Chorea Gravidarum Workup

Updated: Jun 29, 2021
  • Author: Saher K Choudhary, MD; Chief Editor: Selim R Benbadis, MD  more...
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Workup

Laboratory Studies

When making the diagnosis of chorea gravidarum (CG), it is important to keep in mind the diagnositc considerations and to maintain a high index of suspicion and vigilance.

  • Acute rheumatic fever   – erythrocyte sedimentation rate (ESR), throat culture, C-reactive protein, and ASO titer as well as antineuronal antibodies

    See the list below:

    • Husby 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. [49]
  • Wilson disease – serum ceruloplasmin and urinary copper (24 h)
  • Autoimmune disease (APS, SLE, etc) – ESR, antinuclear antibody, anticardiolipin antibodies, and lupus anticoagulant assays. Coagulation times PT and aPTT.
  • Vascular disease   – routine stroke evaluation should include risk factor screenings

    See the list below:

    • Hypercoagulability of pregnancy; investigations for hyperlipidemia, diabetes, valvular heart disease, hyperviscosity states, hemoglobinopathies, or congenital cerebrovascular disease (moyamoya)
    • In young patients with cerebral infarction, in the absence of other clear etiological causes, vasculitides and thrombophilic tendencies must be considered. Hypercoagulable testing including checking for anticardiolipin antibody, antithrombin III levels, prothrombin gene, protein S and protein C resistance, and factor V Leiden should be considered.
  • Hyperthyroidism – thyroxine (T4), thyroid-stimulating hormone (TSH)
  • Hypoparathyroidism – serum calcium and phosphate
  • Wilson disease – serum ceruloplasmin and urinary copper (24 h), liver function tests and electrolytes.
  • Neuro-acanthocytosis  – peripheral smear for acanthocytes
  • Polycythemia – CBC, hemoglobin, and hematocrit.
  • 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
  • Toxicology screening – (serum and urine levels) for amphetamine and cocaine.
  • Lead toxicity – serum lead level
  • Phenothiazine reaction history – therapeutic trial of intravenous (IV) benztropine
  • Adult-onset Tay-Sachs disease – assay of serum lysosomal enzymes
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Imaging Studies

Useful imaging tests for chorea gravidarum (CG) include:

  • Echocardiography: Acute rheumatic fever
  • CT scan: Hypoparathyroidism (may reveal bilateral basal ganglia calcifications)
  • MRI: Evaluate for structural changes or lesions
    • Huntington disease – caudate atrophy
    • Neuroacanthocytosis – basal ganglia atrophy
    • Wilson disease – striatal damage
    • Systemic lupus erythematosus – small arterial damage, locular infarcts
    • Rare basal ganglia tumor
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Other Tests

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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