Cargando…

The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman

Posterior cortical atrophy (PCA) describes a rare heterogenous neurodegenerative syndrome with early visuospatial and visuoperceptual deficits due to atrophy of parieto-occipital brain regions. Here, we describe the case of a 62-year-old woman showing severe cognitive impairments as well as hemianop...

Descripción completa

Detalles Bibliográficos
Autores principales: Ziegler, Georg C., Haarmann, Axel, Daniels, Christine, Herr, Alexandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6900588/
https://www.ncbi.nlm.nih.gov/pubmed/31203704
http://dx.doi.org/10.1177/0891988719856696
_version_ 1783477378669871104
author Ziegler, Georg C.
Haarmann, Axel
Daniels, Christine
Herr, Alexandra
author_facet Ziegler, Georg C.
Haarmann, Axel
Daniels, Christine
Herr, Alexandra
author_sort Ziegler, Georg C.
collection PubMed
description Posterior cortical atrophy (PCA) describes a rare heterogenous neurodegenerative syndrome with early visuospatial and visuoperceptual deficits due to atrophy of parieto-occipital brain regions. Here, we describe the case of a 62-year-old woman showing severe cognitive impairments as well as hemianopsia and all core symptoms of Bálint's syndrome. Years ago, the patient had complained about a “tunnel view” and concentration problems. The diagnostic results point to a case of PCA with underlying Alzheimer pathology. The disease course until diagnosis lasted for 7 years, reflecting the diagnostic difficulties with this still largely unknown syndrome. The unfamiliar symptom presentation including fluctuations in cognitive performance, affective symptoms, cerebrospinal fluid (CSF) biomarkers, which were at first inconspicuous, and a former suspected diagnosis of dissociative pseudodementia, altogether brought considerable uncertainty to the involved health-care professionals. We conclude that cases of “atypical dementia” presenting with visual symptoms, even if appearing unspecific at first, are suspect of PCA. This case report provides an ostensive overview of PCA, including imaging data, CSF-findings, original drawings and handwriting samples from the patient.
format Online
Article
Text
id pubmed-6900588
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher SAGE Publications
record_format MEDLINE/PubMed
spelling pubmed-69005882019-12-12 The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman Ziegler, Georg C. Haarmann, Axel Daniels, Christine Herr, Alexandra J Geriatr Psychiatry Neurol Case Report Posterior cortical atrophy (PCA) describes a rare heterogenous neurodegenerative syndrome with early visuospatial and visuoperceptual deficits due to atrophy of parieto-occipital brain regions. Here, we describe the case of a 62-year-old woman showing severe cognitive impairments as well as hemianopsia and all core symptoms of Bálint's syndrome. Years ago, the patient had complained about a “tunnel view” and concentration problems. The diagnostic results point to a case of PCA with underlying Alzheimer pathology. The disease course until diagnosis lasted for 7 years, reflecting the diagnostic difficulties with this still largely unknown syndrome. The unfamiliar symptom presentation including fluctuations in cognitive performance, affective symptoms, cerebrospinal fluid (CSF) biomarkers, which were at first inconspicuous, and a former suspected diagnosis of dissociative pseudodementia, altogether brought considerable uncertainty to the involved health-care professionals. We conclude that cases of “atypical dementia” presenting with visual symptoms, even if appearing unspecific at first, are suspect of PCA. This case report provides an ostensive overview of PCA, including imaging data, CSF-findings, original drawings and handwriting samples from the patient. SAGE Publications 2019-06-16 2020-01 /pmc/articles/PMC6900588/ /pubmed/31203704 http://dx.doi.org/10.1177/0891988719856696 Text en © The Author(s) 2019 http://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Ziegler, Georg C.
Haarmann, Axel
Daniels, Christine
Herr, Alexandra
The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title_full The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title_fullStr The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title_full_unstemmed The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title_short The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman
title_sort difficult diagnosis of posterior cortical atrophy in a 62-year-old woman
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6900588/
https://www.ncbi.nlm.nih.gov/pubmed/31203704
http://dx.doi.org/10.1177/0891988719856696
work_keys_str_mv AT zieglergeorgc thedifficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT haarmannaxel thedifficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT danielschristine thedifficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT herralexandra thedifficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT zieglergeorgc difficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT haarmannaxel difficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT danielschristine difficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman
AT herralexandra difficultdiagnosisofposteriorcorticalatrophyina62yearoldwoman