Chorea gravidarum


Chorea gravidarum

Description, Causes and Risk Factors:

Chorea gravidarum (CG) is the term given to chorea occurring during pregnancy.

Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. More than 70% of patients gave a previous history of either rheumatic fever or chorea. 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.

Chorea gravidarum

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. 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. 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. The choreic movements may be caused by ischemia or enhanced dopaminergic sensitivity mediated by increased female hormones during pregnancy.

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.

Symptoms:

Patient present with symptoms in the second or third trimester, particularly with mental status changes such as agitation and confusion. These patients are more likely to develop rhabdomyolysis, seizures, hemiplegia, and coma, with hyperthermia have no history of autoimmune disease, so a full evaluation, particularly poor prognostic factor.The symptoms typically present in the first trimester and often subsided in the mild to late second trimester. Antistreptolysin antibodies are elevated and may continue to rise throughout the pregnancy. Cardiac valvular disease is often evident, but patients usually do well with supportive care, reassurance, and medical intervention.

Diagnosis:

A history, physical examination, and pertinent investigations enable a diagnosis in most cases. Given the high rates of reported, otherwise asymptomatic strokes, an imaging study is warranted.

Tests: Brain MRI, toxicology screening, antistreptolysin-O, sedimentation rate, blood cultures, antinuclear antibody, echocardiography, coagulation times (PT and APTT), anticardiolipin antibody, lupus anticoagulant, complete blood count, liver function tests, electrolytes, Slit lamp examination, peripheral RBC smear for acanthocytes.

Treatment:

In choosing treatments, the teratogenic risks to the fetus must be weighed against the benefit of the mother. Most case reports suggest that haloperidol offers the most effective symptomatic relief. Recent studies of the use of phenothiazines for antiemetic effects in pregnancy have confirmed their relative safety.

Traditional therapy has consisted of rest or seclusion and careful feeding. Usually chorea gravidarum is manageable non-pharmacologically. 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.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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