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].

• Hunting

  […] infections, but had anti-basal ganglia antibodies, suggesting immunological basis for the pathophysiology of this chorea.[6][7] Diagnosis[edit] Differential diagnoses[edit] Pelizaeus–Merzbacher disease Pantothenate kinase-associated neurodegeneration Ramsay Hunt [en.wikipedia.org]

• Palmar Erythema

  Vascular changes of the skin in pregnancy; vascular spiders and palmar erythema. Surg Gynecol Obstet. 1949 Jun. 88(6):739-52. [Medline]. Cardoso F, Eduardo C, Silva AP, Mota CC. Chorea in fifty consecutive patients with rheumatic fever. [emedicine.com]

• Facial Grimacing

  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]

  The appearance of facial grimaces slurred speech and impaired coordination may accompany this movement disorder. [symptoma.com]

• Chorea

  […] 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. [en.wikipedia.org]

  According to Willson and Preece,1 the first description of chorea with onset during pregnancy (chorea gravidarum) was made by Horstius in 1661. [jamanetwork.com]

  Rheumatic chorea also known as Sydenham's chorea is a delayed complication of group Aβ haemolytic streptococcal infections and forms one of the major criteria of acute rheumatic fever.1 Sydenham's chorea is usually found in less than 10% of rheumatic [ncbi.nlm.nih.gov]

  Chorea may vary from mild to severe and occurs in approximately 1 per 2,000 to 3,000 pregnancies. [profiles.umassmed.edu]

• Involuntary Movements

  A 22 years-old patient, second pregnancy, without prenatal care, who was admitted to the labor at 36.1 weeks of gestation and psychosis of pregnancy presenting involuntary movements, dysnea and behavior disorder of three days evolution. [pubmed.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]

  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]

  […] chorea [ ko-re´ah ] the ceaseless occurrence of rapid, jerky involuntary movements. adj., adj chore´ic. cho·re·a ( kōr-ē'ă ), Irregular, spasmodic, involuntary movements of the limbs or facial muscles, often accompanied by hypotonia. [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]

  Objectives After completing this article, readers should be able to: Identify the important distinguishing features of chorea, dystonia, myoclonus, and tremor. Recognize important causes of chorea, dystonia, myoclonus, and tremor. [pedsinreview.aappublications.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]

  Palanivelu Medicine Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology 2007 A 20-year-old primipara, of Pakistani origin, referred to the antenatal clinic at term for abnormal involuntary movements and being [semanticscholar.org]

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

  Like the better known chorea minor it shows extrapyramidal symptoms with involuntary movements, lack of coordination, slurred speech and psychic disorders. The neurological state is normal except for a loss of muscle tone. [pubmed.ncbi.nlm.nih.gov]

  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

  Pathology of rheumatic brain disease is of a nonspecific arteritis with endothelial swelling, perivascular lymphocytic infiltration, and petechial hemorrhages. Aschoff bodies are not present in the brain. [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]

• Fibrinoid Necrosis

  necrosis and adjacent microinfarcts, but without demonstrable lesions in the basal ganglia. [semanticscholar.org]

• 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]

[citation needed] Treatment[edit] Drug treatment is indicated for patients with severe disabling chorea. [en.wikipedia.org]

Neuroleptics or benzodiazepines can be used for treatment. When antiphospholipid antibodies are shown to be present, corticosteroids, and sometimes aspirin, are added to the treatment. [ncbi.nlm.nih.gov]

She was treated effectively with anticoagulants and corticosteroids, and is currently asymptomatic and without treatment. [scienceopen.com]

Recurrence of CG in subsequent pregnancies may occur, particularly when associated with antiphospholipid syndrome. [4] Prognosis General prognosis Chorea gravidarum (CG) seldom persists indefinitely. [emedicine.medscape.com]

Prognosis The prognosis of chorea depends on its cause. Huntington's chorea is incurable, leading to the patient's death 10 – 25 years after the first symptoms appear. [encyclopedia.com]

Prognosis Commonly, Sydenham's chorea is self-limiting with spontaneous remission. Symptoms generally improve in a week or two and are better by 8-9 months, rarely lasting a year; however, they may occasionally wax and wane for up to 10 years. [patient.info]

We believe that, if chorea gravidarum is not accompanied by other etiologic factors (such as antiphospholipid antibodies), there is no elevated risk for the mother and the fetus. [ncbi.nlm.nih.gov]

It is not an etiologically or pathologically distinct morbid entity, but a generic term for chorea of any etiology. We report a case of Chorea Gravidarum who doesn�t have the past h/o Rheumatic fever, Sydenham�s chorea in childhood. [biomedscidirect.com]

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 [symptoma.com]

It is not an etiologically or pathologically distinct morbid entity, but a generic term for chorea of any etiology. We report a case of Chorea Gravidarum who doesn't have the past h/o Rheumatic fever, Sydenham's chorea in childhood. [zdoc.site]

Sections address general anatomic, hormonal, epidemiologic, and drug aspects of women's health; neurologic conditions that arise during childhood, pregnancy, adulthood, and old age in females; and particular neurologic conditions that present differently [books.google.com]

Yet, considerable sex differences in epidemiology, clinical features, and treatment exist in these disorders. [thieme-connect.com]

Dietrich, MD, Editor Epidemiology As a group, acute movement disorders ( see Table 1 ) in previously healthy children are rare. [ahcmedia.com]

[citation needed] Pathophysiology[edit] Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. [en.wikipedia.org]

Pathophysiology Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. [ipfs.io]

However, she is still seen on follow-up and is on penicillin prophylaxis to prevent rheumatic heart disease. [ncbi.nlm.nih.gov]

[…] her pregnancy and its outcome rather was helpful to the patient as she was started on penicillin prophylaxis which would prevent her from developing rheumatic heart disease in future. [casereports.bmj.com]

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. [symptoma.com]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.de]

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.