What is cervical dystonia?
Author Ольга Кияница
Cervical dystonia (CD) is a neurological disease that causes abnormal contraction of the neck muscles. This muscle contraction can lead to a head tilt or tilt. The chin can drag up, down, forward or back, and the shoulder can also occupy an unnatural position.
The disorder is also known as spastic torticollis (spastic torticollis, ST). This can cause severe pain and discomfort.
The disease often begins slowly, worsens, and then the patient’s condition stabilizes. In order to diagnose pathology, it may be necessary to consult a neurologist and undergo appropriate instrumental examinations. Treatment is most often aimed at relieving spasms, although anti-inflammatory and anticonvulsant drugs may be prescribed if necessary.
Video: A Patient With Severe Cervical Dystonia
Description of cervical dystonia
Cervical dystonia is a special form of neurological pathology that affects the head and neck. Cervical dystonia causes excessive contraction of the neck muscles. These muscle contractions contribute to the occurrence of involuntary movements and an awkward position of the head, neck, and sometimes shoulders.
Cervical dystonia can cause:
- Twisting the neck.
- Tilt your head to the side.
- Tilt the head all the way forward or backward.
- Raising the shoulder to the ear.
- Mixing the neck away from the midline of the body.
In some cases, these abnormal contractions can be permanent or long lasting. In other cases, they manifest as spasms, which may resemble frequent and minor muscle contractions (tremor).
Neck dystonia is one of the most common forms of muscular dystonia observed in clinical departments with movement disorders.
Prevalence among the population
Cervical dystonia affects women about two times more often than men. This is the most common form of focal dystonia.
Neck dystonia can affect people of any age, but usually develops in people aged 40 to 60 years. Also, cervical dystonia affects people of all ethnic groups.
The exact frequency or prevalence of cervical dystonia in the general population is unknown, but it is estimated that in the United States it is about 60,000 people.
In 10-25% of cases, a positive family history is observed, but a genetic predisposition is not fully proven.
Sometimes cervical dystonia begins in infancy or in early childhood. In such cases, secondary causes should be investigated.
Symptoms of Cervical Dystonia
Symptoms of cervical dystonia can begin to appear slowly and often affect the various muscles of the neck. The position of the head in cervical dystonia may also vary.
The most common abnormal posture associated with dystonia of the neck is the bend of the chin to the shoulder, so that the head is turned sideways ( tortilkollis or torticollis ).
Other abnormal poses associated with cervical dystonia include:
- Anterocollis in which the head leans forward.
- Retrocollis with head tilted back.
- Laterocollis, in which the head is tilted to one side.
There may also be a displacement of the head towards the shoulder in the forward direction (sagittal displacement forward) or backward (sagittal displacement backwards).
Often, cervical dystonia is complex and combines several angles of movement of the head. Some people with isolated CSD may also have postural hand tremor.
Symptoms of neck dystonia vary over the entire period of the disease. Studying the natural history of the disease is necessary to better understand the development of the disease in a particular patient.
Symptoms may temporarily worsen with stress or agitation. Also, the development of the clinic can contribute to certain body positions. Most often, this depends on the physiological characteristics of a particular patient.
Symptoms may improve when performing sensory techniques, such as touching the cheek or the back of the head. A small percentage of people experience spontaneous recovery (remission) after the onset of symptoms. This remission is often temporary, from several days to several years, followed by a relapse.
It is worth pointing out that in less than 1% of the victims permanent remissions are determined.
Sometimes there are secondary problems associated with dystonia of the neck and representing:
- Arthritis of the cervical spine.
- Compression of the nerve roots.
- Constriction of the spinal cord in the neck (cervical stenosis).
The pain associated directly with cervical dystonia is usually localized on the same side as the turned head. The symptom is most often felt as muscle pain in the area of hyperactive muscles. The pain can become severe and sometimes causes a person to become disabled.
Causes of Cervical Dystonia
Scientists believe that the symptoms of dystonia are the result of incorrect signals sent by the nervous system. They cause the muscles to involuntarily contract and the body to assume the wrong position. Researchers and doctors still do not fully understand the neurological mechanisms that cause this abnormal muscle contraction.
Cervical dystonia can be:
- Isolated (primary).
- Acquired (secondary).
The causes of isolated dystonia are not yet fully understood. Isolated cervical dystonia can be genetic and usually affects adults.
Acquired dystonias are usually caused by specific structural or metabolic causes and are often associated with additional neurological symptoms. The most common causes of acquired cervical dystonia are:
- Physical injury (about 10% of reported cases).
- Drug reaction (less than 10% of reported cases).
Drug-induced neck dystonia is most often associated with neuroleptic drugs. Acquired dystonia can occur at different ages, depending on the cause or condition. Children with cerebral palsy may have symptoms of secondary dystonia, including cervical dystonia.
Video: What Causes Dystonia? - Prof Mark Edwards
Diagnosis of cervical dystonia
The diagnosis of cervical dystonia is based on a clinical examination, a detailed elucidation of the patient’s history and the determination of disorders.
A specialist performing the diagnosis and treatment of dystonia is a neurologist with specialized training in the field of movement disorders. Some doctors of osteopathic medicine and otolaryngologists may also have experience in treating neck dystonia, depending on their training and experience.
No specific laboratory or visual test confirms the diagnosis of cervical dystonia. There are also no deviations in laboratory or visual tests.
Magnetic resonance imaging (MRI) of the brain is normal, and MRI of the neck does not help with the diagnosis, if compression of the spinal cord is not suspected.
Electromyography is not shown if there are no additional signs of irritation of the nerve endings.
Cervical dystonia: treatment
Not every treatment option for neck dystonia is successful, so there is no overall exposure strategy suitable for all cases.Most treatments are symptomatic and designed to relieve cramps, pain, and posture or function disorders. Determining the treatment regimen that is most effective in individual cases may require patience and perseverance on the part of the patient and the doctor.
There are three treatment options for cervical dystonia:
- Botulinum toxin injection.
- Oral medications.
- Surgical intervention.
These methods of drug exposure can be used alone or in combination. In addition, the doctor may recommend physiotherapy. It includes exercises for stretching and strengthening muscles. Physical therapy can reduce pain and improve posture and neck movement. Massage and stress relief exercises can also help with pain and discomfort. In some cases, if touching the chin is effective, a soft cervical collar may be helpful.
Botulinum toxin injections are most commonly used to treat neck dystonia.
Botulinum toxin is a neurotoxin that is injected into the dystonic muscles of the neck in small doses.
Botulinum toxin prevents the release of a neurotransmitter, called acetylcholine, which causes the muscles to contract.As a result, reduced muscle tone. Botulinum toxin begins to affect the muscles about 2-3 days after the injection, reaching its peak effect after about 4 weeks. Improvement is observed for about 2-6 months.
Botulinum toxin treatment is the treatment of symptoms. When the action of botulinum toxin ceases, the symptoms of neck dystonia return, so another injection is needed to maintain the effect. In order for botulinum toxin injections to be successful, it is imperative that the injection doctor understands cervical dystonia, the functional anatomy of the neck muscles, and the type and doses of botulinum toxin used.
Currently, there are four brands of botulinum toxin that have been approved by the Food and Drug Administration (FDA) for the treatment of patients with cervical dystonia.
There are three brands of botulinum toxin serotype A, including:
- Onabotulinum Toxin A (BOTOX, Allergan Inc.).
- Abobotulinumtoxin A (Dysport, Tercica Inc).
- Incobotulinumtoxin A (Xeomin, Merz Pharmaceuticals).
There is one type of botulinum toxin type B:
- Rimabotulinumtoxin B (Myobloc, Solstice, US World Meds).
These brands are not interchangeable, and each must be administered as a unique drug. Currently there are no clinical data confirming the advantages of using one over the other.
The most common side effects of botulinum toxin injections are:
- Difficulty swallowing.
- Pain at the injection site
- Weakness in the neck.
These side effects are usually minor and short-lasting, lasting several weeks. The FDA has a black box warning regarding the use of any of these toxins, which indicates that the toxin may spread from the injection site. However, with doses used to correct neck dystonia, the clinical effects of toxin proliferation rarely occur.
Currently, there are no FDA approved oral products for use in dystonia. The most commonly used oral agents are:
- Dopaminergic drugs (levodopa).
- Anticholinergic drugs (benztropine, trihexyphenidyl).
These drugs are usually most effective in children with generalized dystonia. In adults, side effects of these remedies, including memory problems and sedation, often occur before an effective dose can be achieved.
Surgical treatment of neck dystonia are of two types. One of them is selective peripheral denervation, in which the nerves of the dystonic muscles are cut. Although, as reported, the method of action is effective, its implementation is limited due to the difficult access to the nerve involved. Side effects may also occur and considerable surgeon experience is required. Additionally, after surgery there is a need for a long period of rehabilitation.
The operation of deep brain stimulation (GSGM) is effective for neck dystonia and may be suitable for those patients who are not helped by botulinum toxin or have a form of CSD, in particular, anterocollis that is difficult to inject. GPSG involves the placement of electrodes (thin wires) in the brain, called the pale ball on both sides. Electrodes are connected to stimulants that send small electrical impulses to the brain. Although the exact mechanism of such treatment is not fully understood, electrical impulses seem to eliminate the brain glitch and improve dystonic movements.
Life with cervical dystonia
Cervical dystonia is a lifelong disease. But at the same time it is quite possible to live well with cervical dystonia. The early stages of the disease, its diagnosis and the search for an effective method of treatment are often the most difficult stages of everyday coexistence with CD. Symptoms can range from mild to severe, and they often change from day to day. Cervical dystonia often causes significant physical pain and fatigue. People with CDs may have to learn new ways of doing everyday tasks.
People living with cervical dystonia are highly recommended:
- Seek the best medical care.
- Learn more about dystonia and the possibilities of modern treatment.
- Take part in a multi-level support system in the form of groups of people with CSD, online resources, friends and family.
- Contact an experienced mental health professional to diagnose and treat possible concomitant depression or anxiety disorders.
- Explore additional treatments.
- Show activity in the community of people with dystonia.
Additionally, you should try to minimize stress in everyday life. Even if the stress factor does not cause dystonia, the symptoms of the disease may worsen when the patient is under stress.
International organizations supporting patients with dystonia
American Society of Dystonia (American Dystonia Society)
17 Suffolk Lane
Princeton Junction, NJ 08550
Phone: (310) 237-5478
Dystonia Europe (Dystonia Europe)
Square de Meeus 37 - 4th Floor
Brussels, 1000 Belgium
Dystonia Medical Research Foundation
1 East Wacker Drive, Suite 2810 East Wacker Drive
Chicago, IL 60601-1905 United States
Phone: (312) 755-0198
Toll-free: (800) 377-3978
Dystonia Society (Dystonia Society)
89 Albert Embankment, 2nd Floor
London, SE1 7TP United Kingdom
Phone: (845) 458-6211
Toll-free: (845) 458-6322
Genetic and Rare Diseases Information Center (GARD)
PO Box 8126
Gaithersburg, MD 20898-8126
Phone: (301) 251-4925
Toll-free: (888) 205-2311
National Spasmodic Torticollis Association
9920 Talbert Avenue
Fountain Valley, CA 92708
Phone: (714) 378-9837
Toll-free: (800) 487-8385
National Institute of Neurological Disorders and Stroke (National Institute of Neurological Disorders and Stroke)
PO Box 5801
Bethesda MD 20824
Phone: (301) 496-5751
Toll-free: (800) 352-9424
Spastic Torticollis (Spasmodic Torticollis ST / Dystonia, Inc.)
PO Box 28
Mukwonago, WI 53149
Phone: (262) 560-9534
Toll-free: (888) 445-4588
Cervical dystonia is more of a neurological disease that affects certain groups of neck muscles. Presumably, pathology can be transmitted by heredity, but there are also secondary forms of CD associated with other diseases or conditions.
The diagnosis of the disease mainly consists in competently determining the clinical picture and identifying symptoms characteristic of CD. Difficulties may arise with the diagnosis, if the clinic is not too pronounced.
For the treatment of cervical dystonia, botulinum toxin is used primarily to help eliminate symptoms. Tablets are practically ineffective in the treatment of CD. In extreme cases, surgery is performed - selective peripheral denervation or deep brain stimulation. In each embodiment, the appearance of side effects.
Video: Living Comfortably with Cervical Dystonia Through Exercise
Cerebrovascular disease is associated with impaired blood supply to the brain due to deformation or damage to blood vessels. Ischemic stroke most often occurs, although hemorrhagic damage to brain tissue is sometimes determined. In any case, the patient faces severe complications, so appropriate treatment should be carried out.