Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study

Detalhes bibliográficos
Autor(a) principal: Leonardo Cruz de Souza
Data de Publicação: 2019
Outros Autores: Luciano Inácio Mariano, Renata Freire de Moraes, Paulo Caramelli
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Institucional da UFMG
Texto Completo: http://hdl.handle.net/1843/62841
Resumo: Alzheimer’s disease (AD) has heterogeneous clinical presentations. Amnestic progressive disorder leading to dementia is the most typical, but non-amnestic presentations are also recognized. Here we report a case of frontal variant of AD. A right-handed woman, aged 68 years, was referred for progressive behavioral disorders and personality changes. She had a corroborated history of dietary changes, hyperorality, impulsivity, affective indifference and apathy, with functional impairment. Cognitive assessment yielded severe executive deficits. Positron emission tomography with fluorodeoxyglucose showed marked hypometabolism in frontotemporal regions, with relative preservation of parietal regions. CSF AD biomarkers showed low Aβ42, high Tau and high P-Tau. The patient fulfilled criteria for probable behavioral variant frontotemporal dementia. However, considering the AD pathophysiological signature on CSF biomarkers, a diagnosis of frontal variant of AD was established. In the perspective of disease-modifying therapies, it is important to identify atypical Alzheimer presentations, as these patients may be candidates for specific treatments.
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spelling 2024-01-15T21:19:00Z2024-01-15T21:19:00Z201913335636010.1590/1980-57642018dn13-03001519805764http://hdl.handle.net/1843/62841Alzheimer’s disease (AD) has heterogeneous clinical presentations. Amnestic progressive disorder leading to dementia is the most typical, but non-amnestic presentations are also recognized. Here we report a case of frontal variant of AD. A right-handed woman, aged 68 years, was referred for progressive behavioral disorders and personality changes. She had a corroborated history of dietary changes, hyperorality, impulsivity, affective indifference and apathy, with functional impairment. Cognitive assessment yielded severe executive deficits. Positron emission tomography with fluorodeoxyglucose showed marked hypometabolism in frontotemporal regions, with relative preservation of parietal regions. CSF AD biomarkers showed low Aβ42, high Tau and high P-Tau. The patient fulfilled criteria for probable behavioral variant frontotemporal dementia. However, considering the AD pathophysiological signature on CSF biomarkers, a diagnosis of frontal variant of AD was established. In the perspective of disease-modifying therapies, it is important to identify atypical Alzheimer presentations, as these patients may be candidates for specific treatments.A doença de Alzheimer (DA) tem apresentações clínicas heterogêneas. Amnésia progressiva associada a demência é a forma mais comum, mas apresentações não-amnésticas são também reconhecidas. Relatamos um caso de variante frontal da DA. Um mulher destra, de 68 anos, consultou-se devido a transtornos comportamentais progressivos, com alterações de personalidade. Modificações de padrão alimentar, hiperoralidade, impulsividade, indiferença afetiva e apatia, com declínio funcional, foram corroboradas pela família. A avaliação cognitiva evidenciou grave disfunção executiva. A tomografia de emissão de pósitrons com flúordeoxiglicose revelou proeminente hipometabolismo em regiões frontotemporais, com relativa preservação de regiões parietais. A análise de biomarcadores de DA no líquido cefalorraquidiano mostrou redução de níveis de Aβ42, com aumento de níveis de Tau e P-Tau. A paciente preencheu critérios diagnósticos para demência frontotemporal (variante comportamental) provável. Contudo, considerando o perfil de biomarcadores em favor de fisiopatologia da DA, o diagnóstico de variante frontal de DA foi estabelecido. Na perspectiva de tratamentos modificadores da doença, é crucial identificar apresentações atípicas da DA, visto que esses pacientes podem ser candidatos a terapias específicas.porUniversidade Federal de Minas GeraisUFMGBrasilMED - DEPARTAMENTO DE CLÍNICA MÉDICADementia & NeuropsychologiaAlzheimer’s diseaseFrontotemporal DementiaBiomarkersAlzheimer’s diseaseFrontotemporal DementiaBiomarkersBehavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case studyVARIANTE COMPORTAMENTAL DA DEMÊNCIA FRONTOTEMPORAL OU VARIANTE FRONTAL DA DOENÇA DE ALZHEIMER? UM ESTUDO DE CASOinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.1590/1980-57642018dn13-030015Leonardo Cruz de SouzaLuciano Inácio MarianoRenata Freire de MoraesPaulo Caramelliapplication/pdfinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFMGinstname:Universidade Federal de Minas Gerais (UFMG)instacron:UFMGLICENSELicense.txtLicense.txttext/plain; charset=utf-82042https://repositorio.ufmg.br/bitstream/1843/62841/1/License.txtfa505098d172de0bc8864fc1287ffe22MD51ORIGINALBehavioral variant of frontotemporal dementia pdfa.pdfBehavioral variant of frontotemporal dementia pdfa.pdfapplication/pdf4002221https://repositorio.ufmg.br/bitstream/1843/62841/2/Behavioral%20variant%20of%20frontotemporal%20dementia%20pdfa.pdf9386783aaa0b025023bc5b4540610cb1MD521843/628412024-01-15 18:26:30.679oai:repositorio.ufmg.br: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Repositório de PublicaçõesPUBhttps://repositorio.ufmg.br/oaiopendoar:2024-01-15T21:26:30Repositório Institucional da UFMG - Universidade Federal de Minas Gerais (UFMG)false
dc.title.pt_BR.fl_str_mv Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
dc.title.alternative.pt_BR.fl_str_mv VARIANTE COMPORTAMENTAL DA DEMÊNCIA FRONTOTEMPORAL OU VARIANTE FRONTAL DA DOENÇA DE ALZHEIMER? UM ESTUDO DE CASO
title Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
spellingShingle Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
Leonardo Cruz de Souza
Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
title_short Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
title_full Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
title_fullStr Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
title_full_unstemmed Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
title_sort Behavioral variant of frontotemporal dementia or frontal variant of alzheimer's disease? a case study
author Leonardo Cruz de Souza
author_facet Leonardo Cruz de Souza
Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
author_role author
author2 Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
author2_role author
author
author
dc.contributor.author.fl_str_mv Leonardo Cruz de Souza
Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
dc.subject.por.fl_str_mv Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
topic Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
dc.subject.other.pt_BR.fl_str_mv Alzheimer’s disease
Frontotemporal Dementia
Biomarkers
description Alzheimer’s disease (AD) has heterogeneous clinical presentations. Amnestic progressive disorder leading to dementia is the most typical, but non-amnestic presentations are also recognized. Here we report a case of frontal variant of AD. A right-handed woman, aged 68 years, was referred for progressive behavioral disorders and personality changes. She had a corroborated history of dietary changes, hyperorality, impulsivity, affective indifference and apathy, with functional impairment. Cognitive assessment yielded severe executive deficits. Positron emission tomography with fluorodeoxyglucose showed marked hypometabolism in frontotemporal regions, with relative preservation of parietal regions. CSF AD biomarkers showed low Aβ42, high Tau and high P-Tau. The patient fulfilled criteria for probable behavioral variant frontotemporal dementia. However, considering the AD pathophysiological signature on CSF biomarkers, a diagnosis of frontal variant of AD was established. In the perspective of disease-modifying therapies, it is important to identify atypical Alzheimer presentations, as these patients may be candidates for specific treatments.
publishDate 2019
dc.date.issued.fl_str_mv 2019
dc.date.accessioned.fl_str_mv 2024-01-15T21:19:00Z
dc.date.available.fl_str_mv 2024-01-15T21:19:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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dc.identifier.uri.fl_str_mv http://hdl.handle.net/1843/62841
dc.identifier.doi.pt_BR.fl_str_mv 10.1590/1980-57642018dn13-030015
dc.identifier.issn.pt_BR.fl_str_mv 19805764
identifier_str_mv 10.1590/1980-57642018dn13-030015
19805764
url http://hdl.handle.net/1843/62841
dc.language.iso.fl_str_mv por
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dc.relation.ispartof.none.fl_str_mv Dementia & Neuropsychologia
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eu_rights_str_mv openAccess
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dc.publisher.none.fl_str_mv Universidade Federal de Minas Gerais
dc.publisher.initials.fl_str_mv UFMG
dc.publisher.country.fl_str_mv Brasil
dc.publisher.department.fl_str_mv MED - DEPARTAMENTO DE CLÍNICA MÉDICA
publisher.none.fl_str_mv Universidade Federal de Minas Gerais
dc.source.none.fl_str_mv reponame:Repositório Institucional da UFMG
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