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Answers on p 378.
A 24 year old right handed Pakistani woman who was eight weeks' pregnant presented with a two day history of involuntary movements of her left arm, hand, and foot. There was no significant past medical history or family history, and she was taking no regular medication. On examination there were choreiform movements of the left upper limb and left foot. Her speech was normal and there was no other neurological abnormality. She was apyrexial, and examination of the cardiovascular, respiratory, and abdominal systems was unremarkable. Fundoscopy and slit lamp examination of the eyes was normal. Full blood count, urea and electrolytes, liver function tests, and thyroid function tests were normal. She had a polyclonal rise in IgG, with no evidence of autoantibodies. C reactive protein was <6 mg/l and the antistreptolysin O titre 320. Electrocardiography and echocardiography showed no abnormalities. Obstetric ultrasound was performed which showed a viable fetus with estimated gestational age of 10 weeks. Magnetic resonance imaging (MRI) of the brain was normal. She was treated with haloperidol 1 mg three times daily and her chorea improved markedly over the next three days.
On day 8 she suffered a primary focal seizure with violent movements of the right arm leading to a secondary generalised seizure. Emergency computed tomography of the brain with and without contrast showed no abnormality. Cerebrospinal fluid obtained by lumbar puncture was sterile with normal cell counts. An electroencephalogram was asymmetrical with generalised excess of left sided theta and delta waves unaffected by photic stimulation. In the 24 hours after the seizure she developed expressive dysphasia, right sided hemiplegia, and worsening left sided chorea. MRI of the brain was performed on day 10 (fig 1). She was anticoagulated with intravenous unfractionated heparin, and within six hours there was an increase in right sided power to grade 4 out of 5 and improvement of her dysphasia and chorea. After bleeding from her vagina on day 27 miscarriage was diagnosed on obstetric ultrasound. She was discharged after 35 inpatient days: she had mildly reduced right sided power (grade 4 out of 5) and mild dysphasia. Life long treatment with warfarin is planned and she has been advised against future pregnancies.
- Give a differential diagnosis of chorea in pregnancy
- Describe the abnormalities in the MRI image (fig 1)
- What underlying causes should be sought in this patient?