Sydenham Chorea

Jummani, Rahil; Okun, Michael S.

doi:10.1001/archneur.58.2.311

Cited by 17 publications

(11 citation statements)

References 8 publications

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“…The disease equally affects men and women, usually in their 60s-70s (mean age 65 years, range 40-80 years), but there are no reports of patients aged under 40. This reflects the median age observed in patients with classical paraneoplastic syndromes [19][20][21][22], and is higher than the age found in other well-defined chorea syndromes, as Sydenham's chorea (8-9 years) [23], systemic lupus erythematosus (SLE) (21 ± 12 years) [24], and Huntington's disease (40 years) [25]. Onset over 40 years may therefore give grounds for suspecting a paraneoplastic origin.…”

Section: Discussionmentioning

confidence: 89%

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

et al. 2011

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Chorea and other movement disorders are rarely described as paraneoplastic. The aim of this study was to describe 13 patients with paraneoplastic chorea and dystonia collected by the members of the paraneoplastic neurological syndrome (PNS) EuroNetwork and to review 29 cases from the literature. We analyzed neurological symptoms, severity of the neurological syndrome, delay in neurological diagnosis, associated cancer, oncological and neurological treatments received, and outcome. Eleven (1.2%) out of 913 patients with PNS were identified in the EuroNetwork register. Two more patients not included in the register were added. The overall population consisted of 13 patients with a median age of 75 years (range 49-82 years). In most patients, the movement disorder was classical choreoathetosis with symmetric involvement of the trunk, neck, and limbs. A minority of patients presented unilateral chorea, dystonia, and orobuccal dyskinesia. Associated symptoms, as polyneuropathy, encephalitis, psychiatric disturbances, or visual defects, were often present. The movement disorder usually had a subacute course. The most frequently associated cancer was small cell lung cancer (SCLC). Lymphoma, bowel, or kidney cancers were also reported. CV2/CRMP5 was the most frequently associated antibody, followed by Hu. Hyperintense lesions of the basal ganglia on T2-weighted images were seldom observed. Response to cancer therapy was observed in a minority of patients, but survival was short (17 months). As in other neurological diseases, movement disorders should also be suspected as paraneoplastic when they develop subacutely in older patients (usually over 50) and often in the presence of other ancillary neurological symptoms.

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Section: Discussionmentioning

confidence: 89%

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

et al. 2011

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“…19 Later in 1802, an association between SC and rheumatic fever was found and further studies also recognized their common trigger in the streptococcal infection. Antibiotic treatments for streptococcal infections led to a decline in the incidence of SC, however the disease re-emerged in the 1980s with a different phenotype.…”

Section: Immunotherapeutic Approaches To Pediatric Neurodegenerative mentioning

confidence: 98%

The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data

Vitaliti

Tabatabaie

Matin

et al. 2015

Human Vaccines & Immunotherapeutics

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“…Originally referred to as St. Vitus' chorea or St. Vitus dance, Sydenham's chorea (SC) was first described in 1686 by Thomas Sydenham 5. However, a relationship with rheumatic fever (RF) was not recognized until 1810 by Etienne Bouteille 6. Chorea is considered 1 of the major criteria for diagnosis of RF, although it occurs in less than 25% of patients with RF 7.…”

Section: Autoimmune Neuropsychiatric Disorders Associated With Streptmentioning

confidence: 99%

Movement disorders in autoimmune diseases

2012

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Movement disorders have been known to be associated with a variety of autoimmune diseases, including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcus, systemic lupus erythematosus, antiphospholipid syndrome, gluten sensitivity, paraneoplastic and autoimmune encephalopathies. Tremors, dystonia, chorea, ballism, myoclonus, parkinsonism, and ataxia may be the initial and even the only presentation of these autoimmune diseases. Although antibodies directed against various cellular components of the central nervous system have been implicated, the pathogenic mechanisms of these autoimmune movement disorders have not yet been fully elucidated. Clinical recognition of these autoimmune movement disorders is critically important as many improve with immunotherapy or dietary modifications, particularly when diagnosed early. We discuss here the clinical features, pathogenic mechanisms, and treatments of movement disorders associated with autoimmune diseases, based on our own experience and on a systematic review of the literature.

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Sydenham Chorea

Cited by 17 publications

References 8 publications

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience

The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data

Movement disorders in autoimmune diseases

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