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 a causal or pathologically distinct 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][3]
| Chorea gravidarum | |
|---|---|
| Specialty | Neurology, obstetrics  |
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.[citation needed]
Pathophysiology
editSeveral pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. History of either rheumatic fever or chorea is suspected:[2][3] 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.[4][5] 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][3]
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 streptococcal infections, but had anti-basal ganglia antibodies, suggesting immunological basis for the pathophysiology of this chorea.[6][7]
Diagnosis
editDifferential diagnoses
edit- Pelizaeus–Merzbacher disease
- Pantothenate kinase-associated neurodegeneration
- Ramsay Hunt syndrome
- Huntington's 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 lipofuscinosis
- Wilson's disease
- Olivopontocerebellar atrophy
- Familial paroxysmal choreoathetosis
- Benign hereditary chorea[8]
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.[citation needed]
Treatment
editDrug treatment is indicated for patients with severe disabling chorea. It is treated with haloperidol,[9][10][11] chlorpromazine alone or in combination with diazepam, and also pimozide, which is another neuroleptic drug which may have fewer adverse effects than haloperidol.[12] Valproic acid, chloral hydrate, risperidone, or phenobarbital can also be used.[13]
See also
editReferences
edit- ^ Zegart, K. N.; Schwarz, R. H. (1968). "Chorea gravidarum". Obstetrics and Gynecology. 32 (1): 24–7. PMID 5742087.
- ^ a b c Willson, Prentiss (1932). "Chorea Gravidarum". Archives of Internal Medicine. 49 (3): 471–533. doi:10.1001/archinte.1932.00150100128011.
- ^ a b c Willson, Prentiss (1932). "Chorea Gravidarum". Archives of Internal Medicine. 49 (4): 671–97. doi:10.1001/archinte.1932.00150110134008.
- ^ Black, M (1900). "Two cases of chorea in pregnancy". Glasgow Medical Journal: 441–4.
- ^ Matthews, AA (1911). "Chorea complicating pregnancy". Northwest Med. 15: 372.
- ^ Miranda, M; Cardoso, F; Giovannoni, G; Church, A (2004). "Oral contraceptive induced chorea: Another condition associated with anti-basal ganglia antibodies". Journal of Neurology, Neurosurgery, and Psychiatry. 75 (2): 327–8. doi:10.1136/jnnp.2003.019851. PMC 1738905. PMID 14742621.
- ^ Archelos, Juan J.; Hartung, Hans-Peter (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. S2CID 7819141.
- ^ Chorea Gravidarum at eMedicine
- ^ Axley, John (1972). "Rheumatic chorea controlled with haloperidol". The Journal of Pediatrics. 81 (6): 1216–7. doi:10.1016/S0022-3476(72)80272-5. PMID 4643046.
- ^ Patterson, John F. (1979). "Treatment of Chorea Gravidarum with Haloperidol". Southern Medical Journal. 72 (9): 1220–1. doi:10.1097/00007611-197909000-00044. PMID 472859.
- ^ Donaldson, J. O. (1982). "Control of chorea gravidarum with haloperidol". Obstetrics and Gynecology. 59 (3): 381–2. PMID 7078886.
- ^ Shannon, K. M.; Fenichel, G. M. (1990). "Pimozide treatment of Sydenham's chorea". Neurology. 40 (1): 186. doi:10.1212/wnl.40.1.186. PMID 2296371. S2CID 38192455.
- ^ Chorea Gravidarum~treatment at eMedicine