What is Dystonia?
Dystonia is a neurological movement disorder characterized by involuntary muscle contractions, which force certain parts of the body into abnormal, sometimes painful, movements or postures.
What part of the body does Dystonia affect?
Dystonia can affect any part of the body including the arms and legs, trunk, neck, eyelids face, or vocal cords. Although there are different names to describe the various forms of Dystonia, usually based on the affected body or cause of Dystonia, all types fall under the general heading of “Dystonia.”
What causes Dystonia ?
At this time we don’t know exactly what causes Dystonia. Researchers believe Dystonia is due to abnormal functioning of the basal ganglia, which are deep brain structures involved with the control of movement. The basal ganglia assist in initiating and regulating movement. What goes wrong in the basal ganglia is still unknown. An imbalance of dopamine, a neurotransmitter in the basal ganglia, may underline several different forms of Dystonia.
What gets Dystonia ?
Dystonia does not discriminate. Anyone, regardless of race, age, or ethnicity, can manifest symptoms of Dystonia. It is the third most common neurological disorder after Parkinson’s disease and tremor.
Is Dystonia a genetic disorder?
Yes and no. At present researchers have recognized multiple forms of inheritable Dystonia and have identified at least ten genes of chromosomal locations responsible for the various manifestations. It is not yet understood if there are genes responsible for the focal of Dystonia. Also, certain forms of Dystonia are secondary resulting from an apparent outside factor and attributed to specific causes such as exposure to certain medications, trauma, toxins, infections or stroke.
Focal Dystonia
Focal Dystonia usually affect one part of the body and the most common forms include:
Blepharospasam
It affects the muscles of the eyelids forcing them to close. The spasm may become sufficiently request to render the patient unable to see, although the eyes and vision are normal.
Cervical Dystonia
It affects muscles in the neck and holders. The muscle spasms can be painful and cause the neck to twist to one side (Torticollis), forward (antecollis), or backward (retrocollis). The neck may pull, turn or jerk; it may be held persistently in one direction.
Oromandibular Dystonia
Is also called Meige’s syndrome .The muscles of the lower face irregularly pull or contract. The jaw muscle movements that pull the mouth open or closed are involuntary. Usually forced eye blink is also present.
Spasmodic Dysphonia
It involves the muscles inside the larynx or voice box. In spasmodic Dysphonia of the adductor muscles the vocal cords are drawn tightly together particularly when the person tries to talk. The voice typically has a strained, hoarse, choked quality. In spasmodic Dysphonia of the less common abductor type, the vocal cords are pulled apart and the voice has a low, breathy whispered quality; sometimes the person cannot speak at all.
Writer’s Cramp
It is an occupational dystonia in which the hand and forearm muscles contract during the act of writing. The hand may be drawn up so tightly that it cannot move. As soon as the writing instrument is removed from the hand the hand relaxes. A similar cramp may arise in a musician as the violin is bowed or certain fingers are moved in playing a flute or other instrument .Occupational cramps may occur in a wide variety of situations involving repetitive movements.
Hemifacial Spasm
It is not a Dystonia. In this disorder, the muscles on one side of the face irregularly contract. Sometimes this is secondary to inflammation or irritation of the facial nerve.
Secondary Dystonia
Secondary Dystonia results from environmental or disease -related damage to the basal ganglia .Birth injury {particularly due to lack of oxygen}, certain infections, reactions to certain drugs, trauma, or stroke can cause Dystonia symptoms. Dystonia can also be secondary to other illnesses affecting the nervous system including Wilson’s disease.
What goes wrong in dystonia?
Researchers believe that some forms of Dystonia may be caused by breakdown of the dopamine system in the basal ganglia.
A malfunctioning dopamine system in the basal ganglia is responsible for many movement disorders including Parkinson’s disease; but in contrast, in Dystonia there is no visible evidence of damage to the brain.
When Dystonia occurs secondary to certain injuries or small strokes, lesions (areas of damage) are often found in the putamen, a part of basal ganglia, as well as in certain nearby structures.
Even though we can see no microscopic abnormalities of the brain in the great majority of causes of Dystonia, including those with generalized Dystonia, the evidence is so clear in the secondary Dystonia that we believe the same part of the brain is involved in all types.
Can you die from dystonia?
No, Dystonia does not shorten life expectancy or result in death. I n rare cases complications may arise secondary to Dystonia which can cause serious medical problems; however, this is rare condition. While Dystonia is not fatal, it is a chronic disorder and prognosis is difficult to predict. If Dystonia causes any type of impairment, it is because muscle contractions interfere with normal function. Features such as cognition, strength and the senses including vision , hearing are normal.
The Diagnosis of Dystonia
Currently there is no specific laboratory test or x-ray that says whether a person has dystonia. Instead the diagnosis of Dystonia rests solely upon the neurological examination. Therefore in order to correctly diagnose dystonia, doctors must be able to recognize the physical signs and be familiar with the symptoms, particularly because when Dystonia begins often its symptoms may change significantly with different actions.
How is Dystonia Treated ?
Treatment for Dystonia is designed to help lessen the symptoms of spasms, pain, and disturbed postures and functions. Most therapies are symptomatic attempting to cover up or release the Dystonia spasms. The approach for the treatment of Dystonia may be three tiered: oral medications, botulinum toxin injections, and surgery. These therapies may be used in alone or in combination.
Are Botulin Toxin (Botox) injecton’s safe?
Yes, Boutilin toxin, a biological product, is injected into specific muscles where it acts to reduce the involuntary contractions that cause the symptoms of Dystonia. The injections weaken muscle activity sufficiently to reduce a spasm but not enough to cause paralysis.
Role of surgery in the treatment of dystonia?
Surgery may be considered when patients are no longer receptive to other treatments. Surgery is undertaken to interrupt, at various levels of the nervous system, the pathways responsible for the abnormal movements. Some operations intentionally damage small regions of the thalamus (thalamotomy), globus pallidus (Pallidotomy), or other deep centres in the brain. Recently, chronic deep brain stimulation (DBS) has been tried with some success.
How are complementary therapies helpful?
Traditional western practices have long been effective in diagnosing problems and assigning treatments but may do little to address how patients live on a daily basis. Medical fields are slowly incorporating a wider range of knowledge to include treatments outside f the traditional scope to better assist patients and to treat the “whole person”- mind, body, and spirit. Complementary therapy may play and active role in your treatment of Dystonia, and it is intended be used in conjunction with traditional therapies. To avoid any interactions and potential problems, it is important to have open communication among physicians and practitioners who are working with you.
Supportive forms of treatment
By educating yourself with information, you have taken the first step in dealing with Dystonia. Reassurance from family, friends and others who have Dystonia is beneficial.
