brain, spinal cord, and nerve disorders
Dystonia
Types and Symptoms of Dystonia
Muscles in the affected part of the body contract, distorting the position of that body part, and stay contracted for many minutes to hours. Dystonias may affect One part of the body (focal dystonias) Two or more parts of the body that are next to each other (segmental dystonias) Two or more parts that are not next to each other (multifocal dystonias) The trunk plus two different body parts (generalized dystonias) Focal dystonias affect one body part. They typically start after age 20 to 30, but they sometimes start earlier. Segmental dystonias affect several body parts that are next to each other. Initially, contractions (spasms) may occur periodically or related to a particular activity. Certain movements of the affected body part may trigger the spasms, which may disappear during rest. Over days, weeks, or many years, spasms may become more frequent and may continue during rest. Eventually, the affected body part may remain distorted, sometimes in a painful position. As a result, people may be severely disabled. The following are examples of focal or segmental dystonias: Blepharospasm : Cervical Dystonia : Spasmodic dysphonia:The muscles of the vocal cords, which control speech, contract involuntarily. Speech may be impossible or may sound strained, quavery, hoarse, whispery, jerky, creaky, staccato, or garbled and be difficult to understand. Occupational dystonias:These dystonias, also called task-specific dystonias, affect one part of the body and often result from overuse. For example, golfers may have involuntary muscle spasms in the hands and wrists (called the yips). The yips may make putting nearly impossible. What is supposed to be a 3-foot putt can become a 15-foot putt when a golfer loses control because of the yips. Similarly, musicians, especially concert pianists, may have bizarre spasms of the fingers, hands, or arms that prevent them from performing. Musicians who play wind instruments may have spasms of the mouth. Persistent writer’s cramp may be dystonia. Meige disease:This dystonia combines involuntary blinking with jaw grinding and grimacing. Thus, it is also called blepharospasm-oromandibular dystonia. (“Blepharo” refers to the eyelids, “oro” refers to the mouth, and “mandibular” refers to the jaw.) It usually begins in late middle age. These dystonias affect the trunk plus two different body parts. They include the following: Primary generalized dystonia:This rare dystonia, also called idiopathic torsion dystonia, is progressive and often hereditary. In many cases, specific genetic mutations have been identified. The gene most commonly affected is theDYT1gene. The resulting dystonia is calledDYT1dystonia. Involuntary movements result in sustained, often bizarre postures. Typically, symptoms begin during childhood, often with turning the foot in during walking. The dystonia may affect only the trunk or a leg but often affects the whole body, ultimately confining children to a wheelchair. When this dystonia develops in adults, it usually begins in the face or arms and usually does not affect other parts of the body. Mental function is not affected. This rare form of dystonia is hereditary. Symptoms of dopa-responsive dystonia usually begin during childhood. Typically, one leg is affected first. As a result, children tend to walk on tiptoes. Symptoms worsen at night. Walking becomes progressively more difficult, and both arms and legs are affected. However, some children have only mild symptoms, such as muscle cramps after exercise. Sometimes symptoms appear later in life and resemble those of Symptoms . Movements may be slow, balance may be difficult to maintain, and a tremor may occur in the hands during rest. Symptoms lessen dramatically when people are given low doses of Drugs used to treat nausea or psychosis sometimes cause abnormal sustained muscle contractions (dystonias). Botulinum toxin, also used to treat facial wrinkles, is used to treat some dystonias. 