Sydenham's Chorea: A Clinical Follow-Up of 65 Patients

Abstract

Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset of the symptoms was 11.7 +/- 2.6 years (range, 6-17 years). Of the patients, 63% were female and 37% were male (male/female: 1.7/1). Chorea was generalized in 78.5% of the patients, right hemichorea in 12.3%, and left hemichorea 9.2%. There was a history of rheumatic fever in 30.8% of the patients. Echocardiographic study showed cardiac valve involvement in 70.5% of 61 patients. Brain magnetic resonance imaging, which was performed on only 18 patients, was evaluated as normal in all. Electroencephalography was also performed on only 18 patients and showed abnormal waves in 50% of them. Pimozide was mostly the first choice of drug therapy. Nevertheless, drug therapy was not needed in 18.5% of the patients. The recovery period of the first attack of the chorea was 1 to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenham's chorea is still an important health problem in Turkey with respect to its morbidity.

Behavioral, Pharmacological, and Immunological Abnormalities after Streptococcal Exposure: A Novel Rat Model of Sydenham Chorea and Related Neuropsychiatric Disorders

“…These findings suggest that the increase in induced-grooming in GAS-exposed rats represents a form of compulsive responding, consistent with reports of obsessive and compulsive symptoms in SC and PANDAS (Gordon, 2009;Murphy et al, 2006a, b;Swedo et al, 1989). The relevance of impaired food manipulation and increased induced-grooming in GAS-exposed rats to SC and PANDAS was supported by demonstrating that these symptoms were alleviated by administration of haloperidol and paroxetine, respectively, which are used to treat motor and compulsive symptoms in SC and PANDAS (Demiroren et al, 2007;Shannon and Fenichel, 1990). (Paroxetine was administered in the present experiment for 3 days, whereas in OCD patients SSRIs are effective only after several weeks of repeated administration.…”

“…First, we tested whether exposure of the autoimmune-prone Lewis rat strain to GAS antigen leads to behavioral characteristics of SC/PANDAS, including impairments in fine motor control and gait, and increased activity, anxiety, and compulsivity (Gordon, 2009;Marques-Dias et al, 1997;Murphy et al, 2006b;Swedo et al, 1989). Second, we tested whether motor abnormalities and increased compulsivity in GAS rats would respond to the same drugs used to treat motor symptoms and compulsions in SC/PANDAS (D2 blockers and selective serotonin reuptake inhibitors (SSRIs), respectively, Demiroren et al, 2007;Shannon and Fenichel, 1990;Swedo et al, 1998;SE Swedo, unpublished observation). Third, we tested whether sera of GAS rats will have the same immune responses as sera from SC/PANDAS patients, including reaction against GAS antigens (Bronze and Dale, 1993;Husby et al, 1976;Kirvan et al, 2003Kirvan et al, , 2006b) and brain tissue and tubulin (Kirvan et al, 2007), and induction of CaMK II signaling in SK-N-SH neuronal cell line (Kirvan et al, 2003(Kirvan et al, , 2006a.…”

Antibiotic Treatment Attenuates Behavioral and Neurochemical Changes Induced by Exposure of Rats to Group A Streptococcal Antigen

“…Behaviorally, GAS exposure led to impaired food manipulation [13], [14] and increased marble-burying without a concomitant increase in activity level. These behavioral alterations may be relevant to symptoms commonly observed in GAS-related neuropsychiatric disorders, including impaired fine motor control [6], [18], [19], anxiety [19]–[22] and compulsions [18], [20], [22], [23].…”

Successful treatment of Sydenham's chorea with intravenous immunoglobulin

“…SC is a self-limiting disease 4 9. However, symptoms can persist for months or years and relapses are not uncommon, making adequate treatment desirable 2 3 8…”

“…Various medications have been used to treat the chorea, such as dopamine receptor antagonists (eg, haloperidol), antiepileptics (eg, valproic acid or carbamazepine) and prednisone 2 3 8 10 11. Studies examining these medications in patients with SC are small and often observational, objective outcome measurements are limited and study populations are heterogeneous 11.…”