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 Table of Contents  
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 250-251

A rare case of primary antiphospholipid antibody-associated chorea in a child

1 Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre; Department of Pediatric Rheumatology, Apollo Spectra Hospital, Pune, Maharashtra, India
2 Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre, Pune, Maharashtra, India

Date of Web Publication3-Sep-2020

Correspondence Address:
Dr. Jitendra S Oswal
Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre, Pune - 411 043, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_77_20

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How to cite this article:
Kapur SV, Oswal JS. A rare case of primary antiphospholipid antibody-associated chorea in a child. Indian J Rheumatol 2020;15:250-1

How to cite this URL:
Kapur SV, Oswal JS. A rare case of primary antiphospholipid antibody-associated chorea in a child. Indian J Rheumatol [serial online] 2020 [cited 2022 Jan 28];15:250-1. Available from:

Dear Editor,

Antiphospholipid syndrome (APS) is an autoimmune heterogeneous disorder that encompasses clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid (aPL) antibodies.[1] APS can manifest as primary disease or secondary to connective tissue diseases (CTD), such as systemic lupus erythematosus. Limited data exist with regard to the association of chorea in primary APS in children [Table 1]. We report probably the first case from India wherein chorea was the presenting manifestation of a girl with primary APS who improved on anticoagulation and oral corticosteroids.
Table 1: Case reports of chorea in primary antiphospholipid syndrome

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A 14-year-old girl presented with sudden onset of choreiform movement of her arms and legs with dyskinetic movements of the tongue of 2-week duration. There was no history of fever, drug intake, skin rash, oral ulcers, seizures, or focal neurological deficit. She had an episode of the right leg deep vein thrombosis (DVT) 3 months back. There was no significant family history of any neurological condition. Systemic examination was normal. Investigations revealed thrombocytopenia (hemoglobin = 11.3 g/dl, white blood cell = 6.4 × 10.8 × 109/L neutrophils = 48%, lymphocytes = 52%, platelet count = 92,000/cmm). Direct Coombs test and blood cultures were negative. The erythrocyte sedimentation rate, serum electrolytes, urine routine, liver and renal function, X-ray chest, Antistreptolysin O titres, serum ceruloplasmin, two-dimensional ECHO, ultrasonography (abdomen), and ophthalmology examination were all within normal limits, except for prolonged activated partial thromboplastin time (APTT) (63 s [26.9–36.3]). Cerebrospinal fluid examination and magnetic resonance imaging brain were normal. Immunological investigations showed negative antinuclear antibodies, anti-dsDNA, anti-Sm, ANCA, and normal serum complement levels. The anticardiolipin (ACL) IgM (43 U/mL, normal ≤12) and IgG antibody (64 U/mL, normal ≤12), beta-2 glycoprotein-I (β2GPI) IgM antibody (68 RU/ml, normal ≤20), and lupus anticoagulant (LA) were positive. Repeat aPL after an interval of 13 weeks was positive (ACL IgM [40 U/mL] and IgG antibody [50 U/mL], β2GPI IgM antibody [62 RU/ml], and positive [LA]). Given the positive aPL panel and history of DVT in the absence of an underlying CTD, the patient was diagnosed with primary APS. She was started on oral warfarin (5 mg OD) and oral corticosteroids (0.5 mg/kg/day) with significant clinical significant improvement. The patient's choreiform movements completely resolved within the next 8 weeks with normalization of her platelet counts and APTT count following which her steroids were tapered; however, the patient was lost to follow-up.

For the diagnosis of APS to be made, one clinical event, that is, thrombosis or recurrent miscarriages, and the presence of LA or ACL IgG or IgM in the plasma on two or more occasions at least 12 weeks apart are required.[1] In our patient, positive ACL IgM and IgG antibody, β2GPI IgM antibody, and LA with an interval of 13 weeks and demonstration of DVT met the diagnostic criteria of APS. The lack of the history of drug use, infection, and accompanying autoimmune disease also support the diagnosis of primary APS, which is a rare condition in children. In a cohort of 1000 APS patients, the prevalence of chorea, DVT, and thrombocytopenia was 1.3%, 40%, and 30%, respectively.[2] Possible mechanism of pathophysiology of chorea of APS is an antigen–antibody complex-binding phospholipid in the basal ganglia and conferring direct damage to it.[3] Normalization of APTT is unusual with treatment, and to that extent, we are unable to explain the mechanism in our patient. Corticosteroids, anticoagulants, aspirin, immunosuppressants, and dopamine receptor antagonists have been reported to be effective in APS chorea. Long-term anticoagulation is the mainstay of the treatment of APS, especially for patients who have had a prior thrombotic event.

We report this rare case to emphasize the association of chorea with APS in children and prompt investigations that may reveal primary or secondary APS. Early diagnosis allows for appropriate management to minimize complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


  References Top

Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera RH, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006;4:295-306.  Back to cited text no. 1
Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46:1019-27.  Back to cited text no. 2
Asherson RA. Antiphospholipid antibodies and chorea. J Rheumatol 1988;15:377-9.  Back to cited text no. 3
Ayalew Y, Khattak F. Antiphospholipid antibody syndrome presenting with hemichorea. Case Rep Rheumatol 2012;2012:471543.  Back to cited text no. 4
Demonty J, Gonce M, Ribai P, Verellen-Dumoulin C, Hustinx R. Chorea associated with anti-phospholipid antibodies: Case report. Acta Clin Belg 2010;65:350-3.  Back to cited text no. 5
Kiechl-Kohlendorfer U, Ellemunter H, Kiechl S. Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood. Neuropediatrics 1999;30:96-8.  Back to cited text no. 6


  [Table 1]


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