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Sydenham Chorea

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121. Rheumatic Fever (Overview)

common cause of pediatric heart disease in the world. Until 1960, it was a leading cause of death in children and a common cause of structural heart disease. The disease has been known for many centuries. Baillou (1538-1616) first distinguished acute arthritis from gout. Sydenham (1624-1668) described chorea but did not associate it with acute rheumatic fever (ARF). In 1812, Charles Wells associated rheumatism with carditis and provided the first description of the subcutaneous nodules. In 1836, Jean (...) autoimmune neuropsychiatric disorders associated with streptococcal infections. As of 2015, this is considered an unproven hypothesis. [ ] Race In the United States, the attack rate is more a function of crowding than race, though the socioeconomic realities of those crowded conditions is no doubt a factor. Sex No sex predilection exists, except that and Sydenham chorea occur more often in females than in males. Age Although individuals of any age group may be affected, most cases are reported in persons

2014 eMedicine Emergency Medicine

122. Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): An Evolving Concept (PubMed)

is also insufficient. Anti-dopamine receptor antibodies might be relevant to both Sydenham's chorea (SC)-the prototypical post-streptococcal neuropsychiatric disorder-and some rare forms of encephalitis targeting the basal ganglia specifically, but studies exploring their association with children fulfilling Swedo's criteria for PANDAS have been inconclusive. Moreover, we lack evidence in favor of the efficacy of antibiotic prophylaxis or tonsillectomy in patients fulfilling Swedo's criteria

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2013 Tremor and Other Hyperkinetic Movements

123. Movement disorders in autoimmune diseases. (PubMed)

Movement disorders in autoimmune diseases. Movement disorders have been known to be associated with a variety of autoimmune diseases, including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcus, systemic lupus erythematosus, antiphospholipid syndrome, gluten sensitivity, paraneoplastic and autoimmune encephalopathies. Tremors, dystonia, chorea, ballism, myoclonus, parkinsonism, and ataxia may be the initial and even the only presentation

2012 Movement Disorders

124. Proof-of-Concept Safety Study of CPP-109 (Vigabatrin) for Treatment Refractory Tourette's Disorder

. Subjects with a history of seizure disorder (other than febrile seizure). Subjects with history of Sydenham's Chorea. Subjects with autism, schizophrenia, other psychotic disorder, or bipolar disorder. Subjects with a primary diagnosis of a major mood disorder that requires ongoing psychiatric treatment. Subjects with a neurological disorder other than a tic disorder. Subjects with a major medical illness. Female subjects who are unwilling to use birth control or who are pregnant, as determined

2012 Clinical Trials

125. Relationship between movement disorders and obsessive-compulsive disorder: beyond the obsessive-compulsive-tic phenotype. A systematic review. (PubMed)

was found between the choreas (Huntington's disease and Sydenham's chorea) and OCD/OC symptoms. Furthermore, elevated frequencies of OC symptoms were found in small case control series of dystonias. Small family based studies in dystonia subtypes modestly suggest shared familial/genetic relationships between OC symptoms and dystonia.Current data indicate a relationship between OCD/OC symptoms and the choreas. As OCD and the choreas have been associated with dysfunctional frontal-striatal circuits

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2012 Journal of neurology, neurosurgery, and psychiatry

126. Antibiotic Treatment Trial for the PANDAS/PANS Phenotype

-BOCS of more or equal to 16. Parental willingness to accompany their child for multiple study visits and be responsible for medication compliance. Exclusion Criteria: History of Rheumatic Fever including Sydenham's Chorea (heart murmur, frank chorea, EKG PR or QTc prolongation, abnormal reflexes (Gordon-Hey reflex)). Diagnosis of autism (moderate - severe), schizophrenia, mental retardation or chronic degenerative neurological disease. Any illness for which antibiotic treatment may

2012 Clinical Trials

127. Comprehensive treatment of Huntington disease and other choreic disorders. (PubMed)

disorders have specific treatable underlying etiologies, such as vitamin B(12) deficiency or drug-induced dyskinesia. Autoimmune disorders such as Sydenham chorea may be treated with penicillin, corticosteroids, intravenous immunoglobulin, or plasma exchange. Heredodegenerative choreas such as Huntington disease often respond to treatment with tetrabenazine or amantadine. Many other agents may be used nonspecifically for symptom control, including benzodiazepines, neuroleptics, and antiepileptic (...) Comprehensive treatment of Huntington disease and other choreic disorders. The management of choreic disorders presents significant challenges, including identifying the etiology of the disorder, treating and preventing motor symptoms, and managing a range of other neurologic and behavioral complications. Chorea may occur in several neurodegenerative, genetic, or drug-related conditions, and a thorough diagnostic evaluation is needed to identify the specific underlying causes. Some choreic

2012 Cleveland Clinic Journal of Medicine

128. Streptococcus and rheumatic fever. (PubMed)

and cardiac myosin and its peptides appear during progression of rheumatic heart disease. However, autoantibodies against collagen that are not crossreactive may form because of the release of collagen from damaged valve or to responses to collagen bound in vitro by certain serotypes of streptococci. In Sydenham chorea, human mAbs derived from disease target the group A carbohydrate epitope GlcNAc and gangliosides and dopamine receptors found on the surface of neuronal cells in the brain. Human mAbs (...) and autoantibodies in Sydenham chorea were found to signal neuronal cells and activate calcium calmodulin-dependent protein kinase II (CaMKII) in neuronal cells and recognize the intracellular protein biomarker tubulin.To summarize, pathogenic mechanisms of crossreactive autoantibodies which target the valve in rheumatic heart disease and the neuronal cell in Sydenham chorea share a common streptococcal epitope GlcNAc and target intracellular biomarkers of disease including cardiac myosin in the myocardium

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2012 Current Opinion in Rheumatology

129. Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders. (PubMed)

-methyl-d-aspartate receptor. The 12 dopamine-2 receptor antibody-positive patients with encephalitis had movement disorders characterized by parkinsonism, dystonia and chorea. In addition, the patients had psychiatric disturbance with emotional lability, attention deficit and psychosis. Brain magnetic resonance imaging showed lesions localized to the basal ganglia in 50% of the patients. Elevated dopamine-2 receptor immunoglobulin G was also found in 10/30 patients with Sydenham's chorea, 0/22

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2012 Brain

130. A National Prospective Surveillance Study of Acute Rheumatic Fever in Australian Children. (PubMed)

to collect data on children with ARF across Australia. Children up to 15 years of age were included if they had an ARF episode diagnosed between October 1, 2007 and December 31, 2010 that met the case definition.ARF was identified in 151 children: 131 Indigenous Australians, 10 non-Indigenous Australians, 8 Pacific Islanders and 1 African (1 unknown). Common presenting features were joint symptoms, fever and carditis. Sydenham chorea was reported in 19% of children. Aseptic monoarthritis was a major

2012 Pediatric Infectious Dsease Journal

131. Pediatric autoimmune neuropsychiatric disorders associated with streptococcus in identical siblings. (PubMed)

Pediatric autoimmune neuropsychiatric disorders associated with streptococcus in identical siblings. Termed pediatric autoimmune neuropsychiatric disorders associated with Streptococcus (PANDAS), these cases of childhood-onset obsessive compulsive disorder and tic disorders resemble the presentation of Sydenham chorea, in that they have an acute onset following a group A beta-hemolytic streptococcal infection (group A Streptococcus), with accompanying neurological signs, and an episodic

2011 Journal of Child and Adolescent Psychopharmacology

132. A film of patients with movement disorders made in Queen Square, London in the Mid-1920s by Samuel Alexander Kinnier Wilson. (PubMed)

tremor, Parkinson's disease and postencephalitic parkinsonism, hemiballismus, Huntington's chorea, Sydenham's chorea, hysterical palsy and tremor, multiple sclerosis, and progressive lenticular degeneration. Most of the patients are filmed in the square outside the National Hospital. The British Film Institute dates the film to 1924 and the captions to 1925. The case records of 6 of the 14 patients, who were admitted to the National Hospital, Queen Square, under the care of Dr. SAKW have been

2011 Movement Disorders

133. Omega-3 Fatty Acids in Tourette's Disorder

and to understand the nature of the study. Subjects and their legal representatives must be considered reliable. Exclusion Criteria: Organic brain disease, for example, traumatic brain injury residua Meeting criteria for mental retardation as defined by the DSM-IV. A history of seizure disorder (other than febrile seizure). A Subjects with history of Sydenham's Chorea. Autism, schizophrenia or other psychotic disorders. A primary diagnosis of a major mood disorder that requires ongoing psychiatric treatment

2011 Clinical Trials

134. Intravenous Immunoglobulin for PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections)

chronological age). Sleep disorder (insomnia, night terrors, refusal to sleep alone). Handwriting deterioration or other sign of motoric dysfunction (including new onset of motor hyperactivity, or presence of choreiform finger movements). Urinary frequency or increased urge to urinate; daytime or night-time secondary enuresis. EXCLUSION CRITERIA: History of rheumatic fever, including Sydenham chorea (the neurologic manifestation). Presence of symptoms consistent with autism, schizophrenia, or other

2011 Clinical Trials

135. Cognitive and psychiatric phenotypes of movement disorders in children: a systematic review. (PubMed)

cognitive and psychiatric symptoms associated with paediatric movement disorders. We used a systematic approach, via PubMed, and reviewed over 400 abstracts of studies of selected disorders, of which 88 papers reporting paediatric non-motor symptoms are summarized.Obsessive-compulsive disorder was manifest in children with paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections and Sydenham chorea. Children with opsoclonus-myoclonus syndrome had, for the most part

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2011 Developmental Medicine and Child Neurology

136. Movement disorders in paraneoplastic and autoimmune disease (PubMed)

ataxia and pesudoathetosis. Other disorders such as Sydenham's chorea, or chorea related to systemic lupus erythematosus and antiphospholipid syndrome occur in association with multiple antibodies, are not paraneoplastic, and are triggered by molecular mimicry or unknown mechanisms. Recent studies have revealed a new category of disorders that can be paraneoplastic or not, and associate with antibodies against cell-surface or synaptic proteins. They include anti-N-methyl-D-aspartate receptor (anti (...) Movement disorders in paraneoplastic and autoimmune disease The most relevant advances in immune-mediated movement disorders are described, with emphasis on the clinical--immunological associations, novel antigens, and treatment.Many movement disorders previously considered idiopathic or degenerative are now recognized as immune-mediated. Some disorders are paraneoplastic, such as anti-CRMP5-associated chorea, anti-Ma2 hypokinesis and rigidity, anti-Yo cerebellar ataxia and tremor, and anti-Hu

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2011 Current Opinion in Neurology

137. Anti-streptococcal antibodies reacting with brain tissue. I. Immunofluourescent studies. (PubMed)

Anti-streptococcal antibodies reacting with brain tissue. I. Immunofluourescent studies. Serum JD from a 14-year old girl with Sydenhams chorea contained antibodies which gave immunofluorescent staining of the limiting membrane of the brain, ependymal tissue and fibrous astrocytes. These antibodies could be completely absorbed by Str. pyogenes type 24 (NCTC 8305) but only partially if at all by type 6 matt (NCTC 8302) or type 6 glossy (NCTC 8709). In contrast, staining by the same serum (...) reactions of 3/74 could be absorbed out by Str. pyogenes type 24 but not by Str. pyogenes type 6 matt or type 6 glossy. None of these staining patterns given by serum JD or by 3/74 could be absorbed by human uterine smooth muscle. Serum 3/74 stained heart muscle but this reaction could be absorbed without affecting the brain staining reactions. Sera from 4 other patients with Sydenham's chorea were found to give staining of the ependyma and the limiting membrane, 2 only very weakly.

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1976 British journal of experimental pathology

138. Anti-streptococcal antibodies reacting with brain tissue. II. Utrastructural studies. (PubMed)

Anti-streptococcal antibodies reacting with brain tissue. II. Utrastructural studies. The reaction of rabbit anti-Streptococcus pyogenes type 24 with mouse brain has been studied at the ultrastructural level using horseradish peroxidase labelled antiglobulin. It is shown that these antibodies, and by inference those found in Sydenham's chorea, react with the fibrous astrocyte. The cross-reacting antigen is the glial filament or a closely associated cytoplasmic constituent.

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1976 British journal of experimental pathology

139. Involuntary movements in patients taking oral contraceptives (PubMed)

Involuntary movements in patients taking oral contraceptives Involuntary movements developed in five women taking oral contraceptives. In one, the sudden onset of a unilateral disturbance suggested a cerebral thrombosis; this case is considered to be a further example of the increased risk of cerebrovascular disease associated with oral contraception. The four other patients suffered a relapse of Sydenham's chorea between one and four months after starting an oral contraceptive regimen

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1969 British medical journal


equally elevated and were significantly higher than the normal controls. When Group A antibody levels were determined on sera obtained at intervals of 5-12 months and 1-5 yr after the acute illness) it was found that the antibody levels declined within the normal range at the 5-12 month interval in patients with glomerulonephritis as well as in patients with rheumatic fever in whom no valvular involvement had complicated the disease, i.e., patients with pure Sydenham's chorea. However, in patients (...) with rheumatic valvulitis, who had been on penicillin prophylaxis after the last acute episode, the A antibody level showed little decline from the level obtained during the acute illness. The elevated antibody level in patients with rheumatic valvulitis, including patients with Sydenham's chorea with valvulitis, persisted for periods of at least 1 yr and up to 20 yr after the last acute attack. The pattern of the decline of the antibody levels to the A-variant carbohydrate as well as of the antibody titers

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1968 The Journal of experimental medicine

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