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The syndrome of progressive posterior cortical dysfunction: A multiple case study and review

Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose. OBJECTIVES: To describe cases of progressive dementia presenting with prominent visual cortical symptoms. METHODS: We conducted a retrospec...

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Autores principales: Areza-Fegyveres, Renata, Caramelli, Paulo, Porto, Claudia Sellitto, Ono, Carla Rachel, Buchpiguel, Carlos Alberto, Nitrini, Ricardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Associação de Neurologia Cognitiva e do Comportamento 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619011/
https://www.ncbi.nlm.nih.gov/pubmed/29213406
http://dx.doi.org/10.1590/S1980-57642008DN10300014
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author Areza-Fegyveres, Renata
Caramelli, Paulo
Porto, Claudia Sellitto
Ono, Carla Rachel
Buchpiguel, Carlos Alberto
Nitrini, Ricardo
author_facet Areza-Fegyveres, Renata
Caramelli, Paulo
Porto, Claudia Sellitto
Ono, Carla Rachel
Buchpiguel, Carlos Alberto
Nitrini, Ricardo
author_sort Areza-Fegyveres, Renata
collection PubMed
description Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose. OBJECTIVES: To describe cases of progressive dementia presenting with prominent visual cortical symptoms. METHODS: We conducted a retrospective search of cases of progressive dementia with predominant visual symptoms, seen at our dementia unit from 1996 to 2006. RESULTS: Twelve patients (5 men, 7 women) were identified, with ages ranging from 49 to 67 years. At the first examination, the duration of the symptoms ranged from one to ten years and the Mini-Mental State Examination scores from 7 to 27. Eleven patients presented with predominant visuospatial symptoms (partial or complete Balint syndrome) and one with visuoperceptive impairment. Other reported manifestations were: constructional apraxia in 11 patients, partial or complete Gerstmann syndrome in ten, ideomotor apraxia in nine, hemineglect or extinction in four patients, alien hand phenomenon in three, and prosopagnosia in one patient. Memory loss was reported by ten patients, but was not the main complaint in any of these cases. Insight was relatively preserved in five patients even after a long period following the onset of symptoms. Six patients developed parkinsonism during evolution. Clinical diagnoses were possible or probable AD in seven patients, cortico-basal degeneration in four, and dementia with Lewy body in one. CONCLUSIONS: Clinicians should consider this condition especially in presenile patients with slowly progressive higher-order visual symptoms. Although described in association with different conditions, it may also occur in Alzheimer disease.
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spelling pubmed-56190112017-12-06 The syndrome of progressive posterior cortical dysfunction: A multiple case study and review Areza-Fegyveres, Renata Caramelli, Paulo Porto, Claudia Sellitto Ono, Carla Rachel Buchpiguel, Carlos Alberto Nitrini, Ricardo Dement Neuropsychol Original Articles Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose. OBJECTIVES: To describe cases of progressive dementia presenting with prominent visual cortical symptoms. METHODS: We conducted a retrospective search of cases of progressive dementia with predominant visual symptoms, seen at our dementia unit from 1996 to 2006. RESULTS: Twelve patients (5 men, 7 women) were identified, with ages ranging from 49 to 67 years. At the first examination, the duration of the symptoms ranged from one to ten years and the Mini-Mental State Examination scores from 7 to 27. Eleven patients presented with predominant visuospatial symptoms (partial or complete Balint syndrome) and one with visuoperceptive impairment. Other reported manifestations were: constructional apraxia in 11 patients, partial or complete Gerstmann syndrome in ten, ideomotor apraxia in nine, hemineglect or extinction in four patients, alien hand phenomenon in three, and prosopagnosia in one patient. Memory loss was reported by ten patients, but was not the main complaint in any of these cases. Insight was relatively preserved in five patients even after a long period following the onset of symptoms. Six patients developed parkinsonism during evolution. Clinical diagnoses were possible or probable AD in seven patients, cortico-basal degeneration in four, and dementia with Lewy body in one. CONCLUSIONS: Clinicians should consider this condition especially in presenile patients with slowly progressive higher-order visual symptoms. Although described in association with different conditions, it may also occur in Alzheimer disease. Associação de Neurologia Cognitiva e do Comportamento 2007 /pmc/articles/PMC5619011/ /pubmed/29213406 http://dx.doi.org/10.1590/S1980-57642008DN10300014 Text en http://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Articles
Areza-Fegyveres, Renata
Caramelli, Paulo
Porto, Claudia Sellitto
Ono, Carla Rachel
Buchpiguel, Carlos Alberto
Nitrini, Ricardo
The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title_full The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title_fullStr The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title_full_unstemmed The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title_short The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
title_sort syndrome of progressive posterior cortical dysfunction: a multiple case study and review
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619011/
https://www.ncbi.nlm.nih.gov/pubmed/29213406
http://dx.doi.org/10.1590/S1980-57642008DN10300014
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