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An unusual case of chorea gravidarum

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Q1: Give a differential diagnosis of chorea in pregnancy

Although approximately half the cases of chorea gravidarum are idiopathic, rheumatic fever and antiphospholipid syndrome underlie most of the remainder. It is also important to consider Wilson's disease, thyrotoxicosis, Huntington's disease, subcortical infarction, and drug induced choreas.

Q2: Describe the abnormalities in the MRI image

There is extensive cerebral infarction in the middle cerebral artery territory.

Q3: What underlying causes should be sought in this patient?

In a young patient with cerebral infarction, in the absence of hypertension and atrial fibrillation, vasculitides and thrombophilic tendencies must be considered. This patient was normotensive and in sinus rhythm, with no increase in C reactive protein or inflammatory markers. Testing for thrombophilia showed no anticardiolipin antibody, normal antithrombin III levels, normal prothrombin gene, mildly reduced protein S, and activated protein C resistance. She was homozygous for factor V Leiden.

Discussion

Chorea gravidarum is a rare condition which usually presents in the first trimester of pregnancy with bilateral involuntary movements, often with slurred speech and altered affect. It is almost always self limiting and resolves when the pregnancy ends either with delivery, miscarriage, or termination. About half the cases are idiopathic, with rheumatic fever and antiphospholipid syndrome (APLS) underlying most of the remainder.1 It is also important to consider Wilson's disease, thyrotoxicosis, Huntington's disease, subcortical infarction, and drug induced choreas. MRI in both primary and secondary cases is usually entirely normal, though there may be subtle subcortical changes and infarction.2 Treatment of the underlying cause usually leads to resolution of the chorea. Recurrences may occur in subsequent pregnancies, particularly if APLS is the cause. Dopamine antagonists haloperidol and chlorpromazine are useful in symptom control and are seen to be safe when used in low dose.1

This young woman developed rapidly progressing neurology and has a thrombophilic tendency as the underlying cause of her extensive cerebral infarction. Anticoagulation with intravenous heparin arrested the deterioration and her neurological abnormalities improved, leaving her with mild impairment. There is an association between activated protein C resistance and spontaneous abortion, which is likely to be due to placental infarction.3 Although 5%–10% of Europeans are heterozygous for factor V Leiden, the prevalence in Asians is less than 0.5% and reports of arterial thrombosis in Asians due to homozygosity are rare.4 This is the first case of chorea gravidarum and progressive cerebral infarction due to factor V Leiden homozygosity in the literature, and there was a good clinical response to treatment with unfractionated intravenous heparin.

Final diagnosis

Chorea gravidarum and extensive cerebral infarction secondary to underlying homozygosity for factor V Leiden.

References

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