Chorea Gravidarum Treatment & Management

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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Treatment

Medical Care

Declining incidence of chorea gravidarum in modern times reflects, in part, the declining frequency of rheumatic fever. Naturally this results in a situation in which a greater proportion of chorea gravidarum is secondary to other diseases such as systemic lupus erythematosus or Huntington chorea. An increased risk of systemic lupus erythematosus exacerbation is present during pregnancy and especially during the first 2 months postpartum, when the risk is 7 times that of nonpregnant individuals. Although a majority of patients with systemic lupus erythematosus and chorea gravidarum or chorea have improved after starting 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. [43] 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.

Traditional therapy has consisted of rest or seclusion and careful feeding. Usually chorea gravidarum is manageable nonpharmacologically. In mild chorea, patients are generally unaware of the involuntary movements. In general, abnormal choreic movements are more distressing to the observers than to the patient. Early approaches to therapy included sedation and steroids. Phenothiazines have benefited some patients. Chorea gravidarum is not an indication for abortion or premature interruption of pregnancy.

  • Drug treatment is indicated for patients with disabling severe chorea, when chorea interferes with the patient's health in general, 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.

    • In 1972, Axley described the therapeutic value of haloperidol, a butyrophenone, in rheumatic chorea. [44] In 1973, Shenker et al reported its effectiveness in Sydenham chorea. [45] Since then, haloperidol has been effective therapy for Sydenham chorea and moderate-to-severe chorea gravidarum. [46, 47] A potent dopamine antagonist, it usually is administered in doses of 2-6 mg/d, although for more severe cases initial doses of 20 mg/d or more may be required.

    • Limited studies have demonstrated that risk of birth defects attributable to haloperidol is acceptably low as judged from studies of infants born to women who took haloperidol for control of hyperemesis gravidarum [48, 49] , especially if given after the first trimester when embryonic organogenesis is complete. In any instance, discontinue haloperidol therapy 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, acceptably safe treatment for moderately severe to severe chorea gravidarum.

    • Low-dose chlorpromazine either alone (25-50 mg PO/IM tid/qid) or in combination with diazepam (5 mg tid) has proved to be effective in ameliorating chorea.

    • Shannon and Fenichel suggest that pimozide, another neuroleptic drug, may have fewer adverse effects than haloperidol. They suggest that it has virtually no effect on norepinephrine receptors, thus in low doses (2 mg bid) and during short-term treatment has a lower risk for the appearance of tardive dyskinesia while improving Sydenham chorea symptoms. [50]

    • More recently, valproic acid has been reported to be effective at suppressing choreic movements. This drug 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. [51] 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.

  • Treatment of chorea induced by estrogen is as follows:

    • In at least 2 dozen cases [52, 53] , most patients are young nulliparae who have taken oral contraceptives for less than 4 months, and recovery can occur within 2 days of stopping oral contraceptives. Approximately one half previously had Sydenham chorea, rheumatic fever, or chorea gravidarum.

    • Treatment consists of discontinuing the oral contraceptive pill. Use of dopamine antagonists is indicated only if needed. Whether subsequent pregnancies trigger chorea in these women has not been reported.