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Dystonia

Dystonia (literally, "abnormal muscle tone") is a generic term used to describe a neurological movement disorder involving involuntary, sustained muscle contractions. Dystonia may affect muscles throughout the body (generalised), in certain parts of the body (segmental), or may be confined to particular muscles or muscle groups (focal). more...

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Causes

Primary dystonia is caused by a pathology of the central nervous system, likely originating in those parts of the brain concerned with motor function, such as the basal ganglia. The precise cause of primary dystonia is unknown. In many cases it probably involves some genetic predisposition towards the disorder combined with environmental conditions.

Secondary dystonia refers to dystonia brought on by some identified cause, usually involving brain damage or chemical imbalance. Some cases of (particularly focal) dystonia are brought on after trauma, are induced by certain drugs (tardive dystonia), or may be the result of diseases of the nervous system such as Wilson's disease.

Symptoms

Symptoms vary according to the kind of dystonia involved. In all cases, dystonia tends to lead to abnormal posturing, particularly on movement. For many sufferers, pain is also a feature of the condition.

Types of Dystonia

  • Generalised
  • Segmental
  • intermediate

The Focal Dystonias

These are the most common dystonias and tend to be classified as follows:

  • Cervical dystonia (spasmodic torticollis). This affects the muscles of the neck, causing the head to rotate to one side, to pull down towards the chest, or back, or a combination of these postures.
  • Blepharospasm. This affects the muscles around the eyes. The sufferer experiences rapid blinking of the eyes or even their forced closure causing effective blindness.
  • Oromandibular dystonia. This affects the muscles of the jaw and tongue, causes distortions of the mouth and tongue.
  • Spasmodic dysphonia. This affects the muscles of the larynx, causing the voice to sound broken or reducing it to a whisper.

The combination of blepharospasmodic contractions and oromandibular dystonia is called Meige's syndrome.

Treatment

Drugs, such as anticholinergics which act as an inhibitor of the neurotransmitter acetylcholine, may provide some relief. However, for most sufferers their effects are limited. Botulinum toxin injections into affected muscles have proved quite successful in providing some relief for around 3-6 months, depending on the kind of dystonia. The injections have to be repeated and around 15% of recipients develop immunity to the toxin. Surgery, such as the denervation of selected muscles, may also provide some relief. Recently, the procedure of deep brain stimulation has proved successful in a number of cases of severe generalised dystonia.

One type of Dystonia, Dopa-Responsive Dystonia can be completely treated with regular doses of Levadopa/Carbidopa (Simnet). Although this doesn't remove the condition, it does alleviate the symptoms most of the time.

Read more at Wikipedia.org


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Atypical laryngeal dystonia caused by an antiemetic
From American Family Physician, 4/1/04 by Oliver Freudenreich

TO THE EDITOR: Antiemetics that block dopamine receptors (such as metoclopramide or prochlorperazine) are known to potentially cause all of the side effects associated with antipsychotic medications: akathisia, extra-pyramidal side effects, and acute dystonic reactions. (1) Acute dystonic reactions are often dramatic and are potentially life threatening if the closing of the larynx causes asphyxia. Reports of milder or atypical variants of this reaction are rare. (2) This case report describes a patient who has throat discomfort and aphonia as atypical observations of laryngeal dystonia. These subtle manifestations often may be overlooked.

A 36-year-old woman with no history of psychiatric problems and no previous exposure to antipsychotic drugs was prescribed prochlorperazine, 10 mg four times daily, for residual nausea following aborted treatment with erythromycin for upper respiratory symptoms. She had taken three doses of prochlorperazine over 24 hours when she had to stop lecturing her college class because her voice gave out and became a mere whisper. That evening she made herself some hot tea for her "throat discomfort" and "tired voice." Incidentally overhearing this woman describe her day and symptoms to her husband, I was concerned that she might be experiencing an acute dystonic reaction. I advised her to take two 25-mg diphenhydramine tablets from her medicine cabinet immediately and repeat the dose one hour later. She described no other symptoms such as muscle stiffness, neck stiffness, difficulty breathing, or problems with her eyes. Several hours later, the throat discomfort had completely resolved, and she had no further difficulties with her voice. She continued taking 50 mg of diphenhydramine twice daily for another three days.

The time-course and treatment-response of this patient's symptoms are highly suggestive of acute dystonia.As opposed to the treatment given in her case, the optimal treatment of an acute dystonic reaction involves administering parenteral benztropine or parenteral diphenhydramine. (3) Once successfully begun, the anticholinergic or antihistaminergic treatment should be continued orally for another two or three days to prevent recurrence.

I proffer the term "Hot Cup-Of-Tea Sign" for subtle laryngeal dystonia experienced merely as throat discomfort. Use of this term might help to increase the recognition of manifestations other than oculogyric crisis and opisthotonus for acute dystonic reactions. The small but definite risk of the serious reaction of laryngeal dystonia with antidopaminergic antiemetics should be considered before using these agents. Most nonpsychiatric patients are by default neuroleptic-naive and, thus, potentially sensitive to dopamine blockers.

OLIVER FREUDENREICH, M.D.

MGH Schizophrenia Program

Freedom Trail Clinic

25 Staniford St., 2d Fl.

Boston, MA 02114

REFERENCES

(1.) Miller LG, Jankovic J. Drug-induced dyskinesias: an overview. In: Joseph AB, Young RR, eds. Movement disorders in neurology and neuropsychiatry. 2d ed. Malden, Mass.: Blackwell Science, 1999:5-30.

(2.) Koek RJ, Pi EH. Acute laryngeal dystonic reactions to neuroleptics. Psychosomatics 1989;30:359-64.

(3.) Arana GW, Hyman SE, Rosenbaum JF. Handbook of psychiatric drug therapy. 4th ed. Philadelphia, Pa.: Lippincott Williams & Wilkins, 2000.

COPYRIGHT 2004 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group

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