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Chorea Gravidarum

Chorea Gravidarum Syndrome

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].

Streptococcal Pharyngitis
  • 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.[eng.ichacha.net]
  • Recently there has been a decline in incidence which is probably the result of a decline in rheumatic fever, which was a major cause of chorea gravidarum before the use of antibiotics for streptococcal pharyngitis. U.S.[definitions.net]
Hyperthermia
  • In a 23-year-old primigravida with no prior history of rheumatic fever, choreiform movements developed late in the third trimester, and she died in a state of hyperthermia 14 days later.[ncbi.nlm.nih.gov]
  • These patients are more likely to develop rhabdomyolysis, seizures, hemiplegia, and coma, with hyperthermia have no history of autoimmune disease, so a full evaluation, particularly poor prognostic factor.The symptoms typically present in the first trimester[medigoo.com]
  • Such mothers or patients are more prone to develop seizures, rhabdomyolysis , hemiplegia, hyperthermia and coma. The symptoms of chorea gravidarum usually subside in the mid to late second trimester.[epainassist.com]
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 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]
  • The appearance of facial grimaces slurred speech and impaired coordination may accompany this movement disorder.[symptoma.com]
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]
  • […] factors for recurrence of chorea in subsequent pregnancies.[symptoma.com]
  • 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]
  • 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]
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]
  • Abstract Chorea gravidarum is an uncommon condition characterized by involuntary movements, speaking alterations and in the affective status during first trimester pregnancy, the incidence is 1 by each 2275 pregnancies, it is self limiting and resolves[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]
Tremor
  • 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]
  • 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]
  • ( 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]
  • ., postpump chorea in children, cancer-related paraneoplastic syndromes), myoclonus, essential tremor, and dystonia, including dystonia plus syndromes.[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.de]
  • 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]
  • 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.[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]

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.

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]

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]
  • Treatments include neuroleptics for symptomatic relief and therapies targeted toward the underlying pathology.[ncbi.nlm.nih.gov]
  • Treatment of Sydenham's chorea with haloperidol. Dev Med Child Neurol. 1973 Feb. 15(1):19-24. [Medline]. Patterson JF. Treatment of chorea gravidarum with haloperidol. South Med J. 1979 Sep. 72(9):1220-1. [Medline]. Donaldson JO.[emedicine.medscape.com]
  • Therapy with haloperidol, a potent dopamine antagonist, is a reasonably safe treatment for moderate to severe chorea gravidarum during the second and third trimesters of pregnancy. 1982 The American College of Obstetricians and Gynecologists Related Articles[journals.lww.com]
  • Treatment of chorea gravidarum in SLE patients is discussed.[ncbi.nlm.nih.gov]

Prognosis

  • 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 of CG for pregnant women and fetuses are believed to be good. In this report, we present two cases of CG secondary to rheumatic fever that were associated with adverse fetal outcomes.[turkiyeklinikleri.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]
  • In the past, rheumatic disease was generally the etiology, but today, collagen vascular disease should also be considered. Treatments include neuroleptics for symptomatic relief and therapies targeted toward the underlying pathology.[ncbi.nlm.nih.gov]
  • 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.[biomedscidirect.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: 353. CrossRef PubMed Google Scholar 12. Elevli, M, Celebi, A, Tombul, T, Gokalp, AS.[link.springer.com]
  • Epidemiology Typical age of onset is 5-15 years. There is a female preponderance reported in most studies. A predisposition runs in families. The incidence of Sydenham's chorea reflects that of RF. It is rarely seen in developed countries nowadays.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • Get a quick and memorable overview of anatomy, pathophysiology, and clinical presentation from the precision and beauty of Netter and Netter-style plates that highlight key neuroanatomical and neurologic concepts.[books.google.de]

Prevention

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

References

Article

  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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Last updated: 2019-06-28 11:53