Postinfectious Inflammation, Autoimmunity, and Obsessive-Compulsive Disorder: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection, and Pediatric Acute-Onset Neuropsychiatric Disorder.

Postinfectious neuroinflammation has been implicated in multiple models of acute-onset obsessive-compulsive disorder including Sydenham chorea (SC), pediatric acute-onset neuropsychiatric syndrome (PANS), and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS). These conditions are associated with a range of autoantibodies which are thought to be triggered by infections, most notably group A streptococci (GAS). Based on animal models using huma sera, these autoantibodies are thought to cross-react with neural antigens in the basal ganglia and modulate neuronal activity and behavior. As is true for many childhood neuroinflammatory diseases and rheumatological diseases, SC, PANS, and PANDAS lack clinically available, rigorous diagnostic biomarkers and randomized clinical trials. In this review article, we outline the accumulating evidence supporting the role neuroinflammation plays in these disorders. We describe work with animal models including patient-derived anti-neuronal autoantibodies, and we outline imaging studies that show alterations in the basal ganglia. In addition, we present research on metabolites, which are helpful in deciphering functional phenotypes, and on the implication of sleep in these disorders. Finally, we encourage future researchers to collaborate across medical specialties (e.g., pediatrics, psychiatry, rheumatology, immunology, and infectious disease) in order to further research on clinical syndromes presenting with neuropsychiatric manifestations.

[Abnormal mTOR Signaling Pathway Activity in Autism Spectrum Disorders: Prospects of Mechanism-Based Therapy].

Autism spectrum disorder (ASD) is a developmental disorder characterized by the early onset of problems with communication, learning, and behavior. The syndromic form of ASD is caused by monogenic mutations. When it is not possible to find genetic or other known mechanisms, the term "idiopathic autism" is used. A significant part of both syndromic and idiopathic autism is associated with translational deregulation dependent on the mechanistic target of rapamycin (mTOR). In this review, we present both bioinformatic and experimental data that link the mTOR signaling pathway to maternal autoantibody related autism and childhood autoimmune neuropsychiatric disorders such as Sydenham's chorea and pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS). The need for ASD subtyping and the prospects of mechanism-based therapy with inhibitors of the mTOR signaling pathway are also discussed.

Characterization of an experimental model to determine streptococcal M protein-induced autoimmune cardiac and neurobehavioral abnormalities.

Group A streptococcal (GAS) infection is associated with a spectrum of autoimmune diseases including acute rheumatic fever/rheumatic heart disease (ARF/RHD) and neurobehavioral abnormalities. Antibodies against GAS M proteins cross-react with host tissue proteins in the heart and brain leading to the symptomatology observed in ARF/RHD. As throat carriage of Streptococcus dysgalactiae subspecies equisimilis (SDSE) has been reported to be relatively high in some ARF/RHD endemic regions compared with GAS, and both SDSE and GAS express coiled-coil surface protein called M protein, we hypothesized that streptococci other than GAS can also associated with ARF/RHD and neurobehavioral abnormalities. Neurobehavioral assessments and electrocardiography were performed on Lewis rats before and after exposure to recombinant GAS and SDSE M proteins. Histological assessments were performed to confirm inflammatory changes in cardiac and neuronal tissues. ELISA and Western blot analysis were performed to determine the cross-reactivity of antibodies with host connective, cardiac and neuronal tissue proteins. Lewis rats injected with M proteins either from GAS or SDSE developed significant cardiac functional and neurobehavioral abnormalities in comparison to control rats injected with phosphate-buffered saline. Antibodies against GAS and SDSE M proteins cross-reacted with cardiac, connective and neuronal proteins. Serum from rats injected with streptococcal antigens showed higher immunoglobulin G binding to the striatum and cortex of the brain. Cardiac and neurobehavioral abnormalities observed in our experimental model were comparable to the cardinal symptoms observed in patients with ARF/RHD. Here for the first time, we demonstrate in an experimental model that M proteins from different streptococcal species could initiate and drive the autoimmune-mediated cardiac tissue damage and neurobehavioral abnormalities.

Evaluation of executive functions in children with rheumatic heart diseases.

BACKGROUND: Acute rheumatic fever (ARF) is a multisystemic inflammatory disease in children and young adults. The most notable complications of ARF are rheumatic heart disease (RHD) and Sydenham's chorea (SC). There have been many reports about executive dysfunctions with children who have SC. "Executive function" is an umbrella term that is used to describe higher level cognitive functions. The aim of this study is to determine the executive functions of children with RHD. We evaluated executive functions in healthy children with the same sociodemographic characteristics as children with RHD. METHODS: Our study was designed as a cross-sectional randomized study, including children with RHD aged between 12 and 18, and healthy controls. The difference between the patient and control group participants in terms of age, gender, education level, education level of the parents, family income level, and executive functions were investigated. Executive functions composed of Digit Sequence Test, Verbal Fluency Test, Trail-Making Test, Stroop Test, Wisconsin Card Sorting Test. RESULTS: In our study, a total of 30 children with RHD were followed up at the pediatric cardiology outpatient clinic of Bezmialem Vakif University Hospital composed the patient group. The control group was made up of 30 healthy children of the same sex and age group as the patient group. The mean age of the case group was 14.73 ± 1.84 years. The Digit Span Test, Verbal Fluency Test, Trail-Making Test, Wisconsin Card Sorting Test, and Stroop Test produced no statistically significant differences between the RHD patients and the controls. CONCLUSIONS: No statistically significant difference was found between the RHD patients and control patients in any executive function test. It was suggested that executive dysfunction might not develop in RHD patients before developing SC.

Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting.

BACKGROUND: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF. CASE PRESENTATION: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs. CONCLUSIONS: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

Chorea in children: etiology, diagnostic approach and management.

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.

Preliminary data on prednisone effectiveness in children with Sydenham chorea.

The objective of the study is to evaluate the efficacy of corticosteroids in Sydenham chorea. This is a retrospective observational study. Clinical information of children with Sydenham chorea were collected. Outcome of Sydenham chorea was evaluated in consideration of presence or absence of corticosteroid therapy. Thirty patients were enrolled. A total of 15 were treated with prednisone, 15 received symptomatic drugs or no treatment. Patients who were treated with prednisone showed faster improvement (4 vs 16 days; p = 0.002) and shorter median time of remission (30 vs 135 days; p < 0.001).Conclusion: Our study showed that corticosteroid therapy is an effective treatment of Sydenham chorea.What is Known:• Steroid treatment in Sydenham chorea is widely used but it is not standardized.• Few manuscript report a beneficial use of steroids in Sydenham chorea if compared with no treatment.What is New:• Steroid treatment seems to be effective in both clinical remission and clinical improvement of symptoms among patients with Sydenham chorea.• Steroid treatment seems to be superior to conventional treatment.

Efficacy of levetiracetam in the treatment of Sydenham chorea.

BACKGROUND: To study the effect of levetiracetam in treating Sydenham chorea. METHODS: We retrospectively collected the data of 140 patients diagnosed with Sydenham chorea in the pediatric neurology and pediatric cardiology outpatient clinics of Van Training and Research Hospital between January 2010 and December 2018. RESULTS: There were 140 patients, 102 (70%) of whom were girls, with mean age of onset 11.8 ± 2.7 years. Symptomatic treatment was initiated in all patients at the time of diagnosis; this medication was changed during follow up in 15 patients. The most frequently prescribed drugs were haloperidol and sodium (Na) valproate, and the most frequently discontinued one was haloperidol, due to side effects. The second-choice drug was most often levetiracetam. Clinical response often began within the first 2 weeks, with Na valproate (P = 0.002), within 4 weeks with carbamazepine (P = 0.037) but 1-6 months with haloperidol (P = 0.018) and levetiracetam (P = 0.008). Time to full remission was similar with Na valproate, carbamazepine, haloperidol, and levetiracetam (P = 0.276). Our study indicated that levetiracetam was as effective as the other commonly used drugs in the symptomatic treatment of Sydenham chorea. CONCLUSION: Levetiracetam might be an option in the treatment of Sydenham chorea because of its acceptable effect and safety profile. This observation needs further support with evidence obtained through controlled and blinded trials.

Sydenham's Chorea.

BACKGROUND: Sydenham's chorea is the most common acquired movement disorder of adolescence. This clinical manifestation of acute rheumatic fever has a clear and documented relationship with Group A streptococcal infections. The symptoms are involuntary choreiform movements that can affect the face and all extremities. The pathophysiology remains unclear. CASE REPORT: A 12-year-old female was brought to the emergency department with a 2-week history of involuntary muscle spasms of her right arm and leg. Her parents reported intermittent slurred speech and difficulty grasping utensils. Physical examination revealed an awake, alert, age-appropriate female with normal cranial nerves. Patient was found to have choreoathetoid movements on the right extremities with dystonia of right leg with ambulation. Neurology consultation, computed tomography of the head, and magnetic resonance imaging of the brain did not show any acute pathology. Echocardiogram did show mild tricuspid regurgitation, suggestive of rheumatic fever. Anti-streptolysin O titer was markedly elevated, along with DNAse-B antibodies. The patient had marked improvement of movement disorder at just over 1 week later. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Sydenham's chorea is a rare but important movement disorder often related to Group A streptococcus and rheumatic fever. The incidence of rheumatic fever has been decreasing in North America but continues to be much more prevalent in developing countries as well as immigrant populations. This diagnosis is rare and can occasionally be misdiagnosed as a "fidgety" child or as a psychiatric manifestation. Sydenham's chorea is important to diagnose because acute treatment and prophylactic antibiotics can help improve symptoms and minimize cardiac damage.

Neuropsychological manifestations in children with Sydenham's chorea after adjunct intravenous immunoglobulin and standard treatment.

This was an exploratory study comparing neuropsychological manifestations of Sydenham's chorea (SC), 6 months after initiation of treatment, in children who had received intravenous immunoglobulins as an adjunct to standard treatment, with those who had received standard treatment. We included a non-SC control group for comparison. We hypothesized that compared to controls, children with SC who had received prior intravenous immunoglobulins would demonstrate less pronounced impairments compared to those who had received standard care. We conducted a cross-sectional analysis of 17 children with -SC who had received treatment 6 months previously (9 treated with standard of care and 8 augmented with intravenous immunoglobulins) and 17 non-SC, medically well controls. The standard treatment group (n = 9) exhibited significant behavioral difficulties, including significantly poorer co-operation (p = 0.009) compared with the other augmented immunoglobulins and non-SC control groups, and increased impulsivity (p = 0.016) compared with non-SC controls. The standard treatment group scored significantly lower than the other two groups on a measure of executive functioning (p = 0.03). Children with SC may be more at risk for neuropsychological difficulties than non-SC, medically well children. Intravenous immunoglobulins may mitigate some of these impairments.

Changes in motor cortical excitability in patients with Sydenham's chorea.

BACKGROUND AND PURPOSE: The neurophysiological characteristics of motor cortex have been well characterized in patients with Huntington's disease. We present the first data on cortical excitability in patients with Sydenham's chorea. METHODS: Motor cortex excitability was examined using transcranial magnetic stimulation in 16 patients in the early clinical stages of Sydenham's chorea and in 17 age- and sex-matched control subjects. Investigations included resting and active motor threshold, motor evoked potential, input-output curves, contralateral silent period, and transcallosal inhibition. RESULTS: Resting and active motor threshold were significantly higher and motor evoked potentials were significantly smaller in patients in comparison with controls. The input-output curves were shallower in both hemispheres of patients with chorea compared with controls. No significant differences were seen in silent period or transcallosal inhibition duration. CONCLUSION: Sydenham's chorea is characterized by reduced excitability of corticospinal output similar to that observed in Huntington's disease.

Brain SPECT in Sydenham's chorea in remission.

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.

Behavioral and neural effects of intra-striatal infusion of anti-streptococcal antibodies in rats.

Group A ß-hemolytic streptococcal (GAS) infection is associated with a spectrum of neuropsychiatric disorders. The leading hypothesis regarding this association proposes that a GAS infection induces the production of auto-antibodies, which cross-react with neuronal determinants in the brain through the process of molecular mimicry. We have recently shown that exposure of rats to GAS antigen leads to the production of anti-neuronal antibodies concomitant with the development of behavioral alterations. The present study tested the causal role of the antibodies by assessing the behavior of naïve rats following passive transfer of purified antibodies from GAS-exposed rats. Immunoglobulin G (IgG) purified from the sera of GAS-exposed rats was infused directly into the striatum of naïve rats over a 21-day period. Their behavior in the induced-grooming, marble burying, food manipulation and beam walking assays was compared to that of naïve rats infused with IgG purified from adjuvant-exposed rats as well as of naïve rats. The pattern of in vivo antibody deposition in rat brain was evaluated using immunofluorescence and colocalization. Infusion of IgG from GAS-exposed rats to naïve rats led to behavioral and motor alterations partially mimicking those seen in GAS-exposed rats. IgG from GAS-exposed rats reacted with D1 and D2 dopamine receptors and 5HT-2A and 5HT-2C serotonin receptors in vitro. In vivo, IgG deposits in the striatum of infused rats colocalized with specific brain proteins such as dopamine receptors, the serotonin transporter and other neuronal proteins. Our results demonstrate the potential pathogenic role of autoantibodies produced following exposure to GAS in the induction of behavioral and motor alterations, and support a causal role for autoantibodies in GAS-related neuropsychiatric disorders.

Recognizing red flags for alternative diagnoses in pediatric chorea beyond Sydenham's.

BACKGROUND: Chorea is a common movement disorder in children, requiring thorough clinical assessment and appropriate tests for etiological diagnosis. Early identification of treatable conditions can lead to effective treatment, reducing morbidity and improving quality of life. OBJECTIVE: To describe the clinical, demographic, and epidemiological characteristics of children and adolescents diagnosed with chorea. METHODS: A retrospective cross-sectional study of pediatric patients treated in a fourth-level hospital in Bogotá, Colombia, from January 2008 to January 2022. RESULTS: 81 patients with chorea were found. The most frequent etiologies were rheumatic fever (50.6 %), vascular chorea (29.3 %), and chorea secondary to lupus (11.1 %). Patients with chorea secondary to rheumatic fever (Sydenham's chorea) were older compared to other etiologies, 10.36 ± 3.41 years vs 8.29 ± 5.16, p = 0.037. The presence of another movement disorder or abnormalities during the physical examination suggests a different etiology from rheumatic fever. Moreover, the presence of psychiatric symptoms was similar in all etiologies. However, a correlation between age and psychiatric symptoms was observed with an odds ratio of 1.14 95 % CI 1.02-1.29 per year. CONCLUSIONS: Red flags in Sydenham's chorea suggesting an alternate etiology are younger age, the presence of other abnormal movements or other findings in the neurological exam or in the magnetic resonance imaging. No significant statistical differences were found between the etiologies with the presence of neuro-psychiatric manifestations. Nevertheless, these manifestations are very frequent in abnormal movements. This study evidenced the positive correlation between age and the presence of psychiatric symptoms.

Management, treatment, and clinical approach of Sydenham's chorea in children: Italian survey on expert-based experience.

Sydenham's chorea (SC), an autoimmune disorder affecting the central nervous system, is a pivotal diagnostic criterion for acute rheumatic fever. Primarily prevalent in childhood, especially in developing countries, SC manifests with involuntary movements and neuropsychiatric symptoms. Predominantly occurring between ages 5 and 15, with a female bias, SC may recur, particularly during pregnancy or estrogen use. The autoimmune response affecting the basal ganglia, notably against dopamine, underlies the pathophysiology. Clinical management necessitates an integrated approach, potentially involving immunomodulatory therapies. To address discrepancies in SC management, a survey was conducted across Italy, targeting specialists in neurology, pediatrics, child neuropsychiatry, and rheumatology. Of the 51 responding physicians, consensus favored hospitalization for suspected SC, with broad support for laboratory tests and brain MRI. Treatment preferences showed agreement on oral prednisone and IVIG, while opinions varied on duration and plasmapheresis. Haloperidol emerged as the preferred symptomatic therapy. Post-SC penicillin prophylaxis and steroid therapy gained strong support, although opinions differed on duration. Follow-up recommendations included neuropsychological and cardiological assessments. Despite offering valuable insights, broader and more studies are needed in order to guide treatment decisions in this well-known yet challenging complication of acute rheumatic fever, which continues to warrant scientific attention and concerted clinical efforts.

Sydenham's chorea in a 16-year-old female from Bhutan: A case report.

Key Clinical Message: Rheumatic heart disease is a preventable disease. Patients may not present with a typical history of sore throat and polyarthritis but may present with Sydenham's chorea. We should not rely completely on clinical findings to rule out carditis. Echocardiography should be done to rule out subclinical carditis. Abstract: Sydenham's chorea is a major manifestation of rheumatic fever. It occurs primarily in children and is seen rarely after the age of 20 years. We describe a 16-year-old girl who presented with purposeless involuntary movements of her upper and lower limbs. Laboratory blood reports showed raised erythrocyte sedimentation rate and anti-streptolysin O. 2D Doppler Echocardiography confirmed subclinical carditis, thickened mitral and aortic valve with mild mitral regurgitation. She was managed as Acute Rheumatic Fever with oral Phenoxymethyl penicillin and Carbamazepine. At the latest follow-up interviewing the caregiver, the patient had no sequelae. Early diagnosis is key to preventing late consequences of acute rheumatic fever and rheumatic heart disease. Sydenham's chorea is a rare presentation of acute rheumatic fever. The absence of clinical carditis does not rule out carditis.

Chorea as the only presenting clinical feature of rheumatic fever: a case report.

Introduction and importance: Sydenham's chorea (SC), a major neurological manifestation of acute rheumatic fever (ARF), is commonly seen in young children and adolescents. It is characterized by rapid, unpredictable, involuntary, and nonpatterned contractions affecting mostly distal limbs. It can also be associated with clinical or subclinical carditis. SC has been reported as a major manifestation in only 3.87% cases of acute rheumatic fever in Nepal. Case presentation: The authors report a case of a 12-year-old boy with abnormal movement of his right hand and unsteady gait for 12 days. On examination, he had an abnormal hand grip with difficulty maintaining a tetanic contraction (Milkmaid's grip). Laboratory investigations revealed increased anti-Streptolysin O titre and erythrocyte sedimentation rate. Echocardiography revealed subclinical carditis. After thorough clinical examination and pertinent investigations, the final diagnosis of ARF with SC was made. Clinical discussion: SC is a major clinical feature of rheumatic fever according to the revised Jones criteria. It is related to a previous Group A ß-haemolytic Streptococcus pyogenes (GABHS) infection. Approximately 50-65% of the patients with rheumatic fever later develop clinically detectable carditis. Although a self-limiting condition, it might need treatment with antiepileptics, neuroleptics, and phenothiazines. Conclusion: Any child presenting with a movement disorder should also be considered for SC, necessitating additional testing, including a cardiovascular assessment. It needs to be distinguished from other causes of movement disorders as well as psychiatric conditions. Treatment is necessary for moderate to severe chorea that interfere with daily activities. Compliance with subsequent antibiotic prophylaxis is essential for avoiding future cardiac complications.

Rare Recurrence of Sydenham Chorea in an Adult: A Case Report.

Background: Sydenham chorea is thought to be an autoimmune condition that usually develops following a group A beta-hemolytic streptococcal infection.The onset of Sydenham chorea in adults is rare and most of the adult cases usually are secondary to recurrence following childhood illness. Risk factors for chorea recurrence include irregular antibiotic prophylactic use, failure to reach remission within 6 months, and symptom persistence for longer than a year. Case Presentation: A 27-year-old young adult Ethiopian female patient with chronic rheumatic valvular heart disease for the last 8 years experienced repetitive uncontrollable movements of her extremities and torso for three years prior to her current visit. Physical examination was significant for holosystolic murmur at the apical area radiating to the left axilla and choreiform movements apparent on all limbs and trunk. Investigations were significant for mildly raised ESR, echocardiography findings of thickened mitral valve leaflets and severe mitral regurgitation. She was successfully treated with valproic acid and the frequency of penicillin injection was made every 3 weeks with no recurrence for the first 3 months follow-up period. Conclusion: We believe that this is the first case report of adult onset recurrent Sydenham chorea (SC) from a resource-limited setting. Though Sydenham chorea and its recurrence is rare in adults, it should be considered in adults after ruling out other competing differential diagnoses. Because of the lack of evidence on treatment of such rare cases, individualized mode of therapy is advised. Valproic acid is preferred for symptomatic treatment and more frequent benzathine penicillin G injections, for example every three weeks, may help in the prevention of recurrence of Sydenham chorea.

Sydenham Chorea in Sudan; Presentation Panorama.

Introduction: Sydenham's chorea (SC) is the most common form of acquired chorea in childhood, it is considered a neurological complication of streptococcal pharyngitis. In this study, we aimed to determine the clinical pattern, association of Sydenham's chorea with other manifestations of acute rheumatic fever, and the laboratory findings of Sydenham's chorea among Sudanese patients. Methods: A prospective cross-sectional study involving fifty patients of various ages diagnosed with Sydenham's chorea and followed up at The National Center for Neurological Sciences from January 2017 to November 2019. Data were obtained after patients' consent through personal interviews or personal review of patients' records via a structured questionnaire composed of demographic data, symptoms, co-morbid illness, risk factors, physical examination, and related investigations. Results: About 50 patient was enrolled in this study with a median age of 13.7 years. Females were (n=35) (70%) and (30%) (n=15) s were males. Generalized chorea was seen in 33 (66%) and hemichorea in 17 (34%) patients. Weakness (38%) and hypotonia (46%) were common, such as behavior change (44%), dysarthria (70%), gait change (18%), and deterioration of handwriting (12%). Arthritis occurred in (36%), carditis in 30 (60%), both arthritis and carditis in 18 (36%), and pure chorea in 14 (28%). Erythema marginatum and subcutaneous nodules were not observed in our patients. Only 13 patients (26%) gave a history of pharyngitis. Conclusion: Sydenham chorea is more common in young female Sudanese, with a familial predominance and a tendency towards mitral valve disease. All pediatric Patients with chorea should be screened for Sydenham's chorea.

Corticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience.

OBJECTIVE: The purpose of this study was to investigate the effectiveness of corticosteroid therapy for children suffering from Sydenham chorea (SC). METHODS: The design of the study was observational, retrospective and conducted at the single center of the Rheumatology Unit of Policlinic Hospital of Milan, Italy, from May 1995 to May 2022. All data about the patients were collected from medical records. RESULTS: From a total of 59 patients enrolled in the study (44 females and 15 males; median age 9.3 years, range 7.4-10.6 years), 49 were eligible for primary outcome analysis (10 patients were excluded due to incomplete data). Overall, 75% of patients received steroid therapy, while the remaining cases were treated with symptomatic drugs, including neuroleptics and antiseizure drugs. We found that the duration of chorea was significantly shorter in patients treated with corticosteroids in comparison to those receiving symptomatic treatment (median time: 31 vs. 41 days, p = 0.023). Additionally, patients with arthritis at the onset of the disease had a longer duration of chorea than those without arthritis (median time 90.5 vs. 39 days, p = 0.02). We also found that chorea recurred in 12% of the patients and seemed to be linked to a younger age at onset (p = 0.01). CONCLUSIONS: The study suggests that corticosteroid therapy can lead to a faster resolution of SC when compared to neuroleptics and antiseizure drugs treatment.