Chorea Gravidarum Workup
- Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP; Chief Editor: Selim R Benbadis, MD more...
Laboratory Studies
- Maintain a high index of suspicion and vigilance when making the diagnosis of chorea gravidarum and considering the differential diagnosis.
- Acute rheumatic fever- Erythrocyte sedimentation rate (ESR), throat culture, C-reactive protein, and ASO titer
- Wilson disease - Serum ceruloplasmin and urinary copper (24 h)
- Systemic lupus erythematosus - ESR, antinuclear antibody, anticardiolipin antibodies, and lupus anticoagulant assays
- Phenothiazine reaction history - Therapeutic trial of intravenous (IV) benztropine
- Polycythemia - CBC, hemoglobin, and hematocrit
- Hyperthyroidism - Thyroxine (T4), thyroid-stimulating hormone (TSH)
- Hypoparathyroidism - Serum calcium and phosphate
- Vascular disease
- Hypercoagulability of pregnancy; investigations for hyperlipidemia, diabetes, valvular heart disease, hyperviscosity states, hemoglobinopathies, or congenital cerebrovascular disease (moyamoya)
- In a young patient with cerebral infarction, in the absence of hypertension and atrial fibrillation, vasculitides and thrombophilic tendencies must be considered. Testing for thrombophilia with estimation of anticardiolipin antibody, antithrombin III levels, prothrombin gene, protein S, protein C resistance, and factor V Leiden should be considered.
- Meningovascular syphilis - Venereal Disease Research Laboratory test (VDRL), fluorescent treponemal antibody absorption test (FTA-ABS)
- Drugs - Serum levels of anticonvulsants, theophylline, lithium, and tricyclic antidepressants
- Drug toxicity due to amphetamine and cocaine - Serum levels and urine screening
- Lead toxicity - Serum lead level
- Neuroacanthocytosis peripheral smear for acanthocytes
- Adult-onset Tay-Sachs disease - Assay of serum lysosomal enzymes
- Husby has described antineuronal antibodies using an immunofluorescent technique in 46% of patients with Sydenham chorea (n = 30) compared with 14% of patients with rheumatic fever (without chorea) (n = 50) and only 1.8-4% of control subjects (n = 203). He further demonstrated a potential correlation between antibody reactivity and the clinical status, with antibody disappearance on chorea remission.[38]
Imaging Studies
- Hypoparathyroidism: CT scan may reveal bilateral basal ganglia calcificans.
- MRI
- Huntington disease (MRI of the brain to exclude caudate atrophy) and neuroacanthocytosis
- Wilson disease - Striatal damage
- Systemic lupus erythematosus, locular infarcts - Small arterial damage
- Rare basal ganglia tumor
Other Tests
- Obtain ECG whenever a suspicion of rheumatic fever exists to exclude carditis. EEG may show evidence of rheumatic encephalopathy.
- Perform a slit-lamp examination to rule out Kayser-Fleischer rings that would indicate Wilson disease.
Kranick SM, Mowry EM, Colcher A, Horn S, Golbe LI. Movement disorders and pregnancy: a review of the literature. Mov Disord. Apr 30 2010;25(6):665-71. [Medline].
Willson P, Preece AA. Chorea gravidarum. Arch Intern Med. 1932;49:471-533, 671-697.
Zegart KN, Schwarz RH. Chorea gravidarum. Obstet Gynecol. Jul 1968;32(1):24-7. [Medline].
Dike GL. Chorea gravidarum: a case report and review. Md Med J. Sep 1997;46(8):436-9. [Medline].
Greenfield JG, Wolfsohn JM. The pathology of Sydenham's chorea. Lancet. 1922;2:603-606.
Winkelman NW, Eckel JL. The brain in acute rheumatic fever. Arch Neurol Psych. 1932;28:844-870.
Unno S, Iijima M, Osawa M, et al. [A case of chorea gravidarum with moyamoya disease]. Rinsho Shinkeigaku. Apr 2000;40(4):378-82. [Medline].
Koide R, Sakamoto M, Tanaka K, Hayashi H. Opsoclonus-myoclonus syndrome during pregnancy. J Neuroophthalmol. Sep 2004;24(3):273. [Medline].
Black M. Two cases of chorea in pregnancy. Glasgow Med J. 1900;441-444.
Matthews AA. Chorea complicating pregnancy. Northwest Med. 1911;15:372.
Taranta A, Stollerman GH. The relationship of Sydenham's chorea to infection with group A streptococci. Am J Med. Feb 1956;20(2):170-5. [Medline].
TARANTA A. Relation of isolated recurrences of Sydenham's chorea to preceding streptococcal infections. N Engl J Med. Jun 11 1959;260(24):1204-10. [Medline].
Jonas S, Spagnuolo M, Kloth HH. Chorea gravidarum and streptococcal infection. Obstet Gynecol. Jan 1972;39(1):77-9. [Medline].
ARON AM, FREEMAN JM, CARTER S. THE NATURAL HISTORY OF SYDENHAM'S CHOREA. REVIEW OF THE LITERATURE AND LONG-TERM EVALUATION WITH EMPHASIS ON CARDIAC SEQUELAE. Am J Med. Jan 1965;38:83-95. [Medline].
Warren HA, Chornyak J. Cerebral manifestations of acute rheumatic fever. Arch Int Med. 1947;79:589-601.
CHUN RW, SMITH NJ, FORSTER FM. Papilledema in Sydenham's chorea. Am J Dis Child. May 1961;101:641-4. [Medline].
Usher SJ, Jasper HH. The etiology of Sydenham's chorea:electroencephalographic studies. CMAJ. 1941;44:365-371.
Buchanan DN, Walker AE, Case TJ. The pathogenesis of chorea. J Pediatr. 1942;20:555-575.
Miranda M, Cardoso F, Giovannoni G, Church A. Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies. J Neurol Neurosurg Psychiatry. Feb 2004;75(2):327-8. [Medline].
Dale RC. Autoimmunity and the basal ganglia: new insights into old diseases. QJM. Mar 2003;96(3):183-91. [Medline].
Archelos JJ, Hartung HP. Pathogenetic role of autoantibodies in neurological diseases. Trends Neurosci. Jul 2000;23(7):317-27. [Medline].
Wild EJ, Tabrizi SJ. The differential diagnosis of chorea. Pract Neurol. Nov 2007;7(6):360-73. [Medline].
Brett WR. Some paediatric eponyms: IV. Sydenham's Chorea. Br J Child Dis. 1932;29:283.
Marques-Dias MJ, Mercadante MT, Tucker D, Lombroso P. Sydenham's chorea. Psychiatr Clin North Am. Dec 1997;20(4):809-20. [Medline].
Kiessling LS, Marcotte AC, Culpepper L. Antineuronal antibodies in movement disorders. Pediatrics. Jul 1993;92(1):39-43. [Medline].
Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry. Feb 1998;155(2):264-71. [Medline].
Dale RC, Church AJ, Surtees RA, et al. Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis. Mov Disord. Jul 2002;17(4):817-20. [Medline].
Alvarenga PG, Hounie AG, Mercadante MT, Diniz JB, Salem M, Spina G, et al. Obsessive-compulsive symptoms in heart disease patients with and without history of rheumatic fever. J Neuropsychiatry Clin Neurosci. Summer 2006;18(3):405-8. [Medline].
Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol. Aug 2004;61(8):1261-4. [Medline].
Rote NS, Dostal-Johnson D, Branch DW. Antiphospholipid antibodies and recurrent pregnancy loss: correlation between the activated partial thromboplastin time and antibodies against phosphatidylserine and cardiolipin. Am J Obstet Gynecol. Aug 1990;163(2):575-84. [Medline].
Triplet DA. New diagnostic strategies for lupus anticoagulants and antiphospholipid antibodies. Haemostasis. May-Jun 1994;24(3):155-64. [Medline].
Ramsey-Goldman R, Kutzer JE, Kuller LH, et al. Previous pregnancy outcome is an important determinant of subsequent pregnancy outcome in women with systemic lupus erythematosus. Am J Reprod Immunol. Oct-Dec 1992;28(3-4):195-8. [Medline].
Kilpatrick DC, Maclean C, Liston WA, Johnstone FD. Anti-phospholipid antibody syndrome and pre-eclampsia. Lancet. Oct 21 1989;2(8669):987-8. [Medline].
Roubey RA. Autoantibodies to phospholipid-binding plasma proteins: a new view of lupus anticoagulants and other "antiphospholipid" autoantibodies. Blood. Nov 1 1994;84(9):2854-67. [Medline].
Qasim A. An unusual case of chorea gravidarum. Postgrad Med J. Jun 2000;76(896):374, 378-9. [Medline].
Asherson RA, Cervera R, Merrill JT, Erkan D. Antiphospholipid antibodies and the antiphospholipid syndrome: clinical significance and treatment. Semin Thromb Hemost. Apr 2008;34(3):256-66. [Medline].
Kim, Ok; Kim, A; Seo, M W; Kweon, J Y; Ahn, H Y; Park, et al. Chorea gravidarum associated with Moyamoya disease. J Perinatal Med. 2007;35(Suppl II):S187.
Husby G, van de Rijn I, Zabriskie JB, et al. Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever. J Exp Med. Oct 1 1976;144(4):1094-110. [Medline].
Ichikawa K, Kim RC, Givelber H, Collins GH. Chorea gravidarum. Report of a fatal case with neuropathological observations. Arch Neurol. Jul 1980;37(7):429-32. [Medline].
Axley J. Rheumatic chorea controlled with haloperidol. J Pediatr. Dec 1972;81(6):1216-7. [Medline].
Shenker DM, Grossman HJ, Klawans HL. Treatment of Sydenham's chorea with haloperidol. Dev Med Child Neurol. Feb 1973;15(1):19-24. [Medline].
Patterson JF. Treatment of chorea gravidarum with haloperidol. South Med J. Sep 1979;72(9):1220-1. [Medline].
Donaldson JO. Control of chorea gravidarum with haloperidol. Obstet Gynecol. Mar 1982;59(3):381-2. [Medline].
Magnier P. [Apropos of vomiting in pregnancy: therapeutic study of R 1625]. Gynecol Prat. 1964;15(1):17-23. [Medline].
Van Waes A, Van de Velde E. Safety evaluation of haloperidol in the treatment of hyperemesis gravidarum. J Clin Pharmacol. 1969;9:224.
Shannon KM, Fenichel GM. Pimozide treatment of Sydenham's chorea. Neurology. Jan 1990;40(1):186. [Medline].
Pallares AJ, Hurtado L. (Translated title) Carbamazepine in paroxysmal choreoathetosis in Sydenham's chorea. An Esp Pediatr (Anales espanoles pediatrica). 1989;30 (1):41-44.
Pulsinelli WA, Hamill RW. Chorea complicating oral contraceptive therapy. Case report and review of the literature. Am J Med. Sep 1978;65(3):557-9. [Medline].
Nausieda PA, Koller WC, Weiner WJ, Klawans HL. Chorea induced by oral contraceptives. Neurology. Dec 1979;29(12):1605-9. [Medline].
Beresford OD, Graham AM. Chorea gravidarum. J Obstet Gynaecol Br Emp. Aug 1950;57(4):616-25. [Medline].
Fleischman MJ. A case report of chorea gravidarum complicating five pregnancies. Am J Obstet Gynecol. Dec 1953;66(6):1328-30. [Medline].
Ghanem Q. Recurrent chorea gravidarum in four pregnancies. Can J Neurol Sci. May 1985;12(2):136-8. [Medline].
BEAN WB, COGSWELL R. Vascular changes of the skin in pregnancy; vascular spiders and palmar erythema. Surg Gynecol Obstet. Jun 1949;88(6):739-52. [Medline].
Cardoso F, Eduardo C, Silva AP, Mota CC. Chorea in fifty consecutive patients with rheumatic fever. Mov Disord. Sep 1997;12(5):701-3. [Medline].
Donaldson JO. Movement disorders. In: Neurology of Pregnancy. Major Problems In Neurology. Vol 7. Philadelphia: WB Saunders Co; 1978:87-97.
Forrest AD, Hales IB. Severe chorea gravidarum treated with corticotrophin. Lancet. Oct 27 1956;271(6948):874-5. [Medline].
Markham CH, Ansel RD. Drugs and motor behaviour. In: Jarvik ME, ed. Psychopharmacology in the Practice of Medicine. NY: Appleton Century Crofts; 1977:127-139.
Wall C, Andrews HR. On chorea in pregnancy. J Obstet Gynaecol Br. 1903;3:540-547.
Winkelbauer RG, Kimsey LR. Chorea gravidarum treated with chlorpromazine; case report. Am J Obstet Gynecol. Jun 1956;71(6):1353-4. [Medline].

