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

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

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.

Treatment & Management

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Table 1. Primary differential diagnosis

Primary differential diagnosis
Familial paroxysmal choreoathetosis Benign hereditary chorea

Table 2. Secondary differential diagnosis

Secondary differential diagnosis
Drugs/toxicity	Anticonvulsants (eg, phenytoin, carbamazepine, phenobarbital) Antiparkinson agents Neuroleptics (eg, chlorpromazine, haloperidol, pimozide) Noradrenergic stimulants Steroids Estrogens Lead toxicity
Infectious	AIDS Meningovascular syphilis Infectious mononucleosis Lyme disease Sydenham chorea Viral encephalitis Subacute sclerosing panencephalitis Ramsay-Hunt syndrome (ie, progressive myoclonic ataxia)
Genetic	Heredodegenerative/degenerative disorders Ataxia telangiectasia Pantothenate kinase-associated neurodegeneration (PKAN) disease Huntington disease (including Westphal variant) Neuronal ceroid lipofuscinoses Pelizaeus-Merzbacher disease Wilson disease Dentatorubral pallidoluysian atrophy Pallidopontonigral degeneration Multiple system atrophy Olivopontocerebellar atrophy Primary Atrophy of the Pallidal System (progressive pallidal atrophy) Fahr disease Paroxysmal dystonic choreoathetosis Familial intention tremor and lipofuscinosis Dystonia musculorum deformans Dopa-responsive dystonia Spasmodic torticollis Meige syndrome Task-specific tremor (writer's or voice tremor)
Inherited disorders of metabolism	Abetalipoproteinemia Glutaric aciduria Lesch-Nyhan syndrome Pyruvate decarboxylase deficiency Sulfite oxidase deficiency
Metabolic/endocrine disorders	Encephalopathies (eg, hepatic, renal) Hyperparathyroidism Hyperthyroidism Hypoglycemia Hyponatremia Hypernatremia
Vascular/trauma	Cardiac surgery Cerebral hemorrhage Transient cerebral ischemia Vasculitis Antiphospholipid antibody syndrome
Other systemic disorders	Lupus erythematosus Polycythemia vera Neuroacanthocytosis Acquired hepatocerebral degeneration
Miscellaneous	Systemic lupus erythematosus Henoch-Schönlein purpura Peripheral neuropathies (eg, Charcot-Marie-Tooth disease, Guillain-Barré syndrome) Space-occupying lesions of the brain Tic disorders Transient tic disorder Chronic motor or vocal tic disorder Tourette syndrome

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Author

Saher K Choudhary, MD Director, Neurology Residency Program, Center for Neurology-Greer, Prisma Health; Affiliate Clinical Professor, University of South Carolina School of Medicine, Greenville; Locums Clinical Neurohospitalist, Greenville Memorial Hospital; Locums Neurologist in Telemedicine, Greenville Health Systems

Saher K Choudhary, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Professionals in Patient Safety

Disclosure: Nothing to disclose.

Coauthor(s)

Anusha Battineni, MBBS Neurology Research Intern, UMG Neurosciences Associates, Prisma Health-Upstate

Disclosure: Nothing to disclose.

Enrique Urrea-Mendoza, MD Advanced Clinical Research Associate, Neuroscience Associates, Greenville Health System; Clinical Assistant Professor, University of South Carolina School of Medicine, Greenville; Instructor, Clemson University School of Health Research

Enrique Urrea-Mendoza, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, Association of Clinical Research Professionals, Colombian Neurosurgery Association, Colombian Society of Occupational Medicine, Huntington Study Group, International Parkinson and Movement Disorder Society, Parkinson Study Group

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Nestor Galvez-Jimenez, MD, MSc, MHA The Pauline M Braathen Endowed Chair in Neurology, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

Additional Contributors

Stephen T Gancher, MD Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University

Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS † Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, American College of International Physicians, American College of Physicians, American Heart Association, American Stroke Association, National Association of Managed Care Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Sections Chorea Gravidarum
Overview
Presentation
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- Physical
- Causes
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Workup
Treatment
Medication
Tables
References

Chorea Gravidarum Workup

Laboratory Studies

Imaging Studies

Other Tests

References

Tables

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