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Sydenham Chorea

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61. A Study to Evaluate the Benefit of Octagam 5%® in Subjects With Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)

of moderate to severe PANS based on accepted criteria Must be willing to follow study procedures and comply with wash-out period If using prophylactic antibiotics, must be on stable dose for 3 months Exclusion Criteria: History of rheumatic fever, including Sydenham chorea (the neurologic manifestation) Previous IVIG therapy within the last 6 months Allergic reactions to blood products Patients who, in the investigator's opinion, might not be suitable for the trial. Steroid use Contacts and Locations Go

2017 Clinical Trials

62. Prescribing for people with acute rheumatic fever Full Text available with Trip Pro

Journal Article Review 2017 04 03 Australia Aust Prescr 7804938 0312-8008 Sydenham’s chorea benzathine penicillin rheumatic fever rheumatic heart disease Conflict of interest: none declared 2017 5 17 6 0 2017 5 17 6 0 2017 5 17 6 1 ppublish 28507400 10.18773/austprescr.2017.011 austprescr-40-70 PMC5407997 Brain Dev. 2002 Mar;24(2):73-6 11891095 Pediatr Infect Dis J. 2017 Jan 24;:null 28121967 Clin Infect Dis. 2006 Sep 15;43(6):683-9 16912939 J Antimicrob Chemother. 2004 Aug;54(2):447-50 15269193 Clin

2017 Australian Prescriber

63. AAN Guideline on Plasmapheresis in Neurologic Disorders

and may be considered for acute fulminant demyelinating CNS disease (Level C). There is insufficient evidence to support or refute the use of plasmapheresis for myasthenia gravis, pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection, and Sydenham chorea (Class III evidence, Level U). Plasmapheresis, also known as therapeutic plasma exchange, is a procedure that involves separating the blood, exchanging the plasma (typically with donor plasma or albumin solution (...) (35%, p < 0.0009), and tics (49%, p < 0.005) compared to placebo, and these gains were maintained at 1 year post-treatment. Conclusions. There are inadequate data to determine the efficacy of plasmapheresis in the treatment of acute OCD and tic symptoms in the setting of PANDAS (one Class III study). Recommendation. There is insufficient evidence to support or refute the use of plasmapheresis in the treatment of acute OCD and tic symptoms in the setting of PANDAS (Level U). Sydenham chorea

2011 American Association of Neuromuscular & Electrodiagnostic Medicine

64. Plasmapheresis in neurologic disorders

for acute fulminant demyelinating CNS disease (Level C). There is insufficient evidence to support or refute the use of plasmapheresis for myasthenia gravis, pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection, and Sydenham chorea (Class III evidence, Level U). Plasmapheresis, also known as therapeutic plasma exchange, is a procedure that involves separating the blood, exchanging the plasma (typically with donor plasma or albumin solution), and returning the other (...) (35%, p < 0.0009), and tics (49%, p < 0.005) compared to placebo, and these gains were maintained at 1 year post-treatment. Conclusions. There are inadequate data to determine the efficacy of plasmapheresis in the treatment of acute OCD and tic symptoms in the setting of PANDAS (one Class III study). Recommendation. There is insufficient evidence to support or refute the use of plasmapheresis in the treatment of acute OCD and tic symptoms in the setting of PANDAS (Level U). Sydenham chorea

2011 American Academy of Neurology

65. CNS autoimmune disease after Streptococcus pyogenes infections: animal models, cellular mechanisms and genetic factors Full Text available with Trip Pro

CNS autoimmune disease after Streptococcus pyogenes infections: animal models, cellular mechanisms and genetic factors Streptococcus pyogenes infections have been associated with two autoimmune diseases of the CNS: Sydenham's chorea (SC) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus infections (PANDAS). Despite the high frequency of pharyngeal streptococcus infections among children, only a small fraction develops SC or PANDAS. This suggests that several

2016 Future Neurology

66. Efficacy and Safety of Etonogestrel + 17β-Estradiol Vaginal Ring (MK-8342B) in the Treatment of Women With Primary Dysmenorrhea (MK-8342B-060)

pregnancy, middle-ear deafness, Sydenham chorea, or porphyria; known allergy/sensitivity or contraindication to the investigational products or their excipients; known allergy/sensitivity or contraindication to ibuprofen, or has experienced asthma, urticaria, or allergic-type reactions after taking aspirin, or other nonsteroidal anti-inflammatory drugs; history of drug or alcohol abuse or dependence. Known or suspected pregnancy, or had been pregnant or breastfeeding within past 2 months. Has used

2016 Clinical Trials

67. Efficacy and Safety of Etonogestrel + 17β-Estradiol Vaginal Ring (MK-8342B) in Women With Primary Dysmenorrhea (With Optional Extension) (MK-8342B-059)

cancer; any disease that may worsen under hormonal treatment such as disturbances in bile flow, systemic lupus erythematosus, pemphigoid gestationis or idiopathic icterus during previous pregnancy, middle-ear deafness, Sydenham chorea, or porphyria; known allergy/sensitivity or contraindication to the investigational products or their excipients; known allergy/sensitivity or contraindication to ibuprofen, or has experienced asthma, urticaria, or allergic-type reactions after taking aspirin, or other

2016 Clinical Trials

68. NIAID Clinical Center Genomics Opportunity Protocol

in which a strong inherited immunological pathogenic basis has been identified; such as the Pediatric Autoimmune Neuropsychiatric Disorders associated with Streptococcal infections (PANDAS) or Sydenham s chorea. Despite the breadth of clinical presentations under investigation, these immune-mediated disorders share significant overlap in underlying molecular pathophysiology and thus represent a coherent study target. This protocol will facilitate the discovery of genes contributing to selected immune

2015 Clinical Trials

69. Efficacy and Safety of Etonogestrel + 17β-Estradiol Vaginal Ring in Women at Risk for Pregnancy (MK-8342B-061)

such as disturbances in bile flow, systemic lupus erythematosus, pemphigoid gestationis or idiopathic icterus during previous pregnancy, middle-ear deafness, Sydenham chorea, or porphyria; known allergy/sensitivity or contraindication to investigational product or its excipients; history of drug or alcohol abuse or dependence. Recent, current, or suspected pregnancy; or has not had at least 2 menstrual cycles or has not completed two 28-day cycles of a hormonal contraceptive (pill, patch or ring) following

2015 Clinical Trials

70. Efficacy and Safety of Etonogestrel + 17β-Estradiol Vaginal Ring and Levonorgestrel-Ethinyl Estradiol Combined Oral Contraceptive in Adult Women at Risk for Pregnancy (MK-8342B-062)

; history of malabsorptive bariatric surgery. Other medical disorders, including history of malignancy ≤5 years prior to signing informed consent except for adequately treated basal cell or squamous cell skin cancer or in situ cervical cancer; any disease that may worsen under hormonal treatment such as disturbances in bile flow, systemic lupus erythematosus, pemphigoid gestationis or idiopathic icterus during previous pregnancy, middle-ear deafness, Sydenham chorea, or porphyria; known allergy

2015 Clinical Trials

71. Antineuronal Antibodies in a Heterogeneous Group of Youth and Young Adults with Tics and Obsessive-Compulsive Disorder. Full Text available with Trip Pro

Antineuronal Antibodies in a Heterogeneous Group of Youth and Young Adults with Tics and Obsessive-Compulsive Disorder. Antineuronal antibodies have been implicated in tic and obsessive compulsive disorders (OCD) associated with group A streptococcal infections. We investigated antineuronal autoantibody levels as well as antibody-mediated neuronal cell signaling activity, as previously reported for Sydenham chorea and pediatric autoimmune neuropsychiatric disorder associated with streptococci

2015 Journal of Child and Adolescent Psychopharmacology

72. Movement disorders in children with anti-NMDAR encephalitis and other autoimmune encephalopathies. (Abstract)

Movement disorders in children with anti-NMDAR encephalitis and other autoimmune encephalopathies. Accurate recognition of movement disorder phenomenology may differentiate children with anti-N-methyl D-aspartate receptor (NMDAR) encephalitis, autoimmune basal ganglia encephalitis (BGE), and Sydenham's chorea (SC). Three neurologists blinded to the diagnoses recorded dominant and associated movement disorders seen on videos of 31 patients with anti-NMDAR encephalitis (n = 10), BGE (n = 12 (...) ), and SC (n = 9). Stereotypy was only seen in anti-NMDAR encephalitis (8/10) and not in BGE and SC (P < 0.001). Perseveration was only seen in anti-NMDAR encephalitis (5/10) and not in BGE and SC (P < 0.001). Akinesia was more commonly seen in BGE (5/12) than in anti-NMDAR encephalitis (1/10, P = 0.097). Tremor was more commonly seen in BGE (5/12) than in anti-NMDAR encephalitis (1/10, P = 0.097). Chorea was seen in all groups: anti-NMDAR encephalitis (4/10), BGE (3/12), and SC (9/9). Likewise

2014 Movement Disorders

73. Acute-onset choreiform movements in a previously healthy 4-year-old patient. (Abstract)

Acute-onset choreiform movements in a previously healthy 4-year-old patient. We report the case of 4-year-old male with sinus venous thrombosis leading to bilateral thalamic and basal ganglia strokes presenting as generalized choreiform movements. Acute-onset chorea in the pediatric population is most commonly associated with Sydenham chorea, which is a manifestation of acute rheumatic fever. Chorea is a much less commonly noted sign of stroke, and when it occurs, it typically presents

2014 Pediatric Emergency Care

74. Diagnostic criteria of acute rheumatic fever. (Abstract)

preceding Group A Streptococcal infection, in addition to the presence of two major manifestations or one major and two minor manifestations of the Jones criteria. Without documentation of antecedent Group A Streptococcal infection, the diagnosis is much less likely except in a few rare scenarios. Carditis, polyarthritis and Sydenham's chorea are the most common major manifestations of acute rheumatic fever. However, despite the predominance of these major manifestations of acute rheumatic fever

2014 Autoimmunity reviews

75. 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

76. 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

77. 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

78. 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

79. 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

80. 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

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