78 Chorea
Patient 1: Sydenham’s chorea
Salient features
History
• Ask about sore throats if the patient is an adolescent, particularly if female; suspect Syndenham’s chorea (St Vitus dance) in rheumatic fever.
• Take a family history (especially in the middle-aged adult) for Huntington disease.
• Take a history of oral contraceptive use in a young woman or recent pregnancy (chorea gravidorum).
Examination
• Irregular, jerking, ill-sustained, unpredictable, quasipurposive movements of the upper limbs
• The patient is clumsy and keeps dropping objects. Patients with mild disease may show increased fidgeting or restlessness.
• Check the grip of the hands: ask the patient to squeeze your fingers. A squeezing and relaxing motion occurs, which has been described as a ‘milkmaid’s grip’.
• Look at the tongue for any involuntary movements: known as ‘jack-in-the box’ tongue or ‘bag of worms’.
• Test deep tendon reflexes (‘pendular’ or ‘hung-up’ reflexes).
• Tell the examiner that you would like to make enquiries to assess mental status (to exclude premature dementia seen in Huntington disease).
Diagnosis
This young patient has Sydenham’s chorea (lesion) secondary to streptococcal sore throat (aetiology); this condition is usually self-limiting.
Advanced-level questions
What is the prognosis of patients with Sydenham’s chorea?
Most patients recover within 1 month; a few may have relapses. A small proportion may develop valvular heart disease and hence should receive penicillin prophylaxis to prevent recurrence of rheumatic fever.

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