Chorea in the Antiphospholipid Syndrome: Clinical, Radiologic, and Immunologic Characteristics of 50 Patients from Our Clinics and the Recent Literature

Cervera, Ricard; Asherson, Ronald A.; Font, Josep; Tikly, Mohammed; Pallarés, L; Chamorro, Ángel; Ingelmo, M

doi:10.1097/00005792-199705000-00006

Cited by 247 publications

(190 citation statements)

References 27 publications

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“…Estrogenic oral contraceptives and pregnancy (chorea gravidarum) are recognized risk factors for the occurrence of chorea in patients with SLE or antiphospholipid syndrome. 2,25 Anticoagulation therapy is sometimes instituted because an ischemic etiology (secondary to a hypercoagulable state) is assumed. 26 However, no patient in this study had radiologic evidence of subacute ischemia of the basal ganglia.…”

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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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Autoimmune chorea in adults

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“…9 The pathogenesis might be different in those two conditions. It has been hypothesized that in reversible chorea, striatal binding of antiphospholipid antibodies (aPL) results in dysfunction while in persistent chorea, aPL-associated striatal ischemia has been postulated.…”

Section: Discussionmentioning

confidence: 99%

“…Twenty percent of the patients developed chorea soon after starting estrogen-containing oral contraceptives, during pregnancy, or shortly after delivery, suggesting a potential role of estrogen in this disorder. 9 Treatment of APS associated chorea has been tried with a variety of medications and in some with success, including steroids, haloperidol, antiaggregants, anticoagulants 9,12 and immunosuppressive agents such as hydroxycholoroquine and methotrexate. 13,14 Our patient preferred not to try any therapeutic agent to control the movements since she had been adapting psychologically and socially to the problem.…”

Section: Discussionmentioning

confidence: 99%

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Chorea can Present Long before Other Manifestations of Primary Antiphospholipid Syndrome

Al‐Herz

2003

Ann Saudi Med

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A 42-year-old lady presented with acute blue discoloration and ulceration of the left toe. She had also been complaining of a bluish discoloration and pain of all her toes, which were cold, induced for the past three months.The patient has been complaining of involuntary movements that started nearly 20 years ago. They involved both upper and lower limbs on both sides. Initially they were jerky, persistent and embarrassing, but later on became intermittent, and less obvious to the surrounding people.In 1994, she had a stroke with a cerebral infarct involving the left posterior parietal lobe and micro-infarcts scattered in the white matter, manifested as dysphasia and right facial weakness. The stroke was preceded by few transient ischemic attacks with transient muscle weakness, numbness and amaurosis fugax. In the past few years, she was noticed to have levido reticularis rash, and occasional polyarthralgia. On one occasion she was found to have transient thrombocytopenia. She developed hypertension at the age of 20, but a thorough work-up of secondary hypertension did not reveal any abnormality. She is a mother of six children. She had one stillbirth at eight months of pregnancy, but there was no history of miscarriages. A diagnosis of mitral valve prolapse without a regurge was made based on an echocardiogram in 1994. The patient did not show any symptoms suggestive of systemic lupus erythematosus or any other connective tissue disease. She was on baby aspirin once daily, persentine and a daily dose of 50 mg atenolol to control the blood pressure.During the physical examination, she was experiencing involuntary fine movements of both upper and lower limbs which sometimes became jerky. The blood pressure was well controlled. The peripheries were cold and the left second toe had a dark blue color (Figure 1) with an ulcer at the tip (Figure 2). The rest of the digits including the toes and the fingers looked bluish. There were no further areas of ulceration or infarct and there was no telangiectasia or capillary dilatation at the nail bed. Peripheral pulses were normal. There was a levido reticularis rash noticed on both upper and lower limbs. Examination of the heart revealed a mitral valve click with no murmur. The rest of the examination including the musculoskeletal, the abdomen and CNS was unremarkable.Investigations revealed a normal hemogram (platelets 165), electrolytes, creatinine and liver enzymes. ESR was 20 and C-reactive protein was normal. Activated partial thromboplastin time (aPTT) was 62 with a control of 32. The prothrombin time (PT) and the international normalized ratio (INR) were normal. Anticardiolipin antibodies were as follows: IgG 127, IgM normal and IgA 42 with an upper limit of normal being 15. Lupus anticoagulant was weakly positive. Antinuclear antibodies and anti-double stranded DNA were both negative. Protein C, protein S and anti-thrombin III were all negative. Hepatitis screen and antineutrophilic cytoplasmic antibodies (ANCA) were negative. A transthoracic echocardiogram showed a...

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“…Caramelli e col., em 1993, relataram caso de ocorrência de coréia e anticorpos antifosfolípides em paciente com LES, na qual os movimentos anormais desapareceram com o uso de imunossupressor, reaparecendo com a tentativa de retirada da medicação, sugerindo, assim, manifestação de atividade da doença 26 . Em uma sé-rie de 50 pacientes com síndrome antifosfolípide e coréia, o uso do anticoncepcional foi o fator precipitante para a coréia em 6 deles (12%) 27 . Considera-se possível que esta manifestação ocorra por ligação dos anticorpos antifosfolípides nos gânglios da base, ou por aumento da permeabilidade na barreira hemato-encefálica, causada por dano endotelial 28 .…”

Section: Discussionunclassified

Movimentos involuntários anormais como primeira manifestação do lupus eritematoso sistêmico: relato de caso

Kakehasi¹,

Bomtempo²,

Vassalo³

et al. 2001

Arq. Neuro-Psiquiatr.

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RESUMO -Relatamos o caso de uma paciente, de 36 anos de idade, que desenvolveu quadro de coréia após dois meses do início do uso de anticoncepcional oral, acompanhando-se, posteriormente, de trombocitopenia, úlcera mucosa em cavidade oral, artrite, positividade para os anticorpos antinuclear (.AN), anti-DNA e antiSm, preenchendo critérios para lúpus eritematoso sistêmico, segundo o Colégio Americano de Reumatologia. A pesquisa para os anticorpos anticoagulante lúpico e anticardiolipina (IgG e IgM) foi negativa. A paciente foi tratada com prednisona, fenitoína, fenobarbital e clonazepam, obtendo melhora clínica e laboratorial. Discutimos a ocorrência da coréia e outros movimentos anormais como primeira manifestação do lúpus eritematoso sistêmico, sua relação com os anticoncepcionais orais e os anticorpos antifosfolípides.PALAVRAS-CHAVES: lúpus eritematoso sistêmico, coréia, anticoncepcionais orais, anticorpos antifosfolípides. Involuntary movement disorders as first manifestation of systemic lupus erythematosus: case reportABSTRACT We describe a 36 year-old woman who developed chorea two months after starting the use of oral contraceptives. She also developed thrombocitopenia, oral ulcers, arthritis, positive antinuclear antibodies (ANA), anti-Sm and anti-DNA, filling criteria for sistemic lupus erythematosus, as defined by the American College of Rheumatology. The tests for lupus anticoagulant and anticardiolipin (IgG and IgM) were negative. The patient was treated with prednisone, phenitoin, phenobarbital and clonazepam, obtaining clinical and laboratorial improvement. We discuss the ocurrence of chorea and other movement disorders as first manifestation of systemic lupus erythematosus, its relationship with oral contraceptives and antiphospholipid antibodies.KEY WORDS: systemic lupus erythematosus, chorea, oral contraceptives, antiphospholipids antibodies. As manifestações neurológicas no lúpus eritematoso sistêmico (LES) são comuns, ocorrendo em 14 a 75% dos pacientes 1 . Ao contrário do que ocorre com a cefaléia e o acidente vascular cerebral, a coréia é infrequente, incidindo em menos de 4% dos casos, usualmente acompanhada de outros sintomas de disfunção do sistema nervoso central 2 e, raramente, como primeira manifestação da enfermidade. A coréia pode também ocorrer como manifestação da síndrome antifosfolípide primária 3,4 ou como efeito colateral do uso dos anticoncepcionais orais [5][6][7] . Descrevemos um caso clínico onde a coréia foi a primeira manifestação do LES, em paciente com história pregressa de uso recente de anticontraceptivo hormonal, e pesquisa para anticorpos antifosfolípide negativa. CASOMulher de 36 anos, assintomática até dezembro de 1999, quando passou a apresentar movimentos involuntários e repetitivos da cabeça para o lado esquerdo, associados a febre, linfadenomegalia cervical e submandibular. Evoluiu com aparecimento de movimentos nos membros superiores e inferiores, alucinações visuais e idéias persecutórias, tendo sido internada pela Clínica Médica, inici-

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Chorea in the Antiphospholipid Syndrome: Clinical, Radiologic, and Immunologic Characteristics of 50 Patients from Our Clinics and the Recent Literature

Cited by 247 publications

References 27 publications

Autoimmune chorea in adults

Autoimmune chorea in adults

Chorea can Present Long before Other Manifestations of Primary Antiphospholipid Syndrome

Movimentos involuntários anormais como primeira manifestação do lupus eritematoso sistêmico: relato de caso

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