Sodium valproate in Sydenham's chorea

Five patients with a moderate to severe degree of Sydenham's chorea were treated with sodium valproate for their involuntary movements. Within a week of commencement of treatment, the choreic movements disappeared completely. Sodium valproate appears to be a promising drug in the management of Sydenham's chorea.     

Sydenham's chorea and psychosis

The medical histories of 600 psychotic and 369 nonpsychotic subjects were examined for the occurrence of rheumatic chorea. There was significantly more rheumatic chorea in the histories of psychotic patients than in nonpsychotics (p less than 0.01, chi-square). Neuropathological associations are discussed.     

Sydenham's chorea and psychopathology

Sydenham's chorea is a movement disorder seen in rheumatic fever with basal ganglia pathology. This disorder has been associated with an increased frequency of psychopathology in both the acute choreiform stage and later in life. We conducted a prospective study of 29 subjects with Sydenham's chorea and 29 age- and sex-matched controls. The total number of psychiatric symptoms 10 years after the initial contact was much greater in the study group than in controls (p less than 0.001). Similarly, schizophrenia was more common in the study group compared to controls (p less than 0.01). Possible neuropathological associations and treatment are discussed.     

Persistent Sydenham's chorea.

BACKGROUND: Sydenham's chorea (SC) occurs in 26% of patients with rheumatic fever (RF). Despite usually being described as a self-limited condition, few reports indicate that SC may persist in rare subjects. OBJECTIVE: To investigate the proportion of subjects with SC lasting more than 2 years and if clinical features differentiate patients with SC with a duration of less than 2 years (Group 1) from those with SC lasting more than 2 years (Group 2). METHODS: Prospective assessment of all patients with SC seen at our service from July 1993 through March 1998 analyzing the following: gender; age at onset; frequency of arthritis, carditis, family history of RF and SC; topographic distribution; and chorea severity on a 0-4 scale. RESULTS: Thirty-two patients (19 female, 13 male) were studied. In Group 1 (16 subjects, 50%) the follow-up period was 36.2 +/- 20.0 months; 50% were female; age at onset was 10.9 +/- 2.6 years; arthritis and carditis were present in 37.5% and 31.2%, respectively; family history of SC was reported by 18.7%; hemichorea was seen in 25.8% of subjects; and the mean intensity of chorea was 2.6 +/- 0.8. In Group 2, with a follow-up period of 34.1 +/- 18.9 months, 68.8% were female; age at onset was 9.3 +/- 3.9 years; arthritis and carditis were diagnosed in 18.7% and 50%, respectively; no patient reported a family history of SC; hemichorea was observed in 6.2% of subjects; and the mean intensity of chorea was 2.8 +/- 0.5. No difference was statistically significant. CONCLUSIONS: SC persists in half of our patients. Female gender, possibly related to endocrine factors, as well as the presence of carditis, indicating a more severe disease, may be risk factors for a longer duration of SC.     

Physiological observations in Sydenham's chorea.

Involuntary movements in a patient with Sydenham's chorea were usually characterised by bursts of EMG activity lasting more than 100 ms, occurring asynchronously in antagonist muscles. The "hung-up" knee jerk was a choreic movement induced by the tendon tap. Exaggerated voluntary movements were produced with EMG burst longer than normal.     

Nerve conduction study in Sydenham's chorea.

Sydenham's chorea (SC) is a late complication of group A beta-hemolytic streptococci infection presumably caused by an abnormal autoimmune reaction. Despite rare case reports of peripheral neuropathy associated with streptococcal infection, there is no investigation of peripheral nerve in SC. We performed nerve conduction studies in a cohort of patients with SC. The neurophysiology investigation comprised measurement of amplitude and sensory conduction velocity of median, ulnar, and sural nerves; amplitude and motor conduction velocity; and F-wave latency of median, ulnar, fibular, and tibial nerves. Twenty-six patients entered the study (12 females, 14 males; mean age 12.8 +/- 3.6 years). Thirteen subjects had absent or decreased deep reflexes. All investigated neurophysiological parameters fell within the normal range for our population. We failed to find neurophysiological evidence of peripheral nerve involvement in patients with a history of SC. Our findings suggest that the possible autoimmune dysfunction in SC patients is not targeted against epitopes present in peripheral nerves.     

Corticosteroid treatment in patients with Sydenham's chorea

Sydenham's chorea occurs in approximately 10% of acute rheumatic fever and is one of its major manifestations. The disease may last for weeks or months, with a high risk of recurrence; usually only supportive treatment is recommended. This report describes five children diagnosed with Sydenham's chorea and treated with a short course of corticosteroids. Marked improvement of the involuntary movements was observed within 24-48 hours, with complete resolution within 7-12 days after commencement of treatment; there were no relapses. Larger, possibly comparative studies are necessary, but in the meantime treatment with corticosteroids in patients with Sydenham's chorea should be considered.     

Chronic dopaminergic sensitivity after Sydenham's chorea

We studied psychometric performance on the Minnesota Multiphasic Personality Inventory (MMPI) mini-mult and drug-induced choreic reactions in a group of patients with a history of Syndenham's chorea. Action tremor, motor signs, and residual chorea were common. One-half of the patients reported adverse choreic reactions to one or more agents. Patients with adverse reactions to central stimulants and anorectics had statistically significant elevations in the psychotic tetrad of the MMPI. Sydenham's chorea in childhood seems to confer persistent sensitivity to agents that augment central dopaminergic activity, which may be expressed as acute chorea. Central dopaminergic sensitivity may explain earlier reports of psychologic difficulties in survivors of rheumatic chorea.     

Cerebral vasculitis in a case of Sydenham's chorea

A 10-year-old girl with acute-onset hemichorea had multiple areas of abnormal signal seen on magnetic resonance imaging of the brain, associated with middle and anterior cerebral artery vasculitis seen on cerebral angiography. Her serology and clinical course were supportive of the diagnosis of Sydenham's chorea. Other causes of cerebral vasculitis were excluded. Follow-up studies revealed resolution of changes seen on magnetic resonance imaging and partial resolution of angiographic abnormalities. This is the first report of abnormal cerebral angiography in Sydenham's chorea.     

Corticosteroids in the treatment of Sydenham's chorea.

This is a retrospective study of the treatment of eight female patients with Sydenham's chorea by corticosteroid administration. The rationale for use of this medication is based on the concept that Sydenham's chorea is caused by an indolent inflammation of the small vessels in the caudate-putamen complex. All patients in this series responded to corticosteroid treatment relatively rapidly, with considerable diminution in abnormal movements. It is concluded that corticosteroid treatment of this condition is effective and deserves wider use. This report should serve as an impetus for more controlled studies.     

Frequency and significance of vocalizations in Sydenham's chorea

Sydenham's chorea (SC) is a complication of Streptococcus infection characterized by a combination of motor and non-motor features. We have investigated the presence of vocalizations in 89 consecutive patients with SC evaluated during a one-year period in the UFMG Movement Disorders Clinic. Seven (4/3 M/F) of the 89 patients (29/60 M/F) presented with simple vocalizations not preceded by premonitory sensations but in association with facial chorea in five patients. These findings suggest that vocalizations are not a common feature in SC and their phenomenology is quite distinct from the characteristics of vocal tics in tic disorders.     

Executive functioning in adult patients with Sydenham's chorea

Sydenham's chorea (SC) is characterized by a combination of motor and behavioral findings. Cognitive function has been scarcely studied in this condition. The aim of this study is to investigate executive functions in adult patients with SC. We performed neuropsychological tests to evaluate executive functions in controls and adult patients with persistent and in remission SC. Patients with SC have impairment in Tower of London task, reduced verbal fluency, and lower scores in the Stroop test. A subset of adult patients with SC present with executive dysfunction, even when chorea is in remission. © 2010 Movement Disorder Society