Antiphospholipid Antibody-Associated Chorea

Orzechowski, Nicole M.; Wolanskyj, Alexandra P.; Ahlskog, J. Eric; Kumar, Neeraj; Moder, Kevin G.

doi:10.3899/jrheum.080268

Cited by 73 publications

(57 citation statements)

References 20 publications

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“…Symptomatic therapy with dopamine antagonists is usually effective and glucocorticoids in combination with immunosuppressive agents (azathioprine, cyclophosphamide) may be used to control NPSLE disease activity. Antiplatelet and/or anticoagulation therapy is administered in antiphospholipid-positive patients, especially when other antiphospholipid/APS-related manifestations are present 36 61 62…”

Section: Resultsmentioning

confidence: 99%

EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs

et al. 2010

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ObjectivesTo develop recommendations for the diagnosis, prevention and treatment of neuropsychiatric systemic lupus erythematosus (NPSLE) manifestations. Methods The authors compiled questions on prevalence and risk factors, diagnosis and monitoring, therapy and prognosis of NPSLE. A systematic literature search was performed and evidence was categorised based on sample size and study design. Results Systemic lupus erythematosus (SLE) patients are at increased risk of several neuropsychiatric manifestations. Common (cumulative incidence >5%) manifestations include cerebrovascular disease (CVD) and seizures; relatively uncommon (1-5%) are severe cognitive dysfunction, major depression, acute confusional state (ACS), peripheral nervous disorders psychosis. Strong risk factors (at least fi vefold increased risk) are previous or concurrent severe NPSLE (for cognitive dysfunction, seizures) and antiphospholipid antibodies (for CVD, seizures, chorea). The diagnostic work-up of suspected NPSLE is comparable to that in patients without SLE who present with the same manifestations, and aims to exclude causes unrelated to SLE. Investigations include cerebrospinal fl uid analysis (to exclude central nervous system infection), EEG (to diagnose seizure disorder), neuropsychological tests (to assess cognitive dysfunction), nerve conduction studies (for peripheral neuropathy) and MRI (T1/T2, fl uid-attenuating inversion recovery, diffusion-weighted imaging, enhanced T1 sequence). Glucocorticoids and immunosuppressive therapy are indicated when NPSLE is thought to refl ect an infl ammatory process (optic neuritis, transverse myelitis, peripheral neuropathy, refractory seizures, psychosis, ACS) and in the presence of generalised lupus activity. Antiplatelet/anticoagulation therapy is indicated when manifestations are related to antiphospholipid antibodies, particularly thrombotic CVD. Conclusions Neuropsychiatric manifestations in SLE patients should be fi rst evaluated and treated as in patients without SLE, and secondarily attributed to SLE and treated accordingly. involve the central and the peripheral nervous system and that range from overt manifestations such as stroke, seizures and psychosis, to more subtle abnormalities of cognitive function (see supplementary table S1, available online only). Multiple pathological mechanisms are implicated in NPSLE, including antiphospholipid or other autoantibodymediated vascular or neuronal injury, intrathecal production of infl ammatory mediators and accelerated atherosclerosis. Despite substantial advances in the understanding of lupus, NPSLE continues to pose diagnostic and therapeutic challenges to practising physicians. The indicated diagnostic work-up remains unclear, therapies are empiric, and the prognosis after an neuropsychiatric event is often diffi cult to determine. We sought to develop recommendations for the management of systemic lupus erythematosus (SLE) patients presenting with neuropsychiatric manifestations using an evidencebased approach followed by exper...

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Section: Resultsmentioning

confidence: 99%

EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs

et al. 2010

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“…We therefore inferred that chorea did not result from a hypercoagulable state and concluded that anticoagulation should be reserved for treating thrombosis, but not chorea. 27 The infrequency of neural-specific antibody detection in patients with idiopathic autoimmune chorea (5 of 21 patients) leads us to conclude that pertinent autoantibody markers remain to be discovered.…”

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confidence: 99%

Autoimmune chorea in adults

et al. 2013

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Objectives: To determine the characteristics of adult-onset autoimmune chorea, and compare paraneoplastic and idiopathic subgroups.Methods: Thirty-six adults with autoimmune chorea were identified at Mayo Clinic (Rochester, MN) from 1997 to 2012. Medical record and laboratory data were recorded. Nonparaneoplastic (n 5 22) and paraneoplastic cases (n 5 14) were compared.Results: Women accounted for 21 patients (58%). Median age at symptom onset was 67 years (range 18-87 years). We estimated the incidence for Olmsted County was 1.5 per million personyears. Symptom onset was subacute in all. Chorea was focal (20 patients) or generalized (16 patients). Although chorea predominated, other neurologic disorders frequently coexisted (29 patients); abnormal eye movements were uncommon (4 patients). No patient had NMDA receptor antibody or any immunoglobulin (Ig)G yielding a detectable immunofluorescence binding pattern restricted to basal ganglia. Two had synaptic IgG antibodies novel to the context of chorea (GAD65, 1; CASPR2, 1). In the paraneoplastic group, 14 patients had evidence of cancer. Of 13 with a histopathologically confirmed neoplasm, small-cell carcinoma and adenocarcinoma were most common; 6 patients had a cancer-predictive paraneoplastic autoantibody, with CRMP-5-IgG and ANNA-1 being most common. In the idiopathic group, 19 of the 22 patients had a coexisting autoimmune disorder (most frequently systemic lupus erythematosus and antiphospholipid syndrome); autoantibodies were detected in 21 patients, most frequently lupus and phospholipid specificities (19 patients). The paraneoplastic group was older (p 5 0.001), more frequently male (p 5 0.006), had more frequent weight loss (p 5 0.02), and frequently had peripheral neuropathy (p 5 0.008).Conclusions: Autoimmune chorea is a rare disorder with rapid onset. Male sex, older age, severe chorea, coexisting peripheral neuropathy, and weight loss increase the likelihood of cancer. Huntington disease is usually the foremost consideration for a neurologist evaluating adult-onset chorea. An autoimmune etiology is sometimes overlooked: paraneoplastic, parainfectious, or idiopathic. Prototypic disorders include CRMP-5 (collapsin response-mediator protein 5)-immunoglobulin (Ig) G-associated paraneoplastic chorea 1 (usually related to small-cell carcinoma) and idiopathic phospholipid antibody-associated chorea.2 Sydenham chorea, a parainfectious immune-mediated disorder, lacks a proven autoantigen and is rare in later adulthood.3 There is a paucity of published data addressing clinical or serologic characteristics that aid the distinction of adult-onset autoimmune choreas, guide treatment, or inform outcomes. This report describes our 16-year experience with adult-onset autoimmune chorea, and compares paraneoplastic and idiopathic autoimmune forms.METHODS Patients. The study used Mayo Clinic's computerized central diagnostic index, and was approved by the Mayo Clinic Institutional Review Board (IRB 08-6647). We reviewed 2,634 medical records of patients seen f...

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“…[21][22][23] In the literature there are no clinical studies on the use of IVIG in children with aPL-related chorea. On the other hand, there are data on the use of IVIG treatment in SC.…”

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confidence: 99%