and botulinum toxin injections are no longer working or if there are other debilitating factors involved, there are a few different types of surgical treatments available for dystonia. The most recommended surgical option is Deep Brain Stimulation (DBS). It should be noted that DBS surgery is rare in dystonia patients.
The following medications may be used in the treatment for dystonia: Anticholingerics, Benzodiazepines, Baclofen, Tetrabenzine, Dopaminergic agents, Dopamine-depleting agents
The benefits to treating dystonia are primarily to improve a patients quality of life as well as reducing pain. Treating dystonia may be life-altering in such a way that it may allow a patient to engage in daily activities that a patient may otherwise not have been able to engage in. These may include professional, personal, and recreational activities.

Dystonia Education
This unique resource is designed to educate newly diagnosed patients and the general public about Dystonia. The videos answer important questions about the disease itself, treatment options, and emerging research. Dr. Kumar is a movement disorders specialist and clinical expert on movement disorders. He also serves as Medical Director for the Rocky Mountain Movement Disorders Center.
Learn All About Dystonia
Explore what dystonia is and how it effects the body.
Dystonia is a movement disorder characterized by sustained or intermittent muscle contraction usually involving agonist and antagonist muscles. This means that opposite acting muscles are simultaneously contracting which is sometimes called co-‐ contraction. This causes abnormal posture or, repetitive movements, or both.
Causes & Characteristics
The Symptoms, Classifications and Causes of Dystonia
Many genes have been found to cause various forms of familial dystonia. In the majority of cases, the dystonia tends to have onset in childhood or early adulthood. Typically many members of these families develop generalized dystonia. More recently, two or three genes have been discovered to cause focal or segmental cervical or craniocervical segmental dystonia. Most of the dystonia genes exhibit what is called reduced penetrants. This means that even if one harbors the genetic mutation, one may never develop symptoms. Indeed, in general only about 30 to 40 percent of individuals who have mutations in the various dystonia genes ever develop symptoms of dystonia during their life.
A minority of patients have acquired dystonia as a result of non-‐genetic causes. Dystonia may occur as a result of brain injury occurring from head trauma or as a result of oxygen deprivation during birth. Other non-‐traumatic causes such as brain tumors, strokes, or brain infection may also cause dystonia. Dopamine receptor blocking drugs such as those used to treat nausea and vomiting and others used to treat schizophrenia or depression may cause what is termed tardive dystonia. Lastly, dystonia may occur as part of a more widespread brain disorder in certain brain degenerative or metabolic diseases.
Many Dystonia Treatments
Oral Medications, Botox Treatment, Surgery, Non-Drug Therapy
Surgical therapy is typically reserved for patients with substantial disability despite maximal treatment with oral medication and botulinum toxin injections. Surgery may be used in patient with focal dystonia or generalized dystonia, but only a small percent of patients with focal dystonia require surgical treatment. For patients with generalized dystonia, brain surgery involving implantation of electrodes into the brain to block abnormal signal know as deep brain stimulation is typically the treatment of choice. For patients with focal dystonia, deep brain stimulation may be used, but also peripheral surgery in which the overactive muscles are cut or the nerves supplying these muscles are cut to reduce the abnormal contractions may be used. Peripheral surgery is more commonly used in patients who have severe blepharospasm and cervical dystonia.
Meet the Author and Practicing Clinician
Dr. Rajeev Kumar is one of the world’s foremost experts on movement disorders, with unparalleled experience and expertise treating and researching Parkinson’s Disease, Huntington’s Disease, Dystonia, and many other debilitating movement disorders. Trained at the renowned Mayo Clinic, Dr. Kumar has two decades of experience working in the fields of Neurology and Movement Disorders, and as Medical Director of the Rocky Mountain Movement Disorders Center and CNI Movement Disorders Center, he has solidified his status as one of the most trusted and respected professionals in the field.
Dr. Kumar, MD Specializes in Treating Dystonia and Similar Movement Disorders including: General Dystonia, Cervical Dystonia, Miege Dystonia, Oromandibular Dystonia, Writer’s Cramp, Hemifacial Spasm and Essential Tremor .
Will Botulinum Toxin Injections (Botox) Help Me?
80 to 90 percent of patients with focal dystonia are significantly improved with Botox injections. Patients with cervical dystonia experience a reduction in neck pain, abnormal head positioning, neck stiffness, and tremor/jerking, and are better able to turn the head. Patients with eye twitching or involuntary eye closure (blepharospasm) are substantially improved in 80 to 90 percent of cases, resulting in improvement in the ability to read, drive, and carry out day-to-day activities.
Writer’s cramp and other forms of limb dystonia may interfere with only one specific task or with many different tasks requiring manual dexterity. Selective injections can improve the use of the hands for writing, playing an instrument, typing or other activities.
Treatable Symptoms
- Cervical dystonia – Abnormal neck posturing, often with superimposed jerky movements, may cause pain and embarrassment, and interfere with work, driving and social activities.
- Blepharospasm – Eye twitching and involuntary eye blinking can interfere with reading, driving and many other day-to-day activities. These symptoms are commonly worse when patients are outside in sunlight.
- Limb dystonia – Abnormal hand posturing may be task specific, sometimes occurring only when the patient is writing (writer’s cramp) or performing some other fine dexterous activity, such as typing or playing a musical instrument. The abnormal hand posturing may make it difficult or impossible to accomplish these tasks.
Treatment Methods
Botulinum toxin is injected specifically into the overactive muscles causing the foot cramps or eye twitching, or into the salivary glands for drooling. The effect comes on gradually over several days, with the peak effect reached in approximately 2 weeks. The benefit lasts for about 3 months. As a result, injections are repeated at 3 month intervals to maintain ongoing benefits.
Prognosis When Untreated
Dystonia usually slowly worsens over 2 to 3 years, and thereafter continues as a chronic disability. Remission is uncommon, occurring in less than 5 percent of cases.
Prognosis With Treatment
Botulinum toxin (Botox) injections effectively treat cervical dystonia in between 80-90 percent of patients, resulting in improvement in pain, neck position and quality of life. The injections can occasionally cause mild trouble swallowing or excessive neck weakness, but such side effects are typically mild and short lived. Injections are customized for each individual patient with respect to the specific muscles and the specific doses used, to maximize the benefits and to reduce the chance of any adverse effects.
Oral medications are helpful in less than 25 percent of patients, and rarely result in the immediate improvement seen with botulinum toxin injections. In patients with severe and more widespread dystonia (generalized dystonia), deep brain stimulation may provide additional therapeutic benefit.
Blepharospasm is improved with botulinum toxin injections in 80 to 90 percent of patients. Drooping of the eyelids or dry eyes may occur as adverse effects. If these side effects occur, they are usually mild and go away within 2 or 3 weeks. The specific pattern and dose of the injection into the muscles responsible for eye closing is customized in order to maximize benefits and minimize adverse effects.
Task specific limb dystonia (e.g. writer’s cramp or musician’s cramp) can be improved with highly customized injections of small amounts of Botox to selectively weaken the overactive muscles, without causes excessive weakness or reducing dexterity. When done by a highly experienced physician, this improves function in more than 75 percent of patients. Very low doses are typically needed in order to prevent grip weakness or impairment of other tasks.
Schedule a Consultation for Botulinum Toxin Treatment
In patients with Parkinson’s disease who are not currently Deep Brain Stimulation candidates; many of their mobility, and general movement issues can be improved with the use of Botox injections. With management of specific symptoms, patients’ quality of life can vastly improve in many different ways.Patients with different kinds of dystonia, can also benefit from the management of their different symptoms via Botox injections. They can regain their ability to walk, write, drive, and even live life with decreased pain.