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Sydenham Chorea

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61. Obsessive Compulsive Disorder

as controversial in OCDasthatofPANDAS.Thecentralhypothesis of PANDAS derives from the observations of neurobehavioral disturbance accompanying Sydenham chorea, a sequel of rheumatic fever. An immune response to group A -hemolytic streptococcus (GABHS) infections purportedly leadstocrossreactivitywith,andin?ammation of,basalganglia,withadistinctneurobehavioral syndrome that includes OCD, tics, and perhaps hyperactivity. The diagnostic criteria were laid out by Swedo et al., 14 but detractors have argued AACAP (...) suddenly relapses. Neurologic signs, such as chorea, are evidence of rheumatic fever but may not occur for many months after infection. “Soft” neurologic signs, such as tremor and coordination dif?culties on examination, are one criterion of the PANDAS diagnosis. 14,17 Antistreptococcic antibodies such as antistreptolysin O and anti-DNase B are present in most children by early adolescence, but a 0.2 log increase (doubling) in titers is considered evidence of a recent infection. Intercurrent titers may

2012 American Academy of Child and Adolescent Psychiatry

63. The Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease

? pericardial friction rub or effusion • Expanded discussion on cognitive dysfunction with chorea • Upper limits of normal (ULN) for serum streptococcal antibody titres expanded to include children and adults based on Fiji data (Table 3.6) Table 1.1 Levels of evidence for clinical interventions, and grades of recommendation Level of evidence Study design Grade of recommendation I Evidence obtained from a systematic review of all relevant RCT A Rich body of high quality RCT data II Evidence obtained from

2012 Clinical Practice Guidelines Portal

64. Rheumatic fever identification, management and secondary prevention

Islander communities living in rural or remote areas or in disadvantaged suburban areas. Sydenham (rheumatic) chorea is present in roughly 25% of Aboriginal Australians (especially female adolescents) with ARF. 11 As part of the major manifestations, Sydenham chorea is sufficient to diagnose ARF without evidence of previous S. pyogenes infection, provided other causes of chorea have been excluded. 2 The NHFA criteria consider some less specific symptoms, such as polyarthralgia and aseptic monoarthritis (...) -arthritis or polyarthralgia Sydenham chorea Erythema marginatum Subcutaneous nodules Carditis (including subclinical evidence of rheumatic valve disease on echocardiogram) Polyarthritis Sydenham chorea Erythema marginatum Subcutaneous nodules Minor manifestations Fever ESR ≥30 mm/hr or CPR ≥30 mg/L Prolonged P-R interval on ECG Fever Polyarthralgia or aseptic mono-arthritis ESR ≥30 mm/hr or CPR ≥30 mg/L Prolonged P-R interval on ECG ESR = erythrocyte sedimentation rate; CRP = c-reaction protein; ECG

2012 Clinical Practice Guidelines Portal

65. 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

66. 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

67. 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

68. 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

69. Systematic review of immunoglobulin use in paediatric neurological and neurodevelopmental disorders. (PubMed)

(grade C), reduces mortality in acute encephalitis syndrome with myocarditis (grade C), and improves function and stabilizes disease in myasthenia gravis (grade C). IVIG improves outcome in N-methyl-d-aspartate receptor encephalitis (grade C) and opsoclonus-myoclonus syndrome (grade C), reduces cataplexy symptoms in narcolepsy (grade C), speeds recovery in Sydenham chorea (grade C), reduces tics in selected cases of Tourette syndrome (grade D), and improves symptoms in paediatric autoimmune

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2016 Developmental Medicine and Child Neurology

70. CNS autoimmune disease after Streptococcus pyogenes infections: animal models, cellular mechanisms and genetic factors (PubMed)

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

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2016 Future Neurology

71. The link between autoimmune diseases and obsessive-compulsive and tic disorders: A systematic review. (PubMed)

between ADs and OCD/tic disorders until July, 13th 2016. Seventy-four studies met inclusion criteria. Overall, the studies were of limited methodological quality. Rates of OCD were higher in rheumatic fever patients who were also affected by its neurological manifestation, Sydenham's chorea. The literature on other ADs was scarce and the findings inconclusive. Few studies examined the association between ADs and tic disorders. A handful of family studies reported elevated rates of ADs in first-degree

2016 Neuroscience and biobehavioral reviews

72. 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

73. 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

74. 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

75. The usefulness of immunotherapy in paediatric neurodegenerative disorders: A systematic review of literature data. (PubMed)

-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome. In Sydenham Chorea the use of methylprednisolone was found in most cases as efficient as IVIG, while in Tourette's Syndrome, Colecoxib was successfully used in one patient. Pediatric Multiple Sclerosis seems to respond better to immunosuppressant agents (Mitoxantrone, Cyclophosphamide, Natalizumab), as well as Neuromyelitis optica

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2015 Human vaccines & immunotherapeutics

76. Antineuronal Antibodies in a Heterogeneous Group of Youth and Young Adults with Tics and Obsessive-Compulsive Disorder. (PubMed)

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

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2015 Journal of Child and Adolescent Psychopharmacology

77. Anti-basal ganglia antibodies in primary obsessive-compulsive disorder: systematic review and meta-analysis. (PubMed)

Anti-basal ganglia antibodies in primary obsessive-compulsive disorder: systematic review and meta-analysis. Autoimmune-mediated basal ganglia dysfunction is implicated in the pathophysiology of neuropsychiatric disorders commonly manifesting with obsessive-compulsive features (e.g. Sydenham chorea). The relationship between autoimmunity and primary obsessive-compulsive disorder (OCD), however, is less clear.To pool data on serum and cerebrospinal fluid (CSF) anti-basal ganglia antibody (ABGA

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2014 British Journal of Psychiatry

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

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

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

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

80. Evidence-based guideline update: Plasmapheresis in neurologic disorders: Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology (PubMed)

, based on one Class I study (Level B). Plasmapheresis is possibly effective 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).

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2011 EvidenceUpdates

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