Sydenham chorea

IMPORTANCE: Sydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence. OBJECTIVES: To evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome. DATA SOURCES: The systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [Sydenham OR Sydenham's OR rheumatic OR minor] AND chorea)...

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...

No abstract text is available yet for this article.

OBJECTIVES/BACKGROUND: Narcolepsy type 1 (NT1) is an immune-mediated disorder characterized by excessive daytime sleepiness, cataplexy, low levels of hypocretin-1 in the cerebrospinal fluid, and a strong association with the HLA DQB1*06:02 allele. There is evidence for streptococcal infections as one pathogenic factor that may lead to NT1 as part of a multifactorial pathogenesis. Elevated titers of Antistreptolysin-O antibodies and increased inflammatory activity in response to streptococci antigens have been described in patients with NT1...

Group A streptococcus (GAS) is a global pathogen associated with significant morbidity and mortality for which there is currently no licensed vaccine. Vaccine development has been slow, mostly due to safety concerns regarding streptococcal antigens associated with autoimmunity and related complications. For a GAS vaccine to be safe, it must be ensured that the antigens used in the vaccine do not elicit an antibody response that can cross-react with host tissues. In this study, we evaluated the safety of our GAS vaccine candidate called VaxiStrep in New Zealand White rabbits...

Post-infectious neuroinflammation has been implicated in multiple models of acute onset obsessive-compulsive disorder (OCD) including Sydenham's chorea (SC), pediatric acute-onset neuropsychiatric syndrome (PANS), and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). These conditions are associated with a range of autoantibodies which are thought to be triggered by an 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...

There is still no evidence-based guideline and consensus on the treatment Sydenham's Chorea (SC). The first-line medication preference of specialists depends on personal experience and is variable. In this study, we evaluate the treatment results of pediatric patients who were treated with valproic acid (VPA). The medical records of 17 patients diagnosed with SC were reviewed retrospectively. The mean time to clinical improvement was found as approximately 5 days, the mean duration of remission as 13.60 ± 3...

AIM: Sydenham chorea is an immune-mediated neuropsychiatric condition, and a major criterion for diagnosis of acute rheumatic fever (ARF). Children in remote Northern Australia experience disproportionately high rates of ARF, yet studies looking at the epidemiology, clinical presentation and management of Sydenham chorea are limited in this population. METHODS: We conducted a retrospective case series from January 2002 to April 2022 of all paediatric patients aged ≤18 years admitted to Royal Darwin Hospital with Sydenham chorea...

No abstract text is available yet for this article.

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...

OBJECTIVE: In the fourth year of the COVID-19 pandemic, mortality rates decreased, but the risk of neuropsychiatric disorders remained the same, with a prevalence of 3.8% of pediatric cases, including movement disorders (MD) and ataxia. METHODS: In this study, we report on a 10-year-old girl with hemichorea after SARS-CoV-2 infection and immunostained murine brain with patient CSF to identify intrathecal antibodies. Additionally, we conducted a scoping review of children with MD and ataxia after SARS-CoV-2 infection...

BACKGROUND: Chorea following SARS-CoV-2 infection and vaccination, has been increasingly recognized. We aimed to synthesize clinical and paraclinical characteristics, treatment responses, and outcomes of this neurologic complication. METHODS: We systematically reviewed LitCOVID, the WHO database on COVID-19, and MedRxiv up to March 2023, following a published protocol. RESULTS: We included 14 chorea cases in patients with SARS-CoV-2 infection and eight following COVID-19 vaccination...

PURPOSE OF REVIEW: Patients with different types of choreic syndromes, specially those with Huntington's (HD) and Wilson's (WD) diseases, report frequent sleep complaints. This review focuses on the main findings of studies addressing the sleep features in these diseases, and other less frequent causes of chorea associated with sleep disorders, including a new syndrome described in the last decade associated with IgLON5 antibodies. RECENT FINDINGS: Patients with HD and WD showed a bad quality of sleep and high frequency of insomnia and excessive daytime somnolence...

Pediatric acute-onset neuropsychiatric syndrome (PANS) features a heterogeneous constellation of acute obsessive-compulsive disorder (OCD), eating restriction, cognitive, behavioral and/or affective symptoms, often followed by a chronic course with cognitive deterioration. An immune-mediated etiology is advocated in which the CNS is hit by different pathogen-driven (auto)immune responses. This narrative review focused on recent clinical (ie, diagnostic criteria, pre-existing neurodevelopmental disorders, neuroimaging) and pathophysiological (ie, CSF, serum, genetic and autoimmune findings) aspects of PANS...

Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections (PANDAS), Sydenham chorea and other post-infectious psychiatric deteriorations are thought to be caused by inflammatory/autoimmune mechanisms, likely involving the basal ganglia based on imaging studies. Patients have a relapsing-remitting course and some develop severe refractory psychiatric disease. We found that 55/193 (28%) of consecutive patients meeting PANS criteria developed chronic arthritis and 25/121 (21%) of those with related psychiatric deteriorations developed chronic arthritis...

OBJECTIVE: To conduct the first prospective surveillance study of Sydenham's chorea (SC) in the UK and Ireland, and to describe the current paediatric and child psychiatric service-related incidence, presentation and management of SC in children and young people aged 0-16 years. DESIGN: Surveillance study of first presentations of SC reported by paediatricians via the British Paediatric Surveillance Unit (BPSU) and all presentations of SC reported by child and adolescent psychiatrists through the Child and Adolescent Psychiatry Surveillance System (CAPSS)...

INTRODUCTION: Sydenham's chorea (SC) is the most common cause of acquired chorea in children. The existing literature describes it as a benign, self-remitting condition. However, recent evidence discloses the persistence of long-course neuropsychiatric and cognitive complications in adulthood, which imposes to redefine the concept of 'benignity' of such condition. In addition, therapies are mostly empirical and non-evidence based. AREAS COVERED: Here, we conducted an electronic exploration of the PubMed database and selected 165 relevant studies directly correlated to SC treatment...

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...

INTRODUCTION: Sydenham's chorea (SC), prevalent in developing countries and occasionally affecting developed ones, poses a clinical challenge due to the lack of systematic guidelines for diagnosis and treatment. Resulting from Group A Beta-Hemolytic Streptococcus infection, SC presents various symptoms. This review aims to collect and evaluate available data on SC management to propose a cohesive treatment plan. METHODS: We searched PubMed, the Cochrane Library, Google Scholar, and ClinicalTrials...

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)...