Chorea Gravidarum Clinical Presentation

Updated: Aug 11, 2017
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Selim R Benbadis, MD  more...
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Presentation

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. [4]

    • Women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies. [12, 13]

    • Carditis was found in 87% of fatal cases. [4]

    • Sydenham chorea can follow the onset rheumatic fever by as much as 7 months. [14] 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. [15] 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. [16]

  • Rheumatic brain disease: Sydenham chorea, some mental status changes, emotional lability to hysterical traits, psychotic delusions, and hallucinations can occur. [17] Seizures [18] and papilledema [19] can occur.

  • Rheumatic encephalopathy

    • Rheumatic encephalopathy is reflected in the EEG findings. [20]

    • 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. [21] Improvement in EEG pattern parallels recovery from rheumatic carditis and chorea, usually within 6 months.

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Physical

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

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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. [22] However, the presence of antibodies in serum does not necessarily infer pathogenicity; the antibodies could be produced as part of tissue damage. [23] To demonstrate that a disorder is autoimmune, 5 criteria must be fulfilled [24] . 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.

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