Behavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case study

ABSTRACT 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 referre...

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Main Authors: Leonardo Cruz de Souza, Luciano Inácio Mariano, Renata Freire de Moraes, Paulo Caramelli
Format: Article
Language:English
Published: Associação Neurologia Cognitiva e do Comportamento 2019-09-01
Series:Dementia & Neuropsychologia
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642019000300356&tlng=en
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author Leonardo Cruz de Souza
Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
author_facet Leonardo Cruz de Souza
Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
author_sort Leonardo Cruz de Souza
collection DOAJ
description ABSTRACT 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 doaj.art-4aeb714a2d834c66a9d5b691671cfcbc2022-12-22T04:15:56ZengAssociação Neurologia Cognitiva e do ComportamentoDementia & Neuropsychologia1980-57642019-09-0113335636010.1590/1980-57642018dn13-030015Behavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case studyLeonardo Cruz de Souzahttps://orcid.org/0000-0001-5027-9722Luciano Inácio MarianoRenata Freire de MoraesPaulo CaramelliABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642019000300356&tlng=enAlzheimer's diseasefrontotemporal dementiaatypical Alzheimer's diseasebiomarkers
spellingShingle Leonardo Cruz de Souza
Luciano Inácio Mariano
Renata Freire de Moraes
Paulo Caramelli
Behavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case study
Dementia & Neuropsychologia
Alzheimer's disease
frontotemporal dementia
atypical Alzheimer's disease
biomarkers
title 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_short 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
topic Alzheimer's disease
frontotemporal dementia
atypical Alzheimer's disease
biomarkers
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642019000300356&tlng=en
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