SciELO - Scientific Electronic Library Online

 
vol.4 issue1Efficacy and safety of brand-risperidone versus similar-risperidone in elderly patients with neuropsychiatric disorders: A retrospective studyDementia in Fragile X-associated Tremor/Ataxia Syndrome author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

Share


Dementia & Neuropsychologia

Print version ISSN 1980-5764

Dement. neuropsychol. vol.4 no.1 São Paulo Jan./Mar. 2010

http://dx.doi.org/10.1590/S1980-57642010DN40100013 

Case Reports

Progressive posterior cortical dysfunction

Disfunção cortical posterior progressiva

Fábio Henrique de Gobbi Porto1 

Gislaine Cristina Lopes Machado2 

Lilian Schafirovits Morillo3 

Sonia Maria Dozzi Brucki4 

1MD, Behavioral and Cognitive Neurology Unit, Department of Neurology, and Cognitive Disorders Reference Center (CEREDIC); Hospital das Clínicas of the University of São Paulo, São Paulo SP, Brazil.

2MD, Department of Radiology, Hospital A.C. Camargo, São Paulo SP, Brazil.

3MD, MSc, Behavioral and Cognitive Neurology Unit, Department of Neurology, and Cogni­tive Disorders Reference Center (CEREDIC); Hospital das Clínicas of the University of São Paulo, School of Medicine, São Paulo SP, Brazil. Discipline of Geriatric Medicine, Hospital das Clínicas of the University of São Paulo, School of Medicine, São Paulo SP, Brazil.

4MD, PhD, Neurologist from the Cognitive and Behavioral Neurology Group (University of São Paulo); Psychobiology Department (Federal University of São Paulo); Hospital Santa Marcelina, São Paulo SP, Brazil.

Abstract

Progressive posterior cortical dysfunction (PPCD) is an insidious syndrome characterized by prominent disorders of higher visual processing. It affects both dorsal (occipito-parietal) and ventral (occipito-temporal) pathways, disturbing visuospatial processing and visual recognition, respectively. We report a case of a 67-year-old woman presenting with progressive impairment of visual functions. Neurologic examination showed agraphia, alexia, hemispatial neglect (left side visual extinction), complete Balint's syndrome and visual agnosia. Magnetic resonance imaging showed circumscribed atrophy involving the bilateral parieto-occipital regions, slightly more predominant to the right . Our aim was to describe a case of this syndrome, to present a video showing the main abnormalities, and to discuss this unusual presentation of dementia. We believe this article can contribute by improving the recognition of PPCD.

Key words: posterior progressive cortical dysfunction syndrome; Balint's syndrome; visuospatial dysfunction; visual agnosia

Resumo

Disfunção cortical posterior progressiva (DCPP) é um síndrome insidiosa caracterizada por distúrbios do processamento visual superior. Ela afeta tanto a via dorsal (occipito-parietal) como a ventral (occipito-temporal), alterando o processamento visuoespacial e reconhecimento visual, respectivamente. Nós relatamos o caso de uma mulher de 67 anos com dificuldade progressiva nas funções visuais. O exame neurológico mostrou agraphia, alexia, negligência especial (extinção visual à esquerda), síndrome de Balint complete e agnosia visual. A ressonância magnética mostrou atrofia nas regiões parieto-occipitais, mais evidente à direita. Nosso objetivo foi descrever um caso desta síndrome, apresentar um vídeo mostrando as principais anormalidades e discutir esta apresentação incomum de demência. Nós esperamos que o artigo possa facilitar o reconhecimento da DCPP.

Palavras-chave: síndrome da disfunção cortical posterior progressiva; Síndrome de Balint; disfunção visuoespacial; agnosia visual

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

References

1. McMonagle P, Deering F, Berliner Y, Kertesz A. The cognitive profile of posterior cortical atrophy Neurology 2006;66:331-338. [ Links ]

2. Renner JA, Burns JM, Hou CE, McKeel JR. DW, Storandt M, Morris JC. Progressive posterior cortical dysfunction: a clinicopathologic series. Neurology 2004;63:1175-1180 [ Links ]

3. Tang-Wai DF, Graff-Radford NR, Boeve BF, et al. Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. Neurology 2004;63:1168-1174. [ Links ]

4. Areza-Fegyveres R, Caramelli P, Porto CS, et al. The syndrome of progressive posterior cortical dysfunction: a multiple case study and review. Dement Neuropsychol 2007;1:311-319. [ Links ]

5. Damasio AR, Tranel D, Rizzo M. Disorders of Complex Visual Processing. In: Mesulam MM, editor. Principles of Behavioral and Cognitive neurology. 2nd ed. New York: Oxford Press; 2000:332-372. [ Links ]

6. Brucki SMD, Nitrini R, Caramelli P, Bertolucci PHF, Okamoto IH. Sugestões para o uso do mini-exame do estado mental no Brasil. Arq Neuropsiquiatr 2003;61:777-781. [ Links ]

7. Vasconcelos LG, Brucki SMD, Bueno OFA. Cognitive and functional dementia assessment tools: review of Brazilian literature. Dement Neuropsychol 2007;1:18-23. [ Links ]

8. Brucki SMD, Rocha MSG. Category fluency test: effects of age, gender and education on total scores, clustering and switching in Brazilian Portuguese-speaking subjects. Braz J Med Biol Res 2004;37:1771-1777. [ Links ]

9. Bottino CMC, Almeida OP, Tamai S, Forlenza OV, Scalco MZ, Carvalho IAM, (1999). Entrevista estruturada para diagnóstico de transtornos mentais em idosos - CAMDEX The Cambridge Examination for Mental Disorders of the Elderly. Cambridge University Press (Brazilian version, translated and adapted on behalf of the editors). [ Links ]

10. Nitrini R, Caramelli P, Bottino CMC, Damasceno BP, Brucki SMP, Anghinah R. Diagnosis of Alzheimer's disease in Brazil: diagnostic criteria and auxiliary tests. Recommendations of the scientific department of cognitive neurology and aging of the Brazilian academy of neurology. Arq Neuropsiquiatr 2005;63:713-719. [ Links ]

11. Knopman DS, DeKosky ST, Cummings JL, et al. Practice parameter: diagnosis of dementia (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001;56:1143-1153. [ Links ]

12. Benson DF, Davis RJ, Snyder BD. Posterior cortical atrophy. Arch Neurol 1988;45:789-793. [ Links ]

13. Mendez MF, Ghajarania M, Perryman KM. Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer's disease. Dement Geriatr Cogn Disord 2002;14:33-40. [ Links ]

Received: October 20, 2009; Accepted: January 05, 2010

Fábio Henrique de Gobbi Porto - CEREDIC / Centro de Referência em Distúrbios Cognitivos Hospital das Clínicas / FMUSP - Rua Arruda Alvim 206 - 05419-020 São Paulo SP - Brazil. E-mail: portofhg@gmail.com

Disclosure: The authors report no conflicts of interest

Creative Commons License This is an open-access article distributed under the terms of the Creative Commons Attribution License