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Sydenham Chorea

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101. Tardive Dyskinesia (Follow-up)

amphetamines, levodopa, and substances that may result in dyskinesias (see Etiology). Specifically note whether antiemetic medications (especially metoclopramide, prochlorperazine, and related compounds) are being administered. Distinction from similar conditions Unlike TD, Sydenham chorea is a disorder associated with a history of group A streptococcal infection and rheumatic fever in children. It typically affects children and adolescents 6 months or more after an infection with group A streptococci (...) . Prompt administration of antibiotic therapy for infections with group A streptococci dramatically reduces the incidence of Sydenham chorea. The female-to-male ratio is approximately 2:1. Sydenham chorea is characterized by the rapid onset of chorea, muscular weakness, hypotonia, dysarthria, obsessions, compulsions, and other behavioral and emotional disturbances. After an abrupt or insidious onset, Sydenham chorea worsens over 2-4 weeks and then resolves over 3–6 months. Chorea may persist after

2014 eMedicine.com

102. Rheumatic Fever (Treatment)

to prevent rheumatic Fever. Pediatr Infect Dis J . 2009 Jul. 28(7):e259-64. . Kiliç A, Unüvar E, Tatli B, Gökçe M, Omeroglu RE, Oguz F, et al. Neurologic and cardiac findings in children with Sydenham chorea. Pediatr Neurol . 2007 Mar. 36(3):159-64. . Birdi N, Hosking M, Clulow MK, Duffy CM, Allen U, Petty RE. Acute rheumatic fever and poststreptococcal reactive arthritis: diagnostic and treatment practices of pediatric subspecialists in Canada. J Rheumatol . 2001 Jul. 28(7):1681-8. . Gerber MA

2014 eMedicine Emergency Medicine

103. Acute Rheumatic Fever (Diagnosis)

arthritis, which is present in approximately 80% of patients. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Sydenham chorea was once a common late-onset clinical manifestation but is now rare. [ ] Carditis (with progressive congestive heart failure, a new murmur, or pericarditis) may be the presenting sign of unrecognized past episodes and is the most lethal manifestation. Genetics may contribute, as evidenced by an increase in family incidence. No significant (...) , Beaton A, Raghu A, Steer A, Carapetis J, Ferretti JJ, et al. Acute Rheumatic Fever and Rheumatic Heart Disease. 2017 Apr 3. . . Karthikeyan G, Guilherme L. Acute rheumatic fever. Lancet . 2018 Jul 14. 392 (10142):161-174. . Dale RC. Immune-mediated extrapyramidal movement disorders, including Sydenham chorea. Handb Clin Neurol . 2013. 112:1235-41. . Ayoub EM, Barrett DJ, Maclaren NK, Krischer JP. Association of class II human histocompatibility leukocyte antigens with rheumatic fever. J Clin Invest

2014 eMedicine.com

104. Cor Triatriatum (Diagnosis)

echocardiography. Br Heart J . 1984 Feb. 51(2):211-9. . . Parsons CG. Cor triatriatum; concerning the nature of an anomalous septum in the left auricle. Br Heart J . 1950 Oct. 12(4):327-38. . . Thilenius OG, Bharati S, Lev M. Subdivided left atrium: an expanded concept of cor triatriatum sinistrum. Am J Cardiol . 1976 Apr. 37(5):743-52. . de Belder MA, Argano V, Burrell CJ. Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment by cross

2014 eMedicine.com

105. Streptococcal Infection, Group A (Overview)

with cardiac sarcolemma membranes. [ ] During the course of the host's immune response to the GAS, the host's antigens may, as a result of this molecular mimicry, be mistaken as foreign; this leads to an inflammatory cascade with resultant tissue damage. In patients with ARF with Sydenham chorea, common antibodies to antigens found in the S pyogenes cell membrane and the caudate nucleus of the brain are present, further supporting the concept of an aberrant autoimmune response in the development of ARF (...) in past decades; however, incidence is cyclic, depending on the prevalence of toxin-producing strains and the immune status of the population. Modes of transmission, age distribution of cases, and other epidemiologic features are similar to those for streptococcal pharyngitis. Central nervous system diseases The primary evidence for poststreptococcal autoimmune central nervous system (CNS) disease is provided by studies of Sydenham chorea, the neurologic manifestation of rheumatic fever. Reports

2014 eMedicine Pediatrics

106. Rheumatic Heart Disease (Overview)

among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Braunwald E. Rheumatic fever. Heart Disease: A Textbook of Cardiovascular Medicine . 5th ed. Philadelphia, Pa: WB Saunders Co; 1997. Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Cilliers AM, Manyemba J, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever. Cochrane Database Syst Rev . 2003. CD003176. . Clinical trial. The natural

2014 eMedicine Pediatrics

107. Rheumatic Fever (Overview)

of carditis in acute rheumatic fever. Aust N Z J Med . 1994 Oct. 24(5):530-5. . Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Circulation. The natural history of rheumatic fever and rheumatic heart disease. Ten-year report of a cooperative clinical trial of ACTH, cortisone, and aspirin. Circulation (...) % of admissions. [ ] Sex Rheumatic fever occurs in equal numbers in males and females. Females with rheumatic fever fare worse than males and have a slightly higher incidence of chorea. Age Rheumatic fever is principally a disease of childhood, with a median age of 10 years; However, GABHS pharyngitis is uncommon in children younger than 3 years, and acute rheumatic fever is extremely rare in these younger children in industrialized countries. Although less commonly seen in adults compared with children

2014 eMedicine Pediatrics

108. Rheumatic Fever (Follow-up)

. . Abernethy M, Bass N, Sharpe N, et al. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Aust N Z J Med . 1994 Oct. 24(5):530-5. . Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Circulation. The natural history of rheumatic fever and rheumatic heart disease. Ten

2014 eMedicine Pediatrics

109. Rheumatic Heart Disease (Follow-up)

, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Braunwald E. Rheumatic fever. Heart Disease: A Textbook of Cardiovascular Medicine . 5th ed. Philadelphia, Pa: WB Saunders Co; 1997. Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Cilliers AM, Manyemba J, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever. Cochrane Database Syst Rev . 2003 (...) manifestations of acute rheumatic fever consists of salicylates and steroids. Aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea, and the response is typically dramatic. If rapid improvement is not observed after 24-36 hours of therapy, question the diagnosis of rheumatic fever. Attempt to obtain aspirin blood levels from 20-25 mg/dL, but stable levels may be difficult to achieve during the inflammatory phase because of variable GI absorption of the drug

2014 eMedicine Pediatrics

110. Streptococcal Infection, Group A (Diagnosis)

with cardiac sarcolemma membranes. [ ] During the course of the host's immune response to the GAS, the host's antigens may, as a result of this molecular mimicry, be mistaken as foreign; this leads to an inflammatory cascade with resultant tissue damage. In patients with ARF with Sydenham chorea, common antibodies to antigens found in the S pyogenes cell membrane and the caudate nucleus of the brain are present, further supporting the concept of an aberrant autoimmune response in the development of ARF (...) in past decades; however, incidence is cyclic, depending on the prevalence of toxin-producing strains and the immune status of the population. Modes of transmission, age distribution of cases, and other epidemiologic features are similar to those for streptococcal pharyngitis. Central nervous system diseases The primary evidence for poststreptococcal autoimmune central nervous system (CNS) disease is provided by studies of Sydenham chorea, the neurologic manifestation of rheumatic fever. Reports

2014 eMedicine Pediatrics

111. Rheumatic Heart Disease (Diagnosis)

among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Braunwald E. Rheumatic fever. Heart Disease: A Textbook of Cardiovascular Medicine . 5th ed. Philadelphia, Pa: WB Saunders Co; 1997. Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Cilliers AM, Manyemba J, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever. Cochrane Database Syst Rev . 2003. CD003176. . Clinical trial. The natural

2014 eMedicine Pediatrics

112. Rheumatic Fever (Diagnosis)

of carditis in acute rheumatic fever. Aust N Z J Med . 1994 Oct. 24(5):530-5. . Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry . 2005 May 1. 57(9):1073-6. . Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet . 2005 Jul 9-15. 366(9480):155-68. . Circulation. The natural history of rheumatic fever and rheumatic heart disease. Ten-year report of a cooperative clinical trial of ACTH, cortisone, and aspirin. Circulation (...) % of admissions. [ ] Sex Rheumatic fever occurs in equal numbers in males and females. Females with rheumatic fever fare worse than males and have a slightly higher incidence of chorea. Age Rheumatic fever is principally a disease of childhood, with a median age of 10 years; However, GABHS pharyngitis is uncommon in children younger than 3 years, and acute rheumatic fever is extremely rare in these younger children in industrialized countries. Although less commonly seen in adults compared with children

2014 eMedicine Pediatrics

113. Rheumatic Fever (Diagnosis)

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

114. Rheumatic Fever (Follow-up)

Immunother . 2013 Nov. 9 (11):2393-7. . . Lennon D, Kerdemelidis M, Arroll B. Meta-analysis of trials of streptococcal throat treatment programs to prevent rheumatic Fever. Pediatr Infect Dis J . 2009 Jul. 28(7):e259-64. . Kiliç A, Unüvar E, Tatli B, Gökçe M, Omeroglu RE, Oguz F, et al. Neurologic and cardiac findings in children with Sydenham chorea. Pediatr Neurol . 2007 Mar. 36(3):159-64. . Birdi N, Hosking M, Clulow MK, Duffy CM, Allen U, Petty RE. Acute rheumatic fever and poststreptococcal reactive

2014 eMedicine Emergency Medicine

115. 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

116. Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS): An Evolving Concept Full Text available with Trip Pro

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

2013 Tremor and Other Hyperkinetic Movements

117. 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

118. 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

119. Comprehensive treatment of Huntington disease and other choreic disorders. Full Text available with Trip Pro

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

120. Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders. Full Text available with Trip Pro

-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

2012 Brain

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