eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Chorea Gravidarum

Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Contributor Information and Disclosures

Updated: Feb 10, 2009

Introduction

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. Chorea is an involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces.

Chorea gravidarum is regarded as a syndrome rather than a specific disease entity.

Incidence

Most of the more common and serious movement disorders rarely occur during reproductive years. Hence clinicians are not very familiar with chorea gravidarum. Willson and Preece (1932) found that the overall incidence of chorea gravidarum was approximately 1 case per 300 deliveries.1 According to them, the first description of chorea with onset during pregnancy was made by Horstius in 1661. The condition is much more rare now. Zegart and Schwartz (1968) found that one patient had been encountered in the course of 139,000 deliveries in 3 major Philadelphia hospitals.2 The decline 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.

In recent times, most cases of chorea appearing during pregnancy are caused by other diseases (eg, systemic lupus erythematosus [SLE], Huntington disease). In general, about half the cases are idiopathic, with rheumatic fever and antiphospholipid syndrome (APLS) underlying most of the remainder.3

Patient profile

Most patients with chorea gravidarum are young; the average age is 22 years.1 Almost all reported patients have been Caucasians, although this may be due to a bias in the older literature, in which the vast majority of reported cases are among European patients. Of initial attacks, 80% occur during first pregnancies, and one half start during the first trimester.1 One third begin in the second trimester. Of afflicted women, 60% previously had chorea. Recurrences may occur in subsequent pregnancies, particularly if antiphospholipid syndrome is the cause. A family history of transient chorea is not unusual.

Pathophysiology

Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. Willson and Preece noted that nearly 70% of their patients gave a previous history of either rheumatic fever or chorea.1 Of patients who present with chorea and no apparent carditis, 20% may develop rheumatic heart disease after 20 years. Interestingly, 50% of patients with oral contraceptive-induced chorea have a past history of chorea, which in 41% of cases is of rheumatic origin. The suggestion is that estrogens and progestational hormones may sensitize dopamine receptors (presumably at a striatal level) and induce chorea in individuals who are vulnerable to this complication by virtue of preexisting pathology in the basal ganglion.

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, and petechial hemorrhages. Aschoff bodies are not present in the brain.4,5 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. The same pathologic changes have been reported for chorea gravidarum, but all those patients also had cardiac disease.1 Brain tissue from patients with acute rheumatic fever with or without chorea has not been studied for the presence of antistreptococcal antibodies. Presumably, as the inflammation resolves, the chorea disappears and degenerative changes are left in small arterioles.

Several lines of evidence suggest that heightened dopamine activity occurs either by denervation hypersensitivity or by aberrant sprouting of dopamine terminals on the remaining striatal neurons. A possible relationship between chorea gravidarum and moyamoya disease has been reported in a 16-year-old pregnant patient.6 The choreic movements may be caused by ischemia or enhanced dopaminergic sensitivity mediated by increased female hormones during pregnancy.

Koide et al reported that from 1994-2004, 8 patients were diagnosed with clinically definite opsoclonus-myoclonus syndrome (a movement disorder) at TokyoMetropolitanNeurologicalHospital. This rare disorder occurred during pregnancy in 25% of their cases and they raised the possibility of a susceptibility factor in pregnancy. The relationship between opsoclonus-myoclonus syndrome and pregnancy, like chorea gravidarum, remains unclear.7

Clinical

History

Emotional stress aggravates the movements of chorea gravidarum. During sleep, the movements disappear. The chorea may be unilateral hemichorea. The patient may attempt to disguise chorea by incorporating it into a mannerism or gesture. Choreic movements largely affect the extremities but vary greatly in complexity and temporal expression from one patient to another. The patient may be restless and fidgety and is often unaware of it and may not complain about it; hence, the clinician might be misled or totally miss the diagnosis.

Generally, the affected limb is hypotonic; joints are floppy, and knee jerks are pendular. Normally the arms dangle by the sides, but with chorea (ie, hypotonia), they flail about. Wrist and fingers assume the shape of a dinner fork with abduction of the thumb. At times, continuous involuntary movements may be impossible to sustain. Protruded tongue darts in and out uncontrollably. Varying hand strength is referred to as "milkmaid" grip. Choreic movements are rapid, purposeless, irregular, jerky movements that seem to randomly flow from one part of the body to another.

Obtain a thorough past history including a history of rheumatic fever and confidential inquiry about illicit drug use and any psychiatric treatment with neuroleptics or metoclopramide (ie, dopamine antagonists).

  • Relationship to rheumatic heart disease
    • Chorea gravidarum is linked strongly to rheumatic fever. At least 35% of patients have a definite history of acute rheumatic fever and Sydenham chorea; 4% of those with chorea gravidarum had acute rheumatic fever.1
    • Women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies.8,9
    • Carditis was found in 87% of fatal cases.1
    • Sydenham chorea can follow the onset rheumatic fever by as much as 7 months.10 Both conditions are related to group A streptococcal infections. Isolated recurrences of chorea among a group of 60 children with a history of Sydenham chorea followed an episode of streptococcal pharyngitis by a week, 3 months, or even 6 months.11 By the time chorea develops, antistreptolysin O (ASO) titers may have returned to normal. This does not imply that all cases of chorea gravidarum are related to an immediately preceding streptococcal infection; the fact that chorea recurs in the same woman with several pregnancies is statistically against this. Moreover, Jonas et al were able to document that a woman with chorea and a history of acute rheumatic fever had been free of streptococcal infection for 15 months prior to the presentation of chorea in the sixth month of pregnancy.12
  • Rheumatic brain disease: Sydenham chorea, some mental status changes, emotional lability to hysterical traits, psychotic delusions, and hallucinations can occur.13 Seizures14 and papilledema15 can occur.
  • Rheumatic encephalopathy
    • Rheumatic encephalopathy is reflected in the EEG findings.16
    • EEG changes are not limited to patients with clinical manifestations of chorea. Slow waves (3-6 Hz) can occur continually or in intermittent rhythmic paroxysms. They may be generalized or predominantly over the frontal and central regions.
    • Changes may be unilateral in hemichorea.17 Improvement in EEG pattern parallels recovery from rheumatic carditis and chorea, usually within 6 months.

Physical

Physical examination includes a careful general, systemic, and neurologic examination. Look especially for involuntary movements and mental status changes.

Causes

The most probable cause of chorea gravidarum is the reactivation by some mechanism of subclinical damage to basal ganglia resulting from previous rheumatic encephalopathy. Oral contraceptives and possibly other mechanisms may activate the same mechanism.

In 2004, Miranda et al reported of a case of chorea associated with the use of the oral contraceptives, in which antibasal ganglia antibodies have also been detected, suggesting an immunological basis to the pathogenesis of this disorder.18 However, the presence of antibodies in serum does not necessarily infer pathogenicity; the antibodies could be produced as part of tissue damage.19 To demonstrate that a disorder is autoimmune, 5 criteria must be fulfilled20 . The criteria are (1) the presence of autoantibodies, (2) the presence of antibodies in target tissue, (3) the induction of disease in an animal model by passive transfer of the antibody, (4) the induction of disease in an animal model by autoantigen immunization, and (5) improvement of clinical symptoms after removal of the antibodies with plasma exchange.

More on Chorea Gravidarum

Overview: Chorea Gravidarum
Differential Diagnoses & Workup: Chorea Gravidarum
Treatment & Medication: Chorea Gravidarum
Follow-up: Chorea Gravidarum
References
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References

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Further Reading

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Keywords

chorea gravidarum, chorea during pregnancy, Sydenham chorea, rheumatic fever, involuntary movement, abnormal movement, facial grimaces, systemic lupus erythematosus, SLE, Huntington disease, CG, SC, RF, SLE

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Contributor Information and Disclosures

Author

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine
Disclosure: Abbott Labs  Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching

Medical Editor

Stephen T Gancher, MD, Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University
Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and Movement Disorders Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Nestor Galvez-Jimenez, MD, MSc, MHA, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida
Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

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