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Research ArticleArticle

Antiphospholipid Antibody-Associated Chorea

NICOLE M. ORZECHOWSKI, ALEXANDRA P. WOLANSKYJ, J. ERIC AHLSKOG, NEERAJ KUMAR and KEVIN G. MODER
The Journal of Rheumatology November 2008, 35 (11) 2165-2170; DOI: https://doi.org/10.3899/jrheum.080268
NICOLE M. ORZECHOWSKI
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  • For correspondence: nicoleorzechowski@msn.com
ALEXANDRA P. WOLANSKYJ
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J. ERIC AHLSKOG
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NEERAJ KUMAR
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KEVIN G. MODER
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Abstract

Objective

To describe the clinical features, treatment, and outcomes of patients with antiphospholipid antibody (aPL)-associated chorea.

Methods

The study cohort consisted of consecutive patients with chorea evaluated between 1990 and 2005 with documented aPL at time of their neurologic diagnosis.

Results

Eighteen patients were identified, 4 with systemic lupus erythematosus (SLE). The 14 non- SLE patients experienced 1.6 vascular thromboses/pregnancy losses per person, while patients with SLE experienced 0.5 events/person. Four non-SLE patients (29%) and no SLE patients met criteria for antiphospholipid antibody syndrome (APS). None of these 4 tested positive for IgM anticardiolipin antibody (aCL). In contrast, 10 (71%) non-APS patients tested positive for IgM aCL. Chorea was most often bilateral, mild to moderate, and occurred once with a median age at onset of 44 and 33 years in non-SLE and SLE patients, respectively. Therapy included immunosuppression in 3 (21%) non-SLE patients and in all SLE patients. Antidopaminergic agents were used in 7 (39%). All patients responded to treatment. Five patients received anticoagulation for thrombosis and 2 died of bleeding complications, both non-SLE patients.

Conclusion

aPL-associated chorea occurs most often in women and severity is mild to moderate. Clinical expression of chorea does not differ between those with and without SLE. Anticoagulation should be reserved for thrombosis treatment and not simply for chorea in the presence of aPL, as 2 patients died of bleeding. The absence of IgM aCL in patients with APS supports prior evidence that IgG aCL and lupus anticoagulant may be the more clinically relevant antibodies for thrombosis. However, IgM aCL may be important in patients with chorea

Key Indexing Terms:
  • ANTICARDIOLIPIN ANTIBODY
  • CHOREA
  • ANTIPHOSPHOLIPID ANTIBODIES
  • ANTIPHOSPHOLIPID ANTIBODY SYNDROME
  • LUPUS ANTICOAGULANT

Footnotes

  • N.M. Orzechowski, DO, Instructor of Medicine, Division of Rheumatology; A.P. Wolanskyj, MD, Assistant Professor of Medicine, Division of Hematology; J.E. Ahlskog, MD, Professor of Neurology; N. Kumar, MD, Associate Professor of Neurology, Department of Neurology; K.G. Moder, MD, Associate Professor of Medicine, Division of Rheumatology, Mayo Clinic College of Medicine.

    • Accepted for publication June 14, 2008.
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The Journal of Rheumatology
Vol. 35, Issue 11
1 Nov 2008
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Antiphospholipid Antibody-Associated Chorea
NICOLE M. ORZECHOWSKI, ALEXANDRA P. WOLANSKYJ, J. ERIC AHLSKOG, NEERAJ KUMAR, KEVIN G. MODER
The Journal of Rheumatology Nov 2008, 35 (11) 2165-2170; DOI: 10.3899/jrheum.080268

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Antiphospholipid Antibody-Associated Chorea
NICOLE M. ORZECHOWSKI, ALEXANDRA P. WOLANSKYJ, J. ERIC AHLSKOG, NEERAJ KUMAR, KEVIN G. MODER
The Journal of Rheumatology Nov 2008, 35 (11) 2165-2170; DOI: 10.3899/jrheum.080268
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