Sydenham chorea

Sydenham chorea (SC) is common in childhood with extensive differential diagnoses, including inherited disease, autoimmunity, endocrine disorders, and infections. SC due to acute rheumatic fever (ARF) is rare. Herein, we present a case of SC in an eight-year-old child who presented with choreiform movements of her face and limbs, including facial grimacing, difficulty walking, and slurred speech. She also had a runny nose and odynophagia. She had two episodes of sore throat in the last two months, and her physical examination was unremarkable except for hypertrophic tonsils and generalized hypotonia...

The pathogenesis of Acute Rheumatic Fever/Rheumatic Heart Disease (ARF/RHD) and associated neurobehavioral complications including Sydenham's chorea (SC) is complex. Disease complications triggered by Group A streptococcal (GAS) infection are confined to human and determining the early events leading to pathology requires a robust animal model that reflects the hallmark features of the disease. However, modeling these conditions in a laboratory animal, of a uniquely human disease is challenging. Animal models including cattle, sheep, pig, dog, cat, guinea pigs rats and mice have been used extensively to dissect molecular mechanisms of the autoimmune inflammatory responses in ARF/RHD...

Bacterial coinfection and COVID-19 have been reported in pediatric populations. We describe a case of Sydenham's chorea, which is exceedingly rare in developed countries, with concurrent COVID-19. Discussed here is the clinical course of an 8-year-old COVID-positive female with pure Sydenham's chorea and subclinical carditis from acute rheumatic fever. To our knowledge, there are no documented reports of acute rheumatic fever in a pediatric patient with coexisting COVID-19 infection.

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...

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BACKGROUND: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. OBJECTIVES: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management...

Sydenham chorea, also known as St. Vitus dance, is a major clinical criterion for the diagnosis of acute rheumatic fever. Clinically, it results in a combination of movement disorders and complex neuropsychiatric symptoms. Cardiac damage due to rheumatic fever may also predispose to neurologic complications later in life. Rheumatic heart disease (RHD) is associated with heart remodeling, cardiac arrhythmias, and ischemic stroke. Furthermore, chronically damaged heart valves are predisposed to infection. Septic brain embolism, a known complication of infective endocarditis, may result in brain ischemia, hemorrhage, and spread of the infection to the brain...

Initial reports supporting the possibility of inflammation in the brain in obsessive-compulsive disorder (OCD) evolved from the models of Sydenham's Chorea, and Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS), which implicated excessive autoimmune responses following exposure to group A B-hemolytic streptococcal infections. Subsequently, this model was expanded to Pediatric Autoimmune Neuropsychiatric Syndrome (PANS) which applied the same concept but included other infections...

The coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2 appeared in Wuhan, China in December 2019 and quickly spread around the world and is considered a global pandemic. This disease, which is pre-infected with respiratory and cardiovascular system symptoms, can also occur in many organ systems. Since the beginning of the pandemic, cases related to neurological involvement have been reported in the literature and studies coercing neurological findings and complications have been published. COVID-19 can cause wide spectrum of neurological phenotypes from severe to milder...

Pediatric movement disorders (PMDs) consist of a heterogeneous group of signs and symptoms caused by numerous neurological diseases. Different neurological disorders in children also share overlapping movement disorders making a diagnosis of the underlying cause of the movement disorder challenging. The similarity of the symptoms across multiple disease types suggests that there may be a final common motor pathway causing the overlapping movement disorders. There are numerous disorders in children associated with disturbances in tone and involuntary movements...

PURPOSE: To report on a patient with probable catastrophicantiphospholipid syndrome (CAPS) with eye, brain and skin involvement. METHODS: Descriptive presentation of the case and of the relevant clinical photographs. RESULTS: A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, lower limb skin ulcers and livedo reticularis. Workup showed anemia, elevated ESR, CRP, and positive anti-phospholipid antibodies. Right eye funduscopy showed cotton-wool spots along the inferotemporal arcade with arteriolar occlusion, perivascular retinal hemorrhages, and diffuse retinal ischemia in the temporal peripheral retina...

BACKGROUND: Our aim in this study was to evaluate the efficacy of magnetic resonance imaging (MRI) studies in the detection of brain regions effected by Sydenham chorea and to determine whether they provided data regarding the pathogenesis of Sydenham chorea. To this end, we assessed basal ganglia structures in Sydenham chorea patients and control group by quantitative MRI volumetric analysis. METHODS: Patients with a recent onset of chorea and control subjects matched for age and gender were included in the study...

Rheumatic heart disease (RHD) as a chronic sequela of repeated episodes of acute rheumatic fever (ARF), remains a cause of cardiac morbidity in Egypt although it is given full attention through a national RHD prevention and control program. The present report reviews our experience with subjects presenting with ARF or its sequelae in a single RHD centre and describes the disease pattern over the last decade. A cross-sectional study was conducted in El-Mahalla RHD centre between 2006 and 2018. A total of 17014 individual were enrolled and evaluated...

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...

The writer Bertolt Brecht (1898-1956) is known for his poems, dramas and the "epic or dialectical theater" he founded. He was retrospectively postulated to have had rheumatic fever because of heart problems and neuromuscular symptoms in his youth. Based on current rheumatological knowledge, it cannot be deduced with certainty from the available documents that Brecht had rheumatic fever. At most, a very unusual manifestation of rheumatic fever can be suspected with atypical rheumatic chorea and a very atypical course of rheumatic carditis...

Movement, behavioral, and neuropsychiatric disorders in children have been linked to infections and a group of anti-neuronal autoantibodies, implying dopamine receptor-mediated encephalitis within the basal ganglia. The purpose of this study was to determine if anti-neuronal biomarkers, when used as a group, confirmed the acute disease in Sydenham chorea (SC) and pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS). IgG autoantibodies against four neuronal autoantigens (tubulin, lysoganglioside GM1 , and dopamine receptors D1 and D2) were detected in SC sera (N=8), sera and/or cerebrospinal fluid (CSF) from two groups of PANDAS cases (N=25 first group and N=35 second group), sera from Tourette's syndrome (N=18), obsessive-compulsive disorder (N=25), attention deficit hyperactivity disorder (N=18), and healthy controls (N=28) by direct enzyme-linked immunosorbent assay (ELISA)...

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62...

Sydenham chorea (SC) is the most common acquired cause of chorea in children. (SC) is the neurological manifestation of rheumatic fever. Rheumatic fever is still reported in Saudi Arabia, although less frequently. According to modified Jones criteria, carditis and arthritis are the major manifestations of acute rheumatic fever (ARF) in children. SC is often seen in isolated form; however, it can occur in association with other clinical manifestations of ARF like carditis. It has been reported that silent, mild valvular regurgitation has been found in patients with pure chorea...

Huntington's disease was an important example for discussing the problem of predictive genetic testing. Like other movement disorders, it includes non-motor symptoms and a prodromal phase. As a rapidly progressive monogenetic disease, it is an important model disease for the study of neurodegenerative pathomechanisms and one of the first movement disorders for which causal therapies seem to be reachable.

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...