Newswise — Dystonia is a very complex, highly variable movement disorder characterized by involuntary muscle contractions. According to the American Association of Neurological Surgeons (AANS), dystonia affects as many as 250,000 people in the United States. It is a condition that knows no age, ethnic, or racial boundaries " it can affect young children to older adults of all races and ethnicities.
Dystonia results from abnormal functioning of the basal ganglia, a deep part of the brain which controls coordination of movement. These regions of the brain control the speed and fluidity of movement and prevent unwanted movements. Patients with dsytonia may experience uncontrollable twisting, spasms, repetitive movements, or abnormal postures and positions. Dystonia can affect any part of the body and may cause impairment when muscle contractions interfere with normal bodily functions. The condition is usually not associated with pain, but it certainly can lead to pain in affected areas, particularly when the neck is involved.
Dystonia is classified by three main factors: the age at which symptoms develop; the areas of the body affected; and the underlying cause.
The likelihood that dystonia will affect multiple body parts is generally linked to the age of onset. The younger one is at onset, the greater the chance that symptoms will spread. Conversely, the older one is at onset, the more likely that the disorder will remain more moderate.
Dystonia classification by age:
"¢Childhood onset " 0 to age 12"¢Adolescent onset " age 13 to 20"¢Adult onset " older than age 20
Dystonia classification by body part:
Focal Dystonia " is limited to one area of the body and can affect the neck (cervical dystonia or spasmodic torticollis), eyes (blepharospasm), jaw/mouth/lower face (oromandibular dystonia), vocal cords (laryngeal dystonia), or arms/legs (limb dystonia). It more commonly affects people in their 40s and 50s and is frequently referred to as adult-onset dystonia. Women are affected about three times more frequently than men. In general, focal dystonias are classified as primary (idiopathic) and are not hereditary.
Segmental Dystonia " affects two or more parts of the body that are adjacent or close to one another. Up to 30 percent of people with focal dystonia have spasms in areas adjacent to the primary site. A common form of this segmental dystonia affects the eyelids, jaw, mouth, and lower face.
Other types of dystonia include multifocal, which involves two or more body parts distant from one another; hemidystonia, which affects half of the body; and generalized, which begins with leg involvement, but generally spreads to one or more additional regions of the body.
Dystonia classification by cause:
Primary (idiopathic) " dystonia is the only sign, and secondary causes have been ruled out. In some cases, this form of dystonia is linked to specific gene mutations.
Secondary (symptomatic) " dystonia that results primarily from environmental causes, from underlying diseases which may or may not be genetic, or from brain or spinal cord injuries.
Dystonia-plus syndromes " dystonia that results from nondegenerative, neurochemical disorders associated with other neurological conditions, such as myoclonus or parkinsonism.
Heredodegenerative dystonia " dystonia that generally results from neurodegenerative disorders in which other neurological symptoms are present and in which heredity plays a role.
"While there are commonalities to symptoms, each patient experiences dystonia somewhat uniquely, and treatment options must be approached on an individualized basis. There are many organizations that provide resources for patients with dystonia, such as local support groups and Web forums," said Alex B. Valadka, MD, FACS, AANS spokesperson. "Accessing these services may be helpful for patients and family members."
There is a three-tiered approach to treating dystonia: botulinum toxin (botox) injections, 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.
Surgery may be 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 centers in the brain. Deep brain stimulation (DBS) has been tried recently with some success. Other surgeries include cutting nerves deep in the neck where they lead to the nerve roots close to the spinal cord (anterior cervical rhizotomy) or removing the nerves at the point where 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.
To find out more information about neurological disorders or to locate a qualified neurosurgeon in your area, visit: http://www.NeurosurgeryToday.org.
Founded in 1931 as the Harvey Cushing Society, the American Association of Neurological Surgeons (AANS) is a scientific and educational association with more than 6,800 members worldwide. The AANS is dedicated to advancing the specialty of neurological surgery in order to provide the highest quality of neurosurgical care to the public. All active members of the AANS are certified by the American Board of Neurological Surgery, the Royal College of Physicians and Surgeons (Neurosurgery) of Canada or the Mexican Council of Neurological Surgery, AC. Neurological surgery is the medical specialty concerned with the prevention, diagnosis, treatment and rehabilitation of disorders that affect the entire nervous system, including the spinal column, spinal cord, brain, and peripheral nerves.
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