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Chorea Gravidarum
Chorea Pregnancy Ind

Chorea gravidarum is a term denoting the appearance of non-voluntary, non-rhythmic and abrupt movements during pregnancy, often without an identifiable cause. Antiphospholipid syndrome, rheumatic fever, and use of oral contraceptives are considered as most important conditions that can induce chorea gravidarum in pregnant women. The diagnosis requires a detailed patient history and a thorough laboratory workup.

Presentation

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].

Entire Body System

  • Movement Disorder

    Read More Movement disorders in pregnancy. [pubfacts.com]

    […] complicates a pre-existing movement disorder. [scienceopen.com]

    Movement disorders in pregnancy. Marsha S A SmithM. [semanticscholar.org]

    Antineuronal antibodies in movement disorders. Pediatrics. 1993 Jul. 92(1):39-43. [Medline]. Swedo SE, Leonard HL, Garvey M, et al. [emedicine.com]

  • Galactorrhea

    Fetal distress · Vasa praevia · Uterine rupture · Hemorrhage ( Postpartum ) · placenta ( Placenta accreta ) · Umbilical cord prolapse · Amniotic fluid embolism Puerperal disorders Puerperal fever · Peripartum cardiomyopathy · Postpartum thyroiditis · Galactorrhea [thefullwiki.org]

Gastrointestinal

  • Vomiting

    More: Evidence Summaries Nausea/vomiting in pregnancy Source: Clinical Knowledge Summaries - 01 October 2018 gestation and usually resolve around the 20th week of pregnancy.Hyperemesis gravidarum is a diagnosis of exclusion characterized by... [evidence.nhs.uk]

    Phenothiazines can also be prescribed for nausea and vomiting. Other treatment approaches for chorea gravidarum consist of steroids and sedation. Chlorpromazine can also be prescribed alone or with diazepam. [epainassist.com]

    [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. [emedicine.com]

Cardiovascular

  • Orthostatic Hypotension

    Attention is required for side effects (which may include depression, parkinsonism, akathisia, or orthostatic hypotension). Newer VMAT2 blockers, such as valbenazine or deutetrabenazine, may have fewer side effects. [emcrit.org]

Musculoskeletal

  • Arthritis

    Since initiation of OCP, she noted that her muscles would become painful at times, accompanied by migratory arthralgias and arthritis (with joint swelling and redness) involving the wrist and finger joints. [cureus.com]

    Because Sydenham chorea is a complication of rheumatic fever, some individuals will have additional symptoms of joint arthritis or arthralgia, inflammation of the heart valves causing permanent damage to the valves, and ongoing fever. [rarediseases.org]

    Semin Arthritis Rheum 1987; 16: 253. CrossRef PubMed Google Scholar 17. Cervera, R, Asherson, RA, Font, J, et al. Chorea in the antiphospholipid syndrome. [link.springer.com]

    Associated diseases Most patients with Sydenham's chorea will have other manifestations of RF [ 20 ] : 60-80% have cardiac involvement, particularly of the mitral valve. 30% have arthritis and joint involvement. [patient.info]

  • Arthralgia

    Since initiation of OCP, she noted that her muscles would become painful at times, accompanied by migratory arthralgias and arthritis (with joint swelling and redness) involving the wrist and finger joints. [cureus.com]

    Because Sydenham chorea is a complication of rheumatic fever, some individuals will have additional symptoms of joint arthritis or arthralgia, inflammation of the heart valves causing permanent damage to the valves, and ongoing fever. [rarediseases.org]

  • Myalgia

    Note that a child who presents with abnormal movements and personality changes should be evaluated for anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, especially in the setting of a viral prodrome (e.g., headache, fever, myalgias). [ahcmedia.com]

Face, Head & Neck

  • Facial Grimacing

    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. [wikiwand.com]

    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]

Neurologic

  • Chorea

    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]

    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]

    Chorea gravidarum is a rare condition where the patient develops chorea during her pregnancy.1 Chorea gravidarum is a complication of pregnancy and it is related with eclampsia. [epainassist.com]

  • Involuntary Movements

    […] 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]

    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]

    Any of various disorders of the nervous system marked by involuntary, jerky movements, especially of the arms, legs, and face, and by incoordination. [thefreedictionary.com]

    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]

  • 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]

    Keywords Parkinson's disease - restless legs syndrome - Tourette syndrome - chorea gravidarum - essential tremor - estrogen - sex differences - pregnancy [thieme-connect.com]

    Tremor is best distinguished by its rhythmicity. [ahcmedia.com]

  • Slurred Speech

    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. [sonar.ch]

  • 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.de]

Workup

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.

Treatment

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]

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

Prognosis

Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. Conclusion: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good. [go.gale.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]

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 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]

Etiology

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]

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]

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]

Epidemiology

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.de]

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

Rheumatic chorea in northern Australia: a clinical and epidemiological study. Arch Dis Child. 1999;80(4):353-358. doi:10.1136/adc.80.4.353 ↑ Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. [wikem.org]

Arch Intern Med. 1966; 117: 389 Scopus (42) Crossref Google Scholar Lewis-Jonsson J Chorea: its nomenclature, etiology and epidemiology in clinical material from Malmohus County. [amjmed.com]

Pathophysiology

[citation needed] Pathophysiology[edit] Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. [wiki.alquds.edu]

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

Prevention

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

References

  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.
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