Sydenham's chorea in a family with Huntington's disease: case report and review of the literature

Detalhes bibliográficos
Autor(a) principal: Santos-Silva,Rita
Data de Publicação: 2011
Outros Autores: Corujeira,Susana, Almeida,Ana Filipe, Granja,Sofia, Moura,Cláudia, Azevedo,Inês, Leão,Miguel, Maia,Ana
Tipo de documento: Artigo
Idioma: eng
Título da fonte: São Paulo medical journal (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400011
Resumo: CONTEXT: Sydenham's chorea affects almost 30% of patients with acute rheumatic fever. It is more frequent in females and is rare in the first decade of life, and genetic vulnerability underlies it. Because of easy access to antibiotics, it is now rare in so-called developed countries. CASE REPORT: A 6-year-old boy with a family history of Huntington's disease, who was the only child of an unscreened and asymptomatic mother, was brought for a consultation because of migratory arthralgia, depressed mood, and rapid, abrupt and unintentional movements of his right arm and leg, that had evolved over a three-week period. On physical examination, he presented a grade III/VI systolic heart murmur and right-side choreic movements, giving rise to a deficit of active mobilization. Laboratory tests revealed elevated erythrocyte sedimentation rate (63 mm/h), C-reactive protein (25 mg/l) and antistreptolysin O titer (1,824 U/ml). Cardiovascular evaluation showed mild aortic insufficiency, moderate mitral insufficiency and a prolonged PR interval. A clinical diagnosis of Sydenham's chorea/acute rheumatic fever was made, and therapy consisting of penicillin, haloperidol, captopril and furosemide was instituted, with excellent results. CONCLUSION: In developed countries, Sydenham's chorea seems forgotten and, because of this, little is known about its clinical course and controversy surrounds the therapeutic options available. This occurrence of rheumatic chorea in a family with Huntington's disease highlights the importance of the differential diagnosis for the different forms of chorea.
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spelling Sydenham's chorea in a family with Huntington's disease: case report and review of the literatureChoreaHuntington diseaseRheumatic feverAntistreptolysinGenetic counselingCONTEXT: Sydenham's chorea affects almost 30% of patients with acute rheumatic fever. It is more frequent in females and is rare in the first decade of life, and genetic vulnerability underlies it. Because of easy access to antibiotics, it is now rare in so-called developed countries. CASE REPORT: A 6-year-old boy with a family history of Huntington's disease, who was the only child of an unscreened and asymptomatic mother, was brought for a consultation because of migratory arthralgia, depressed mood, and rapid, abrupt and unintentional movements of his right arm and leg, that had evolved over a three-week period. On physical examination, he presented a grade III/VI systolic heart murmur and right-side choreic movements, giving rise to a deficit of active mobilization. Laboratory tests revealed elevated erythrocyte sedimentation rate (63 mm/h), C-reactive protein (25 mg/l) and antistreptolysin O titer (1,824 U/ml). Cardiovascular evaluation showed mild aortic insufficiency, moderate mitral insufficiency and a prolonged PR interval. A clinical diagnosis of Sydenham's chorea/acute rheumatic fever was made, and therapy consisting of penicillin, haloperidol, captopril and furosemide was instituted, with excellent results. CONCLUSION: In developed countries, Sydenham's chorea seems forgotten and, because of this, little is known about its clinical course and controversy surrounds the therapeutic options available. This occurrence of rheumatic chorea in a family with Huntington's disease highlights the importance of the differential diagnosis for the different forms of chorea.Associação Paulista de Medicina - APM2011-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400011Sao Paulo Medical Journal v.129 n.4 2011reponame:São Paulo medical journal (Online)instname:Associação Paulista de Medicinainstacron:APM10.1590/S1516-31802011000400011info:eu-repo/semantics/openAccessSantos-Silva,RitaCorujeira,SusanaAlmeida,Ana FilipeGranja,SofiaMoura,CláudiaAzevedo,InêsLeão,MiguelMaia,Anaeng2011-09-21T00:00:00Zoai:scielo:S1516-31802011000400011Revistahttp://www.scielo.br/spmjhttps://old.scielo.br/oai/scielo-oai.phprevistas@apm.org.br1806-94601516-3180opendoar:2011-09-21T00:00São Paulo medical journal (Online) - Associação Paulista de Medicinafalse
dc.title.none.fl_str_mv Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
title Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
spellingShingle Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
Santos-Silva,Rita
Chorea
Huntington disease
Rheumatic fever
Antistreptolysin
Genetic counseling
title_short Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
title_full Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
title_fullStr Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
title_full_unstemmed Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
title_sort Sydenham's chorea in a family with Huntington's disease: case report and review of the literature
author Santos-Silva,Rita
author_facet Santos-Silva,Rita
Corujeira,Susana
Almeida,Ana Filipe
Granja,Sofia
Moura,Cláudia
Azevedo,Inês
Leão,Miguel
Maia,Ana
author_role author
author2 Corujeira,Susana
Almeida,Ana Filipe
Granja,Sofia
Moura,Cláudia
Azevedo,Inês
Leão,Miguel
Maia,Ana
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Santos-Silva,Rita
Corujeira,Susana
Almeida,Ana Filipe
Granja,Sofia
Moura,Cláudia
Azevedo,Inês
Leão,Miguel
Maia,Ana
dc.subject.por.fl_str_mv Chorea
Huntington disease
Rheumatic fever
Antistreptolysin
Genetic counseling
topic Chorea
Huntington disease
Rheumatic fever
Antistreptolysin
Genetic counseling
description CONTEXT: Sydenham's chorea affects almost 30% of patients with acute rheumatic fever. It is more frequent in females and is rare in the first decade of life, and genetic vulnerability underlies it. Because of easy access to antibiotics, it is now rare in so-called developed countries. CASE REPORT: A 6-year-old boy with a family history of Huntington's disease, who was the only child of an unscreened and asymptomatic mother, was brought for a consultation because of migratory arthralgia, depressed mood, and rapid, abrupt and unintentional movements of his right arm and leg, that had evolved over a three-week period. On physical examination, he presented a grade III/VI systolic heart murmur and right-side choreic movements, giving rise to a deficit of active mobilization. Laboratory tests revealed elevated erythrocyte sedimentation rate (63 mm/h), C-reactive protein (25 mg/l) and antistreptolysin O titer (1,824 U/ml). Cardiovascular evaluation showed mild aortic insufficiency, moderate mitral insufficiency and a prolonged PR interval. A clinical diagnosis of Sydenham's chorea/acute rheumatic fever was made, and therapy consisting of penicillin, haloperidol, captopril and furosemide was instituted, with excellent results. CONCLUSION: In developed countries, Sydenham's chorea seems forgotten and, because of this, little is known about its clinical course and controversy surrounds the therapeutic options available. This occurrence of rheumatic chorea in a family with Huntington's disease highlights the importance of the differential diagnosis for the different forms of chorea.
publishDate 2011
dc.date.none.fl_str_mv 2011-01-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400011
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dc.language.iso.fl_str_mv eng
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dc.relation.none.fl_str_mv 10.1590/S1516-31802011000400011
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
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dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Associação Paulista de Medicina - APM
publisher.none.fl_str_mv Associação Paulista de Medicina - APM
dc.source.none.fl_str_mv Sao Paulo Medical Journal v.129 n.4 2011
reponame:São Paulo medical journal (Online)
instname:Associação Paulista de Medicina
instacron:APM
instname_str Associação Paulista de Medicina
instacron_str APM
institution APM
reponame_str São Paulo medical journal (Online)
collection São Paulo medical journal (Online)
repository.name.fl_str_mv São Paulo medical journal (Online) - Associação Paulista de Medicina
repository.mail.fl_str_mv revistas@apm.org.br
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