Chorea gravidarum is becoming a rare entity in clinical practice, but its early recognition is vital in preventing both maternal and fetal morbidity and mortality [1]. The clinical presentation is distinguished by the onset of involuntary, non-rhythmic, non-repetitive and abrupt but brief movements of any of the limbs (known as chorea) during the first trimester of pregnancy [1] [2] [3]. Based on the extent of body involvement, several forms are recognized - generalized, focal, multifocal or hemichorea (involving only one side of the body) [4]. The appearance of facial grimaces slurred speech and impaired coordination may accompany this movement disorder [1] [2] [3]. In the majority of cases, chorea is self-limiting, and spontaneously resolves after pregnancy, but rare cases of spontaneous abortion were documented [4] [5]. Huntington's disease, drug-induced chorea, Wilson's disease, thyrotoxicosis, systemic lupus erythematosus (SLE), and syphilis have all been confirmed as possible causes, but in the absence of an evident etiology, many cases are diagnosed as idiopathic [1] [5]. However, history of rheumatic fever (particularly in developing countries where it is widely present) and use of oral contraceptives, as well as antiphospholipid syndrome, are the most important causes of chorea gravidarum, and they are also potent risk factors for recurrence of chorea in subsequent pregnancies [1] [4] [6].

• Facial Grimacing

  Chorea gravidarum is a rare 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. [en.wikipedia.org]

  Chorea gravidarum Chorea gravidarum is a rare 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. [encyclo.co.uk]

  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. [ipfs.io]

• 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. [en.wikipedia.org]

  Background Chorea gravidarum (CG) is the term given to chorea occurring during pregnancy. This is not an etiologically or pathologically distinct morbid entity but a generic term for chorea of any cause starting during pregnancy. [emedicine.com]

  Chorea gravidarum. Arch Intern Med. 1932. 49:471-533, 671-697. Zegart KN, Schwarz RH. Chorea gravidarum. Obstet Gynecol. 1968 Jul. 32(1):24-7. [Medline]. Dike GL. Chorea gravidarum: a case report and review. Md Med J. 1997 Sep. 46(8):436-9. [emedicine.medscape.com]

  Heightened dopaminergic activity in the striatum is postulated as the basis for the chorea. Indications for the use of haloperidol in chorea gravidarum are discussed. [ncbi.nlm.nih.gov]

• Involuntary Movements

  The clinical picture is of extrapyramidal symptoms such as involuntary movements, lack of coordination and slurred speech. Neuroleptics or benzodiazepines can be used for treatment. [ncbi.nlm.nih.gov]

  More: Evidence Summaries Abnormal Involuntary Movements Source: Patient Abnormal involuntary movements (AIMs) are also known as dyskinesias. several varieties of dyskinesia are measured on an Abnormal Involuntary Movements scale. [evidence.nhs.uk]

  Also found in: Dictionary, Thesaurus, Encyclopedia, Wikipedia. chorea [ ko-re ah ] the ceaseless occurrence of rapid, jerky involuntary movements. adj., adj chore ic. cho·re·a grav·i·da'r·um Sydenham chorea occurring during pregnancy. chorea gravidarum [medical-dictionary.thefreedictionary.com]

• Tremor

  ( Essential tremor, Intention tremor ) · Restless legs · Stiff person Dementia Tauopathy : Alzheimer's ( Early-onset ) · Frontotemporal dementia / Frontotemporal lobar degeneration ( Pick's, Dementia with Lewy bodies ) Multi-infarct dementia Mitochondrial [thefullwiki.org]

  Werner Poewe, Joseph Jankovic Cambridge University Press, ٢٠‏/٠٢‏/٢٠١٤ Movement disorders - ranging from parkinsonism to a variety of hyperkinetic disorders, such as tremors, dystonic, chorea and myoclonus - can be the presenting or a prominent clinical [books.google.com]

  Werner Poewe, Joseph Jankovic Cambridge University Press, 20.02.2014 Movement disorders - ranging from parkinsonism to a variety of hyperkinetic disorders, such as tremors, dystonic, chorea and myoclonus - can be the presenting or a prominent clinical [books.google.de]

• Abnormal Involuntary Movement

  More: Evidence Summaries Abnormal Involuntary Movements Source: Patient Abnormal involuntary movements (AIMs) are also known as dyskinesias. several varieties of dyskinesia are measured on an Abnormal Involuntary Movements scale. [evidence.nhs.uk]

  The remaining chapters focus on the abnormal involuntary movements associated with each disease or syndrome. [books.google.com]

  This condition is characterized by abrupt, abnormal, involuntary movements that are non-rhythmic, brief and sporadic movements of any limb. These movements are commonly associated with non-patterned grimacing of the face. [epainassist.com]

• Extrapyramidal Symptoms

  The clinical picture is of extrapyramidal symptoms such as involuntary movements, lack of coordination and slurred speech. Neuroleptics or benzodiazepines can be used for treatment. [ncbi.nlm.nih.gov]

  The clinical picture is of extrapyramidal symptoms such as involuntary movements, lack of coordination and slurred speech. Neuroleptics or benzodiazepines can be used for treatment. Read More Chorea gravidarum. [pubfacts.com]

  Extrapyramidal symptoms associated with antidepressants – a review of the literature and an analysis of spontaneous reports. Ann Clin Psychiatry 2010;22:148–156. 12. [tremorjournal.org]

The diagnosis of chorea gravidarum can be made only through a meticulous and complete physical examination that will identify the characteristic abnormal movements. A clinical suspicion must be present if non-rhythmic and abrupt movements are confirmed during the first trimester of pregnancy. In that case, several laboratory tests should be performed [2], in the attempt to establish the underlying cause. Before laboratory testing, a detailed patient history can reveal key information and is a vital component of the diagnostic workup. Previous use of oral contraceptives, family history of diseases in which chorea is a symptom (for example Huntington's disease) or recent infection by streptococcus pyogenes (primarily pharyngitis) and the subsequent development of rheumatic fever may be noted, although only 20-30% of cases have documented evidence of this infection [1]. For this reason, titers of antistreptolysin O (ASO) antibodies, throat cultures, as well as levels of rheumatoid factor (RF), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and a complete blood count (CBC) are mandatory for exclusion of rheumatic fever as a possible cause [1]. Having in mind the diverse etiology of chorea gravidarum, anticardiolipin antibodies, venereal disease research laboratory (VDRL) test, anti-nuclear antibodies (ANA), ceruloplasmin and urinary copper levels, a full thyroid workup (T3, T4, thyroid-stimulating hormone, or TSH), and assessment of serum electrolytes (primarily calcium and phosphate) must be conducted [1] [2] [5] [7]. In some patients, imaging studies of the endocranium, primarily in the form of magnetic resonance imaging (MRI) may be of great benefit.

• Lymphocytic Infiltrate

  Pathologic changes found at autopsy in chorea gravidarum include perivascular degenerative changes in the caudate nucleus.Pathology of rheumatic brain disease is of a nonspecific arteritis with endothelial swelling, perivascular lymphocytic infiltration [medigoo.com]

  Rheumatic brain disease shows pathology of nonspecific arteritis with perivascular lymphocytic infiltration, endothelial swelling and petechial hemorrhages. There is absence of Aschoff bodies. These changes are more apparent in the corpus striatum. [epainassist.com]

  Pathology of rheumatic brain disease is of a nonspecific arteritis with endothelial swelling, perivascular lymphocytic infiltration, and petechial hemorrhages. [emedicine.com]

• Aschoff Body

  Aschoff bodies are not present in the brain. These changes are evident to some extent throughout the cerebrum but are most prominent in the corpus striatum. Severe neuronal loss occurs in the caudate nucleus and putamen. [medigoo.com]

  There is absence of Aschoff bodies. These changes are more apparent in the corpus striatum. [epainassist.com]

  Aschoff bodies are not present in the brain. [8, 9] These changes are evident to some extent throughout the cerebrum but are most prominent in the corpus striatum. Severe neuronal loss occurs in the caudate nucleus and putamen. [emedicine.com]

1. Kim A, Choi CH, Han CH, Shin JC. Consecutive pregnancy with chorea gravidarum associated with moyamoya disease. J Perinatol. 2009;29(4):317-319.
2. Germes Piña F. Chorea gravidarum. A case report [Article in Spanish]. Ginecol Obstet Mex. 2009;77(3):156-159.
3. Pathania M, Upadhyaya S, Lali BS, Sharma A. Chorea gravidarum: a rarity in West still haunts pregnant women in the East. BMJ Case Rep. 2013;2013:bcr2012008096.
4. Maia DP, Fonseca PG, Camargos ST, Pfannes C, Cunningham MC, Cardoso F. Pregnancy in patients with Sydenham's Chorea. Parkinsonism Relat Disord. 2012;18(5):458-461.
5. Miranda M, Cardoso F, Giovannoni G, Church A. Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies. J Neurol Neurosurg Psychiatry. 2004;75(2):327-328.
6. Karageyim AY, Kars B, Dansuk R, Aygun E, Unal O, Turan MC. Chorea gravidarum: a case report. J Matern Fetal Neonatal Med. 2002;12(5):353-354.
7. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.