Congenital prosopagnosia: A case report

Schultz, Rodrigo Rizek; Bertolucci, Paulo Henrique Ferreira

doi:10.1590/S1980-57642011DN05010010

Abstract

Prosopagnosia is a visual agnosia characterized by an inability to recognize previously known human faces and to learn new faces. The aim of this study was to present a forty-six year-old woman with congenital prosopagnosia, and to discuss the neural bases of perception and recognition of faces. The patients had a lifetime impairment in recognizing faces of family members, close friends, and even her own face in photos. She also had impairment in recognizing animals such as discriminating between cats and dogs. The patient's basic visual skills showed impairment in identifying and recognizing the animal form perception on the coding subtest of the WAIS-R, recognizing overlapping pictures (Luria), and in identifying silhouettes depicting animals and objects (VOSP). Unconventional tests using pictures evidenced impairment in her capacity to identify famous faces, facial emotions and animals. Her face perception abilities were preserved, but recognition could not take place. Therefore, it appears that the agnosia in this case best fits the group of categories termed "associative".

Key words:
prosopagnosia; congenital; agnosia; face recognition

Resumo

Prosopagnosia é uma agnosia visual caracterizada por uma incapacidade de reconhecer faces humanas vistas anteriormente e aprender outras. O objetivo é apresentar uma mulher de 46 anos com prosopagnosia congênita e discutir as bases neurais da percepção e do reconhecimento de faces. Ela nos procurou referindo apresentar desde a infância problemas no reconhecimento de faces de membros da família, amigos próximos e mesmo para sua própria imagem numa fotografia. Também diz apresentar prejuízo no reconhecimento de animais, como discriminar cães de gatos. Apresentou dificuldades em identificar e reconhecer animais desenhados; reconhecer figuras sobrepostas (Luria), incorrendo em paragnosias visuais e identificar silhuetas de animais (VOSP). Em testes não convencionais, usando figuras, evidenciou diminuição da capacidade em identificar faces famosas, expressões faciais e animais, mas não em estimar o sexo e a idade das pessoas. Concluindo, suas habilidades perceptuais para face estão preservadas, mas há um déficit de reconhecimento. Tudo indica que sua agnosia pertence ao grupo das associativas.

Palavras-chave:
prosopagnosia; congenital; agnosia; reconhecimento de face

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References

Damasio AR, Tranel D, Rizzo M. Disorders of complex visual processing. In: Mesulan MM, editor. Principles of behavioral and cognitive neurology. 2nd Ed. New York: Oxford University; 2000:332-372.
Behrmann M, Avidan G. Congenital prosopagnosia: face-blind from birth. Trends Cogn Sci 2005;9:180-187.
Bruce V, Young AW. Understanding face recognition. Br J Psychol 1986;77:305-327.
Ariel R, Sadeh M. Congenital visual agnosia and prosopagnosia in a child: a case report. Cortex 1996;32:221-240.
Grueter M, Grueter T, Bell V, et al. Hereditary prosopagnosia: the first case series. Cortex 2007;43:734-749.
Frota NAF, Pinto LF, Porto CS, Aguia PHP, Castro LHM, Caramelli P. Visual agnosia and prosopagnosia secondary to melanoma metastases: case report. Dement Neuropsychol 2007;1:104-107.
Grüter T, Grüter M, Bell V, Carbon CC. Visual mental imagery in congenital prosopagnosia. Neurosci Lett 2009;453:135-140.
Tree JJ, Wilkieb J. Face and object imagery in congenital prosopagnosia: A case series. Cortex 2010;46:1189-1198.
Behrmann M, Avidan G, Gao F, Black S. Structural imaging reveals anatomical alterations in inferotemporal cortex in congenital prosopagnosia. Cereb Cortex 2007;17:2354-2363.
Thomas C, Avidan G, Humphreys K, Jung K, Gao F, Behrmann M. Reduced structural connectivity in ventral visual cortex in congenital prosopagnosia. Nat Neurosci 2009;12:29-31.
Garrido L, Furl N, Draganski B, et al. Voxel-based morphometry reveals reduced grey matter volume in the temporalcortex of developmental prosopagnosics. Brain 2009;132: 3443-3455.
Dailey MN, Cottrell. Prosopagnosia in modular neural network models. Prog Brain Res 1999;121:165-184.
Hasson U, Avidan G, Deouell LY, Bentin S, Malach R. Face-selective activation in a congenital prosopagnosic subject. J Cogn Neurosci 2003;15:419-431.
Damasio A, Damasio H, van Hoesen GW. Prosopagnosia: anatomic basis and behavioral mechanisms. Neurology 1982; 32:331-341.
Rapcsak SZ, Polster MR, Glisky ML, Comer JF. False recognition of unfamiliar faces following right hemisphere damage: neuropsychological and anatomical observations. Cortex 1996;32:593-611.
Behrmann M, Avidan G, Marotta JJ, Kimchi R. Detailed exploration of face-related processing in congenital prosopagnosia: 1. Behavioral findings. J Cogn Neurosci 2005;17:1130-1149.
Barton JJS. Disorders of color and object recognition. Continuum Lifelong Learning Neurol 2010;16:111-127.
Warrington EK, James M. The visual objects and space perception battery (VOSP). Bury St Edmunds: Thames Valley Test Company, 1991.
Nascimento E, Figueiredo VLM. WISC-III e WAIS-III: alterações nas versões originais americanas decorrentes das adaptações para uso no Brasil. Psicol Refl e Crít 2002;15:603-612.
Luria A. The working brain: an introduction to neuropsychology. New York: Penguin Books 1973:400.

Publication Dates

Publication in this collection
Jan-Mar 2011

History

Received
10 Dec 2010
Accepted
10 Feb 2011

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

[1] Damasio AR, Tranel D, Rizzo M. Disorders of complex visual processing. In: Mesulan MM, editor. Principles of behavioral and cognitive neurology. 2nd Ed. New York: Oxford University; 2000:332-372.

[2] Behrmann M, Avidan G. Congenital prosopagnosia: face-blind from birth. Trends Cogn Sci 2005;9:180-187.

[3] Bruce V, Young AW. Understanding face recognition. Br J Psychol 1986;77:305-327.

[4] Ariel R, Sadeh M. Congenital visual agnosia and prosopagnosia in a child: a case report. Cortex 1996;32:221-240.

[5] Grueter M, Grueter T, Bell V, et al. Hereditary prosopagnosia: the first case series. Cortex 2007;43:734-749.

[6] Frota NAF, Pinto LF, Porto CS, Aguia PHP, Castro LHM, Caramelli P. Visual agnosia and prosopagnosia secondary to melanoma metastases: case report. Dement Neuropsychol 2007;1:104-107.

[7] Grüter T, Grüter M, Bell V, Carbon CC. Visual mental imagery in congenital prosopagnosia. Neurosci Lett 2009;453:135-140.

[8] Tree JJ, Wilkieb J. Face and object imagery in congenital prosopagnosia: A case series. Cortex 2010;46:1189-1198.

[9] Behrmann M, Avidan G, Gao F, Black S. Structural imaging reveals anatomical alterations in inferotemporal cortex in congenital prosopagnosia. Cereb Cortex 2007;17:2354-2363.

[10] Thomas C, Avidan G, Humphreys K, Jung K, Gao F, Behrmann M. Reduced structural connectivity in ventral visual cortex in congenital prosopagnosia. Nat Neurosci 2009;12:29-31.

[11] Garrido L, Furl N, Draganski B, et al. Voxel-based morphometry reveals reduced grey matter volume in the temporalcortex of developmental prosopagnosics. Brain 2009;132: 3443-3455.

[12] Dailey MN, Cottrell. Prosopagnosia in modular neural network models. Prog Brain Res 1999;121:165-184.

[13] Hasson U, Avidan G, Deouell LY, Bentin S, Malach R. Face-selective activation in a congenital prosopagnosic subject. J Cogn Neurosci 2003;15:419-431.

[14] Damasio A, Damasio H, van Hoesen GW. Prosopagnosia: anatomic basis and behavioral mechanisms. Neurology 1982; 32:331-341.

[15] Rapcsak SZ, Polster MR, Glisky ML, Comer JF. False recognition of unfamiliar faces following right hemisphere damage: neuropsychological and anatomical observations. Cortex 1996;32:593-611.

[16] Behrmann M, Avidan G, Marotta JJ, Kimchi R. Detailed exploration of face-related processing in congenital prosopagnosia: 1. Behavioral findings. J Cogn Neurosci 2005;17:1130-1149.

[17] Barton JJS. Disorders of color and object recognition. Continuum Lifelong Learning Neurol 2010;16:111-127.

[18] Warrington EK, James M. The visual objects and space perception battery (VOSP). Bury St Edmunds: Thames Valley Test Company, 1991.

[19] Nascimento E, Figueiredo VLM. WISC-III e WAIS-III: alterações nas versões originais americanas decorrentes das adaptações para uso no Brasil. Psicol Refl e Crít 2002;15:603-612.

[20] Luria A. The working brain: an introduction to neuropsychology. New York: Penguin Books 1973:400.

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