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Sydenham’s chorea

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https://www.readbyqxmd.com/read/29666090/rare-case-of-chorea-hyperglycaemia-basal-ganglia-c-h-bg-syndrome
#1
Sajjad Ahmad, Priya Mohan Babu, Lavanya Shenbagaraj, Lindsay George
An 83-year-old woman presented with acute-onset haemichorea and haemiballism particularly affecting the left side of the body. She was known to have type 2 diabetes, which was poorly controlled with sitagliptin. She was hyperglycaemic but not ketotic or acidotic. After she was started on insulin and good glycaemic control was achieved, her abnormal movements dramatically improved. MRI of the brain showed a T1-weighted hyperintense lesion on the right basal ganglia, which is typical of chorea-hyperglycaemia-basal ganglia syndrome...
April 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29519947/acute-hyperkinetic-movement-disorders-in-italian-paediatric-emergency-departments
#2
Umberto Raucci, Pasquale Parisi, Nicola Vanacore, Giacomo Garone, Claudia Bondone, Antonella Palmieri, Lucia Calistri, Agnese Suppiej, Raffaele Falsaperla, Alessandro Capuano, Valentina Ferro, Antonio Francesco Urbino, Ramona Tallone, Alessandra Montemaggi, Stefano Sartori, Piero Pavone, Margherita Mancardi, Federico Melani, Lucrezia Ilvento, Maria Federica Pelizza, Antonino Reale
INTRODUCTION: Limited data exist on epidemiology, clinical presentation and management of acute hyperkinetic movement disorders (AHMD) in paediatric emergency departments (pED). METHODS: We retrospectively analysed a case series of 256 children (aged 2 months to 17 years) presenting with AHMD to the pEDs of six Italian tertiary care hospitals over a 2-year period (January 2012 to December 2013). RESULTS: The most common type of AHMD was tics (44...
August 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29471551/bilateral-striatal-necrosis-after-sydenham-s-chorea-in-a-7-year-old-boy-a-2-year-follow-up
#3
C Canavese, C Davico, M Casabianca, C Olivieri, S Mancini, G Migliore, A Versace, A Tocchet, B Vitiello
Child bilateral striatal necrosis (BSN) is a rare and etiologically heterogeneous condition. An association with group A streptococcus (GAS) infection was previously reported in two cases of BSN in infancy and early childhood. We here report on a 7-year-old boy who developed chorea and dystonia 20 days after symptomatic recovery from Sydenham's chorea. Repeated brain magnetic resonance imaging scans, obtained before, soon after the onset of the post-Sydenham symptoms, and 1 year later were consistent with an evolution from bilateral striatal microbleeding to necrosis, and consequently reduced basal ganglia volume and enlargement of the frontal horns...
June 2018: Neuropediatrics
https://www.readbyqxmd.com/read/29406902/autoimmune-and-paraneoplastic-movement-disorders-an-update
#4
REVIEW
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29350121/-a-boy-with-involuntary-movements
#5
G M Kalisvaart, Y Koopman-Keemink
A 12-year-old Dutch boy came to the paediatrician with involuntary movements of the right half of his body. He was diagnosed with Sydenham chorea as a consequence of acute rheumatic fever. Acute rheumatic fever is a rare disease in Western countries and should be recognised in time. Antibiotic prophylaxis is indicated in patients who have had acute rheumatic fever, at least until the age of 40.
2018: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29289523/principles-and-approaches-to-the-treatment-of-immune-mediated-movement-disorders
#6
REVIEW
Shekeeb S Mohammad, Russell C Dale
Immune mediated movement disorders include movement disorders in the context of autoimmune encephalitis such as anti-NMDAR encephalitis, post-infectious autoimmune movement disorders such as Sydenham chorea, paraneoplastic autoimmune movement disorders such as opsoclonus myoclonus ataxia syndrome, and infection triggered conditions such as paediatric acute neuropsychiatric syndrome. This review focuses on the approach to treatment of immune mediated movement disorders, which requires an understanding of the immunopathogenesis, whether the disease is destructive or 'altering', and the natural history of disease...
March 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29287833/corticosteroid-treatment-in-sydenham-s-chorea
#7
C Fusco, C Spagnoli
Sydenham's chorea (SC) is an immune-mediated hyperkinetic movement disorder, developing after group A Beta-hemolytic streptococcal (GABHS) infection. Aside from conventional symptomatic treatment (carbamazepine, valproate, neuroleptics), the use of steroids has also been advocated, mainly in severe, drug-resistant cases or if clinically disabling side effects develop with first line therapies. Based on the description of 5 cases followed in the Child Neurology Unit of Santa Maria Nuova Hospital in Reggio Emilia and on the available medical literature on this topic, we propose considering the use of corticosteroids therapy in children with SC, with the administration of IV methyl-prednisolone followed by oral deflazacort in severe cases and of oral deflazacort alone in mild and moderate degrees of involvement...
March 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29103424/autoantibody-associated-movement-disorders-in-children-proven-and-proposed
#8
REVIEW
Harvey S Singer
Movement disorders secondary to autoantibodies in children represent a rapidly expanding group of conditions. Once considered to be limited to poststreptococcal Sydenham's chorea or rare cases of childhood systemic lupus erythematosus, a variety of antibody-related movement abnormalities are now seen as part of noninfectious autoimmune encephalitis or within an expanding list of postinfectious disorders. In this article, several proposed autoantibody-mediated movement disorders in children are reviewed. In each one, there is a hypothesized antibody biomarker that is believed to be pathogenic and cause the clinical symptoms...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28979614/-exceptionally-good-response-to-sodium-valproate-in-patients-with-recurrent-sydenham-s-chorea
#9
Siham Bouchal, Ouarda Ouali, Mouhamed Faouzi Belahsen
Sydenham's chorea is the most common acquired cause of chorea in the third world. We report a case of recurrent chorea successfully treated with sodium valproate. Miss A.C, aged 16, with a history of recurrent angina reported an episode of choreic movements 2 years before requiring treatment with haloperidol and prevention of rheumatic fever. The patient interrupted her treatment with occurrence of a relapse a few months later. Brain MRI and transthoracic ultrasound were normal. Preventive treatment with extencilline and haloperidol was restarted without any improvement, hence the treatment with sodium valproate...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28745221/rheumatic-heart-disease-pathogenesis-and-vaccine
#10
L Guilherme, S Freschi de Barros, K F Kohler, S R Santos, F Morais Ferreira, W R Silva, R Alencar, E Postol, J Kalil
Rheumatic fever (RF) and rheumatic heart disease (RHD) follow untreated S. pyogenes throat infections in children who present susceptible genes that favor the development of autoimmune reactions. In this review, we focus on the genes that confer susceptibility and on the autoimmune reactions that occur due to molecular mimicry between human-tissue proteins and streptococcal M protein. Polyarthritis is the initial manifestation, which can evolve to carditis and severe valve damage; these culminate in rheumatic heart disease (RHD) or Sydenham's chorea, which affects the central nervous system...
2018: Current Protein & Peptide Science
https://www.readbyqxmd.com/read/28589057/treatment-of-sydenham-s-chorea-a-review-of-the-current-evidence
#11
REVIEW
Shannon L Dean, Harvey S Singer
BACKGROUND: Sydenham's chorea (SC), the neurologic manifestation of rheumatic fever, remains the most prevalent form of chorea in children. Suggested treatments of chorea in SC include prophylactic penicillin, symptomatic (antipsychotic and anticonvulsant) medications, and immunomodulatory therapy (steroids, intravenous immunoglobulin (IVIG), and plasma exchange). In this manuscript, we undertook a systematic review of the published literature to examine the data supporting these therapeutic recommendations...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28442182/a-retrospective-study-acute-rheumatic-fever-and-post-streptococcal-reactive-arthritis-in-japan
#12
Satoshi Sato, Yoji Uejima, Eisuke Suganuma, Tadamasa Takano, Yutaka Kawano
BACKGROUND: Acute rheumatic fever (ARF) and post-streptococcal reactive arthritis (PSRA) are immune-mediated consequences of group A streptococcal pharyngitis. ARF has declined in developed nations. No prevalence survey of PSRA has been conducted. This study evaluated the incidence and characteristics of ARF and PSRA in Japanese children. METHODS: From 2010 to 2015, ARF and PSRA were evaluated using clinical data retrospectively collected by chart review from 528 hospitals...
October 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/27920268/clinical-value-of-perfusion-abnormalities-of-brain-on-technetium-99m-hmpao-single-photon-emission-computed-tomography-in-children-with-sydenham-chorea
#13
Sara Maria Delle Acque Giorgio, Maria Grazia Caprio, Flavia Galante, Giustina Russo, Alfonso Romano, Emilia Vergara, Maria Alessio, Alberto Cuocolo
We evaluated whether perfusion brain abnormalities by single-photon emission computed tomography (SPECT) imaging improves diagnostic and prognostic assessment in Sydenham chorea. Twenty-three children with acute autoimmune chorea underwent technetium-99m hexamethylpropyleneamine oxime brain SPECT imaging. In 16 children, SPECT was repeated during the follow-up. A pattern of basal ganglia hyperperfusion was observed in 20 (87%) patients. In 4 of 10 patients with generalized chorea, perfusion was comparable in right and left striatum and right and left thalamus...
March 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/27919056/autoimmune-choreas
#14
REVIEW
Francisco Cardoso
Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Emphasis is given particularly to Sydenham's chorea, systemic lupus erythematosus, primary antiphospolipid antibody syndrome, paraneoplastic chorea and anti- N -methyl-d-aspartate receptor encephalitis...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27815733/acute-rheumatic-fever-outbreak-in-southern-central-european-country
#15
Urška Kočevar, Nataša Toplak, Blaž Kosmač, Luka Kopač, Samo Vesel, Natalija Krajnc, Matjaž Homan, Rina Rus, Tadej Avčin
A decline in the incidence of acute rheumatic fever (ARF) in developed countries over the past century can be attributed to the improved public hygiene and to widespread use of antibiotics. ARF seemed to be a rare disease in southern central European country, Slovenia, up to 2010 when we noticed an increase in the number of patients with ARF. In order to assess the current incidence of ARF, we performed a retrospective study of all patients with ARF treated at the University Children's Hospital Ljubljana from January 2008 until the end of December 2014...
January 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27706426/abnormal-eye-movements-in-three-types-of-chorea
#16
REVIEW
Tiago Attoni, Rogério Beato, Serge Pinto, Francisco Cardoso
Chorea is an abnormal movement characterized by a continuous flow of random muscle contractions. This phenomenon has several causes, such as infectious and degenerative processes. Chorea results from basal ganglia dysfunction. As the control of the eye movements is related to the basal ganglia, it is expected, therefore, that is altered in diseases related to chorea. Sydenham's chorea, Huntington's disease and neuroacanthocytosis are described in this review as basal ganglia illnesses that can present with abnormal eye movements...
September 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27687817/the-link-between-autoimmune-diseases-and-obsessive-compulsive-and-tic-disorders-a-systematic-review
#17
REVIEW
Ana Pérez-Vigil, Lorena Fernández de la Cruz, Gustaf Brander, Kayoko Isomura, Caroline Gromark, David Mataix-Cols
Immunological factors are increasingly recognized as being important in a range of neuropsychiatric disorders. We aimed to summarize the disperse and often conflicting literature on the potential association between autoimmune diseases (ADs) and obsessive-compulsive disorder (OCD) and tic disorders. We searched PubMed, EMBASE, and PsycINFO for original studies evaluating the relationship between ADs and OCD/tic disorders until July, 13th 2016. Seventy-four studies met inclusion criteria. Overall, the studies were of limited methodological quality...
December 2016: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/27370066/-evaluation-of-a-neuropsychiatric-disorder-from-pandas-to-pans-and-cans
#18
Muharrem Burak Baytunca, Tuğba Donuk, Serpil Erermiş
PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) syndrome is a disorder seen before adolescence that possesses an abrupt onset of obsessive-compulsive disorder symptoms and/or tics. Swedo and colleagues defined this disorder in 1998 as a syndrome related to Group A streptoccoccus (GAS) infection with neurological issues, such as motor hyperactivation and choreiform movements. The progress of the disorder may be described as wax-and-waning, apart from abrupt onset, and this relapse and remission course is associated with exacerbating infections, according to the creators of PANDAS syndrome...
2016: Türk Psikiyatri Dergisi, Turkish Journal of Psychiatry
https://www.readbyqxmd.com/read/27262149/stiff-person-syndrome-and-other-immune-mediated-movement-disorders-new-insights
#19
REVIEW
Bettina Balint, Kailash P Bhatia
PURPOSE OF REVIEW: This review highlights the recent developments in immune-mediated movement disorders and how they reflect on clinical practice and our understanding of the underlying pathophysiological mechanisms. RECENT FINDINGS: The antibody spectrum associated with stiff person syndrome and related disorders (SPSD) has broadened and, apart from the classic glutamic acid decarboxylase (GAD)- and amphiphysin-antibodies, includes now also antibodies against dipeptidyl-peptidase-like protein-6 (DPPX), gamma-aminobutyric acid type A receptor (GABAAR), glycine receptor (GlyR) and glycine transporter 2 (GlyT2)...
August 2016: Current Opinion in Neurology
https://www.readbyqxmd.com/read/27209549/predictors-of-recurrence-in-sydenham-s-chorea-clinical-observation-from-a-single-center
#20
Esra Gurkas, Zeynep S Karalok, Birce D Taskin, Ummu Aydogmus, Alev Guven, Aydan Degerliyurt, Omer Bektas, Cahide Yilmaz
OBJECTIVE: Sydenham's chorea is the most common cause of acquired chorea in children and is the major manifestation for acute rheumatic fever. Despite being known as a benign, self-limiting condition, recurrences and persistence of symptoms can be seen. In this study, we aimed to evaluate retrospectively the clinical and laboratory features of patients with Sydenham's chorea and the rate and the course of recurrences, and to assess the risk of recurrences. METHODS: The study was a retrospective study conducted in a tertiary hospital...
October 2016: Brain & Development
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