Case report

A 42-year-old woman with a background of migraine with aura developed chorea gravidarum aged 33 years within the first week post partum after her first pregnancy, and again 14 weeks into her second pregnancy when aged 35 years (figure 1: 1–2). She developed asymmetric hyperkinetic choreiform movements of the limbs and oromandibular area. Her family history included pulmonary embolism (father) and rheumatoid arthritis (aunt). There was no prior history of combined oral contraceptive use or exposure to dopamine-receptor blocking drugs.

• Download figure
• Open in new tab
• Download powerpoint

Figure 1

Timeline illustrating the course of relapsing-remitting chorea and trials of treatment. aPL, antiphospholipid antibodies; IVIg, intravenous immunoglobulin; LMWH, low-molecular-weight heparin.

Investigations included high titre triple positivity of aPL (anti-cardiolipin antibodies, anti-β-2-glycoprotein-I (anti-β-2-GPI) antibodies and positive lupus anticoagulant) and intermittently raised antistreptolysin O titre (400 IU/mL (normal 0–200)). Recent aPL titre levels remained persistently raised: IgG anti-cardiolipin 1160.9 u/mL (reference: 0.0–12.1 U/mL), IgG anti-β-2-GPI 3298.5 U/mL (reference: 0.0–10.0 U/mL), dilute Russell’s viper venom time (dRVVT) 1.73 ratio (reference: 0.85–1.17 ratio) with persistence presence of a lupus anticoagulant by dRVVT and dilute activated partial thromboplastin time analysis. Blood tests were normal or negative for thyroid-stimulating hormone, calcium, copper, caeruloplasmin, anti-DNAase B, basal ganglia antibodies, antinuclear antibody, anti-dsDNA, Caspr2/LGi1/NMDAR antibodies.

MR scan of brain showed an incidental pineal cyst and no basal ganglia lesion or signal change with serial imaging showing stable appearances with no cause for chorea identified. Brain fluorodeoxy glucose positron-emission tomography (FDG-PET)-CT raised the possibility of hypermetabolism in the left basal ganglia and mild temporal hypometabolism bilaterally (figure 2).

• Download figure
• Open in new tab
• Download powerpoint

Figure 2

FDG-PET-CT ((A) coronal, (B) axial) showing hypermetabolism (A, B) in the left basal ganglia (increased tracer uptake in the left caudate and left lentiform nucleus) and very mild temporal hypometabolism (A) bilaterally. FDG-PET-CT, fluorodeoxy glucose positron-emission tomography-CT.

The chorea remitted within 9 months of her first delivery, noting she took only aspirin during her first pregnancy. In the last trimester of her second pregnancy, the choreiform movements were not particularly marked. Around 7 weeks post partum, she developed very marked choreiform movements and became clumsy and somewhat impulsive and repetitive. She received treatment with low-molecular-weight-heparin (for 6 weeks for thromboprophylaxis), prednisolone and intravenous immunoglobulin, while breast feeding. Tetrabenazine was trialled, as the involuntary movements were troublesome, and this helped to suppress chorea, though lowered her mood. The chorea remitted within 24 months. She developed a recurrence of unprovoked chorea 3 years later (figure 1: 3), when she was not pregnant or taking hormone therapy. Olanzapine was trialled with benefit and her movements and mood symptoms of hypomania improved. She relapsed with a fourth recurrence of chorea 2 years later (figure 1: 4) and restarted olanzapine. She received pulsed oral methylprednisolone 500 mg for 5 days, started immunotherapy with azathioprine then weaned off olanzapine. On last review she reached a dose of azathioprine 150 mg daily with minor symptomatic improvement in the chorea, though reported possibly better control of choreic involuntary movements when taking olanzapine. She stopped azathioprine in May 2022 and reported no discernible change or difference in the chorea since coming off this medication.