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- Dystonia (24)
- Blepharospasm (8)
- Torsion Dystonia (1)
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Dystonia
From Wikipedia, the free encyclopedia
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| ICD-10 | G24.9 | |
|---|---|---|
| ICD-9 | 333 | |
| DiseasesDB | 17912 | |
| MeSH | C10.597.350.300 | |
Dystonia is a neurological movement disorder in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures.[1] The disorder may be inherited or caused by other factors such as birth-related or other physical trauma, infection or reaction to drugs.[1]
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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, and the GABA producing Purkinje neurons. The precise cause of primary dystonia is unknown. In many cases it may involve 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 by some unidentified cause such as 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 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 due to muscle spasms is also a feature of the condition. Dystonia video: [1]
- Generalised
- Segmental
- Intermediate
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.
- Oculogyric crisis. An extreme and sustained (usually) upward deviation of the eyes often with convergence causing diplopia. It is frequently associated with backwards and lateral flexion of the neck and either widely opened mouth or jaw clenching. Frequently a result of antiemetics such as the neuroleptics (e.g. prochlorperazine) or metoclopramide.
- 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.
Drugs, such as anticholinergics which act as an inhibitor of the neurotransmitter acetylcholine, may provide some relief. Clonazepam, an anti-seizure medicine, is also sometimes prescribed. 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. There is a Type A and Type B toxin approved for treatment of dystonia; often those that develop resistance to Type A may be able to use Type B.[2]
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.[3]
One type of dystonia, dopa-responsive dystonia can be completely treated with regular doses of L-dopa in a form such as Sinemet (carbidopa/levodopa). Although this doesn't remove the condition, it does alleviate the symptoms most of the time.
A baclofen pump has been used to treat patients of all ages exhibiting muscle spasticity along with dystonia. The pump delivers 20/40mg of baclofen to the spinal chord in the cervical region. The pump is placed below muscle dura of the abdomen.[4]
Physical therapy can sometimes help with focal dystonia. A structured set of exercises are tailored to help the affected area.
- ^ a b Dystonia fact sheet: National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/disorders/dystonias/detail_dystonias.htm
- ^ Brin MF, Lew MF, Adler CH, Comella CL, Factor SA, Jankovic J, O'Brien C, Murray JJ, Wallace JD, Willmer-Hulme A, Koller M (1999). "Safety and efficacy of NeuroBloc (botulinum toxin type B) in type A-resistant cervical dystonia". Neurology 53 (7): 1431-8. PMID 10534247.
- ^ Bittar RG, Yianni J, Wang S, Liu X, Nandi D, Joint C, Scott R, Bain PG, Gregory R, Stein J, Aziz TZ (2005). "Deep brain stimulation for generalised dystonia and spasmodic torticollis". J Clin Neurosci 12 (1): 12-6. PMID 15639404.
- ^ Jankovic, Dr. Joseph; Dr. Eduardo Tolosa (2007). Parkinson's Disease & Movement Disorders, fifth edition, Philadelphia, Penn.: Lippincott Williams & Wilkins, pp. 349-350. ISBN 0-7817-7881-6.