Over half of cases of chorea gravidarum are idiopathic; the remaining cases are secondary to various etiologies. While chorea gravidarum was initially associated with rheumatic fever, it is now more commonly associated with autoimmune disorders such as systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome, vascular disease such as stroke or moyamoya, thyrotoxicosis, drug-induced chorea, Huntington disease, and Wilson disease.
Most cases of chorea gravidarum occur during a first pregnancy, and the majority of affected patients have a previous history of chorea. Symptom onset is typically in the first trimester. In idiopathic cases, chorea resolves in one-third of patients prior to delivery and in two-thirds of patients soon after delivery. Symptoms may recur during subsequent pregnancies depending on the underlying cause.
G25.5 – Other chorea
25113000 – Chorea Gravidarum
Differential Diagnosis & Pitfalls
- Huntington disease
- Sydenham chorea (see rheumatic fever)
- Wilson disease
- Systemic lupus erythematosus and/or antiphospholipid antibody syndrome
- Paraneoplastic, eg, anti-CRMP5 associated with small cell lung carcinoma
- Nonketotic hyperglycemia
- Drug-induced chorea (cocaine, amphetamines, dopamine antagonists, steroids, etc)