Chorea Gravidarum Treatment & Management

Updated: Jun 29, 2021
  • Author: Saher K Choudhary, MD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Medical Care

Usually, chorea gravidarum (CG) is manageable non-pharmacologically and traditional therapy consists of rest or seclusion and careful feeding. In mild chorea, patients are generally unaware of the involuntary movements and may have no complaints. In general, abnormal choreic movements are more distressing to the observers than to the patient. The mainstay of pharmacologic treatment is neuroleptic drugs, such as haloperidol, and steroids.

As noted previously, the declining incidence of CG in modern times reflects, in part, the declining frequency of rheumatic fever. This results in a situation in which a greater proportion of CG is secondary to other diseases such as autoimmune disorders. SLE exacerbation risk is 7 times higher during pregnancy and the first 2 months postpartum compared to nonpregnant individuals. Although a majority of patients with SLE and CG or chorea improve after starting or increasing steroid therapy, spontaneous remissions have occurred without change of steroid dose or with haloperidol therapy alone. Patients whose symptoms did not respond to steroids or haloperidol benefited from other drugs. Ichikawa et al reported morphologic alterations of an acute or relatively acute nature in the corpus callosum in at least 11 of the cases they reviewed. [7] This suggests that the response to steroid therapy may depend on whether the primary vascular lesion involving the basal ganglion is of an acute or chronic nature.

In regards to oral contraceptive pill-/estrogen-induced chorea, whether subsequent pregnancies trigger chorea in these women is not known. The mainstay of treatment consists of discontinuing the oral contraceptive pill and using a dopamine antagonist only if needed (ie, symptoms persist after discontinuing the oral contraceptive pill). In at least 2 dozen cases, [50, 9]  most patients with estrogen-induced chorea were noted to be young, nulliparous women who had taken oral contraceptives for less than 4 months. A majority of these patients recovered within 2 days of stopping oral contraceptives. Of note, approximately 50% of patients had a history of Sydenham chorea, rheumatic fever, or CG. 

Pharmacologic treatment

Drug treatment is indicated for patients with disabling severe chorea, when chorea interferes with the patient's health, or when the fetus is in danger due to dehydration, malnutrition, disturbed sleep, or injury.

  • Reserpine is potentially toxic to the fetus and is relatively contraindicated during pregnancy.
  • Haloperidol, a potent dopamine antagonist, remains a first line agent with starting dose of 2–6 mg/day up to 20 mg/day in severe cases
    • In 1972, Axley described the therapeutic value of haloperidol in rheumatic chorea. [51]  In 1973, Shenker et al reported its effectiveness in Sydenham chorea. [52]  Since then, haloperidol has been effective therapy for Sydenham chorea and moderate-to-severe CG. [53, 54]    
  • Limited studies have demonstrated that risk of birth defects attributable to haloperidol is low as judged from studies of infants born to women who took haloperidol for control of hyperemesis gravidarum, [55, 56] especially if given after the first trimester when embryonic organogenesis is complete.
  • It is recommended that haloperidol therapy be discontinued as soon as possible to minimize the risk of tardive dyskinesia. Most common adverse reactions include extrapyramidal effects. Thus far, haloperidol has proven to be an effective, relatively safe treatment for moderate-to-severe CG.
  • Chlorpromazine (25–50 mg PO/IM TID/QID) either as monotherapy or in combination with diazepam (5 mg TID) has proved to be effective in ameliorating chorea.
  • Pimozide (2 mg BID), another neuroleptic drug may have fewer adverse effects than haloperidol
    • Shannon and Fenichel suggest that pimozide has virtually no effect on norepinephrine receptors, thus in low doses and during short-term treatment has a lower risk for the appearance of tardive dyskinesia while improving Sydenham chorea symptoms. [57]
  • Valproic acid has been more recently reported to be effective at suppressing choreic movements.
    • Valproic acid enhances the activity of GABA, an inhibitory neurotransmitter of the striatonigral and striatopallidal circuit, which is decreased markedly in brains of individuals with chorea. The use of sodium valproate in Sydenham chorea shows evidence of response within 10 days, with a dose of 15–20 mg/kg/d.
  • Carbamazepine also may have a positive effect in Sydenham chorea.
    • Pallares and Hurtado reported a large improvement in the first week of treatment of one patient using 20 mg/kg/d. [58]  They suggest that the cholinergic action of carbamazepine in the striatum increases the acetylcholine level, inducing a new equilibrium in the balance of the dopaminergic and cholinergic systems.
  • In patients with Sydenham chorea, the dopaminergic system is hyperactive, and the cholinergic system is hypoactive. The stimulus by carbamazepine on the cholinergic system promotes a relative decrease in dopamine, similar to the action produced by neuroleptic drugs. [53, 54]