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Sydenham Chorea

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81. Acute Rheumatic Fever (Follow-up)

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 . 1986 Jun. 77 (6):2019-26. . . Yoshinoya S, Pope RM. Detection of immune complexes (...) Treat Options Cardiovasc Med . 2017 Feb. 19 (2):15. . . [Guideline] Shulman ST, Bisno AL, Clegg HW, Gerber MA, Kaplan EL, Lee G, et al. Clinical practice guideline for the diagnosis and management of group A streptococcal pharyngitis: 2012 update by the Infectious Diseases Society of America. Clin Infect Dis . 2012 Nov 15. 55(10):e86-102. . Sydenham Chorea Information Page. National Institute of Neurological Disorders and Stroke. Available at . Accessed: November 4, 2018. Barash J, Mashiach E, Navon

2014 eMedicine.com

82. Tourette Syndrome (Overview)

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

83. 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

84. 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

85. Cor Triatriatum (Follow-up)

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 sectional echocardiography. Br Heart J . 1992 Jul. 68(1):9-11. . . Alboliras ET, Edwards WD

2014 eMedicine.com

86. Streptococcus Group A Infections (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.com

87. Rheumatic Fever (Diagnosis)

not have a clear-cut sexual predilection, although certain clinical manifestations, such as and Sydenham chorea, are more common in females who have gone through puberty. Age ARF is most common among children aged 5-15 years. It is relatively rare in infants and uncommon in preschool-aged children. ARF occurs in young adults, but the incidence of first episodes of ARF falls steadily after adolescence and is rare after age 35 years. [ ] The lower rate of ARF in adults may represent a decreased risk (...) 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: a case report and review of the literature. Pediatr Emerg Care . 2007 Jan. 23(1):20-4. . Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Jani D, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med . 2007 Aug 2. 357(5):470-6

2014 eMedicine.com

88. 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

89. Tardive Dyskinesia (Treatment)

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

90. Acute Rheumatic Fever (Treatment)

heart failure usually responds to rest and corticosteroid therapy. Digoxin can be useful in patients with severe carditis, but its use should be monitored closely because of the possibility of heart block. Nocturnal tachycardia may be a sign of cardiac involvement that may be responsive to digoxin. Vasodilators and diuretics also may be used. Sydenham chorea requires long-term antimicrobial prophylaxis, even if no other manifestations of rheumatic fever evolve. The signs and symptoms of chorea (...) usually do not respond well to treatment with antirheumatic agents. Complete physical and mental rest is essential because the manifestations of chorea may be exaggerated by emotional trauma. Glucocorticoids or salicylates have little or no effect on chorea. Because chorea disappears with sleep, adequate sedation should be provided. A number of drugs have been used off label for symptomatic treatment of Sydenham chorea, including anticonvulsants (eg, valproate, carbamazepine) and neuroleptics (eg

2014 eMedicine.com

91. Inherited Metabolic Disorders (Treatment)

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

92. Cor Triatriatum (Treatment)

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 sectional echocardiography. Br Heart J . 1992 Jul. 68(1):9-11. . . Alboliras ET, Edwards WD

2014 eMedicine.com

93. Rheumatic Fever (Treatment)

,. 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: a case report and review of the literature. Pediatr Emerg Care . 2007 Jan (...) to reduce the risk of subsequent rheumatic heart disease. Bed rest is a traditional part of ARF therapy and is especially important in those with carditis. Patients are typically advised to rest through the acute illness and to then gradually increase activity; some clinicians monitor the patient’s ESR and restart activity only as it normalizes. [ , ] Intravenous immunoglobulin has not been shown to reduce the risk of rheumatic heart disease or to substantially improve the clinical course. [ ] Chorea

2014 eMedicine.com

94. Tourette Syndrome and Other Tic Disorders (Treatment)

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

95. Rheumatic Fever (Overview)

not have a clear-cut sexual predilection, although certain clinical manifestations, such as and Sydenham chorea, are more common in females who have gone through puberty. Age ARF is most common among children aged 5-15 years. It is relatively rare in infants and uncommon in preschool-aged children. ARF occurs in young adults, but the incidence of first episodes of ARF falls steadily after adolescence and is rare after age 35 years. [ ] The lower rate of ARF in adults may represent a decreased risk (...) 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: a case report and review of the literature. Pediatr Emerg Care . 2007 Jan. 23(1):20-4. . Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Jani D, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med . 2007 Aug 2. 357(5):470-6

2014 eMedicine.com

96. Tardive Dyskinesia (Overview)

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

97. Botulinum Toxin (BOTOX&reg (Overview)

syndrome and other tic disorders. Immunologic response found in patients with Sydenham chorea is also found in patients with Tourette syndrome and obsessive-compulsive disorder. Points on the graph represent percent expression of D8/17 antigen on circulating B lymphocytes. Tourette syndrome and other tic disorders. In a randomized controlled trial of habit reversal therapy (HRT), results differed significantly from those of a control therapy (massed practice; P< .001, analysis of variance). The HRT

2014 eMedicine.com

98. Tourette Syndrome and Other Tic Disorders (Overview)

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

99. Cor Triatriatum (Overview)

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

100. Streptococcus Group A Infections (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.com

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