eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Chorea Gravidarum: Follow-up

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

Follow-up

Prognosis

  • General prognosis
    • Chorea gravidarum seldom persists indefinitely. Without treatment, the disease abates in 30% of patients before they give birth. In almost two thirds of patients, the chorea lasts until puerperium. Symptoms often dramatically disappear in the days after childbirth. In some patients, neurological sequelae may continue in the form of various degrees of incoordination, tremor, and clumsiness.
    • The absence of a control group (ie, women without chorea gravidarum in pregnancy) from Beresford and Graham's analysis of chorea gravidarum in pregnancy makes interpretation of the statistics difficult; they report that death occurred in 1.5% of pregnancies, fetal death in 3.3%, and premature labor in 6.6%.49
    • Death is now rare38 ; the mortality rate of 12% reported by Willson and Preece1 reflects death due to underlying rheumatic heart disease.
    • In the case of drug-induced chorea gravidarum, movements clear on drug withdrawal, and specific antidote therapy often is not needed. Individual susceptibility for adverse effects from these drugs may be due to preexisting basal ganglia abnormalities, such as prior Sydenham chorea or hypoxic encephalopathy.
    • In the case of contraceptive-induced chorea gravidarum, researchers know from animal experiments that female hormones enhance postsynaptic dopaminergic sensitivity. By binding to presynaptic dopaminergic transporter sites, cocaine blocks dopamine reuptake, thus potentiating dopaminergic transmission. It also may influence postsynaptic receptor sensitivity.
  • Fetal prognosis
    • Spontaneous abortion occurs at a normal rate2 , and infants are healthy.
    • Willson and Preece mentioned two 19th century cases of neonatal chorea. One case involved a microcephalic child with athetoid cerebral palsy. The other case was said to involve transient chorea, but the movements were not described further.1
    • In view of the current rarity of chorea gravidarum, fetal mortality is difficult to assess; however, in Beresford and Graham's series, fetal loss was 6.6%, and only one half of this loss was directly attributable to chorea.49 In chorea gravidarum, maternal mortality is reportedly less than 1%.
  • Future pregnancy
    • Of women with chorea gravidarum, 21% have recurrent chorea with subsequent pregnancies.1
    • Several cases have been described in which attacks occurred in 3, 4, and even 5 pregnancies.50,51

Miscellaneous

Medicolegal Pitfalls

  • For the neurologic community to ignore the legal system is naïve and unrealistic. Accurate diagnosis is important, for this influences the next step, namely therapy. With the gradual disappearance of rheumatic fever, other causes of chorea have become more important.
  • Since the cause of chorea gravidarum is multifactorial, adequate attention should be paid while ordering tests, including genetic testing, to informed consent and confidentiality issues. Although patients choose to undergo these diagnostic tests for personal, life-planning, and reproductive reasons, keep in mind that these patients may be faced with barriers in obtaining health insurance, keeping jobs, adopting children, and other forms of discrimination. Undergoing the test alone may result in stigma.
  • Ethical duty of the physician that dates far back to ancient times should tie hand in hand in with the more recent legal duty. This contributes much to maintaining the patient's confidence, which is a long-standing tenet of the patient-doctor relationship.
  • The use of long-acting penicillin undoubtedly has diminished the cardiac complications.
 


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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