Chorea gravidarum

Chorea gravidarum
Classification and external resources
ICD-10	G25.5, O99.3
eMedicine	neuro/61
MeSH	D020150

Chorea gravidarum is a rare^[1] type of chorea which presents with involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. It is a complication of pregnancy which can be associated with eclampsia and its effects upon the basal ganglia. It is not an etiologically or pathologically distinct morbid entity but a generic term for chorea of any cause starting during pregnancy. It is associated with history of Sydenham's chorea. It mostly occurs in young patients; the average age is 22 years.^[2]

Recently there has been a decline in incidence which is probably the result of a decline in rheumatic fever (RF), which was a major cause of chorea gravidarum before the use of antibiotics for streptococcal pharyngitis.

Pathophysiology

Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. History of either rheumatic fever or chorea is suspected^[2]: the suggestion is that estrogens and progesterone may sensitize dopamine receptors (presumably at a striatal level) and induce chorea in individuals who are vulnerable to this complication by virtue of preexisting pathology in the basal ganglia. The relation to rheumatic fever was strengthened by many studies that showed that women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies.^[3][4] At least 35% of patients have a definite history of acute rheumatic fever and Sydenham chorea; 4% of those with chorea gravidarum had acute rheumatic fever.^[2]

It has been suggested that use of oral contraceptives is an infrequent cause of chorea. A patient developed this chorea with no definite evidence of previous Sydenham's chorea or recent streptoccocal infections, but had anti-basal ganglia antibodies, suggesting immunological basis for the pathophysiology of this chorea.^[5][6]

Differential Diagnoses

Cerebellar Hemorrhage Pelizaeus-Merzbacher Disease Hallervorden-Spatz Disease Ramsay Hunt Syndrome Huntington Disease Striatonigral Degeneration Lesch-Nyhan Syndrome

Systemic Lupus Erythematosus Lyme Disease Torticollis Multiple System Atrophy Tourette Syndrome and Other Tic Disorders Neuroacanthocytosis

Viral Encephalitis Neuronal Ceroid Lipofuscinoses Wilson disease Olivopontocerebellar Atrophy Familial paroxysmal choreoathetosis Benign hereditary chorea[7]

Chorea can also be a manifestation of drug toxicity (for example anticonvulsants, antiparkinson agents, neuroleptics, steroids, and estrogen), or a result of an infectious disease such as meningovascular syphilis, Lyme disease, viral encephalitis, and many others.

Treatment

Drug treatment is indicated for patients with severe disabling chorea. It is treated with haloperidol^[8][9][10], chlorpromazine alone or in combination with diazepam, and also pimozide, which is another neuroleptic drug which may have fewer adverse effects than haloperidol^[11]. Valproic acid, chloral hydrate, risperidone, or phenobarbital can also be used.^[12]

See also

• chorea

References

1. ^ Zegart KN, Schwarz RH (July 1968). "Chorea gravidarum". Obstetrics and Gynecology 32 (1): 24–7. PMID 5742087. 
2. ^ ^{a b c} Willson P, Preece AA. Chorea gravidarum. Arch Intern Med. 1932;49:471-533, 671-697.
3. ^ Black M. Two cases of chorea in pregnancy. Glasgow Med J. 1900;441-444.
4. ^ Matthews AA. Chorea complicating pregnancy. Northwest Med. 1911;15:372.
5. ^ Miranda M, Cardoso F, Giovannoni G, Church A (February 2004). "Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies". Journal of Neurology, Neurosurgery, and Psychiatry 75 (2): 327–8. PMC 1738905. PMID 14742621. http://jnnp.bmj.com/cgi/pmidlookup?view=long&pmid=14742621. 
6. ^ Archelos JJ, Hartung HP (July 2000). "Pathogenetic role of autoantibodies in neurological diseases". Trends in Neurosciences 23 (7): 317–27. doi:10.1016/S0166-2236(00)01575-7. PMID 10856942. http://linkinghub.elsevier.com/retrieve/pii/S0166-2236(00)01575-7. 
7. ^ http://emedicine.medscape.com/article/1149725-diagnosis
8. ^ Axley J (December 1972). "Rheumatic chorea controlled with haloperidol". The Journal of Pediatrics 81 (6): 1216–7. doi:10.1016/S0022-3476(72)80272-5. PMID 4643046. 
9. ^ Patterson JF (September 1979). "Treatment of chorea gravidarum with haloperidol". Southern Medical Journal 72 (9): 1220–1. PMID 472859. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0038-4348&volume=72&issue=9&spage=1220. 
10. ^ Donaldson JO (March 1982). "Control of chorea gravidarum with haloperidol". Obstetrics and Gynecology 59 (3): 381–2. PMID 7078886. 
11. ^ Shannon KM, Fenichel GM (January 1990). "Pimozide treatment of Sydenham's chorea". Neurology 40 (1): 186. PMID 2296371. 
12. ^ http://emedicine.medscape.com/article/1149725-treatment

External links

• Palanivelu LM (2007). "Chorea gravidarum". Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology 27 (3): 310. doi:10.1080/01443610701241134. PMID 17464821.