Search

Background: Huntington's disease (HD) is a rare neurodegenerative disorder with protean clinical manifestations. Its management is challenging, consisting mainly of off-label treatments., Objectives: The International Parkinson and Movement Disorder Society commissioned a task force to review and evaluate the evidence of available therapies for HD gene expansion carriers., Methods: We followed the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. Eligible randomized controlled trials were identified via an electronic search of the CENTRAL, MEDLINE, and EMBASE databases. All eligible trials that evaluated one or more of 33 predetermined clinical questions were included. Risk of bias was evaluated using the Cochrane Risk of Bias tool. A framework was adapted to allow for efficacy and safety conclusions to be drawn from the balance between the GRADE level of evidence and the importance of the benefit/harm of the intervention., Results: Twenty-two eligible studies involving 17 interventions were included, providing data to address 8 clinical questions. These data supported a likely effect of deutetrabenazine on motor impairment, chorea, and dystonia and of tetrabenazine on chorea. The data did not support a disease-modifying effect for premanifest and manifest HD. There was no eligible evidence to support the use of specific treatments for depression, psychosis, irritability, apathy, or suicidality. Similarly, no evidence was eligible to support the use of physiotherapy, occupational therapy, exercise, dietary, or surgical treatments., Conclusions: Data for therapeutic interventions in HD are limited and support only the use of VMAT2 inhibitors for specific motor symptoms. © 2021 International Parkinson and Movement Disorder Society., (© 2021 International Parkinson and Movement Disorder Society.)

Chorea is defined by the presence of abnormal, involuntary, continuous, random movements that results from a number of autoimmune, hereditary, vascular, metabolic, drug-induced and functional (psychogenic) causes. Chorea may present at all stages of life, from newborns to elderly individuals. While Huntington disease is the main suspicion in adults presenting with chorea, once a drug-induced or parkinsonian dyskinesia have been ruled out; Huntington disease exceptionally presents with chorea in children. Sydenham chorea is considered the most common cause of acute childhood-onset chorea, but its prevalence has decreased in Western countries. However, in younger children other etiologies such as dyskinetic cerebral palsy, anti-NMDAR receptor encephalitis, other autoimmune conditions, or mutations in NKX2-1, ADCY-5, FOXG1, GNAO1, GPR88, SLC2A1, SQSTM1, ATP8A2, or SYT-1 should be considered. In this manuscript, we review the main causes, diagnosis and management of chorea in children.

Diseases with a choreic phenotype can be due to a variety of genetic etiologies. As testing for Huntington's disease (HD) becomes more available in previously resource-limited regions, it is becoming apparent that there are patients in these areas with other rare genetic conditions which cause an HD-like phenotype. Documentation of the presence of these conditions is important in order to provide appropriate diagnostic and clinical care for these populations. Information for this article was gathered in two ways; the literature was surveyed for publications reporting a variety of genetic choreic disorders, and movement disorders specialists from countries in Latin America and the Caribbean were contacted regarding their experiences with chorea of genetic etiology. Here we discuss the availability of molecular diagnostics for HD and for other choreic disorders, along with a summary of the published reports of affected subjects, and authors' personal experiences from the regions. While rare, patients affected by non-HD genetic choreas are evidently present in Latin America and the Caribbean. HD-like 2 is particularly prevalent in countries where the population has African ancestry. The incidence of other conditions is likely determined by other variations in ethnic background and settlement patterns. As genetic resources and awareness of these disorders improve, more patients are likely to be identified, and have the potential to benefit from education, support, and ultimately molecular therapies., (Copyright © 2018. Published by Elsevier Ltd.)

Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Emphasis is given particularly to Sydenham's chorea, systemic lupus erythematosus, primary antiphospolipid antibody syndrome, paraneoplastic chorea and anti- N -methyl-d-aspartate receptor encephalitis., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Chorea is an abnormal movement characterized by a continuous flow of random muscle contractions. This phenomenon has several causes, such as infectious and degenerative processes. Chorea results from basal ganglia dysfunction. As the control of the eye movements is related to the basal ganglia, it is expected, therefore, that is altered in diseases related to chorea. Sydenham's chorea, Huntington's disease and neuroacanthocytosis are described in this review as basal ganglia illnesses that can present with abnormal eye movements. Ocular changes resulting from dysfunction of the basal ganglia are apparent in saccade tasks, slow pursuit, setting a target and anti-saccade tasks. The purpose of this article is to review the main characteristics of eye motion in these three forms of chorea.

The mechanisms involved in the symptoms of Sydenham's chorea (SC) remain obscure. Taking into account the autoreactive antibody-mediated hypothesis of SC pathogenesis, the persistence of chorea may be associated with increased levels of B1 lymphocytes and other lymphocyte subsets. We evaluated lymphocyte subsets, including B1 and T cells, in patients with remitted (RSC) and persistent (PSC) SC by flow cytometry. Our results showed neither difference in the frequency of T and B lymphocytes subpopulations nor in their activation and functional states. These findings undermine the view of PSC as a sustained cytotoxic cellular-mediated condition. Alternative mechanisms may explain the pathogenesis of PSC.

Background: Neuropsychiatric comorbidities are frequent in Sydenham chorea. However, cognitive impairment in Sydenham chorea has not been sufficiently described. The objective of this study was to evaluate expressive and receptive language deficits in adolescents with Sydenham chorea., Methods: Twenty patients with Sydenham chorea were compared with 20 patients with rheumatic fever without chorea and 20 healthy controls. Participants were matched for age and gender. Participants were assessed with verbal fluency tasks (phonemic and semantic) and with verbal comprehension tasks (Token Test). Patients with Sydenham chorea were also assessed with the Universidade Federal de Minas Gerais Sydenham Chorea Rating Scale., Results: Performance in verbal fluency and in verbal comprehension tasks differed significantly (P < 0.01) among the three groups. Patients with Sydenham chorea performed significantly worse than healthy control group in phonemic and semantic verbal fluency tasks as well as in the Token Test. The group with rheumatic fever also performed worse than healthy controls in phonemic verbal fluency. Severity of motor signs in Sydenham chorea inversely correlated with performance in phonemic verbal fluency (words beginning with letter S, and total sum of words beginning with letters F, A, and S)., Conclusions: Adolescents with Sydenham chorea show difficulties in verbal fluency and in verbal comprehension. Patients with rheumatic fever also have some degree of language impairment. Future studies must investigate language impairment in difference stages of Sydenham chorea (acute, persistent, and remission) and putative biological markers., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Sydenham's chorea (SC) has been associated with increased frequency of psychiatric disorders. The objective of the present study was to determine whether there is any difference in the frequency of psychiatric disorders between SC patients in remission and patients with persistent chorea., Methods: Fifty consecutive patients with SC (mean age ± SD, years; 21.5 ± 6.7; M/F; 10/40) were subjected to a comprehensive and structured psychiatric evaluation., Results: The most frequent psychiatric disorders observed in SC patients were: major depression (14%); generalized anxiety disorder (16%), social phobia (24%) and obsessive-compulsive disorder (24%). The frequency of psychiatric disorders did not differ between SC patients in remission in comparison with patients with persistent chorea, except for depressive disorders which were more frequent in the later, Conclusions: Psychiatric disorders are common among young adults with SC regardless of the status of motor symptoms., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: Sydenham's chorea, a major manifestation of rheumatic fever, is characterized by chorea, behavioral changes, and cognitive dysfunction. Perfusion changes in the basal ganglia are the most frequent imaging findings observed in patients with Sydenham's chorea., Methods: Twelve adult women with Sydenham's chorea in remission underwent brain single-photon emission computed tomography (SPECT). Their scans underwent a quantification process to evaluate the perfusion of Brodmann's areas of the frontal lobes and basal ganglia. The results were compared with the findings from a control group that was matched by age., Results: A pattern of hyperperfusion in the left putamen was observed in the patient group (P = 0.02). No significant difference was observed in relation to other brain regions., Conclusions: The findings of brain SPECT suggest that perfusion abnormalities of the basal ganglia may persist even after the remission of abnormal movements in patients with Sydenham's chorea., (© 2013 Movement Disorder Society.)

Objective: To analyze the case notes of 127 patients with chorea admitted to the National Hospital at Queen Square, London, under the care of William Richard Gowers and review his contribution to the study of choreas., Methods: We consulted the case books available at the Queen Square Library, from 1878 to 1911, comprising 42 volumes., Results: 97 patients (76.3%) were female and the age of presentation ranged from 4 to 60 years (mean 14.3). 43 patients (33.8%) experienced recurrent attacks of chorea. 29 patients (22.8%) had a family history of chorea. Past history of rheumatic fever was observed in 46 patients (36.2%). 54 patients (42.5%) had speech impairment while a similar number had a cardiac murmur. Generalized chorea occurred in 87.4% and hemichorea in 11.8%. Gowers diagnosed different forms of chorea: Huntington's disease, paralytic, persistent, recurrent, tetanoid, functional, maniacal, hemichorea and chorea gravidarum., Conclusions: Gowers was one of the pioneers in recognizing chorea as a physical sign found in a myriad of etiologies. He also provided a comprehensive description of the clinical features and natural history of Sydenham's chorea in his work., (Copyright © 2012 S. Karger AG, Basel.)

Background: Sydenham's chorea (SC) is a neurological manifestation of rheumatic fever. Autoimmune mechanism of SC is supported by clinical improvement with immunomodulatory therapy; presence of circulating serum anti-basal ganglia antibodies; increase in Th2 group of cytokines in serum and CSF of patients. However, a role of the antibodies in the pathogenesis can only be established by their passive transfer. Chorea is a manifestation clearly related to increased dopaminergic (DA) activity. The purpose of this study was to investigate the potential of antibodies from patients with Sydenham's chorea to cause behavior alterations on rats with unilateral post-synaptic dopamine receptor up-regulation., Methods: Rats previously submitted to 6-hydroxidopamine (6-OH-DA) unilateral lesion of substantia nigra pars compacta (SNc) and tested with apomorphine to ensure DA receptors up regulation, received intrastriatal infusion of antibodies from SC patients (n=4) or healthy controls (n=3) during 48 h. 24h post infusion initiation (24PI) and 48 h post infusion initiation (48PI), we registered the occurrence of spontaneous contra lateral rotations (CLR)., Findings: SC group exhibited significantly higher number of CLR than control group at 24PI (p=0.049) and 48PI (p=0.048)., Conclusion: The limited sample of the present study restricts us to affirm that SC is really an immune-mediated condition. However the significant result of this pilot study points to preliminary evidence that SC antibodies may affect DA activity in rats with up-regulated striatal DA receptors., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Sydenham's chorea is associated with dysfunction of fronto-striatal circuits induced by cross-reactive antibodies to group A β-hemolytic streptococcus. High susceptibility of extrapyramidal effects of neuroleptics in patients with Sydenham's chorea suggests underlying nigro-striatal dysfunction., Objective: To study the presence of parkinsonism in patients with a history of Sydenham's Chorea., Methods: We used the UFMG Sydenham's Chorea Rating Scale (USCRS) and the Unified Parkinson's Disease Rating Scale (UPDRS) part III, respectively, to determine the presence of chorea and parkinsonian symptoms and signs in 25 adults with a history of previous Sydenham's Chorea currently without chorea or use of anti-choreic drugs., Results: Bradykinesia was found in 64% of subjects. There was a statistically significant correlation between bradykinesia and hemichorea (-0.412; p = 0.036) and bradykinesia and generalized chorea (0.412; p = 0.036). There was no correlation between bradykinesia and use of anti-choreic drugs., Conclusions: Bradykinesia is common in patients with Sydenham's Chorea in remission. This finding suggests an immune-mediated dysfunction of the nigro-striatal system., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: Sydenham's Chorea is a frequent cause of chorea during pregnancy, chorea gravidarum. The aim of this article is to describe the effect of pregnancy in a consecutive series of patients with diagnosis of Sydenham's Chorea., Methods: A chart review was performed of all patients with the diagnosis of Sydenham's Chorea followed up at our institution from 07/1993 through 08/2010 and who became pregnant., Results: From 66 patients, 20 became pregnant. Of these 20 patients, 15 (75%) developed chorea gravidarum. Generalized chorea was found in 67% of these 15 patients, focal or multifocal chorea was identified in 20% and 13.4% developed hemichorea. In 80% of cases chorea began in the first 6 months of gestation. Three women with previous persistent chorea experienced worsening of the movement disorder during pregnancy. Remission occurred after delivery in 11 patients whereas the other four remained with non-disabling chorea during the first 12 months after delivery. Abortion occurred in two patients (13%). All patients with chorea gravidarum subsequently treated with oral contraceptives developed recurrence of chorea., Conclusions: Chorea gravidarum is a frequent complication of pregnancy in patients with previous history of Sydenham's Chorea and an increased risk of miscarriage should be considered. Our findings confirm the notion that chorea gravidarum results from hormonal changes acting on previously dysfunctional basal ganglia., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Sydenham's chorea (SC) is the neurologic expression of acute rheumatic fever (ARF). Despite the declining incidence of ARF worldwide, it remains the most common cause of acute chorea in children. It is characterized by a combination of motor and nonmotor features. In addition to chorea, among the first are decreased muscle tone and tics. Nonmotor features include obsessions, compulsions, attention deficit, emotional lability, decreased verbal fluency, and executive dysfunction. Most patients present with nonneurologic features of ARF, such as carditis and arthritis. The pathogenesis is thought to involve streptococcus-induced antibodies which cross-react with antigens of the basal ganglia. The diagnosis is made on purely clinical grounds since there is no biological marker of the illness. The management is based on use of antichoreic agents, such as valproic acid and neuroleptics, and prophylaxis of new bouts of streptococcus infection with antibiotics. Although the motor features of SC come into spontaneous remission in the majority of patients, a significant proportion of individuals remain with persistent chorea., (Copyright © 2011 Elsevier B.V. All rights reserved.)

There are few studies of language and speech in patients with Sydenham's chorea (SC). We have done an acoustic analysis of fundamental frequency (F0), duration and intensity of declarative and interrogative sentences made by 20 SC patients, 20 patients with rheumatic fever (RF) without chorea, and compared them with 20 healthy age-matched controls (CO). Each group included 12 females. We found that there is no difference between the RF and CO groups in all studied parameters. Patients with SC, however, presented with a speech characterized by decreased F0 range (difference between minimum and maximum F0), shorter duration of sentences, and higher intensity of the first syllable of sentences. The findings were not influenced by the nature of the sentences (i.e. , declarative or interrogative), but for all variables they were significantly more severe in males than females. In conclusion, we have demonstrated that patients with acute SC have an impairment of modulation of F0 and longer duration of emission of sentences, resulting in a monotone and slow speech. This pattern is similar to what has been described in other basal ganglia illnesses, such as Parkinson's disease, Huntington's disease and Wilson's disease.

Sydenham's chorea (SC) is the neurologic manifestation of rheumatic fever. In addition to involuntary movements, SC patients show behavioral changes, such as hyperactivity, obsessions, and compulsions. Brain-derived neurotrophic factor (BDNF) is related to neuronal development and differentiation. Since BDNF serum levels are altered in a series of neuropsychiatric disorders, such as schizophrenia and Huntington's disease, we investigated the serum levels of BDNF in SC patients. Eighteen patients with acute SC, 4 with persistent SC and 27 control subjects were included in this study. BDNF was determined by ELISA. There was no significant difference between BDNF serum levels of control and acute SC groups (P = 0.12). Persistent SC patients presented decreased BDNF levels when compared to both control and acute SC groups (P < 0.001). Our results suggest that the persistence of symptoms in SC may be related to structural changes in the central nervous system as expressed by altered BDNF levels.

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., ((c) 2010 Movement Disorder Society.)

Until now, there are no conclusive data about the mechanisms involved in motor symptoms of Sydenham's chorea (SC). Taking into account the autoreactive antibody-mediated hypothesis of SC pathogenesis, the SC may be associated with uncontrolled immune mechanisms. Besides the antibody hypothesis, the innate immune system has been underappreciated. Hence, we evaluated the activation state of monocytes, cells that are precursors of macrophages, to characterize the inflammation profile of patients. We assessed the surface molecules CD80, CD86, and human leukocyte antigen DR expression in patients with SC by flow cytometry analysis. Our results showed a decreased CD14(+) (monocyte) frequency, with concomitant increased CD14(-) frequency inside monocyte population. Although monocyte population showed a decreased human leukocyte antigen DR and CD86 frequencies, the CD14(-) population showed an increased frequency of CD80(+) monocyte from SC compared with controls. These data suggest that monocytes showed a reduced costimulatory potential in SC.

Chorea is defined as a syndrome characterized by brief, abrupt involuntary movements resulting from a continuous flow of random muscle contractions. There are genetic and non-genetic causes of chorea. The most common genetic cause of chorea is Huntington's disease (HD). Non-genetic forms of chorea include vascular choreas, auto-immune choreas, metabolic and toxic choreas, and drug-induced choreas. This chapter provides an overview of clinical features, pathogenesis and management of HD, other important genetic causes of chorea, Sydenham's chorea, other autoimmune choreas and vascular choreas.

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.

Sydenham's chorea (SC) is characterized by motor, mainly choreic involuntary movements, and psychiatric symptoms, including anxiety, depression, obsessive-compulsive and attention-deficit/hyperactivity disorders. Symptoms of SC may be considered as the result of basal ganglia dysfunction determined by autoimmune mechanisms elicited by streptococcal infection. We report on a case of a 13-year-old boy with brief psychotic episode following acute SC. His hallucinations and delusions remitted after treatment with risperidone, paralleling motor improvement. The neurobiological basis of psychotic symptoms remains largely elusive. This case report provides further insight about the involvement of basal ganglia dysfunction on psychosis.

Chorea is one of the major types of involuntary movement disorders originating from dysfunctional neuronal networks interconnecting the basal ganglia and frontal cortical motor areas. The syndrome is characterised by a continuous flow of random, brief, involuntary muscle contractions and can result from a wide variety of causes. Diagnostic work-up can be straightforward in patients with a positive family history of Huntington's disease or acute-onset hemichorea in patients with lacunar stroke, but it can be a challenging and complex task in rare autoimmune or genetic choreas. Principles of management focus on establishing an aetiological classification and, if possible, removal of the cause. Preventive strategies may be possible in Huntington's disease where genetic counselling plays a major part. In this review we summarise the current understanding of the neuroanatomy and pathophysiology of chorea, its major aetiological classes, and principles of diagnostic work-up and management.

Background: Sydenham's chorea (SC) is a disorder associated with rheumatic fever and related to Streptococcus-induced immune reaction cross-reactive with basal ganglia antigens. Obsessive-compulsive disorder and other behavioral abnormalities have been described in SC. There are scarce data of cognitive function in this condition., Objective: To assess verbal fluency in patients with SC., Patients and Methods: We have compared the semantic (SVF) and phonemic verbal fluency (PVF) of 20 subjects with SC and 40 controls matched by age, gender and years of school., Results: The scores of the control group on the SVF and PVF tests were 26.3 +/- 5.7 and 18.0 +/- 6.4 whereas the SC patients scored 25.1 +/- 6.9 and 12.5 +/- 8.2. PVF was significantly decreased in the SC group (p < 0.01)., Conclusion: SC is associated with reduction of PVF. This finding may reflect disruption of the dorsolateral prefrontal-striatal circuit caused by the auto-immune process characteristic of SC.

The authors analyse the correspondence between Eça de Queiroz, a well-known Portuguese writer, and his wife, in 1900, describing the neurological disease of their second son, José Maria d'Eça de Queiroz, know as Zezé. In those letters, the writer mentions chorea as his son diagnosis, explaining in detail its clinical picture. In addition, the writer reveals the medical thoughts of the time about the aetiology, treatment and natural history of Sydenham's Chorea.

Sydenham's chorea (SC), a major manifestation of acute rheumatic fever (RF), is characterized by chorea and other motor and non-motor features. Among the latter are behavioral symptoms, including obsessive-compulsive disorder. Although SC is typically a self-limited condition, up to 50% of patients may evolve with persistent chorea. There is evidence that Gustav Mahler had a movement disorder, but its nature remains undetermined. There are witnesses describing him as having facial dyskinesia and a gait disorder consistent with chorea. His conducting performance was notorious for obsessive attention to details of the staging and musical production. Mahler was diagnosed with a valvulopathy in 1907 and died of subacute bacterial endocarditis in 1911. It is possible that the composer suffered from RF in childhood with carditis and SC, which may left him with valvulopathy, obsessive-compulsive disorder, and persistent chorea.

Recently we developed and validated the Universidade Federal de Minas Gerais (UFMG) Sydenham's chorea Rating Scale (USCRS) to systematically assess SC patients. In this study, we assessed 97 children and adults with SC (mean age +/- SD, 15.5 +/- 5.9; male/female, 31/66) seen at the Movement Disorders Clinic at UFMG employing the USCRS. The patients were divided into 4 groups according to their clinical status: acute (n=19), recurrent (n=17), persistent (n=19) and remission (n=42). The mean +/- SEM USCRS scores for each group were: 47.7 +/- 4.7 for acute group, 29.5 +/- 2.6 for recurrent group, 17.6 +/- 3.1 for persistent group and 1.1 +/- 0.2 for remission group. All pair comparisons were statistically significant (p<0.05). Our results indicate that the USRSC can reasonably discriminate groups of SC patients in different clinical stages of the disease.

Serious adverse effects with antichoreic drugs can occur in patients with acute Sydenham's chorea (SC). The response to conventional treatment in severe SC may also be poor. Thus alternative therapeutic strategies have been developed. We report on four patients with severe acute SC (two with chorea paralytica and two with neuroleptic-induced parkinsonism) treated with i.v. methyl-prednisolone pulse-therapy followed by oral prednisone. The mean+/-SEM clinical rating score dropped from 63.7+/-10.2 to 33.5+/-10.6 in the first month post-treatment but in a lesser degree thereafter. Only one patient developed weight gain and 'moon facies'. This case series suggests that immune suppression with corticosteroids may be an effective and safe treatment in complicated and severe acute SC.

We compared the frequency of migraine among Sydenham's chorea (SC) patients, rheumatic fever (RF) patients without neurological symptoms and matched controls. Migraine was more frequent in SC patients (12/55, 21.8%) than in controls (9/110, 8.1%) and as common as in the RF group (10/55, 18.2%). Our data are in agreement with previous studies reporting higher frequency of migraine in other basal ganglia disorders, such as essential tremor and Tourette's syndrome.

The proposed pathogenesis of Sydenham's chorea (SC) is an autoantibody-mediated basal ganglia dysfunction. Our study has shown that incubation of PC12 cells with complement-inactivated serum from SC patients was associated with a significant increase in Ca2+ levels evoked by KCl stimulus (mean +/- SEM, 341.0 +/- 8.7% of fluorescence intensity, arbitrary units) when compared with incubation with control serum (313.8 +/- 8.7% of fluorescence intensity, arbitrary units; P = 0.01). The increase in Ca2+ levels determined by SC patients sera correlated directly with the enzyme-linked immunosorbent assay optical density values for anti-basal ganglia antibodies. Our study supports the hypothesis that antibodies against basal ganglia in SC may cause their dysfunction., (Copyright 2005 Movement Disorder Society.)

The authors investigated obsessive-compulsive behavior, obsessive-compulsive disorder (OCD), and attention deficit and hyperactivity disorder (ADHD) in 50 healthy subjects, 50 patients with rheumatic fever without chorea, and 56 patients with Sydenham chorea. Obsessive-compulsive behavior, OCD, and ADHD were more frequent in the Sydenham chorea group (19%, 23.2%, 30.4%) than in the healthy subjects (11%, 4%, 8%) and in the rheumatic fever without chorea group (14%, 6%, 8%). ADHD was more common in persistent Sydenham chorea.

Despite the renewed interest in Sydenham's chorea (SC) in recent years, there were no valid and reliable scales to rate the several signs and symptoms of patients with SC and related disorders. The Universidade Federal de Minas Gerais (UFMG) Sydenham's Chorea Rating Scale (USCRS) was designed to provide a detailed quantitative description of the performance of activities of daily living, behavioral abnormalities, and motor function of subjects with SC. The scale comprises 27 items and each one is scored from 0 (no symptom or sign) to 4 (severe disability or finding). Data from 84 subjects, aged 4.9 to 33.6 years, support the interrater reliability and internal consistency of the scale. The USCRS is a promising instrument for rating the clinical features of SC as well as their functional impact in children and adults., (Copyright 2005 Movement Disorder Society.)

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., (2004 Movement Disorder Society.)

Purpose of Review: The aim of this article is to review the literature on the non-genetic causes of chorea. The differential diagnosis of the large number of causes of sporadic chorea is often a challenging task. Interest has also been growing in the possibility that the mechanism responsible for Sydenham's chorea plays a role in the pathogeneis of other neuropsychiatric disorders., Recent Findings: Stroke is the main cause of sporadic chorea. Sydenham's chorea shares clinical features with tic disorders, such as obsessive-compulsive disorder and attention deficit hyperactivity disorder. However, there are unequivocal differences between Sydenham's chorea and Tourette's syndrome. There is initial evidence suggesting the beneficial effect of immunosuppression in Sydenham's chorea. Other autoimmune causes of chorea include systemic lupus erythematosus as well as paraneoplastic syndromes. The growing list of drugs associated with chorea include lamotrigine, methadone and lithium. Among infectious agents, HIV is the leading reported cause of chorea., Summary: Patients with sporadic chorea require a thorough work up because numerous causes can lead to this condition. It remains unconfirmed whether the pathogenic mechanisms of Sydenham's chorea are responsible for other conditions such as isolated obsessive-compulsive disorder or Tourette's syndrome. Drugs and infectious agents, especially HIV, are often implicated in the causes of chorea.

Sydenham's chorea (SC) is thought to result from the action of streptococcus-induced antibodies that cross react with basal ganglia antigens. Much less is known, however, about the involvement of cellular mechanisms in its pathogenesis. Since chemokines seem to play a role in several CNS inflammatory disorders, we sought to investigate the chemokine profile of patients with SC. Increased serum levels of CXCL9, formerly monokine induced by interferon-gamma (Mig), and CXCL10, formerly interferon-gamma-inducible protein of 10 kDa (IP-10) were demonstrated in acute SC patients, suggesting that a particular group of chemokines may be involved in SC pathogenesis.

Use of oral contraceptives is a recognised but infrequent cause of chorea. This type of chorea has usually been considered a reactivation of Sydenham's chorea by an unknown mechanism. A patient developed a chorea triggered by the use of oral contraceptives with no definite evidence of previous Sydenham's chorea or recent streptoccocal infections. However, the patient had positive anti-basal ganglia antibodies, which supports an immunological basis for the pathophysiology of this chorea.

Despite treatment with valproic acid and neuroleptics, a significant proportion of patients with Sydenham chorea (SC) remain with chorea. We evaluated the effect of intravenous methyl-prednisolone followed by oral prednisone in patients with SC refractory to conventional treatment. Patients were enrolled in the study if they failed to improve with conventional treatment, despite the development of side effects. Chorea was rated on a 0 to 4 score. Five patients, 3 of them women, were included in the study. The median pretreatment rating score of the chorea was 3 (range, 3-4) and dropped to 1 (range, 0-2) after a median follow-up of 7 months (range, 3-7 months). Two patients developed Cushing syndrome. Our data suggest that intravenous methyl-prednisolone followed by oral prednisone is an effective and well-tolerated treatment of refractory SC.

Previous investigations have suggested that Sydenham's chorea (SC) may be an autoantibody mediated disorder. We examined this autoimmune hypothesis by measuring Th1 (IFN-gamma, IL-12) and Th2 (IL-4, IL-10) cytokines, oligoclonal bands (OCB) and anti-basal ganglia antibodies (ABGA). CSF IL-4 was elevated in 31% of acute SC and 50% of persistent SC. CSF IL-10 was also elevated in 31% of acute SC but 0% of persistent SC. CSF IFN-gamma was undetectable in all patients. Serums IL-4, IL-10 and IL-12 were elevated in acute compared to persistent SC. OCB were found in 46% of acute SC, ABGA were in 93% of acute SC and 50% of persistent SC was of IgG(1) and IgG(3) subclass. These findings support an autoantibody pathogenesis.

Objective: To determine the sensitivity and specificity of methods to detect anti-basal ganglia antibodies (ABGA) in Sydenham's chorea (SC)., Background: SC is a delayed manifestation of group Abeta hemolytic streptococcal infection typically associated with rheumatic fever (RHF). SC is characterized by chorea and motor and neuropsychiatric symptoms. Patients with SC produce antibodies that cross-react with streptococcal, caudate, and subthalamic nuclei antigens detected using an immunofluorescent (IF) method with inconsistent reports of positivity., Methods: The authors developed ELISA and Western immunoblotting (WB) methods to detect ABGA and compared these assays to IF. They investigated samples from patients with acute SC (n = 20), persistent SC (n = 16), control samples from RHF (n = 16), and healthy pediatric volunteers (n = 11)., Results: ABGA ELISA had a sensitivity of 95% and specificity of 93% in acute SC. Both WB and IF had a sensitivity of 100% and specificity of 93%. In the persistent SC group, ABGA sensitivity dropped to 69% using WB and to 63% using IF. Three common basal ganglia antigens were identified by WB in both acute and persistent SC (40 kDa [n = 15], 45 kDa [n = 15], and 60 kDa [n = 13]). There was no antibody reactivity to cerebellum, cerebral cortex, or myelin antigen preparations in any group., Conclusions: These results support the hypothesis that Syndenham's chorea is an autoantibody-mediated disorder. Western immunoblotting and immunofluorescence are the best methods for detecting anti-basal ganglia antibodies, and reactivity to basal ganglia antigens of 40, 45, and 60 kDa were commonly seen in both acute and persistent cases of SC.

We describe a 36 year-old woman who developed chorea two months after starting the use of oral contraceptives. She also developed thrombocytopenia, oral ulcers, arthritis, positive antinuclear antibodies (ANA), anti-Sm and anti-DNA, filling criteria for systemic 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 labatorial 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.

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.

Rheumatic fever (RF) remains a significant public health problem in developing countries. Although its incidence has been declining in developed areas, several outbreaks of RF have been identified in the United States since 1985. To study the prevalence and clinical features of Sydenham's chorea (SC), a well-recognized expression of RF, we examined 50 consecutive patients with RF seen at our Pediatric Cardiology Clinic. Chorea was rated on a scale of 0-4 (0 = no chorea; and 4 = severe chorea, patient unable to walk). Chorea, active or in remission, occurred in 13 patients (26%). The male-to-female ratio was 1.08 among the total population and 0.86 among the SC group. The mean age of the 50 patients at onset of RF was 8.4 years and the standard deviation (SD) 3.1 years. Carditis was diagnosed in 84% of patients with and without chorea, but arthritis was more common in the latter group (83.8%) than in the SC group (30.8%). The mean age at onset of SC was 9.2 years (SD 2.7). Involuntary movement, noticed by nine patients, was the most common initial symptom. Chorea went into remission in eight patients after 9.9 months (SD 13.4). Chorea was rated 3 in ten patients, 1 in two patients, and 4 in one patient. Among the five patients with active SC, three had hemichorea and two had generalized chorea. We recognized abnormal saccades in 80% of patients with active chorea, whereas just 13.5% of patients without chorea displayed this abnormality. SC remains an important cause of chorea in countries plagued by RF.