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Sydenham Chorea

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101. Inherited Metabolic Disorders (Overview)

only having a propensity or a risk of developing the disease. Whether the problem is an enzyme deficiency in carbohydrate metabolism or the excessive triplet repeats that characterize the mutation for Huntington chorea does not matter. The biochemical or genetic abnormality is no more a definition of a disease state than a positive result on a purified–protein-derivative (PPD) skin test is for tuberculosis. The issue is not an academic one. In view of the desire of insurance carriers to avoid pre (...) (by analyses of nuclear and mitochondrial DNA in individuals, even individuals dead for millions of years, and in species and in populations of species). Genetic mutations have been presumed to be the basis of inherited disease since the time of Mendel and Garrod. Even now, some diseases that have been recognized for 100-150 years have known genetic defects but the protein products are not well characterized. Chediak-Higashi syndrome is an example of such an inherited metabolic defect; Huntington chorea

2014 eMedicine.com

102. Acute Rheumatic Fever (Overview)

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

103. Rheumatic Fever (Follow-up)

. 2013 Jul 4. 369(1):75-80. . Atatoa-Carr P, Lennon D, Wilson N,. Rheumatic fever diagnosis, management, and secondary prevention: a New Zealand guideline. N Z Med J . 2008 Apr 4. 121(1271):59-69. . Gewitz MH,Baltimore RS, Tani LY,et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation . 2015 May 19. 131 (20):1806-18. . Weiner SG, Normandin PA. Sydenham chorea (...) Medication). Compliance with long-term secondary antibiotic prophylaxis is often poor, and close follow-up is mandatory. Next: Further Inpatient Care Most patients with acute rheumatic fever (ARF) can be treated at home. Inpatient care may be appropriate when the patient has severe constitutional symptoms, chorea, carditis with CHF, or major toxicity with the anti-inflammatory drugs. Previous Next: Inpatient & Outpatient Medications Patients with ARF need prolonged antibiotic prophylaxis to prevent

2014 eMedicine.com

104. Inherited Metabolic Disorders (Follow-up)

only having a propensity or a risk of developing the disease. Whether the problem is an enzyme deficiency in carbohydrate metabolism or the excessive triplet repeats that characterize the mutation for Huntington chorea does not matter. The biochemical or genetic abnormality is no more a definition of a disease state than a positive result on a purified–protein-derivative (PPD) skin test is for tuberculosis. The issue is not an academic one. In view of the desire of insurance carriers to avoid pre (...) (by analyses of nuclear and mitochondrial DNA in individuals, even individuals dead for millions of years, and in species and in populations of species). Genetic mutations have been presumed to be the basis of inherited disease since the time of Mendel and Garrod. Even now, some diseases that have been recognized for 100-150 years have known genetic defects but the protein products are not well characterized. Chediak-Higashi syndrome is an example of such an inherited metabolic defect; Huntington chorea

2014 eMedicine.com

105. Tourette Syndrome and Other Tic Disorders (Follow-up)

al. Relationship of birth weight to the phenotypic expression of Gilles de la Tourette''s syndrome in monozygotic twins. Neurology . 1992 Mar. 42(3 Pt 1):652-8. . Kirvan CA, Swedo SE, Heuser JS, Cunningham MW. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Nat Med . 2003 Jul. 9(7):914-20. . Snider LA, Swedo SE. Post-streptococcal autoimmune disorders of the central nervous system. Curr Opin Neurol . 2003 Jun. 16(3):359-65. . Swedo SE, Leonard HL, Rapoport JL (...) . The pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) subgroup: separating fact from fiction. Pediatrics . 2004 Apr. 113(4):907-11. . Asbahr FR, Ramos RT, Negrao AB, Gentil V. Case series: increased vulnerability to obsessive-compulsive symptoms with repeated episodes of Sydenham chorea. J Am Acad Child Adolesc Psychiatry . 1999 Dec. 38(12):1522-5. . Mell LK, Davis RL, Owens D, et al. Association between streptococcal infection and obsessive-compulsive disorder

2014 eMedicine.com

106. 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

107. 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

108. 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

109. 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

110. 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

111. 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

112. 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

113. 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

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 (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

116. Tardive Dyskinesia (Diagnosis)

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

117. Tourette Syndrome (Diagnosis)

on chromosomes 5 and 11. [ ] In the future, major advances in our understanding of the neurobiology of TS will likely depend on progress in elucidating genetic mechanisms. Autoimmune theory The autoimmune theory of TS posits that antibodies directed against an antecedent infection (eg, streptococcal infection) cross-react with neuronal structures in the central nervous system. This is the presumed mechanism of action for Sydenham chorea and pediatric autoimmune neuropsychiatric disorder associated

2014 eMedicine.com

118. Inherited Metabolic Disorders (Diagnosis)

only having a propensity or a risk of developing the disease. Whether the problem is an enzyme deficiency in carbohydrate metabolism or the excessive triplet repeats that characterize the mutation for Huntington chorea does not matter. The biochemical or genetic abnormality is no more a definition of a disease state than a positive result on a purified–protein-derivative (PPD) skin test is for tuberculosis. The issue is not an academic one. In view of the desire of insurance carriers to avoid pre (...) (by analyses of nuclear and mitochondrial DNA in individuals, even individuals dead for millions of years, and in species and in populations of species). Genetic mutations have been presumed to be the basis of inherited disease since the time of Mendel and Garrod. Even now, some diseases that have been recognized for 100-150 years have known genetic defects but the protein products are not well characterized. Chediak-Higashi syndrome is an example of such an inherited metabolic defect; Huntington chorea

2014 eMedicine.com

119. Tourette Syndrome and Other Tic Disorders (Diagnosis)

arthritis, carditis, or serologic abnormality. In the 1970s, patients with Sydenham chorea were demonstrated to have high levels of antibodies that react to human brain. These antibodies have since been shown to cross-react to certain proteins on group A beta-hemolytic streptococci (GABHS). [ ] Although tics and chorea can be differentiated clinically, the definitions were less clear in the 19th century. For instance, Charcot and Gilles de la Tourette distinguished tics and chorea primarily on grounds (...) in a small subgroup of patients, the precise relationship between such infections, antineuronal antibodies, and TS remains unknown. Some observations support a connection between GABHS infection and tics. [ ] OCD occurs more frequently in children with Sydenham chorea than in healthy controls or those who have rheumatic fever without chorea. [ ] In a large case-control study, children with OCD or a chronic tic disorder were more than twice as likely as controls to have had a documented GABHS infection

2014 eMedicine.com

120. 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

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