Progressive posterior cortical dysfunction

Porto, Fábio Henrique de Gobbi; Machado, Gislaine Cristina Lopes; Morillo, Lilian Schafirovits; Brucki, Sonia Maria Dozzi

doi:10.1590/S1980-57642010DN40100013

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

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References

¹
McMonagle P, Deering F, Berliner Y, Kertesz A. The cognitive profile of posterior cortical atrophy Neurology 2006;66:331-338.
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
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.
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.
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.
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.
⁷
Vasconcelos LG, Brucki SMD, Bueno OFA. Cognitive and functional dementia assessment tools: review of Brazilian literature. Dement Neuropsychol 2007;1:18-23.
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.
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).
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.
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.
¹²
Benson DF, Davis RJ, Snyder BD. Posterior cortical atrophy. Arch Neurol 1988;45:789-793.
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.

Publication Dates

Publication in this collection
Jan-Mar 2010

History

Received
20 Oct 2009
Accepted
05 Jan 2010

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

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

[2] 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

[3] 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.

[4] 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.

[5] 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.

[6] 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.

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

[8] 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.

[9] 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).

[10] 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.

[11] 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.

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

[13] 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.

Brasil