Chorea Gravidarum Workup

Updated: Aug 11, 2017
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Selim R Benbadis, MD  more...
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Workup

Laboratory Studies

See the list below:

  • Maintain a high index of suspicion and vigilance when making the diagnosis of chorea gravidarum and considering the differential diagnosis.

  • Acute rheumatic fever- Erythrocyte sedimentation rate (ESR), throat culture, C-reactive protein, and ASO titer

  • Wilson disease - Serum ceruloplasmin and urinary copper (24 h)

  • Systemic lupus erythematosus - ESR, antinuclear antibody, anticardiolipin antibodies, and lupus anticoagulant assays

  • Phenothiazine reaction history - Therapeutic trial of intravenous (IV) benztropine

  • Polycythemia - CBC, hemoglobin, and hematocrit

  • Hyperthyroidism - Thyroxine (T4), thyroid-stimulating hormone (TSH)

  • Hypoparathyroidism - Serum calcium and phosphate

  • Vascular disease

    • Hypercoagulability of pregnancy; investigations for hyperlipidemia, diabetes, valvular heart disease, hyperviscosity states, hemoglobinopathies, or congenital cerebrovascular disease (moyamoya)

    • In a young patient with cerebral infarction, in the absence of hypertension and atrial fibrillation, vasculitides and thrombophilic tendencies must be considered. Testing for thrombophilia with estimation of anticardiolipin antibody, antithrombin III levels, prothrombin gene, protein S, protein C resistance, and factor V Leiden should be considered.

  • Meningovascular syphilis - Venereal Disease Research Laboratory test (VDRL), fluorescent treponemal antibody absorption test (FTA-ABS)

  • Drugs - Serum levels of anticonvulsants, theophylline, lithium, and tricyclic antidepressants

  • Drug toxicity due to amphetamine and cocaine - Serum levels and urine screening

  • Lead toxicity - Serum lead level

  • Neuroacanthocytosis peripheral smear for acanthocytes

  • Adult-onset Tay-Sachs disease - Assay of serum lysosomal enzymes

  • Husby has described antineuronal antibodies using an immunofluorescent technique in 46% of patients with Sydenham chorea (n = 30) compared with 14% of patients with rheumatic fever (without chorea) (n = 50) and only 1.8-4% of control subjects (n = 203). He further demonstrated a potential correlation between antibody reactivity and the clinical status, with antibody disappearance on chorea remission. [42]

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Imaging Studies

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  • Hypoparathyroidism: CT scan may reveal bilateral basal ganglia calcificans.

  • MRI

    • Huntington disease (MRI of the brain to exclude caudate atrophy) and neuroacanthocytosis

    • Wilson disease - Striatal damage

    • Systemic lupus erythematosus, locular infarcts - Small arterial damage

    • Rare basal ganglia tumor

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Other Tests

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  • Obtain ECG whenever a suspicion of rheumatic fever exists to exclude carditis. EEG may show evidence of rheumatic encephalopathy.

  • Perform a slit-lamp examination to rule out Kayser-Fleischer rings that would indicate Wilson disease.

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