Chorea,or (rarely)choreia,(/kəˈriə/) is an abnormal involuntarymovement disorder,characterized by quick movements of the hands or feet. It is one of a group of neurological disorders calleddyskinesias.The termchoreais derived fromAncient Greekχορεία(choreia)'dance', as the movements of the body is comparable to dancing.
| Chorea | |
|---|---|
| Specialty | Neurology |
The termhemichorearefers to chorea of one side of the body, such as chorea of one arm but not both (analogous tohemiballismus).
Presentation
editChorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next. These 'dance-like' movements of chorea often occur withathetosis,which adds twisting and writhing movements. Walking may become difficult and include odd postures and leg movements. Unlikeataxia,which affects the quality of voluntary movements, orParkinsonism,which is a hindrance of voluntary movements, the movements of chorea andballismoccur on their own, without conscious effort. Thus, chorea is said to be ahyperkineticmovement disorder. When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to asballism,or ballismus.[citation needed]
Causes
editHuntington's disease
editHuntington's diseaseis a neurodegenerative disease and most common inherited cause of chorea. The condition was formerly called Huntington's chorea but was renamed because of the important non-choreic features includingcognitivedecline and behavioural change.[1]
Other genetic causes
editOther genetic causes of chorea are rare. They include the classical Huntington's disease 'mimic' orphenocopysyndromes, calledHuntington's disease-like syndrometypes 1, 2 and 3; inheritedprion disease,thespinocerebellar ataxiastype 1, 3 and 17,neuroacanthocytosis,dentatorubral-pallidoluysian atrophy(DRPLA),brain iron accumulation disorders,Wilson's disease,benign hereditary chorea,Friedreich's ataxia,mitochondrial diseaseandRett syndrome.[2]
Acquired causes
editThe most common acquired causes of chorea arecerebrovascular diseaseand, in the developing world,HIVinfection—usually through its association withcryptococcal disease.[2]
Sydenham's choreaoccurs as a complication of streptococcal infection. Twenty percent (20%) of children and adolescents withrheumatic feverdevelop Sydenham's chorea as a complication. It is increasingly rare, which may be partially due to penicillin, improved social conditions, and/or a natural reduction in the bacteria (Streptococcus) it has stemmed from. Psychological symptoms may precede or accompany this acquired chorea and may be relapsing and remitting. The broader spectrum of paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection can cause chorea and are collectively referred to asPANDAS.[2]
Chorea gravidarumrefers to choreic symptoms that occur during pregnancy. If left untreated, the disease resolves in 30% of patients before delivery but, in the other 70%, it persists. The symptoms then progressively disappear in the next few days following the delivery.[2]
Chorea may also be caused by drugs (commonlylevodopa,anti-convulsantsandanti-psychotics).[2]
Other acquired causes includeCSF leak,[3]systemic lupus erythematosus,antiphospholipid syndrome,thyrotoxicosis,polycythaemia rubra vera,[2]transmissible spongiform encephalopathies,coeliac disease[4]andgluten ataxia.
Treatment
editThere is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Although there are many drugs that can control it, no cure has yet been identified.
| Form | Treatment |
|---|---|
| Huntington's-related | A common treatment isdopaminergic antagonists,although treatment is largely supportive.Tetrabenazine,deutetrabenazineandvalbenazineare FDA-approved drugs for the treatment of Huntington's disease-related chorea. |
| Sydenham's chorea | Haloperidol,carbamazepineandvalproic acid.Usually involvesantibioticdrugs to treat the infection, followed by drug therapy to prevent recurrence. |
| Chorea gravidarum | haloperidol,[5][6][7]chlorpromazinealone or in combination withdiazepam,alsopimozidecan also be used. |
| Wilson's disease | Reducing levels of copper in the body using D-penicillamine,trientine hydrochloride,tetrathiomolybdate,and otherchelatingagents |
| Drug-induced chorea | Adjusting medication dosages. |
| Metabolic and endocrine-related choreas | Treated according to their causes. |
History
editHistorically, choreas like Huntington disease and Sydenham's chorea were calledSaint Vitus' dance,related to a series ofsocial phenomena of the same name.[8]
See also
editReferences
edit- ^Gillian, Bates; Sarah, Tabrizi; Lesley, Jones (2014).Huntington's disease(4th ed.). Oxford University Press.ISBN978-0199929146.
- ^abcdefWild, EJ; Tabrizi, SJ (November 2007)."The differential diagnosis of chorea".Practical Neurology.7(6): 360–73.doi:10.1136/pn.2007.134585.PMID18024776.S2CID31059440.
- ^Mokri, Bahram (December 2014). "Movement disorders associated with spontaneous CSF leaks: a case series".Cephalalgia: An International Journal of Headache.34(14): 1134–1141.doi:10.1177/0333102414531154.ISSN1468-2982.PMID24728303.S2CID3100453.
- ^Bushara, Khalafalla O. (2005)."Neurologic presentation of celiac disease".Gastroenterology.128(4 Suppl 1): S92–7.doi:10.1053/j.gastro.2005.02.018.PMID15825133.
- ^Axley, John (1972)."Rheumatic chorea controlled with haloperidol".The Journal of Pediatrics.81(6): 1216–7.doi:10.1016/S0022-3476(72)80272-5.PMID4643046.
- ^Patterson, John F. (1979). "Treatment of Chorea Gravidarum With Haloperidol".Southern Medical Journal.72(9): 1220–1.doi:10.1097/00007611-197909000-00044.PMID472859.
- ^Donaldson JO (March 1982). "Control of choreia gravidarum with haloperidol".Obstetrics and Gynecology.59(3): 381–2.PMID7078886.
- ^Lawrence, David M. (2009).Huntington's Disease.Infobase Publishing.ISBN9780791095867.