Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Arquivos de neuro-psiquiatria (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000300142 |
Resumo: | ABSTRACT The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia. |
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Arquivos de neuro-psiquiatria (Online) |
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Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodiesautoimmunitycerebellar ataxiadiabetes mellitusABSTRACT The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia.Academia Brasileira de Neurologia - ABNEURO2017-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000300142Arquivos de Neuro-Psiquiatria v.75 n.3 2017reponame:Arquivos de neuro-psiquiatria (Online)instname:Academia Brasileira de Neurologiainstacron:ABNEURO10.1590/0004-282x20170011info:eu-repo/semantics/openAccessAguiar,Tiago SilvaFragoso,AndreaAlbuquerque,Carolina Rouanet deTeixeira,Patrícia de FátimaSouza,Marcus Vinícius Leitão deZajdenverg,LenitaAlves-Leon,Soniza VieiraRodacki,MelanieLima,Marco Antônio Sales Dantas deeng2017-03-20T00:00:00Zoai:scielo:S0004-282X2017000300142Revistahttp://www.scielo.br/anphttps://old.scielo.br/oai/scielo-oai.php||revista.arquivos@abneuro.org1678-42270004-282Xopendoar:2017-03-20T00:00Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologiafalse |
dc.title.none.fl_str_mv |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
title |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
spellingShingle |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies Aguiar,Tiago Silva autoimmunity cerebellar ataxia diabetes mellitus |
title_short |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
title_full |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
title_fullStr |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
title_full_unstemmed |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
title_sort |
Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies |
author |
Aguiar,Tiago Silva |
author_facet |
Aguiar,Tiago Silva Fragoso,Andrea Albuquerque,Carolina Rouanet de Teixeira,Patrícia de Fátima Souza,Marcus Vinícius Leitão de Zajdenverg,Lenita Alves-Leon,Soniza Vieira Rodacki,Melanie Lima,Marco Antônio Sales Dantas de |
author_role |
author |
author2 |
Fragoso,Andrea Albuquerque,Carolina Rouanet de Teixeira,Patrícia de Fátima Souza,Marcus Vinícius Leitão de Zajdenverg,Lenita Alves-Leon,Soniza Vieira Rodacki,Melanie Lima,Marco Antônio Sales Dantas de |
author2_role |
author author author author author author author author |
dc.contributor.author.fl_str_mv |
Aguiar,Tiago Silva Fragoso,Andrea Albuquerque,Carolina Rouanet de Teixeira,Patrícia de Fátima Souza,Marcus Vinícius Leitão de Zajdenverg,Lenita Alves-Leon,Soniza Vieira Rodacki,Melanie Lima,Marco Antônio Sales Dantas de |
dc.subject.por.fl_str_mv |
autoimmunity cerebellar ataxia diabetes mellitus |
topic |
autoimmunity cerebellar ataxia diabetes mellitus |
description |
ABSTRACT The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-03-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000300142 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000300142 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/0004-282x20170011 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
dc.source.none.fl_str_mv |
Arquivos de Neuro-Psiquiatria v.75 n.3 2017 reponame:Arquivos de neuro-psiquiatria (Online) instname:Academia Brasileira de Neurologia instacron:ABNEURO |
instname_str |
Academia Brasileira de Neurologia |
instacron_str |
ABNEURO |
institution |
ABNEURO |
reponame_str |
Arquivos de neuro-psiquiatria (Online) |
collection |
Arquivos de neuro-psiquiatria (Online) |
repository.name.fl_str_mv |
Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologia |
repository.mail.fl_str_mv |
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