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Miranda Crealey - One of the best experts on this subject based on the ideXlab platform.
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ps 249 Sydenhams Chorea the irish experience
2014Co-Authors: Miranda CrealeyAbstract:Sydenham’s Chorea (SC) is an autoimmune movement disorder characterised by uncoordinated involuntary movements (Chorea), emotional instability and hypotonia. It is one of the major Jones diagnostic criteria of rheumatic fever caused by group A streptococcus bacterium (GAS). Aims Report on the incidence of SC diagnosis in Ireland (including Northern Ireland) July 2006–July 2013. Describe the clinical presentation, investigation and management. Methods A retrospective observational study was undertaken of all children with a clinical diagnosis of SC or “SC like” presentation attending the paediatric neurology departments in Ireland. A questionnaire and chart review was performed on all cases. Results 16 cases were documented by paediatric neurologists from 2006- 2013 giving an incidence of 0.21/100,000. 13 occurred in females with a median age of 10 years. 75% presented predominantly with a hemiChorea. All had functional impairment with 11/16 having behavioural or mood change. 5 had carditis diagnosed by echo. 13 had serological evidence of previous GAS infection. All had a normal MRI brain. 15/16 received medication. valproate was the first choice in 11 cases, steroids in 2 and valproate in combination with steroids in 2. 8 were treated with penicillin for >11 months. Conclusion The incidence of Sydenhams Chorea appears to be rising. It is a clinical diagnosis and important to recognise. In our cohort we used a combination of steroids and valproate which gave a good response. There was a trend towards long-term penicillin. Acknowledgements Collaborators: David Webb (Crumlin), Tony Bouldin (Crumlin), Deidre Peake (Belfast), Sanjay Tirupathi (Belfast), Niamh Mc Sweeney (Cork).
Mercadante, Marcos Tomanik - One of the best experts on this subject based on the ideXlab platform.
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Obsessive-compulsive disorder, tic disorders, Tourette syndrome and other psychiatric disorders in rheumatic fever with or without Sydenham's Chorea patients
2005Co-Authors: Mercadante, Marcos TomanikAbstract:Transtornos psiquiátricos têm sido descritos com maior freqüência em pacientes com Coréia de Sydenham (CS) do que em pacientes com Febre Reumática(FR) sem CS. Os objetivos desse estudo forma o de verificar: se existe uma freqüência aumentada de transtornos psiquiátricos em pacientes com FR comparados a um grupo controle; se estes transtornos psiquiátricos apresentam freqüência aumentada em grupo de pacientes com Coréia de Sydenham, a manifestação da FR no Sistema Nervoso Central, comparado ao grupo de pacientes com FR sem CS; e, por fim, verificar a relação temporal entre o início destes diversos transtornos e o início da FR. Concluiu-se que a presença de FR está associada a uma maior freqüência de transtornos psiquiátricos, mesmo na ausência de CS. O TDHA e o TT, neste estudo, foram indicados como fatores de risco para o desenvolvimento de CS em pacientes com FR.Psychiatric disorders have been described as more frequent in Sydenhams Chorea patients SC) than in rheumatic fever without SC (RF). The aim of this study was to investigate it the prevalence of psychiatric disorders in RF is associated with the occurrence of SC. Furthermore, age of onset of the various symptoms was determined in order to clarify the temporal relationship between the presence of psychiatric symptoms and either rheumatic fever or Sydenhams Chorea. Using semi-structured diagnostic interviews for DSM-IV and ratings scales, the authors assessed 22 SC patients, 20 RF patients and 20 pediatric controls. Statistical Analyses were performed using Pearson chi-square (Fischers exact test for 2x2 tables) for comparisons of categorial variables. Comparisons of continuous variables among groups were carried out using ANOVA and the Student t-test, when only groups were analyzed. In order to establish the risk for the development of SC and OCD given to presence of other co-morbid conditions, a logistic regression was applied. The level of significance adopted was 0.05. Both the SC and RF groups showed a greater prevalence of psychiatric disorders. The SC sample showed higher frequency of major depression disorder (MDD) (x2 = 19,1, df = 2, p = 0,00007), tic disorder (TD) (x2 = 21,1, df = 2, p = 0,00001) and attention-deficit hyperactivity disorder (ADHD) (x2-21,7, df = 2, p = 0,0002). Although Obsessive-Compulsive Disorder (OCD) was not statiscally higher in the SC and RF groups, Obsessive-Compulsive Symptoms were more frequent in both RF and SC groups compared to the controls (x2 = 7,3, df = 2, p = 0,025). The age of onset for both ADHD and TD predicted the risk for development of SC. The risk of development of OCD in SC children was also associated with the age of onset of ADHD. RF seems to confer increased risk to develop neuropsychiatric disorders even in patients without SC. In this sample, ADHD and TD was an important risk factor for the occurrence of co-morbid illnesses
Dua Priyamvada - One of the best experts on this subject based on the ideXlab platform.
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Functional effects of anti-neuronal antibodies in patients with encephalitis lethargica and related disorders associated with streptococcal infection
2014Co-Authors: Dua PriyamvadaAbstract:PhDEncephalitis lethargica affected a large number of people in the pandemic in the early 1900s (von Economo, 1930). Histological and biochemical data suggest that autoimmune mechanisms play an important role in this disorder and recently serum anti-basal ganglia antibodies (ABGA) have been detected in affected sporadic cases associated with evidence of recent streptococcal infection (Dale et al., 2004a). ABGA are also associated with other neuropsychiatric disorders including Sydenhams Chorea, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, Tourettes syndrome and obsessive compulsive disorder. The precise frequency, presentation, disease course, treatment response and causes of these disorders are still unknown. As ABGA are strongly associated with recent streptococcal infection, these disorders represent a potentially good model for the study of molecular mimicry and autoimmunity. The present study focussed on various aspects of this group of disorders. Profiling of group A streptococcus isolates from both patients with postulated post-streptococcal disorders of the CNS and controls was done which highlighted differences in virulence factors like M protein and superantigens between the two groups. Also in the present study we demonstrated the pathogenicity of anti-neuronal antibodies found in patients in both an in vivo and in vitro setting. An animal model of the disorders was produced by passive transfer of antibodies from patients which resulted in symptoms reminiscent of diseases like encephalitis lethargica and dystonia. An active immunization animal model using GABHS proteins and recombinant proteins (putative autoantigens) was also developed. Furthermore, the autoantibodies from patients and animal models were analysed on both neuronal and non-neuronal cells where they demonstrated to have a functional effect on cytotoxicity, apoptosis, calcium flux and enolase activity. N-methyl D-aspartate glutamate receptor and voltage gated potassium channel have been recently been implicated in a 16 range of neurological disorders, hence we also tested the patient sera for antibodies against these receptors and found a group of patients to be positive. In summary, EL and other ABGA-associated disorders are still an emerging entity, with major implications for neuropsychiatry. As auto-antibody mediated diseases respond to immunomodulatory therapy, identifying and defining the pathogenesis of these disorders is important so that patients can be appropriately treated.The Sophie Cameron Trus
Hounie, Ana Gabriela - One of the best experts on this subject based on the ideXlab platform.
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Obsessive-compulsive spectrum disorders and rheumatic fever: a family study.
2003Co-Authors: Hounie, Ana GabrielaAbstract:Resumo HOUNIE, A.G. Transtornos do espectro obsessivo-compulsivo e febre reumática: um estudo de transmissão familiar. São Paulo, 2003. 174p. Tese (Doutorado) Faculdade de Medicina, Universidade de São Paulo. A febre reumática (FR) é uma doença autoimune causada por anticorpos desenvolvidos contra o estreptococo beta hemolítico do grupo A (SBHGA). Estudos recentes amparam a noção de que a FR esteja associada ao transtorno obsessivo-compulsivo (TOC), independentemente da presença de outras manifestações no sistema nervoso central (ex.: coréia de Sydenham - CS). O TOC freqüentemente surge acompanhado por outros transtornos, como aqueles denominados de transtornos do espectro obsessivo (TEO), entre eles, a síndrome de Tourette (ST), o transtorno de tiques crônicos (TTC) e o transtorno dismórfico corporal (TDC). Alguns deles parecem apresentar uma relação geneticamente determinada. Um subgrupo de TOC é geneticamente relacionado à ST, podendo ser considerado uma expressão fenotípica alternativa de seu genótipo. Um recente estudo de famílias de portadores de TOC encontrou alta freqüência de TDC nos seus familiares de primeiro grau, além de transtornos de tiques. Neste estudo de famílias buscamos verificar a freqüência de TOC e TEO relacionados em familiares de portadores de FR, com ou sem CS e em um grupo controle, na tentativa de determinar se existe agregação familiar desses transtornos em famílias com FR. Foram avaliados 98 probandos e seus 381 familiares de primeiro grau (FPG). Dos 98 probandos, 31 eram portadores de FR sem CS, 28 tinham FR com CS e 39 eram controles sem FR recrutados em um ambulatório de ortopedia. Entrevistadores treinados avaliaram os sujeitos por meio de entrevistas semi-estruturadas (KSADS e SCID). Vinhetas clínicas foram elaboradas e avaliadas por psiquiatras independentes que faziam a melhor estimativa diagnóstica (best estimate diagnosis). Estes avaliadores eram cegos quanto a se os sujeitos eram probandos ou familiares e quanto ao grupo a que pertenciam. As comparações entre as freqüências dos transtornos entre os três grupos foram feitas por meio dos testes de Fisher, qui-quadrado, pelas Equações de Estimação Generalizadas (GEE) e pela análise de Kaplan-Meier. Resultados foram considerados estatisticamente significantes quando o nível descritivo (p, bicaudado) do teste foi menor ou igual a 0,05. Os TEO combinados foram mais freqüentes em FPG de portadores de FR como um todo (com e sem CS) do que em FPG de probandos controles (p=0,03). O TTC foi mais freqüente em FPG de probandos com CS do que FPG de controles (p=0,05). O TAG foi mais freqüente em FPG de portadores de FR do que em FPG de controles (p=0,008). Subdividindo os probandos baseados na presença de um TEO, os transtornos de tiques somados (p,GEE=0,01), o TDC (p,GEE=0,02), e o TAG (x2=0,004) individualmente e os TEO combinados (p,GEE=0,02) foram mais freqüentes em FPG de portadores de FR na presença de TEO comparados aos FPG de controles. Os TEO foram mais freqüentes (sem alcançar significância estatística, p=0,09) nos FPG de portadores de FR sem um TEO do que nos FPG de controles. A febre reumática nos probandos elevou o risco de seus familiares apresentarem um TEO. Esse risco foi ainda maior se o probando, além de apresentar FR, tinha ele próprio um TEO. É possível que um subgrupo da FR seja relacionado a TEO e que fatores de susceptibilidade para FR e para TEO tenham um efeito aditivo. Portanto, a FR pode ser considerada um fator de risco para TEO.Summary Hounie, A.G. Obsessive-compulsive spectrum disorders and rheumatic fever: a family study. São Paulo, 2003. 174p. Tese (Doutorado) Faculdade de Medicina, Universidade de São Paulo. Rheumatic fever (RF) is an autoimmune disease caused by antibodies against group A beta hemolytic streptococcus (GABHS). Some studies support the notion that RF be associated with obsessive-compulsive disorder (OCD) independent of its manifestations on the central nervous system (eg., Sydenhams Chorea - SC). OCD is often accompanied by other disorders, which are known as obsessive-compulsive spectrum disorders (OCSD), such as Tourette syndrome (TS), chronic tic disorder (CTD) and body dysmorphic disorder (BDD). Some of those putative OCSD seem to share a genetic relationship. An OCD subgroup is genetically related to TS, being considered an alternative phenotype. A recent OCD family study found higher rates of BDD and tic disorders among first-degree relatives (FDR) of OCD probands. This is a family study that assessed the frequency of OCD and OCSD in first-degree relatives of RF probands (with and without SC) in order to verify if there was aggregation of those disorders in RF families. We assessed 98 probands and their 381 first-degree relatives. Of the 98 probands, 31 had RF without SC, 28 had RF with SC and 39 were controls without RF recruited in an orthopedic clinic. Trained interviewers assessed subjects with semi-structured interviews (KSADS and SCID). Clinical vignettes were elaborated and evaluated by independent psychiatrists that assigned best estimate diagnosis. These raters were blind to subject status regarding group and if they were probands or relatives. Comparisons between frequencies of disorders were done with Fisher and chi-square tests, generalized estimated equations (GEE), and Kaplan-Meier survival analyses. Results were considered statistically significant if their level of significance was less or equal to 0.05 (p, two-tailed). OCSD combined were more frequent in FDR of RF probands (with or without SC) than in FDR of controls (p=0.03). CTD was more frequent in FDR of SC probands than FDR of controls (p=0.05). Generalized anxiety disorder (GAD) was more frequent in FDR of RF probands than in FDR of controls (p=0.008). Subdividing probands based on the presence of an OCSD, tic disorders combined (GEE, p=0.01), BDD (GEE, p=0.02), GAD (x2=0.004), and OCSD combined (GEE, p=0.02) were more frequent in FDR of RF probands with an OCSD than in FDR of controls. OCSD were also more frequent (non-significantly, p=0.09) in FDR of RF probands without an OCSD than in FDR of controls. RF in the probands increased the risk of OCSD in their family members. This risk was even higher if the proband had RF plus an OCSD. It is possible that a subgroup of RF is associated to OCSD and susceptibility factors linked to RF and to OCSD may have an additive effect. Therefore RF may be a risk factor for OCSD
Glynn L. E. - One of the best experts on this subject based on the ideXlab platform.
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Anti-streptococcal antibodies reacting with brain tissue. I. Immunofluourescent studies.
2024Co-Authors: Kingston D., Glynn L. E.Abstract:Serum JD from a 14-year old girl with Sydenhams Chorea contained antibodies which gave immunofluorescent staining of the limiting membrane of the brain, ependymal tissue and fibrous astrocytes. These antibodies could be completely absorbed by Str. pyogenes type 24 (NCTC 8305) but only partially if at all by type 6 matt (NCTC 8302) or type 6 glossy (NCTC 8709). In contrast, staining by the same serum of the choroid plexus, the periphery of hepatocytes, the periphery of the cells of the gastric mucosa, and tubules in the kidney could be absorbed out by the type 6 matt and type 24 strains (but not by the type 6 glossy or Staph. aureus NCTC 6571). A rabbit anti-streptococcal serum (3/74) raised against disintegrated washed cells of Str. pyogenes type 24 stained and the same structures in the brain to high titre, but not the choroid plexus and not the other structures stained by serum JD. These staining reactions of 3/74 could be absorbed out by Str. pyogenes type 24 but not by Str. pyogenes type 6 matt or type 6 glossy. None of these staining patterns given by serum JD or by 3/74 could be absorbed by human uterine smooth muscle. Serum 3/74 stained heart muscle but this reaction could be absorbed without affecting the brain staining reactions. Sera from 4 other patients with Sydenham's Chorea were found to give staining of the ependyma and the limiting membrane, 2 only very weakly
